surgical techniques alveolar bone grafting

Brief Clinical Notes

A Patient of Severe CervicofacialSubcutaneous EmphysemaAssociated With Munchausen’sSyndrome

Fuat Tosun, MD, Cem Ozer, MD, Timur Akcam, MD,Mustafa Gerek, MD, Sertac Yetiser, MD

Ankara, Turkey

Abstract: Subcutaneous cervicofacial emphysema is an entity withthe presence of air within the fascial planes of the head and neck,which results from various causes. A case of severe subcutaneouscervicofacial emphysema associated with Munchausen’s syn-drome is presented. Detailed diagnostic investigations to findout the organic cause of the condition were failed. Episodes of facialswelling were prevented by psychiatric support. Munchausen’ssyndrome should be kept in mind among the etiological factorsof subcutaneous cervicofacial emphysema cases, in whom no or-ganic causes can be found out.

Key Words: Subcutaneous emphysema, factitiousdisorder, head, neck

S ubcutaneous cervicofacial emphysema is the ab-normal presence of air within the fascial planes of

the head and neck.1 Several organic causes of thisphenomenon are categorized into four groups as iatro-genic, traumatic, infectious, and spontaneous.2 Mostof the cases are benign and treated by supportivemeasures. However, patients must be followed-upclosely for the potential fatal complications such asairway obstruction.3 A rare, distinct group of thisclinical entity is self-induced subcutaneous emphy-sema and is first described by Gershwin et al in1971 as an unusual form of Munchausen’s syndrome,which is characterized primarily by intentional pro-duction of physical symptoms, signs, and laboratoryfindings of an illness.4 We report an unusual case ofa severe subcutaneous emphysema extending fromperiorbital region down to the upper part of the chestwall in a 21-year-old male, which was precipitatedby trauma and has been continued on the basis ofMunchausen’s syndrome.

CASE REPORT

A 21-year-old male presented with a complaint of re-current episodes of swelling over his head, neck, andupper part of the chest wall, which followed fallingdown from a berth two months ago. He had been ex-amined elsewhere and was given antiallergic medica-tion. The patient also noted difficulty in breathingduring his swelling attacks. There was no historyof allergy, fever, cough, emesis, strenuous effort, as-piration of foreign body, or any surgery to initiatethe disease. The past medical history was unremark-able without initial trauma.

Physical examination revealed a crepitant swell-ing of the facial, cervical, and upper part of thethoracic regions. There were several papules in hisneck and face (Fig 1). Examination of the oral cavityrevealed no mucosal alteration and no dental abnor-mality. Flexible nasal endoscopy showed normalanatomy of the nasopharynx, the pharynx, and thelarynx. There was no sign of infection on physicalexamination.

Routine blood count, urine tests, liver and renalfunction tests, erythrocyte sedimentation rate werenormal. Chest radiograph was normal. Culturestaken from the blood, the nasal cavity, and the phar-ynx for gas-producing bacteria, were found to be neg-ative. Computed tomography (CT) of the neck andthe thorax revealed subcutaneous emphysema ofthe periorbital region, face, cervical region, and upperpart of the chest wall (Fig 2). There was no sign ofother pathologies including pneumomediastinumand pneumothorax on CT scan. A contrast swallow-ing study showed normal esophageal function andanatomy. Panendoscopy of head and neck and bron-choscopy revealed normal findings. Consultationsfrom maxillofacial surgery, chest disease, dentistry,infectious disease, allergy, and internal medicinerevealed no pathology. Only, psychiatry consultationrevealed a depressive mood of the patient.

The episode of subcutaneous emphysema re-gressed slowly and disappeared spontaneously. How-ever during his stay at the hospital, he continuedto develop recurrent episodes of subcutaneous em-physema extending from periorbital region downto the upper chest wall. Loss of vision due to eyelidclosure, swallowing difficulties as a result of mucosaledema, and severe dyspnea was observed duringthe last episode and a tracheotomy was performedto secure his airway.

His symptoms resolved quickly after tracheot-omy, and he was discharged from the hospital toget rest at home for 45 days. Following completion ofthe rest period, he was decannulated and hospitalized

From the Department of ORL & HNS, Gulhane Medical School,Etlik 06018, Ankara, Turkey.

Address correspondence to Fuat Tosun, MD, Associate Profes-sor, Gulhane Medical School, Dept. of ORL & HNS, Etlik 06018,Ankara-Turkey; E-mail: [email protected]

Presented at the Seventh Congress of Balkan Military MedicalCommittee, 6–10 October 2002, Athens, Greece.

Submitted for publication, Accepted for publication.

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to follow up for possible recurrences. A new episodeof subcutaneous emphysema occurred before givinghim the duty. All diagnostic procedures were re-peated, but no etiologic factor could be found out.

One night, he was seen by a nurse while he was ad-ministrating intravenous diazepam. A new psychia-try consultation revealed that he was a drug abuser,and he is using his disease to attract attention and toobtain secondary gain. Then we postulated thattrauma precipitated the first episode of subcutaneousemphysema, but other swelling episodes were cre-ated by the patient intentionally by straining or injec-tion of the air subcutaneously. However, he deniedany self-destructive behavior. At the end, the patientwas referred to the psychiatry department and he isstill under outpatient control without any recurrencesfor the last 18 months.

DISCUSSION

The most common causes of subcutaneous cervicofa-cial emphysema are the iatrogenic ones includinghead and neck surgical interventions, dental proce-dures, endoscopies, traumatic intubations, and pul-monary barotrauma secondary to mechanical ventila-tion.2 Traumatic lesions of the oral cavity, thepharynx, the larynx, the trachea, and the esophagus,resulting in a perforation of the mucosa may be thesource of subcutaneous emphysema.5 Neoplasmsand foreign bodies within the aerodigestive tractscan also produce emphysema by the same mecha-nisms.1 Rarely, infections of the head and neck sec-ondary to gas-producing microorganisms may causesubcutaneous emphysema. The most common infec-tion leading to cervicofacial emphysema is the retro-pharyngeal abscess.6 Another important cause ofsubcutaneous emphysema is the rupture of the alve-olar membrane secondary to increased intraalveolarpressure, which may be precipitated by Valsalva’smaneuver, strenuous effort, coughing, vomiting,and parturition.1

The clinical presentation of subcutaneous cervi-cofacial emphysema correlates with the extent of dis-section of the subcutaneous air. Swelling of the faceand neck with crackles under the skin are the primarysymptoms. Other symptoms include neck pain, dys-phagia, dysphonia, dyspnea, and otalgia. Typicalcrepitus by palpating the skin of the head and neckis noted on physical examination. Rarely fever, in-creased white cell count, and electrocardiographicchanges of low voltages can be seen. Streaks or pocketsof subcutaneous air throughout the face, the neck, andthe chest wall can be seen in radiographs.1,2,5,7,8

A detailed investigation beyond a careful historyand physical examination is generally needed for thediagnosis of subcutaneous cervicofacial emphysema.Routine blood tests, erythrocyte sedimentation rate,

Fig 1 Severe subcutaneous emphysema of the head andneck.

Fig 2 Computed tomography of the patient shows thepresence of air within the fascial planes of the head andneck (indicated by arrows).

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cultures from the blood, the nasal cavity, and thenasopharynx, direct radiographs from the head, theneck, and the chest should be performed initially.High-resolution spiral tomography of the head andneck can help to find out the location of the defectthat causes air leak. Contrast swallowing study mayreveal an esophageal defect. Panendoscopy of theesophagus, the larynx, and the tracheobronchial treeshould be performed in addition to radiological stud-ies to detect all mucosal injuries of the aerodigestivetract.1,2,5,8 A broad differential diagnosis includesallergic reaction, edema, hematoma, and infection.9

These investigations may not be sufficient, and amultidisciplinary study is generally needed to findout exact etiology as in our case.

Subcutaneous cervicofacial emphysema is abenign, self-limited disease and usually disappearsspontaneously in 5 to 10 days. Although majorityof patients requires no treatment, close observationfor the development of potential life-threateningcomplications such as airway obstruction is manda-tory. Other complications of subcutaneous emphy-sema include pneumomediastinum, pneumothorax,pneumopericardium, mediastinitis, air embolus,and deep neck abscess. Prophylactic antibiotic ther-apy may be helpful especially in cases of traumafor preventing the bacterial contamination of the neckand mediastinum from the aerodigestive tract.1,2,8

Cough suppressants, sedatives, analgesics, decon-gestants, antihistamines, and stool softeners are ad-vocated for reducing the straining. Administrationof 100% oxygen increases the gradient of nitrogenresorption from tissues by reducing its partial pres-sure.10 Subcutaneous drainage of emphysema byangiocatheters is a safe, easy, and effective methodto achieve symptomatic relief.11 Tracheotomy is indi-cated in cases with airway compromise. It also pre-vents straining and helps to release the trapped airin the facial planes through the neck incision.1,2

In this case, the patient presented with recurrentepisodes of subcutaneous cervicofacial emphysemaextending from the face down to the upper chest wall.Although a history of trauma was preceded to pa-tient’s complaints, no organic cause could be founddespite extensive investigations. Final psychiatryconsultation revealed that he was a drug abuserand that he was using his disease to attract attentionand to obtain secondary gain. The patient was awareof an ongoing medical problem, which would makehimself excluded from the duty. Then we considereda self-induced etiology, which was first reported byGershwin et al in 1971. They regarded this disorderas an abnormal type of Munchausen’s syndrome,which is a factitious disorder, described by Asher

in 1951. The name of this syndrome was dedicatedto Baron Heironymus Karl Friedrick Munchausen(1720–1797), a German cavalry officer who becamewell-known after his retirement, for telling exagger-ated tales of him as a soldier that resembled an epic.Asher described 3 subtypes of this clinical entity inhis original report as acute abdominal type, hemor-rhagic type, and neurologic type.12 Litersater on,other abnormal types such as dermatitis autogenica,cardiac arrhytmias etc. have been described by sev-eral authors.13

The incidence of self-induced subcutaneousemphysema is not known. Usually the condition ismerely cosmetic; however, respiratory failure maybe associated in severe cases. In 1983, Winans et alreported a case of self-induced orbital emphysemathat resulted in orbital exenteration. The diagnosisof self-induced subcutaneous emphysema is difficult.Winans et al made the diagnosis of Munchausen’ssyndrome in their patient after a ten years follow-up.14 All possible organic causes of spontaneous sub-cutaneous emphysema should be eliminated beforethe diagnosis has been set.

CONCLUSION

In conclusion, subcutaneous emphysema may resultfrom various etiological factors. In cases withoutany known organic origin, Munchausen’s syndromeshould be kept in mind as an etiological factor. Thereis no specific treatment protocol for these cases. How-ever, patients should be followed-up closely for po-tential airway compromise and a psychiatric supportis needed to prevent new episodes of the disease.

REFERENCES

1. Breznick DA, Saporito JL. Iatrogenic retropharyngeal emphy-sema with impending airway obstruction. Arch OtolaryngolHead Neck Surg 1989;115:1367–1372

2. Rossiter JL, Hendrix RA. Iatrogenic subcutaneous cervicofacialand mediastinal emphysema. J Otolaryngol 1991;20:315–319

3. Oliver AJ, Diaz EM, Helfrick JF. Air emphysema secondary tomandibular fracture: Case report. J Oral Maxillofac Surg 1993;51:1143–1145

4. Gershwin ME, Gude JK, Petralli J. Factitious subcutaneous em-physema. Ann Intern Med 1971;75:585–587

5. Goudy SL, Miller FB, Bumpous JM. Neck crepitance: Evalua-tion and management of suspected upper aerodigestive tractinjury. Laryngoscope 2002;112:791–795

6. Maunder RJ, Pierson DJ, Hudson LD. Subcutaneous and medi-astinal emphysema: Pathophysiology, diagnosis and manage-ment. Arch Intern Med 1984;144:1447–1453

7. Capes JO, Salon JM, Wells DL. Bilateral cervicofacial, axillary,and anterior mediastinal emphysema: A rare complication ofthird molar extraction. J Oral Maxillofac Surg 1999;57:996–999

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8. Sansevere JJ, Badwal RS, Najjar TA. Cervical and mediastinalemphysema secondary to mandibular fracture: Case report andreview of the literature. Int J Oral Maxillofac Surg 1993;22:278–281

9. Demas PN, Braun TW. Infection associated with orbital subcu-taneous emphysema. J Oral Maxillofac Surg 1991;49:1239–1242

10. Ali A, Cunliffe DR, Watt-Smith SR. Surgical emphysema andpneumomediastinum complicating dental extraction. Br DentJ 2000;188:589–590

11. Leo F, Solli P, Veronesi G, Spaggiari L, Pastorino U. Efficacy ofmicrodrainage in severe subcutaneous emphysema. Chest2002;122:1448–1449

12. Asher R. Munchausen’s syndrome. Lancet 1951;1:339–34113. Karnik AM, Farah S, Khadadah M, Al-Duwaisan H, Denath

FM. A unique case of Munchausen’s syndrome. Br J Clin Pract1990;44:699–701

14. Winans JM, House LR, Robinson HE. Self-induced orbitalemphysema as a presenting sign of Munchausen’s syndrome.Laryngoscope 1983;93:1209–1211

Unusual Late VascularComplications of Sagittal SplitOsteotomy of the MandibularRamus

Jui-Pin Lai, MD,* Ching-Hua Hsieh, MD,* Yu-Ray Chen, MD,y

Chi-Cheng Liang, MD*

Kaohsiung, Taiwan

Abstract: Intraoperative or early postoperative vascular complica-tions are not uncommon problems in sagittal split osteotomies ofthe mandibular ramus; however, reports of late complications areconsiderably rarer. Here, we present two patients who sustainedlate vascular complications after the sagittal split osteotomy. Thefirst patient had a delayed bleeding, which presented itself as a rap-idly expanding swelling of the left cheek from the left external ca-rotid artery 18 days postoperatively. During exploration, a 2 mmlaceration of the external carotid artery located just proximal tothe bifurcation of the internal maxillary artery and the superficialtemporal artery was successfully repaired. The prominent bonyspike of the cut end of medial cortex of the set-back mandibularramus was found against the arterial wall and could possibly havecaused the progressive necrosis of the wall with subsequent spon-taneous rupture. The second patient suffered from a mild noise inthe right ear 2 weeks after the initial surgery; however, a pre-auricular arteriovenous fistula between the right external carotidartery and the external jugular vein was discovered 1 year postop-eratively. The diagnosis was confirmed by angiography, and the

lesion was treated successfully by therapeutic embolization at thattime. To avoid vascular injury, sufficient protection of the soft tissueduring exposure of the mandibular ramus is mandatory. In addi-tion, the direction of the cut of medial cortex is suggested to avoidthe cranialward inclination that creates a sharp, bony end againstthe artery. Awareness of the possible late vascular complications tofacilitate early detection and management is also important.

KeyWords: Sagittal split osteotomy, mandible, hemor-rhage, pseudoaneurysm, arteriovenous fistula

T he sagittal split osteotomy of the ramus for cor-rection of various mandibular deformities has

been well documented since it was first introducedby Trauner and Obwegeser1 in 1957. Of the varioussurgical techniques that have been advocated for re-duction of the prognathic mandible,2–6 the Dal Pontvariation2 of the Trauner and Obwegeser operation ismost frequently used. Being performed by way of themouth, this technique leaves no external scar, and therisk of damaging the facial nerve is very low. It alsopermits considerable displacement in either the for-ward or backward direction with a great area of bonecontact between the proximal and distal segments, fa-voring the process of consolidation. However, manysurgical complications of this procedure have beenidentified over the years, including injury to the facialnerve, infection, fragmentation of the ramus, unex-pected condylar fracture, osteonecrosis, nonunionof bone, and bleeding.4,7–9 As a complication arisingfrom sagittal split osteotomies of the ramus, the fre-quency of hemorrhage has decreased with improvedexperience. Thirty-eight percent of surgeons whoresponded to a questionnaire by Behrman7 in 1972reported hemorrhaging, whereas only 10 cases ofalmost 800 replies (approximately 1.25%) in 5,000questionnaires that were mailed to every oral andmaxillofacial surgeon in North America by Laniganet al9 in 1986 reported hemorrhages. It is of note thatall the vascular complications were reported as occur-ring intraoperatively or just after the surgery, and thedescriptions of any late vascular complications in theliterature was rare.9,10

In the past 24 years between July1981 and July2004, of 960 patients who received bilateral sagittalsplit osteotomy operation by the authors Lai JPand Chen YR, two cases with intraoperative vas-cular complications as well as another two caseswith late vascular complications were encountered.The authors would like to present the details ofthose late vascular complications and to discussthe cause, the management, and proposed preven-tive methods.

From the *Department of Plastic and Reconstructive Surgery,Chang Gung Memorial Hospital in Kaohsiung, Taiwan, and theyCraniofacial Center, Department of Plastic and ReconstructiveSurgery, Chang Gung University, Chang Gung Memorial Hospital,Kaohsiung, Taiwan.

Address correspondence to Dr. Jui-Pin Lai, Department of Plasticand Reconstructive Surgery, Chang Gung Memorial Hospital inKaohsiung, 123, Ta-Pei Road, Niao-Sung Hsiang, Kaohsiung Hsien,Taiwan; E-mail: [email protected]


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CASE REPORTS

Case 1

A 29-year-old man was admitted for a bilateral sag-ittal split osteotomy of the mandible to correct man-dibular prognathism, chin deviation, and class IIImalocclusion in September 1992. The mandible wasset back and rotated left to align the midline by thestandard intraoral approach. The bone ends were sta-bilized by means of trans-osseous wires over the up-per borders. There was no unexpected bleeding notedduring the operation. Total blood loss during the op-eration was approximately 500 ml. Mandibulomaxil-lary fixation with an occlusal splint was applied tosecure immobilization of bony fragments. After thesurgery, the patient recovered well until the 18thpostoperative day when he discovered a rapidlygrowing swelling in his left cheek. The progressivelyworsening throbbing pain compelled him to visit ourclinic the day after the onset of swelling. He deniedany history of facial trauma during the postoperativeperiod. The physical examinations revealed an ill-defined, tense mass in the left cheek. Neither localheat nor erythema was noted. Tenderness was pres-ent, but mild. Although no bruit was heard, bimanualpalpation could detect pulsation over the swollenarea. An enhanced facial computed tomography scanwas performed and showed a blood-density masswith enhancement located lateral to the left mandib-ular ramus (Fig 1). An arteriogram revealed leakageof dye from the left external carotid artery (LECA)just proximal to the bifurcation of the internal max-illary artery (IMA) and the superficial temporalartery (STA) (Fig 2). Because the swelling expanded

rapidly, exploration was performed immediately af-ter the arteriography examination. The LECA was ap-proached by way of the extended submandibular andpre-auricular incisions. After controlling the LECAproximally and ligation of the STA, the hematomawas evacuated. A 2 mm laceration of the LECA wasfound just proximal to the bifurcation of IMA andSTA, as shown on the arteriogram. The lacerationof the LECA was repaired with 7–0 Prolene sutures.The patient recovered uneventfully after the secondoperation. There have been no further complicationsin the following years.

Case 2

A 43-year-old man underwent orthognathic surgicalcorrection of mandible deviation, facial asymmetry,and class III malocclusion in October 1992. Throughthe intraoral approach, the mandible was set backand rotated to the right side using bilateral sagittalsplit osteotomy technique without any intra-operativecomplications. The recorded blood loss was minimal.The postoperative course was uneventful except thatthe patient noted a mild noise in his right ear 2 weeksafter the initial operation. The consultant otologistdid not find a reasonable cause of that unilateral‘‘tinnitus’’ at first. The noises persisted and evenexaggerated in the following 1 year until a thrill with

Fig 2 Case 1. Arteriogram shows extravasation of dye (ar-rowhead) from left external carotid artery at the level prox-imal to bifurcation of STA and IMA. Anteroposterior view.

Fig 1 Case 1. Computed tomography scan reveals a blood-density mass (arrowhead) located laterally to the left man-dibular ramus. Axial view.

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pulsation was noted over the right pre-auricular areaduring a routine follow-up. During this period oftime, there was neither history of trauma nor abnor-mal physical examination. Under the impression ofa vascular lesion over the external carotid system,an angiogram of the right carotid artery was per-formed and showed a shunting between the right ex-ternal carotid artery and external jugular vein poste-rior to the right mandibular ramus (Fig 3). By usingan inflatable balloon, the fistula was occluded (Fig 4).The noise, thrill, and pulsation all vanished after em-bolization. There were no neurologic deficits or othercomplications during the following years.

DISCUSSION

Intraoperative or early postoperative hemorrhagingis not an uncommon problem in sagittal split osteot-omies of the mandibular ramus.4,7,8 Traumatic vascu-lar injuries can be caused by contusion, perforation,incision, or laceration.11 An incomplete laceration ofthe vessel, especially the artery, tends to cause exten-sive bleeding because the partial retraction of thevessel wall creates a persistent orifice. The principlesources of hemorrhage were reported to be lacera-tions of the maxillary, facial, and inferior alveolar ves-sels.4,9 The retromandibular venous plexus7,8 has alsobeen reported as a troublesome bleeding source. Al-though the bleeding caused by an injury during theoperative procedure might be severe enough to abortthe surgery or require extensive blood transfusions,7,9

most hemorrhages can be controlled by ligation,

suture, pressure packing with gauze, or even digitcompression.

Late complications of vascular injury, includingthrombosis, delayed bleeding, formation of arteriove-nous fistula, or pseudoaneurysm, are uncommon andusually attributed to improper management of acuteinjury.12–14 Delayed bleeding after intraoral verticalramus osteotomies have been reported 6 to 10 dayspostoperatively as declotting of the previously in-jured artery and required exploration in the operativeroom to ligate the bleeder.9 In addition to declottingof previously injured vessels, there are other possiblereasons accounting for the delayed bleeding, includ-ing secondary hemorrhage caused by infection, rup-ture of a pseudoaneurysm, and erosion of vessel wallcaused by contusion. Contusions can occur froma crushing injury, with impingement of the arteryagainst a fractured bone or other firm object. Erosionor necrosis of the wall leads to rupture of the vessel.In the first case, we found no evidence of infection,and the intraoperative blood loss during the initialosteotomy procedure was minimal, suggesting adirect injury with a vessel as great as the externalcarotid artery. During the exploration, we found thelaceration of the external carotid artery was very closeto, if not actually against, the prominent bony spike ofthe set-back mandibular ramus. This bony spike mayhave caused progressive necrosis of the arterial wall,leading to spontaneous rupture and bleeding at the18th postoperative day. From the anatomic view-point, the external carotid artery begins at the levelof the upper border of the thyroid cartilage, passesinward cranially, medial to the posterior belly ofthe digastric and stylohyoideus muscles, and then

Fig 3 Case 2. Pre-embolization arteriogram shows earlypresentation of tortuous jugular veins and communicationbetween external carotid artery and jugular vein (arrow-head). Lateral view.

Fig 4 Case 2. Post-embolization arteriogram shows the de-tached balloon (arrowhead) occluded the communication.Lateral view.


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inclines dorsally to the space behind the neck of themandible, where it divides into the superficial tempo-ral and maxillary arteries.15 It is located slightly me-dial and posterior to, not directly behind, the mandib-ular ramus at the level of the transverse cut of themedial cortex. It only can be injured by chisel, burr,or drill if performing the medial cut without adequateprotection. In the case of rotation and setting back ofthe mandible, the cut end of the medial cortex hasgreat potential for impingement on the external ca-rotid artery, especially when the surrounding tissueis edematous. To prevent such kinds of vascular com-plications in sagittal split osteotomy, special careshould be taken to avoid direct injury to the vessel,and protecting the soft tissue is mandatory while ex-posing the mandibular ramus and the cutting of themedial cortex. We suggest that the direction of trans-verse cut of the medial cortex be keep parallel to theocclusal plane, thus avoiding cranialward inclinationthat can create a sharp bony end against the artery.

Transmural injury to the arterial side-wall allowsfor the development of an adjacent hematoma that re-mains in continuity with the arterial lumen. Liquefac-tion and cavitation rapidly follow with the concur-rent development of an endothelial lining at theperipheral of the hematoma, thus resulting in the for-mation of a pseudoaneurysm after a period of time.Arterial pseudoaneurysms can occur as a result ofblunt or, more commonly, sharp trauma to the vesselwall.10,16 Although the occurrences have been rare, ithas been reported after Le Fort I and bilateral sagittalsplit osteotomies10 as well as after facial bone frac-tures.17,18

Furthermore, if the arterial injury is accompa-nied by a nearby venous injury, a fistula tract may re-sult, forming an arteriovenous shunt. In the secondcase, the most likely cause of arteriovenous fistula be-tween the external carotid artery and the jugular veinis direct injury to both of these vessels, either by a burror the drill used in placing the transverse cut of themedial cortex. The lesion in the right face and neckarea become prominent with tortured vessels, withformation of an arteriovenous shunt during thecourse of a year. Clinical symptoms of bruit and thrillindicate the confirmation of this diagnosis.

Most of the postoperative hematomas can bemanaged conservatively by compression, head eleva-tion, and bed rest. The hematoma will be absorbed in4 to 6 weeks. But, if the hematoma expands rapidly,resulting in severe symptoms or unstable vital signs,more aggressive procedures should be taken. Al-though the diagnosis of a vascular lesion could besuspected by clinical history and physical findingsin these two cases, the arteriography is useful in

not only helping to confirm the diagnosis and loca-tion of the injury site but also in providing the optionof embolization of the bleeding artery. Therapeuticembolization of the artery for control of bleedingis a well-known procedure that has been availablesince the 1970s.15,17 Although the procedure of em-bolization in the head and neck is not without itshazards, therapeutic embolization performed byan experienced specialist allows the arteries to beoccluded selectively closer to the bleeder, and nogeneral anesthesia is required; it also gives the sur-geon a degree of control before exploring the lesion.

Surgical exposure to ligate the bleeder is notalways easily accomplished, especially in the cra-niofacial area, and should be reserved for criticalcases or cases failing conservative treatment andtherapeutic embolization. To prevent excessive hem-orrhage during exploration of the lesion site, prox-imal and distal control of the suspected bleedingvessel should first be obtained. In the first case,we controlled the external carotid artery in theneck area and the STA pre-auricularly before di-rectly attacking the hematoma. The vessels shouldbe ligated as close to the bleeding as possible be-cause the more proximal the ligation, the morelikely that distal collateral vessels will reconstitutethe damaged vessel and increase the likelihood ofsubsequent bleeding.

It is noted that these two presented complica-tions happened in the very early period but not inthe following practice of the authors. Occurrenceof such complications may be minimized in thoseexperienced surgeons. In addition, awareness ofthe possible late vascular complications to facilitateearly detection and management should also bestressed.

The authors thank Philip Lieu at Johns Hopkins University forEnglish revision of the manuscript.

REFERENCES

1. Trauner R, Obwegeser H. The surgical correction of mandibularprognathism and retrognathia with consideration of genio-plasty. Oral Surg Oral Med Oral Pathol 1957;10:677–689

2. Dal Pont G. Retromolar osteotomy for the correction of prog-nathism. J Oral Surg Anesth Hosp Dent Serv 1961;19:42–47

3. Leonard MS, Ziman P, Bevis R, et al. The sagittal split osteotomyof the mandible. Oral Surg Oral Med Oral Pathol 1985;60:459–466

4. MacIntosh RB. Experience with the sagittal osteotomy of themandibular ramus: a 13-year review. J Maxillofac Surg 1981;9:151–165

5. Wolford LM, Bennett MA, Rafferty CG. Modification of themandibular ramus sagittal split osteotomy. Oral Surg OralMed Oral Pathol 1987;64:146–155

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6. Wolford LM, Davis WM. The mandibular inferior border split:A modification in the sagittal split osteotomy. J Oral MaxillofacSurg 1990;48:92–94

7. Behrman SJ. Complications of sagittal osteotomy of the man-dibular ramus. J Oral Surg 1972;30:554–561

8. Deeb ME, Wolford L, Bevis R. Complications of orthognathicsurgery. Clin Plast Surg 1989;16:825–840

9. Lanigan DT, Hey J, West RA. Hemorrhage following mandib-ular osteotomies: a report of 21 cases. J Oral Maxillofac Surg1991;49:713–724

10. Bradley JP, Elahi M, Kawamoto HK. Delayed presentation ofpseudoaneurysm after Le Fort I osteotomy. J Craniofac Surg2002;13:746–750

11. Freeark RJ, Baker WH. Arterial injuries. Textbook of Surgery,13th ed. London: W.B. Saunders, 1986:1886–1897

12. Fomon JJ. Late complications of peripheral arterial injuries.Arch Surg 1965;91:610–616

13. Lindenauer SM. Late complications of traumatic arteriovenousfistulas. Surg Gynecol Obstet 1969;129:525–532

14. Rich NM. Traumatic arteriovenous fistulas and false aneur-ysms: A review of 558 lesions. Surgery 1975;78:817–828

15. Stein BR, Kerber CW. Therapeutic arterial embolization for posttraumatic hemorrhage: report of case. J Oral Surg 1981;39:439–441

16. Conner WC,III, Rohrich RJ, Pollock RA. Traumatic aneurysmsof the face and temple: a patient report and literature review,1644 to 1998. Ann Plast Surg 1998;41:321–326

17. Clark S, Wood N. Traumatic aneurysms of the internal maxil-lary artery and their treatment by embolisation: a report of twocases. Br J Oral Maxillofac Surg 1990;28:302–305

18. Gerbino G, Roccia F, Grosso M, et al. Pseudoaneurysm of theinternal maxillary artery and Frey’s syndrome after blunt facialtrauma. J Oral Maxillofac Surg 1997;55:1485–1490

Fronto-Orbital LeptomeningealCyst Manifested withXophthalmus

Orhan Sen,* Omer Refik Ozerdem,y Osman Kizilkilic,z

Lokman Aktas,§ Levent Sener,* Tamer Seyhan,y Nur Altinorsy

Adana, Turkey

Abstract: A 41-year-old female patient with fronto-orbital leptome-ningeal cyst is presented. After decompression of the cyst, repair ofbone and dura defects were achieved with autogenous bone graftsand pericranial flap. Our case was very interesting in several as-pects: a head trauma at 3 years of age and manifestation of clinicalsymptoms (headaches and exophthalmia) after almost 4 decadesthat had been progressive for 6 months and a rare location (fronto-orbital region) that required a special management. A thoroughhistory is crucial in such cases because clinical symptoms mightappear after decades. An old fracture that is surrounded by thin

calvarial tissue in conjunction with intracranial cystic formationshould make one take a leptomeningeal cyst into considerationin differential diagnosis. Extensive dissection and adequate accessosteotomies followed by repair with autologous and vascular tis-sues are the key factors for a successful outcome in the manage-ment of fronto-orbital pathologies.

Key Words: Leptomeningeal cyst, fronto-orbitaltumors, exophthalmus

L eptomeningeal cysts, which are thought to occuras a result of herniation of the leptomeninges

under the mechanical force of brain pulsations afterdural rupture underlying the fracture site (Taverasand Ransohoff as cited by Britz et al,1) are rare in boththe adults and children. Britz et al. (1998), Weinberget al. (1999) Ozer et al. (1999) found 5, 19 and 47 adultcases (including their own cases), respectively in theliterature.1–3 Ozer et al3 reported that of 47 cases, 16(34.04%) located out of the cranial vault and 23(48.94%) occurred after a childhood trauma.

Tumors that invaded the fronto-orbital area pres-ent a challenge in their diagnosis and surgical man-agement. Any intervention in this area would involvethe intracranial and extracranial (nasopharyngeal space,orbits, sinuses, etc.) structures. Meticulous planningof the surgical intervention and the choice of the re-pair material (alloplastic vs. autogenous, vascularizedvs. nonvascularized, etc.) are very important to avoidlife threatening infections, recurrence, aesthetic im-pairment, and dysfunction of the local structures. Ac-cess to the pathologic area, tumor ablation, and repairof the resulting defect are equally important steps inthe management of fronto-orbital tumors.4–7

In this case report, we present a rare pathologicformation in an uncommon location with particularclinical symptoms and treatment: a leptomeningealcyst of the fronto-orbital region that was manifestedwith exophthalmia.

CASE REPORT

A 41-year-old female patient who had complainedof headaches and progressive left eye bulging for6 months was admitted to our clinic (Figs 1 and 2).A definitive history revealed head trauma from a highfall when she was 3 years old. No surgical interven-tion was performed after this accident. General exam-ination did not reveal any significant finding otherthan left nonpulsatile exophthalmia (Fig 2). Therewas no impairment of eye movements and visionor any other neurologic function. Two- and three-dimensional computed tomographic (CT) pictures

From the *Department of Neurosurgery, the yDepartment ofPlastic and Reconstructive Surgery, the zDepartment of Radiology,and the §Department of Ophthalmology, Baskent University,School of Medicine, Adana, Turkey.

Address correspondence to Dr. Orhan Sen, or Dr. Omer RefikOzerdem, Basxkent Universitesi Adana Hastanesi, 01250 YO1/4regir,Adana, Turkey; E-mail: [email protected]


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demonstrated an old fracture that was surrounded bythin calvarial tissue in the left fronto-orbital region(Fig 3). Orbital magnetic resonance imaging (MRI)showed proptosis of the left eye globe, bony defectof the left orbital roof, and extension of cerebrospinalfluid (CSF) into the orbit (Fig 4). These clinical andradiologic findings strongly suggested a growingfracture (leptomeningeal cyst). Surgical intervention

was initiated with coronal incision followed by eleva-tion of the bilateral epicranial flaps that were con-nected to temporal muscles. CSF leakage throughsmall bone defects on irregular supraorbital andorbital roof bone was noticed during dissection. Thecystic tissue and orbital roof defect were exposedafter left frontotemporal osteotomy (Fig 5). After de-compression of the cystic tissue, a wide dural defect

Fig 1 Preoperative anteroposterior appearance of thepatient.

Fig 2 Preoperative submentovertex appearance of thepatient that demonstrates left proptosis.

Fig 3 Preoperative coronal CTimages that show orbital roofdefect on the left side (arrow).

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appeared on the left frontal lobe. Bone grafts wereobtained from the inner cortex of the osteotomymaterial. Cranialization of the left frontal sinus wasachieved with removal of the posterior sinus wall,and the frontonasal canal was plugged with epicra-nial and bone grafts. Bone graft blocks were placedon the anterior cranial base and fixed to the supraor-bital rim to overlap the bone defects on the anteriorcranial base (Fig 6). The left epicranial flap was

brought to the fronto-orbital area to cover the bonegraft and for duraplasty. This flap, which was wideenough to cover the whole frontal dura including thedefective areas, was sutured to the intact dura andthe osteotomy edges through multiple holes (Fig 6).Fibrin glue was also added to the duraplasty areato reinforce the repair. The osteotomy material wasput back in its original place and fixed with screw-plate systems. Onlay bone grafts were also placedon the thin left supraorbital bone to cover the bonedefect in this area and also to provide symmetry withthe contralateral side. The postoperative period wasuneventful. Follow-up examination after 2 monthsrevealed regression of left eye proptosis and head-aches (Figs 7 and 8). CT images, obtained at this time,showed wel-adapted bone grafts without any com-plication (Fig 9).

DISCUSSION

Our case was very interesting in several aspects:a head trauma at 3 years of age and manifestationof clinical symptoms (headaches and exophthalmia)after almost 4 decades that had been progressive for6 months and a rare location (fronto-orbital region)that required a special management. A forgotten headtrauma from long ago might make a diagnosis of lep-tomeningeal cyst difficult to establish. A thoroughhistory in conjunction with detection of a cystic for-mation underlying the irregular calvarial bone shouldprompt a diagnosis of leptomeningeal cyst. In the pe-diatric population, the most common presentation isthat of a nontender, pulsatile scalp mass associated

Fig 4 Preoperative coronal MRI showing a left fronto-orbital cyst that is filled with opaque material. Extensionof the opaque material into the orbit through the orbitalroof defect is also seen (arrow).

Fig 5 Intraoperative view of the leptomeningeal tissue(grasped by the forceps) and orbital roof defect (shownwith the aspirator).

Fig 6 Autogenous calvarial bone graft to repair orbitalroof (asterisk) and pericranial flap (arrow) to cover the bonegraft and for duraplasty.


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with neurologic findings, whereas a nontender non-pulsatile mass and headaches without neurologic find-ings constitute the common clinical manifestation inadults.1 Fronto-orbital leptomeningeal cases havealso been reported.8,9 Menku et al8 reviewing suchcases, found diplopia, eyelid swelling, proptosis, lim-itation of ocular movements, and pulsatile orbitalmasses as possible symptoms. Clinical symptomsin our case were consistent with these general clinicalpresentations, and the mass effect of the pathologyappeared as progressive exophthalmus.

It has been stated that the dural defect is widerthan the fracture edges in leptomeningeal cases, andadvocated more extended osteotomy to detect theboundaries of the intact dura has been advocated.1,10

In the fronto-orbital region, a coronal incision followedby frontal or frontotemporal osteotomies wouldprovide adequate exposure. Ideal reconstructionof the bony and soft tissues would be probable afterextensive dissection and appropriate osteotomies.

The materials most often used are free peri-cranium or fascia for duraplasty and bone grafts or

Fig 9 Postoperative (2 months)coronal and vertical CT imagesthat show orbital roof repairedwith bone graft (arrow).

Fig 7 Postoperative (2 months) anteroposterior appear-ance of the patient.

Fig 8 Postoperative (2 months) submentovertex appear-ance of the patient that demonstrates relieved proptosis.

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alloplastic materials to repair bone defects synchro-nously in cases with leptomeningeal cysts.1,3,8–11

We used autogenous bone grafts to repair the fronto-orbital bone and a pericranial flap both for duraplastyand to cover the bone grafts in our case. Autogenousbone graft is the most ideal choice for craniofacialbone repair.12–14 These become consolidated into thesurrounding skeleton, which no foreign substancecan, and they have an extraordinary resistance to in-fection.13 Moreover, autogenous bone should be theonly source of repair for defects in the frontal regionnear or extending into the frontal sinus to avoid in-fection.14 Dural repair is an important step in recon-struction to avoid CSF fistula.4,5 We believe thatautogenous bone grafts that are covered with vascu-larized tissues such as epicranial flaps offer the bestchoice to repair cranial and dural defects. Pericranialflaps that are attached to the temporal muscles couldbe raised during the initial dissection for most orbi-tocranial procedures (Tessier’s method, as cited byWolfe).15 These flaps could be used as a protectivevascular layer for bone grafts.15 A dural patch thatis connected with a nonvascularized bone graft or analloplastic material would enhance the risk of CSFfistula. Our combination offers an ideal situationfor wound healing.

It has been debated whether asymptomatic casesshould be treated.1,3,10 Our case is a good examplethat leptomeningeal cysts might become clinically ev-ident and progressive even after decades. We believethat surgical treatment of leptomeningeal cysts wouldbe better performed after diagnosis.

In conclusion, a thorough history is crucial be-fore establishing the diagnosis of leptomeningeal cyst;determination of a head injury that occurred in thepast is very important, and clinical symptoms mightappear even after decades. CT and MRI examinationsare greatly helpful to detect the pathology. An oldfracture that is surrounded by thin calvarial tissuein conjunction with intracranial cystic formationshould make one to take a leptomeningeal cyst intoconsideration in differential diagnosis. Extensive dis-section and adequate access osteotomies followed byrepair with autologous and vascular tissues are thekey factors for successful outcome in the manage-ment of fronto-orbital pathologies.

REFERENCES

1. Britz G, Kim K, Mayberg M. Traumatic leptomeningeal cyst inan adult: a case report and review of the literature. Surg Neurol1998;50:465–469

2. Weinberg JS, LeRoux PD, Panasci D, et al. Adult growing skullfracture mimicking a skull tumor. Acta Neurochir (Wien) 1999;141:545–546

3. Ozer H, O-zdemir N, Mertol T. Traumatic leptomeningeal cystin an adult: a case report and review of the literature. Norol BilD (Turkish, serial online). October–December 1999;16. Availablefrom: http://www.med.ege.edu.tr/norolbil/1999/nbd06699.html.Bornova, Izmir, Turkey: Ege University, Accessed December 20,1999

4. Langstein HN, Chang DW, Robb GL. Coverage of skull basedefects. Clin Plast Surg 2001;28:375–382

5. Spinelli HM, Persing JA, Walser B. Reconstruction of the cranialbase. Clin Plast Surg 1995;22:555–561

6. Chang DW, Lagstein HN, Gupta A, et al. Reconstructive man-agement of cranial base defects after tumor ablation. Plast Re-constr Surg 2001;107:1346–1355

7. Ozerdem OR, Wolfe SA. Fronto-orbital tumorlere yaklasim(Management in fronto-orbital tumors). From the Proceedingsof the 25th National Congress of Turkish Association of Plastic,Reconstructive and Aesthetic Surgery, Samsun, 2003

8. Menku A, Koc RK, Tucer B, et al. Growing fracture of the orbitalroof. Neurosurg Rev. 2004;27:133–136

9. Amirjamshidi A, Abbassioun K, Tary AS. Growing leptomenin-geal cyst of the roof of the orbit presenting with unilateral ex-ophthalmus. Surg Neurol 2000;54:178–182

10. Ziyal M, Aydın Y, TO1/4rkmen CS, et al. The natural historyof late untreated growing skull fractures: report on two cases.Acta Neurochir (Wien) 1998;140:651–654

11. Kurosu A, Fujii T, Ono G. Posttraumatic leptomeningeal cystmimicking a skull tumor in an adult. Br J Neurosurg 2004;18:62–64

12. Millard DR and Yates BM. Practical variations of cranioplasty.Am J Surg 1964;107:802–809

13. Wolfe SA. Autogenus bone grafts versus alloplastic materials. In:Wolfe SA, Berkowitz S, eds. Plastic Surgery of the Facial Skeleton.Philadelphia: Lippincott, Williams & Wilkins; 1989:25–38

14. Wolfe SA. Cranial defects. In: Wolfe SA, Berkowitz S, eds. Plas-tic Surgery of the Facial Skeleton. Philadelphia: Lippincott,Williams & Wilkins; 1989:687–719

15. Wolfe SA. The utility of pericranial flaps. Ann Plast Surg 1978;1:146–153

Reconstruction Necessitated bySevere Hypoplasia ora Columella Defect

Megumi Taji, MD,* Tsuyoshi Takato, MD, PhD,y

Takafumi Susami, DDS, PhD,y Tomoaki Eguchi, MD, PhDy

Tokyo, Japan

Abstract: In reconstruction necessitated by severe hypoplasia ora columella defect, the surgeon must consider various factors ineach case, such as the characteristic columellar shape, color match,texture, patient age, original disease, and surrounding scars. Inthese cases, reconstruction of both the cartilaginous strut and

From *Plastic and Reconstructive Surgery, Yaizu City Hospital,Shizuoka, Japan; yDepartment of Oral and Maxillofacial Surgery,Orthodontics, University of Tokyo Hospital, Tokyo, Japan.

Address correspondence to Dr. Taji, Yaizu City Hospital, Plasticand Reconstructive Surgery, 1000, Dohara, Yaizu-shi, Shizuoka,Japan, 425-8505; E-mail: [email protected]


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the overlying skin is necessary and important to obtain good re-sults. The authors report three cases of reconstruction of the colu-mella with satisfactory results. Case 1 involved a 6-year-old girlwith complete bilateral cleft lip and severe hypoplasia of the pre-maxilla and prolabium. Columellar reconstruction was performedwith small triangular flaps at the columella base, together with a ribchondral graft for cartilaginous support. Case 2 involved a 12-year-old girl with a complete bilateral cleft lip and cleft palate. Columel-lar reconstruction was performed with small triangular flaps at thecolumella base, together with bilateral conchal cartilage grafts.Case 3 involved a 17-year-old boy with a right complete cleft lipand columellar defect caused by previous infection after secondarycheiloplasty. Columellar reconstruction was performed using a leftnasal vestibular flap and septal cartilage grafting, together witha bilateral conchal cartilage graft beneath the flap. The authors con-sider the unilateral nasal vestibular flap to be very useful in care-fully selected unilateral cleft cases.

R econstruction necessitated by severe hypoplasiaor a columella defect is still a challenging proce-

dure because surgeons must pay attention to manyfactors in each case, such as the characteristic colu-mellar shape, color match, texture, patient age, orig-inal disease, and surrounding scars. In a severelyhypoplastic columella, reconstruction of both the car-tilaginous strut and the overlying skin is needed. Re-construction of these two components is the key toobtaining good results. We report three cases of re-construction of the columella with satisfactory resultsand review some references.

CASE REPORTS

Case 1

A 6-year-old girl presented with a complete bilateralcleft lip and severe hypoplasia of the premaxilla andprolabium (Fig 1A). Holoprosencephaly (type Vby DeMyer classification) was suspected. The pa-tient showed a slight degree of mental retardation.At 3 months of age, she underwent primary cheilo-plasty by the method of Veau in our hospital. The col-umella was short and narrow. In reconstruction, thecolumella was widened by small triangular flapsat the columellar base (Figs 1B, 1C). Furthermore,

columellar elongation was simultaneously performedwith rib chondral grafting for cartilaginous support(Fig 1D).

Case 2

A 12-year-old girl presented with a bilateral completecleft lip and cleft palate (Fig 2A). She underwent pri-mary cheiloplasty and palatoplasty in another hospi-tal. Orthodontic treatment started at 7 years of age.Iliac bone grafting was performed to repair a rightalveolar cleft at 13 years of age. Her columella wasshort and narrow. The columella was elongated bysmall triangular flaps at the columellar base. Bilateralconchal cartilages were inserted under the columellaskin for a cartilaginous strut (Figs 2B, 2C). Conchalgrafting was chosen because the patient declined har-vesting of the rib cartilage. Closure of the left alveolarcleft is planned (Fig 2D).

Case 3

A 17-year-old boy presented with a right completecleft lip and columellar defect (Fig 3A). Primary chei-loplasty was performed at 5 months of age. Second-ary cheiloplasty was performed at 8 years of age, butstreptococcal toxic shock syndrome occurred postop-eratively, resulting in necrosis of the columellar skinand underlying septal cartilage. Columellar recon-struction was performed using a left nasal vestibularflap and septal cartilage graft (Figs 3B, 3C). The flapwas carefully designed to avoid including the resid-ual scar tissues caused by previous cheiloplasty andinfection. A 15 *3 7-mm vestibular flap was elevatedto cover the frontal part of the columella, with the do-nor part primarily closed. We harvested a 12 3 20-mmseptal cartilage graft to reconstruct the cartilaginousstrut. The mucosal defects of lateral parts of the col-umella were covered with bipedicled nasal septalmucosa (Fig 3D). The flap remained viable despitethe presence of distal epidermal necrosis. Fourmonths after the first operation, bilateral conchal car-tilage grafts were inserted under the transposed flap

Fig 1 Case 1. (A) Preoperative view. (B, C) Design of surgery. (D) Result at 18 months after columellar reconstruction.

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through a stab incision at the nasal tip for augmenta-tion of the columella (Fig 3E).

RESULTS

Good results were obtained in all three cases.

DISCUSSION

Columellar defects are caused by acquired infection,trauma, or tumor resection or are accompanied withcongenital anomalies.1–6 Columellar reconstruction isgenerally indicated after 2 or 3 years of age. We preferto perform the procedure after puberty to obtainbetter final results, because reconstruction of boththe cartilaginous and soft tissues is necessary in thesecolumellar reconstructions. However, the operationis sometimes performed around the preschool yearsin consideration of psychological pressure.

To improve the columellar hypoplasia accompa-nying a bilateral cleft lip, so-called short columella,several well-known methods have been used conven-tionally, such as the method of Cronin, a forked flap,V-Y advancement, and a stellate flap.7–9 Small tri-

angular flaps, nasal vestibular flaps, and reverse-Uflaps used in secondary operation provide visualeffects such as slight lengthening of the upper col-umella.10–12 Composite grafting from the auricleproduces good results in some cases.13–15 In patientswith more severe deficits of columellar tissue, thereis a tendency to use an arterial flap6,16 or a free flaptransfer.2,5 However, there are no standard surgicalmethods that are considered the first choice for var-ious situations.3

In cases of mild columellar hypoplasia, we usu-ally choose nasal tip tightening or columella elonga-tion using small local flaps. However, in severe cases,an autologous bone graft or cartilage graft is neededas a framework, and local flaps are required to coverthe skin deficit. We believe that inserting a cartilagi-nous framework into the subcutaneous tissue underthe columella yields favorable results in patients withsevere columellar hypoplasia.16

The most desirable donor site is a local flap nearthe columella with sufficient volume and blood sup-ply, whereas the septal cartilage graft is the best do-nor site in view of stiffness and an invisible remainingscar. We use costal cartilage grafting in children so as

Fig 3 Case 3. (A) Defect and nasalvestibular flap planned. (B, C) De-sign of surgery. (D) Flaps trans-posed. (E) Result at 6 monthsafter conchal cartilage grafting.

Fig 2 Case 2. (A) Preoperative view. (B, C) Design of surgery. (D) Result at 14 months after columellar reconstruction.


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not to impair nasal growth. If neither of these grafts isavailable in an adult patient, we then use auricularcartilage. However, the auricular cartilage is inferiorin hardness, and two-piece grafts from both ears arepreferable. The local flap should be designed to main-tain blood supply and avoid scar tissues caused bythe previous operations. In case 1, we banked the na-sal vestibular tissues at the primary cheiloplasty andused them to reconstruct the lateral wall of the colu-mella in the current operation. The columella waswidened with these flaps. In case 3, in which thepatient had a larger defect, we chose a sufficientlysized vestibular flap, which provided enoughvolume without leaving a visible donor site scar.The nasal vestibular flap was originally reportedby Vecchione12 in 1980, which left mild undulationand a noticeable scar at the center of the reconstructedcolumella. In some of their cases with hairy colu-mella, further skin grafting was performed using pre-auricular skin to obtain a hairless and color-matchedappearance. The transverse forked flap reported byEarley and Chantarasak1 resembled to some extentthe procedure we used in case 3. Earley and Chantar-asak used a flap with the body derived from the pro-labium tissue and thus had a disadvantage of short-ening the upper lip height, especially in youngerpatients. We planned a unilateral nasal vestibular flapto overcome this drawback. Although scars causedby the previous surgery precluded the harvest of bi-lateral flaps, the unilateral flap provided enough hair-less tissue because hair follicles were destroyed byprevious infection. We consider the unilateral nasalvestibular flap to be very useful in carefully selectedunilateral cleft cases.

REFERENCES

1. Earley MJ, Chantarasak ND. The transverse forked flap in col-umella reconstruction. Br J Plast Surg 1989;42:270–273

2. Gucer T. Retroauricular prefabricated chondrofasciocutaneousflap for reconstruction of the columella. Plast Reconstr Surg2002;109:1090–1093

3. Mavili ME, Akyurek M. Congenital isolated absence of the na-sal columella: reconstruction with an internal nasal vestibularskin flap and bilateral labial mucosa flaps. Plast Reconstr Surg2000;106:393–399

4. Ozek C, Hakan G, Bilkay U, et al. Nasal columella reconstruc-tion with a composite free flap from the root of auricular helix.Microsurgery 2001;22:53–56

5. Pribaz JJ, Falco N. Nasal reconstruction with auricular micro-vascular transplant. Ann Plast Surg 1993;31:289–297

6. Yanai A, Nagata S, Tanaka H. Reconstruction of the columellawith bilateral nasolabial flaps. Plast Reconstr Surg 1986;77:129–132

7. Converse JM. Reconstructive Plastic Surgery. Philadelphia:W.B. Saunders, 1977:2173, 2189, 2192

8. Cronin TD. Lengthening columella by use of skin from nasalfloor and alae. Plast Reconstr Surg 1958;21:412–423

9. Millard DR. Columellar lengthening by a forked flap. Plast Re-constr Surg 1958;22:454–457

10. Nakajima T, Yoshimura Y, Nakanishi Y, et al. Comprehensivetreatment of bilateral cleft lip by multidisciplinary team ap-proach. Br J Plast Surg 1991;44:486–494

11. Nakajima T, Yoshimura Y, Kami T. Refinement of the‘‘reverse-U’’ incision for the repair of cleft lip nose deformity.Br J Plast Surg 1986;44:486–494

12. Vecchione TR. Columella reconstruction using internal nasalvestibular flaps. Br J Plast Surg 1980;33:399–403

13. Burget GC, Menick FJ. Nasal support and lining: the marriageof beauty and blood supply. Plast Reconstr Surg 1988;84:189–203

14. Cho BC, Park JW, Baik BS. Correction of severe secondary cleftlip nasal deformity using a composite graft: current approachand review. Ann Plast Surg 2002;48:131–137

15. Shin KS, Lee DH. Columella lengthening in nasal tip plasty oforientals. Plast Reconstr Surg 1994;94:446–453

16. Yui S, Yonehara Y, Nakatsuka T, et al. Correction of the bilateralcleft lip-associated nasal deformities after adolescence: opera-tive techniques in our institutions. J Jpn Plast Reconstr Surg2001;21:153–164

A Custom-Made DistractionDevice for ExperimentalMandibular DistractionOsteogenesis

Muhitdin Eski, MD,* Mustafa Nisanci, MD,* Yakup Cil, MD,*Mustafa Sxengezer, MD,* Ayhan Ozcany

Ankara, Turkey

Abstract: Distraction osteogenesis has recently become popularin craniofacial reconstruction, after it was first described for longbones. Despite the widespread clinical use of this technique,currently, lack of an ideal experimental model for small animalshampers investigations focused on its molecular basis, whichwarrants further elucidation. The authors developed a new de-vice for distraction of the rat mandible and, to asses its effective-ness, practiced on 30 animals assigned to either an acute 3-mmdistraction group (n = 7) or a gradual distraction group (0.25 mmtwice a day for 6 days; n = 23). The authors applied the de-vices to a localization that allowed them to perform the ostomiesposterior to the molar teeth. Because of a U-shaped plate that theauthors used for posterior pin fixation, their device maintained itsstability until the end of the study, despite the delicate anatomy ofthe bone at the posterior part of the mandible. Additionally, the

From the *Department of Plastic and Reconstructive Surgery;yDepartment of Pathology, Gulhane Military Medical Academy,Etlik, Ankara, Turkey.

Address correspondence to Dr. Eski, GATA Plastik, Rekonstruktifve Estetik Cerrahi A.D. 06018 Etlik, Ankara, Turkey; E-mail: [email protected]

This study was supported by the Experimental Research Centerof Gulhane Military Medical Academy.

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authors described a practical and easy method of transient mandib-ular stabilization method that facilitates the manipulations whilethe mouth is open for safer airway control during surgery. Thisnew nutrition technique with enteral feeding solution solved theproblem of progressive weight loss after surgery. Application ofthe device was easy and practical, without demanding complexmanipulations. The authors did not observe any device dislodge-ment or a high rate of accidental fractures during the manipula-tions. They demonstrated that the device works properly and isable to create ossified regenerate bones that fill the entire distrac-tion gap, which can be used for various investigations during dis-traction osteogenesis.

A fter its introduction by Codivilla in 1905,1 distrac-tion osteogenesis (DO) was popularized by

Ilizarov2 for lengthening of long bones. The experi-mental and clinical studies conducted by IIizarov3,4

contributed much to both the establishment of basicguidelines for DO and demonstration of its clinical ef-ficacy on a scientific basis. Snyder et al5 were the firstto use DO in the craniofacial region of a canine model.After the report of Snyder et al of a pioneering exper-imental study in the craniofacial region, two successfulmandibular lengthening studies in dogs, accom-plished by using a special intraoral distraction device,were published by Michieli and Miotti.6 Later, detailedhistologic analysis of the ossification process aftermandibular distraction was published as a result ofensuing successful experimental studies using caninemodels.7,8 In clinical practice, after the preliminary re-port regarding its first use in the human mandible byMcCarthy et al9 in 1992, use of DO in the craniofacialregion rapidly became popular among surgeons. Thefirst clinical application was performed by Aytemiz in11 adult patients for the correction of mandibular de-formities.10,11 Recently, numerous reports of successfulcalvarial, full-face, and midface distractions havemade this technique the treatment of choice in surgicalcorrection of many deformities involving the craniofa-cial skeleton.

Despite the widespread use of DO in currentclinical practice, some questions regarding the osteo-genesis process itself and resultant bone still remainto be clarified. Although the histologic and ultrastruc-tural changes associated with DO were investigatedto some extent using larger animal models, molecularmechanisms governing these changes were not eluci-dated well until the description of a rat model formandibular distraction.7,8,12 A rat mandible modelfor distraction has certain critical advantages incomparison to the models in larger animals (sheep,rabbits, dogs, pigs), including availability of molecu-lar reagents, lower cost, and ability to perform manymore procedures to acquire data that can be analyzedstatistically.7,8,13–17 However, studies in which vari-

ous rat models described in the literature were usedhave proved to have some drawbacks that werethought to stem from the designs of the devices forsuch a small and delicate mandible.12,18,19

This study was undertaken to develop a new anda better distraction device, specially designed to elim-inate the well-known disadvantages and shortcom-ings of the ones previously described in the literature.In this article, the device we improved is described,and the efficacy of the device is demonstrated with anexperimental study.

MATERIALS AND METHODS

Design of the Device

All parts of the device were designed by one of the au-thors (M.E.) and manufactured by Modelsan (Ankara,Turkey). The distraction device was synthesized us-ing a 12-mm jackscrew with each end embedded ina specially designed polyvinyl chloride block, anda metal rod was placed in the inferior aspect of thedevice to prevent torque around its long axis. Thepolyvinyl chloride block also had a vertical holefor pins. A pair of specially designed pins servedfor fixation of the device. The pins consisted of twoparts welded together: a bolt, where the distal endwas thickened to screw the pin easily into the man-dible, and a 23 8-mm titanium screw (Fig 1). The endof the pin, which was composed of a titanium screw,was intended to provide mandibular fixation,whereas the proximal end was to fix the distractorto the pin through a washer and a nut. The anteriorpin was to be secured into the body, whereas the

Fig 1 The custom-made distraction device, which consistsof a 12-mm jackscrew, a 12-mm metal rod, polyvinyl chlo-ride blocks, and a pair of specially designed pins.


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posterior one was to be placed into the angle of themandible. It is well known that the angular regionof the rat mandible is thin and delicate (approximately0.3 mm), rendering this region prone to pin looseningthat may lead to failure of the whole procedure. Toovercome this shortcoming, a U-shaped plate wasdesigned to reinforce the posterior pin fixation. TheU-shaped plate was formed in a fashion such thatthe thin bone of the angle barely fit into the groovebetween the open limbs of the U when it was placedup from the inferior rim (Fig 2). This special construc-tion of the plate allowed its stabilization on the lowerrim like a clip, obviating the need for an additionalmeans of securing during the drilling and screwingprocedures. For the posterior pin fixation, each limbof the U-shaped plate had a hole on the same axis.The hole on the medial limb of the U-shaped platewas smaller in diameter than the one on the laterallimb. This feature allowed compression of the bonebetween two leaves of the U-shaped plate whenthe pin was screwed and tightened through theseholes, as occurs in the lag screw principle. Use of thisU-shaped plate for posterior pin fixation into the thinbone of the angle increased the stability of the device.One quarter turn of the device provided 0.25 mm ofseparation between the bone edges.

Application of the Device

General anesthesia was given with 10% intramuscu-lar ketamine (90 mg/kg) and 2% xylazine (10 mg/kg).Cefazolin (10 mg/kg) was administered for 3 postop-

erative days for infection prophylaxis. The surgicalfield on the right hemimandibular area was shavedand prepared for surgery. Transient interincisive fix-ation was achieved with a piece of plastic tube (1 cmin length) obtained from an intravenous fluid infu-sion set. Insertion of this tube segment vertically be-tween the upper and lower incisive teeth stabilizedthe mobile mandible (Fig 3).This maneuver not onlyfacilitated manipulation of the mandible during thesurgery, but also helped to prevent accidental bonefractures during the drilling and osteotomy proce-dures. Then, the body and the angulus of the righthemimandible were exposed through a 1-cm incisionon the inferior mandibular rim. The osteotomy sitewas determined and marked posterior to the molarteeth. Four millimeters anterior to the marked line,a 1.6-mm bicortical hole was drilled at low rotationspeed (,3000 rpm) with a hand drill under irrigation(Dremel Europe Konijnenberg 60; Breda, The Nether-lands). The anterior pin was screwed into this holeunder vision. Four millimeters posterior to the pre-dicted osteotomy line, a limited area of bone was freedfrom its muscular attachments for posterior pin fixa-tion at the lower border of the thin angulus. First, theU-shaped plate mentioned above was placed onto theinferior rim of the mandible, where the bone wasthick and strong. Then, a 1.6-mm bicortical hole wasdrilled in the bone through the larger hole on the lat-eral leaf of the plate. Because the diameter of the holeon the medial or inner leaf of the plate was smaller,the posterior pin acted like a lag screw, pressing thebone between the leaves of the plate as it was screwedin. Despite the delicate structure of the bone in thisarea, this type of posterior pin fixation providedstronger and firmer stabilization by increasing the

Fig 2 Appearance of the distraction device, attached to themandible, from the bottom. The anterior pin is secured tothe body of the mandible, whereas the posterior pin is se-cured to the angle of the mandible with a U-shaped plate.Note the location of osteotomy site (dotted line) posterior tothe last molar.

Fig 3 Transient interincisive fixation performed with a1-cm segment of polyvinyl chloride tube. The pins areplaced and the osteotomy is completed.


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contact surface of fixation between the leaves of theplate. Furthermore, the special U-shaped constructionof the plate served as a conduit for transfer of theforce toward the thicker and stronger inferiorborder of the angle. After placement of the pins,through the exposure provided by retraction of themasseter muscle, osteotomy was accomplished witha 1-mm diamond burr running at low speed underirrigation. Care was taken to avoid violation of theoral mucosa as well as the molar teeth roots. After ir-rigation of the surgical field for the last time, the mas-seter muscle was reapproximated with absorbablesutures while the skin was closed primarily withnonabsorbable silk sutures. After this, the distractor(jackscrew assembly) was secured to the pins witha washer and a nut (Fig 4). Intraoperative activationof the distractor not only confirmed the complete os-teotomy, but also demonstrated that the device worksproperly.

Animals and Experimental Protocol

Thirty-five adult male Sprague-Dawley rats, weigh-ing 250 to 300 g, were obtained from the ExperimentalResearch Center at Gulhane Military Medical Acad-emy. The animals were caged individually at roomtemperature with a 12-hour light/dark cycle andhad free access to water and standard laboratory foodfor rats. The study and the experimental protocolwere approved by Animal Research Committee ofGulhane Military Medical Academy. All animalsused in this study received humane care in compli-ance with the Guide for the Care and Use of LaboratoryAnimals published by the ethics council.

Five rats that could not complete experimentwere excluded from the study, and the remaining

30 rats were assigned to one of two experimentalgroups: One group, which consisted of 7 animals,underwent immediate lengthening (3 mm) aftersurgery, and the other group, which consisted of 23animals, underwent gradual distraction for 6 daysafter a 3-day latency period. The distraction rhythmwas set at two times per day and 0.25 mm each time(0.5 mm/d, 3 mm total lengthening). In both groups,the devices were kept in place for rigid external fix-ation for 4 more weeks after distraction.

Radiographic Techniques

Cephalograms were obtained (Ultranet SA; GE Med-ical Systems by Medys, Manzo, Italy) immediatelyafter the operation to see whether the pins and thedevice were in proper position, with the end of thepins penetrating the medial and lateral holes ofthe U-shaped plate. Radiologic examination was alsoused for investigation of the bone formation at theend of the activation period, at the second week,and at the end of the consolidation period.

Tissue Processing

After the animals were killed, the mandibles weredissected free from the surrounding tissue. Radio-graphs were obtained and then fixed in 10% formalin,decalcified in a 50:50 (vol/vol) solution of 40% formicacid and 6.26% sodium formate for 5 days, and em-bedded in paraffin. Five-micrometer sagittal sectionswere mounted on slides, and hematoxylin and eosinstaining was performed using standard protocols.

RESULTS

We operated on 35 animals. Of the 35, 30 completedthe study. During the learning curve of the proce-dures, 3 of the first 8 animals sustained mandibularfractures intraoperatively; these fractures occurredespecially during manipulation of the mandible.We then developed a transient interincisive-fixationmethod. After improvement of our technique withpolyvinyl chloride tube fixation, no more accidentalintraoperative fractures occurred.

No intraoperative deaths occurred as a compli-cation during the study. This was attributed to theimproved intraoperative airway patency, thanks tothe wide-open mouth resulting from our transientinterincisive-fixation method. However, at the begin-ning of the study, another two animals died on post-operative day 10 as a result of inadequate nutritionand excessive weight loss. To increase nutrition, a di-luted enteral feeding solution (Biosorb Drink; Nutri-cia, Zoetermeer, The Netherlands) was provided

Fig 4 The distractor applied to the right hemimandible ofthe rat.


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(50% water and 50% enteral feeding solution) as a sec-ond bottle in addition to water (Fig 5). After additionof this supplement, no animal died as a result of se-vere weight loss postoperatively, revealing that ade-quate nutrition was provided. Rats tolerated the sur-gical procedures and distraction device withoutdifficulty, returning to normal activities shortly aftersurgery (Fig 6). Wound infection or dehiscence didnot occur, but pin track infection was observed innine animals. However, this did not cause device dis-lodgement or failure of the procedure. After comple-tion of the distraction, animals displayed an anteriorcrossbite deformity, indicating that unilateral in-crease in the length of the right hemimandible wasachieved. We did not observe any device dislodge-ment during the distraction or consolidation period,revealing that the U-shaped plate fulfilled what wasexpected.

Radiologic Investigation

Radiographs obtained immediately after the opera-tion demonstrated that pins and the device were inproper position, with the end of the pins penetratingthe medial and lateral holes of the U-shaped plate(Fig 7). No evidence of bone formation was observedin the radiologic examinations performed at the endof the activation period and at the second week of theconsolidation period in both groups. In the radio-graphs obtained at the end of the consolidation pe-riod, the gradually distracted group demonstrated com-plete mineralization and bony union (Fig 8), whereasno radiologic evidence of new bone was noted in the3-mm immediate lengthening group (Fig 9).

Gross Anatomy

After the 4 weeks of consolidation, all animals werekilled, and the mandibles were prepared for grossanatomical and histologic analyses. On gross analysisof the mandibles obtained at the end of study, newlyformed bone was observed at the distraction site inthe mandibles of the gradual distraction group (Fig 10).It was noted that mandibles from the 3-mm immedi-ate lengthening group demonstrated fibrous union,whereas gradually distracted mandibles developedsolid regenerate. However, bone resorption wasnoted at the posterior pin fixation area that was com-pressed between the leaves of the U-shaped plate.

Histologic Analysis

In all mandibles obtained from both the immediatelengthening and gradually distracted groups, axialsections traversing through the osteotomy sites were

Fig 5 A rat sucking the enteral feeding solution.

Fig 6 The distractor fitted in a rat.Fig 7 Radiograph showing the mandible and the distrac-tor immediately after the operation.


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prepared and stained with hematoxylin and eosin forhistologic analysis. Ossified regenerate bone that filledthe entire distraction gap was noted under 43magni-fication (Fig 11) and 253magnification (Fig 12) in thesections obtained from the gradually distracted group.Atrophic fibrous union with disorganized collagenbundles and a few rudimentary bone spicules wereobserved under 253magnification in the immediatelengthening group.

DISCUSSION

A device for rat mandibular distraction was first de-scribed by Rowe et al.12 Although their first designallowed placement of the device in a posterior local-ization that enables an osteotomy line posterior to themolar teeth, a device dislodgement rate of 90% wasreported in their preliminary study. Because of thisexperience with a high incidence of failure, in theirongoing studies, they preferred more anterior appli-cation of the device on the body of the mandible,

Fig 10 The arrow points to the new regenerated bone onthe distraction site on gross examination.

Fig 11 Hematoxylin and eosin stains of axial sections ob-tained from the gradually distracted rat mandible at the endof the consolidation period (under 43magnification). Thelarge arrow indicates the posterior edge of the lower incisortooth root, and the small arrows indicate the osteotomyedges. Between these two arrows is ossified regeneratebone that completely filled the entire distraction gap.

Fig 8 Radiograph showing complete mineralization of thegradually distracted zone.

Fig 9 No bone formation on acutely lengthened zone ofthe mandible.


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allowing an osteotomy line between two molar teeth.However, obligatory localization of the osteotomyline between two molar teeth became the point thatshould have been criticized for this model, becausesuch an ostomy line includes a large amount of dentalprecursor tissue, and proximity to the oral cavity in-creases the risk of infection.18,19 Following theirmodel, different custom-made distractors were de-scribed in an attempt to place the osteotomy lineposterior to the molar teeth. Use of a custom-madedistractor composed of microplates and a methylme-thacrylate block was reported by Connolly et al.18

Buchman et al19 described a custom-manufacturedsteel-and-titanium frame-type bilateral distractor thatallowed placement of the osteotomy line posterior tothe molar teeth. In the device of Connolly et al, a two-hole microplate with a methylmethacrylate blockprovided posterior pin fixation, whereas bilateralramal fixation with a locking washer and a smallhex nut was used in the device of Buchman et al.Both models were suggested to offer enough stabilitywhile enabling placement of the ostomy posterior tothe molar teeth. However, application of these dis-tractors seemed technically difficult and demanding,increasing the risk for inadvertent bone fractures. Inshort, because of the shortcomings mentioned above,none of these devices are ideal for the distraction ofrat mandible.

Basically, performing gradual distraction in therat mandible has two contradicting drawbacks: (1)a high incidence of device dislodgement when thedevice is applied in a position that allows placementof the osteotomy posterior to the molar teeth (becauseof the delicate anatomy of the angle and the ramus of

the rat mandible) and (2) a high incidence of infectionin addition to inclusion of dental precursors at theosteotomy site when the osteotomy line is placedin an anterior position between the molar teeth (be-cause of the proximity of osteotomy to the oralcavity). Hence, an ideal distractor should maintainits stability until the end of the procedure, includingthe consolidation period, when it is placed at a local-ization that allows an osteotomy posterior to the mo-lar teeth. In addition, it should be simple enough foreasy application to avoid inadvertent fractures andother complications due to demanding manipulations.

Despite its posterior fixation on a delicate part ofthe mandible, because of the U-shaped plate workingwith the lag screw principle, our device maintainedits stability until the end of the study. Accordingly, thisenabled us to perform the osteotomies posterior to themolar teeth to eliminate the infection risk of an osteot-omy line between the teeth. Additionally, the simpleconstruction of the device made its application easyand practical, without the risk of accidental fracturesdue to complex manipulations.

Although no intraoperative or immediate post-operative deaths occurred in our study, a relativelyhigh incidence of death was reported, as with anes-thesia complications in similar studies.12,18,19 Most ofthe deaths were reported to have occurred intraoper-atively or shortly after the surgery, indicating a prob-able airway problem during the surgery. This mighthave been because of lack of a careful airway controlwith proper traction of the tongue during the surgery,because it is obviously easier to work on a stable man-dible when the mouth is closed, ignoring proper air-way control. In our study, the transient interincisive-fixation method stabilized the mandible for an easiermanipulation while keeping the mouth wide open forsafer airway control with proper traction of thetongue. That is why accomplishment of our studywithout any intraoperative death was deemed to re-sult from this new transient interincisive-fixationmethod. Furthermore, our method of mandibular fix-ation also decreased the incidence of inadvertent frac-tures during manipulations. Because the rat mandi-ble is quite mobile, stabilization of the bone isinevitably required while performing certain proce-dures, such as drilling holes and creating osteoto-mies. The surgical clamp that was commonly usedfor this purpose in previous studies was blamedfor these accidental fractures of the mandible duringmanipulations.18 Fixation of the mandible with ourtechnique offered enough stability without usingmore destructive and risky instruments, such asa clamp or a fixation device. Additionally, it shouldbe emphasized that gentle manipulation with

Fig 12 Regenerated bone under 253magnification at thedistracted zone of the mandible shown in Fig 11. The largearrow indicates the posterior edge of the lower incisor toothroot, and the small arrows indicate the osteotomy edge.


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patience is also of great importance to avoid inadver-tent fractures while working on the delicate rat man-dible. Especially while drilling the posterior hole,applying too much pressure on the drill should beavoided while using low-speed rotation.

Another problem that we faced at the beginningof the study was the feeding of the animals. In spite ofthe fact that a soft diet was given after surgery, twoanimals died at postoperative day 10 because of pro-gressive weight loss. After enrichment of the dietwith a diluted enteral feeding solution as describedabove, we did not observe and further lethal weightloss. We believed that nutrition of the animals withliquid food also contributed to the maintenance ofthe stability of the device during the study. Althoughthey were fed with soft food, rats bit and chewed thefood using their jaws. Because the jaws are not usedeffectively for sucking, the force imposed on the de-vice from chewing must be less when enteral feedingsolution is given for the rats’ nutrition.

Although infectious complications or wound de-hiscence were not observed in our study, the pin-reaction rate seemed relatively high. It was thoughtto stem from the configuration of the pins because,in some animals, the outer thickened back of thepin and the skin were noted to come into contact witheach other, applying pressure onto the sutured inci-sion line due to postoperative edema and swelling.To eliminate this risk, a longer screw could be usedfor the distal part of the pin.

Although no device dislodgement occurred dur-ing the study, indicating that U-shaped plate workedwell, bone resorption was noted between the leaves ofthe plate in the gross anatomical examinations at theend of the study. This was deemed to result from thecompression effect on a wider bone segment imposedby the plate working with the lag screw principle. Onthe other hand, this did not cause device loosening ordislodgement until the experiments were completedin 37 days. Bone resorption around the pins and underthe methylmethacrylate block in the angular region ofmandible was also reported in previous studies.18,20

The design of the device was anticipated to maintainits stability only until the new bone formation was con-cluded. Moreover, osteoclastic resorption was com-mon around the bicortical pins that were evaluated af-ter having served for osseous stability. It seems thatlate bone resorption around the pin and betweenthe leaves of the plate is unavoidable, but it doesnot occur to an extent to cause device loosening or dis-lodgement during the course of a distraction study un-til a stage when new bone formation can be shown. Inour study, although minimal mobility in some animalswas observed close to the end of the experiment, our

device offered satisfactory stability for a time periodthat is long enough to complete the study without dis-lodgement.

The optimal parameters for a successful distrac-tion in the rat mandible, such as latency, rate, andrhythm, have been studied well.12,23 Because theaim of this study was to demonstrate that the devicewe developed works well with satisfactory stabiliza-tion for new bone formation at an osteotomy site pos-terior to the molars, we used the parameters recom-mended by the authors who used the similar devicemodels (latency, 3 days; rate, 0.5 mm; rhythm, 0.25 mmtwo times per day).12,21–24 These parameters pro-vided ossified regenerate bone that filled the entiredistraction gap, which can be used for histologicand biochemical investigation.

In our study, the 3-mm immediate lengtheninggroup showed fibrous union, corresponding to theprevious studies in which a similar distractor modelwas used for 3-mm acute defects.22 However, a 37%bony union rate was reported in a study in whicha frame-type bilateral distractor was used for the samesize of acute defect.19 This discordance must be dueto a different type of distractor design or different os-teotomy site that was vertical from the sigmoid notchto the inferior border of the mandible. In our study, weplaced the osteotomies just posterior to the molar teeththat correspond to the ramus of the mandible.

CONCLUSION

In terms of meeting the needs of an ideal distractorfor rat mandible, we believe that our device is supe-rior in comparison to the ones previously described.The main advantages of our device are that it is (1)able to maintain its stability during the procedurewhen it is placed in a position that allows an osteot-omy posterior to the molar teeth and (2) simpleenough for easy application without complex manip-ulations that may lead to accidental damage of thesusceptible rat mandible. In this study, we demon-strated that the device works properly and is ableto create ossified regenerate bones that fill the entiredistraction gap, which can be used for various inves-tigations about DO. Additionally, our interincisive-fixation technique and postoperative nutrition methoddescribed here can be considered to further refine thesurgical technique and animal care for future exper-imental studies with DO.

The authors thank Vets. Tayfun Ide, Elvin Akdag, and Ersin Can-polat for their assistance with performing this study.


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REFERENCES

1. Codivilla A. On the means of lengthening in the lower limbs,the muscle and tissues which are shortened through deformity.Am J Orthop Surg 1905;2:353–359

2. Ilizarov GA. Clinical application of the tension-stress effect forlimb lengthening. Clin Orthop 1990;250:8–26

3. Ilizarov GA. The tension-stress effect on the genesis and growthof tissues: I. The influence of stability of fixation and soft-tissuepreservation. Clin Orthop 1989;238:249–281

4. Ilizarov GA. The tension-stress effect on the genesis and growthof tissues: II. The influence of the rate and frequency of distrac-tion. Clin Orthop 1989;239:263–285

5. Snyder CC, Levine GA, Swanson HM, et al. Mandibular length-ening by gradual distraction: preliminary report. Plast ReconstrSurg 1973;51:506–508

6. Michieli S, Miotti B. Lengthening of mandibular body by gradualsurgical-orthodontic distraction. J Oral Surg 1977;35:187–192

7. Karp NS, Thorne CH, McCarthy JG, et al. Bone lengthening inthe craniofacial skeleton. Ann Plast Surg 1990;24:231–237

8. Karp NS, McCarthy JG, Schreiber JS, et al. Membranous bonelengthening: a serial histological study. Ann Plast Surg 1992;29:2–7

9. McCarthy JG, Schreiber JS, Karp N, et al. Lengthening of humanmandible by gradual distraction. Plast Reconstr Surg 1992;89:1–10

10. Aytemiz C, Sengezer M. Gradual distraction technique in thecorrection of mandibular deformities. In: Hinderer UT, ed. Pro-ceedings of the Xth Congress of the International Confederationfor Plastic and Reconstructive Surgery. Amsterdam: ExcerptaMedica, 1992:297

11. Sengezer M. Mandibular lengthening by gradual distraction.Plast Reconstr Surg 1993;92:372–373

12. Rowe NM, Mehrara BJ, Dudziak ME, et al. Rat mandibular dis-traction osteogenesis: I. Histologic and radiographic analysis.Plast Reconstr Surg 1998;102:2022–2032

13. Costantino PD, Shybut G, Friedman CD, et al. Segmental man-dibular regeneration by distraction osteogenesis: an experi-mental study. Arch Otolaryngol Head Neck Surg 1990;116:535–545

14. Karaharju-Suvanto T, Karaharju ED, Ranta R. Mandibular dis-traction: an experimental study on sheep. J CraniomaxillofacSurg 1990;18:280–283

15. Komuro Y, Takato T, Harii K, et al. The histologic analysis ofdistraction osteogenesis of the mandible in rabbits. PlastReconstr Surg 1994;94:152–159

16. Califano L, Cortese A, Zupi A, et al. Mandibular lengthening byexternal distraction: an experimental study in the rabbit. J OralMaxillofac Surg 1994;52:1179–1183

17. Troulis MJ, Glowacki J, Perrott DH, et al. Effects of latency andrate on bone formation in a porcine mandibular distractionmodel. J Oral Maxillofac Surg 2000;58:507–513

18. Connolly JP, Zi-Jun L, Wang L, et al. A custom mandibulardistraction device for the rat. J Craniofac Surg 2002;13:445–452

19. Buchman SR, Ignelzi MA, Radu C, et al. Unique rodent modelof distraction osteogenesis of mandible. Ann Plast Surg 2003;111:511–519

20. Mehrara BJ, Rowe NM, Dudziak ME, et al. Rat mandibular dis-traction osteogenesis: II. Molecular analysis of transforminggrowth factor beta-1 and osteocalcin gene expression. Plast Re-constr Surg 1999;103:536–543

21. Warren SM, Mehrara BJ, Steinbrech DS, et al. Rat mandibulardistraction osteogenesis: III. Gradual distraction versus acutelengthening. Plast Reconstr Surg 2001;107:441–453

22. Paccione MF, Mehrara BJ, Warren SM, et al. Rat mandibular dis-traction osteogenesis: latency, rate, and rhythm determine theadaptive response. J Craniofac Surg 2001;12:175–182

23. Rowe NM, Mehrara BJ, Luchas JS, et al. Angiogenesis duringmandibular distraction osteogenesis. Ann Plast Surg 1999;42:470–475

24. Schulten AJM, Zimmermann CE, Glowacki J. Osteoclastic boneresorption around intraosseous screws in rat and pig mandi-bles. Microsc Res Tech 2003;61:533–539

Longitudinal CephalometricAnalysis After MaxillaryDistraction Osteogenesis

Michael Krimmel, MD, DMD,*Carl-Peter Cornelius, PhD, MD, DMD,* Margit Bacher, DMD,y

Dirk Gulicher, MD, DMD,*Siegmar Reinert, PhD, MD, DMD*

Tubingen, Germany

Abstract: The aim of this study was to analyze the long-term den-toskeletal changes in patients who received distraction osteogene-sis of the maxilla. Seventeen patients aged 12 to 31 years underwentrigid external distraction osteogenesis for treatment of maxillaryhypoplasia. Cephalograms were evaluated retrospectively regard-ing linear and angular measurements. Follow-up times rangedfrom 1 to 5 years. In two patients, there was no consolidation ofthe Le Fort I segment to the midface. After the 0- to 12-month pe-riod, the maxilla had a stable position in all patients. Nevertheless,there was further decrease of SNA and ANB and an increase of thefacial concavity thereafter. Ongoing growth of the facial skeletonmust be considered when distraction osteogenesis is chosen forthe advancement of the maxilla in adolescents.

D istraction osteogenesis has become a widely ac-cepted technique for the treatment of skeletal

craniofacial deformities. After its first applicationto the mandible by McCarthy et al, the techniquehas been extended to almost every part of the cranio-facial skeleton.1,2 Different techniques were intro-duced for the advancement of the maxilla.3–7 Amongthe different systems, the rigid external distractionsystem, as described by Polley and Figueroa8 in1997, has gained wide acceptance because it offersthe advantage of rigid anchorage and predictablehorizontal and vertical movement of the maxilla.Since then, several authors have reported its useand were most enthusiastic about the achieved imme-diate results after distraction.9–11 However, in these

From the *Department of Oral and Maxillofacial Surgery, Uni-versity Hospital Tubingen, Germany; yDepartment of Orthodon-tics, University Tubingen, Germany.

Address correspondence to Dr. Krimmel, Klinik und Poliklinikfur Mund-, Kiefer-, und Gesichtschirurgie, Universitat Tubingen,Osianderstr. 2–8, D-72076 Tubingen, Germany; E-mail: [email protected]

BRIEF CLINICAL NOTES / Krimmel et al

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studies, only short-term surveys were reported on theoutcome of the procedure. Because the technique wasmostly applied to growing adolescents with severemaxillary hypoplasia, long-term assessment of the fa-cial development is necessary. The number of studieswith longer follow-up is very limited. Therefore, thepurpose of this study was to examine long-term den-toskeletal changes in patients who had undergonemaxillary distraction osteogenesis.

PATIENTS AND METHODS

Seventeen patients aged 12 to 31 years (mean, 16.3years) were treated with rigid external distractionosteogenesis (Martin Medizin-Technik, Tuttlingen,Germany) between 1997 and 2000. Eight of these pa-tients were male and nine were female. Ten patientshad unilateral cleft lip and palate, and four patientshad bilateral cleft lip and palate. Three patients pre-sented with maxillary or nasomaxillary dysplasiaassociated with hypodontia and a median facial cleftsyndrome.

Preoperatively, an intraoral splint was fixed tothe teeth in all but one patient, who was almost eden-tulous and who required skeletal fixation of the dis-traction device. A complete Le Fort I osteotomy anddownfracture were performed. The rigid externaldistraction system was securely fixed to the craniumand connected to the intraoral splint. After a latencyperiod of 5 days, distraction was performed at a rateof 1 mm/d. After the desired maxillary advancement,a consolidation period of 6 weeks followed. For anadditional 3 months, the patients had to wear a facemask during the night. Orthodontic treatment wasthen continued.

The follow-up of the operated patients rangedfrom the date of operation to the date of the mostrecent cephalometric radiograph.

CEPHALOMETRIC ANALYSIS

Longitudinal dentoskeletal changes were studied ret-rospectively using acetate paper tracings of lateralcephalograms taken shortly before osteotomy andduring follow-up. Eight dentoskeletal anatomicallandmarks were identified: sella (S), nasion (N), pointA (A), point B (B), pogonion (Pog), posterior nasalspine (Spp), upper incisor edge (U1), and lower inci-sor edge (L1).

For linear measurements, horizontal and verticalplanes were created. The line SN served as the x-axis,and a line perpendicular to this plane through the

sella was used as the y-axis. By subtraction of the xand y values of the respective studied landmarks be-fore and after distraction, the displacement becameevident.

Skeletal changes were furthermore examined bythe following skeletal angular measurements: SNA,ANB, and NAPog. The longitudinal developmentwas calculated by subtraction of the measured anglesbefore surgery and during follow-up. The longitudi-nal dental overjet was also measured.

RESULTS

In two cases, the Le Fort I segment did not consolidateto the midface after the desired advancement of themaxilla. The patients were 24 and 31 years old andwere the eldest in the study group. The 24-year-oldfemale patient had a bilateral cleft lip and palateand a mobile premaxilla without bone grafting tothe alveolar cleft. The 31-year-old male patient hada unilateral cleft lip and palate and was almost eden-tulous secondary to poor oral hygiene.

From the remaining 15 patients, 3 were lost to fol-low-up. Their data were therefore not included in thestudy. The remaining 12 patients could be followedup between almost 1 year and more than 5 years(average, 2.8 years). Their average age was 14.9 years(range, 12–20 years).

The development of SNA during and after dis-traction is shown in Fig 1. SNA increased signifi-cantly after completed distraction. However, afterreaching a maximum, there was also a considerabledecrease of SNA during the first 12 months, indicat-ing maxillary relapse in the horizontal plane. Afterthe first year, the amount of SNA decrease slowed

Fig 1 Differences of the SNA angle after maxillary distrac-tion osteogenesis (rigid external distraction system).


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down to 0.5� in 1 year but still continued. Remark-ably, all of the curves of SNA change after the firstyear after distraction ran parallel, indicating a com-mon pattern behind this phenomenon.

The development of ANB (Fig 2) showedchanges similar to those of SNA during the first post-operative year. Thereafter, further decrease occurred;however, in contrast to SNA, the ANB decreaseranged from 0.5� to 1� in 1 year. Additional mandib-ular changes were responsible for this discrepancy(Fig 3).

The change of NAPog indicating facial concavityand convexity is shown in Fig 4. Again, there wasconsiderable improvement via distraction osteogene-sis and also relapse during the first year. Althoughinconsistent, there was an ongoing tendency towardfacial concavity in the following period.

Absolute values calculated for the horizontal (x)and vertical (y) displacement of the A-point areshown in Figs 5 and 6. The A-point moved anteriorlyand downward in all patients. Relapse in the horizon-tal plane occurred during the first 12 months (range,2–4 mm); thereafter, the horizontal position of theA-point was stable. In the vertical direction, the move-ment of distraction was less homogenous, ranging

Fig 3 a-d: Lteral cephalograms beforeduring and after maxillary distractionosteogenesis demonstrating ongoingmandibular growth. (A) Before distrac-tion osteogenesis (B) During distractionosteogenesis (C) 6 months follow-up(D) 3 years and 6 months follow-up.

Fig 2 Differences of the ANB angle after maxillarydistraction osteogenesis (rigid external distraction system).


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from none to downward. No upward movementwas seen. Postdistraction changes showed relapsein terms of upward movement or continuing down-ward movement.

All patients had an anterior crossbite preopera-tively. Maxillary advancement resulted in a positivedental overjet in all cases, with a maximum of 14 mmin one case. Within the observation period, the overjetdecreased, and a positive anterior dental step couldbe maintained in all patients (Fig 7). The greaterthe overjet was after distraction, the higher its de-crease was in the follow-up period.

DISCUSSION

Since the first description of rigid external distractionby Polley and Figueroa, several reports have beenpublished on its application.9–12 The possibility of

greater advancement of the maxilla with distractionosteogenesis compared with conventional orthog-nathic surgery has mostly been emphasized. Boneconsolidation or skeletal fixation was addressed onlymarginally.

We found two patients in our series who pre-sented with nonunion of the Le Fort I segment andrequired internal fixation after successful advance-ment. Whether nonunion was due to their olderage or to their special circumstances (three maxillarysegments, multiple tooth loss) remains unclear. Thiscomplication has not been described by other au-thors; however, their patients were mostly childrenand adolescents.

The number of studies on the long-term effect ofdistraction to the face is very limited.13–15 The currentstudy revealed that during the 0- to 12-month period,significant relapse of the maxilla in the horizontal

Fig 4 Differences of the NAPog angle after maxillary dis-traction osteogenesis (rigid external distraction system).

Fig 5 Horizontal movement of the A-point after distrac-tion osteogenesis.

Fig 6 Vertical movement of the A-point after distractionosteogenesis.

Fig 7 Dental overjet before and after distraction osteo-genesis.


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plane occurred after advancement. In the long-term(after 12 months), the displaced maxilla had a stableposition in the horizontal plane. Suzuki et al14 founda relapse rate of 22.3% in the horizontal dimensionduring the first 6-month follow-up period and stableconditions thereafter. A report from Ko et al15

described a relapse rate of 12.6% in a 12-month pe-riod. These studies and our findings suggest thatskeletal stability is reached between 6 and 12 monthsafter distraction. The different durations found forskeletal consolidation may result from differencesin the examined populations (age, underlying dis-ease, dentition), postdistraction protocol (consolida-tion period, face mask), and different evaluationintervals. The amount of relapse in maxillary distrac-tion osteogenesis found in the study of Suzuki et aland our study (A-point (x): mean, 2.5 mm, 6-month fol-low-up) seems higher compared with rigid internalfixation. Heliovaara et al16 reported 0.4 and 0.5 mmmean horizontal relapses in unilateral and bilateralcleft lip and palate patients after Le Fort I osteotomywith miniplate fixation and bone grafting.

To our knowledge, there is no report on thedevelopment of the facial skeleton 1 year after dis-traction. Although the maxilla had reached a stableposition after 12 months, SNA and ANB continuedto decrease, and NAPog continued to increase. Thisdevelopment can only be explained by furthergrowth of the neighboring facial bones.17 The dis-crepancy in the change of SNA and ANB indicatesthat all patients had similar further growth in thenasofrontal area but different amounts of growth inthe mandible. This development may deterioratethe facial balance reached after distraction and mayrequire a second surgical correction. In our series,none of the patients underwent repeated distractionor secondary orthognathic surgery. However, thepossible necessity must be considered and discussedbefore performing maxillary distraction osteogenesisin adolescents. Polley and Figueroa9 even proposedtreatment of maxillary hypoplasia with the rigid ex-ternal distraction system from the age of 5 years andup. Because future facial growth can be foreseen evenless in this young age group, we cannot accept thisproposal.

To compensate for relapse and future facialgrowth, overcorrection is advocated. Our observedrelapse rate justifies an overcorrection of at least2 to 4 mm in all patients. Future facial growth isdifficult to anticipate because of its variability. Tominimize the risk of undercorrection in patientswith maxillary hypoplasia, a safe method wouldbe to postpone the distraction process to lateadolescence.

CONCLUSION

Distraction osteogenesis is a superior technique totreat extreme maxillary hypoplasia. Its applicationin children and adolescents, however, is critical, be-cause the achievable result may not show long-termstability. Ongoing growth of the facial skeleton mustbe considered. Although bone consolidation may beless favorable in adults and internal fixation may berequired secondarily, the final result is more predict-able and stable.

REFERENCES

1. McCarthy JG, Schreiber J, Karp N, et al. Lengthening the man-dibular body by gradual distraction. Plast Reconstr Surg 1992;89:1–10

2. Cohen SR. Craniofacial distraction with a modular internal dis-traction system: evolution of design and surgical techniques.Plast Reconstr Surg 1999;103:1592–1608

3. Cedars MG, Linck DL, Chin M, et al. Advancement of the mid-face using distraction techniques. Plast Reconstr Surg 1999;103:429–441

4. Block MS, Cervini D, Chang A, et al. Anterior maxillary ad-vancement using a tooth-supported distraction osteogenesis.J Oral Maxillofac Surg 1995;53:561–565

5. Molina F, Ortiz-Monasterio F. Maxillary distraction: three yearsof clinical experience. In: Proceedings of the 65th Annual Meet-ing of the American Society of Plastic and Reconstructive Sur-geons, Plastic Surgical Forum. Chicago, IL: Vol XIX. 1996:54

6. Rachmiel A, Aizenbud D, Ardekian L, et al. Surgically-assistedorthopedic protraction of the maxilla in cleft lip and palate pa-tients. Int J Oral Maxillofac Surg 1999;28:9–14

7. Rachmiel A, Potparic T, Jackson IT, et al. Midface advancementby gradual distraction. Br J Plast Surg 1993;46:201–207

8. Polley JW, Figueroa M. Management of severe maxillary defi-ciency in childhood and adolescence through distraction osteo-genesis with an external, adjustable, rigid distraction device.J Craniofac Surg 1997;8:181–185

9. Polley JW, Figueroa M. Rigid external distraction: its applica-tion in cleft maxillary deformities. Plast Reconstr Surg 1998;102:1360–1372

10. Swennen G, Colle F, De Mey A, et al. Maxillary distraction incleft lip palate patients: a review of six cases. J Craniofac Surg1999;10:117–120

11. Krimmel M, Cornelius CP, Roser M, et al. External distraction ofthe maxilla in patients with craniofacial dysplasia. J CraniofacSurg 2001;12:458–463

12. Ahn JG, Figueroa M, Braun S, et al. Biomechanical con-siderations in distraction of the osteotomized dentomaxillarycomplex. Am J Orthod Dentofacial Orthop 1999;116:264–270

13. Hollier LH, Kim JH, Grayson B, et al. Mandibular growth afterdistraction in patients under 48 months of age. Plast ReconstrSurg 1999;103:1361–1370

14. Suzuki EY, Motohashi N, Ohyama K. Longitudinal dento-skeletal changes in UCLP patients following maxillary distrac-tion osteogenesis using RED system. J Med Dent Sci 2004;51:27–33

15. Ko EW, Figueroa AA, Polley JW. Soft tissue profile changes aftermaxillary advancement with distraction osteogenesis by use ofa rigid external distraction device: a 1-year follow-up. J OralMaxillofac Surg 2000;58:959–969


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16. Heliovaara A, Ranta R, Hukki J, et al. Skeletal stability of Le FortI osteotomy in patients with isolated cleft palate and bilateralcleft lip and palate. Int J Oral Maxillofac Surg 2002;31:358–363

17. Enlow DH. Craniofacial growth and development: normal anddeviant patterns. In: Polley JC. Craniofacial and MaxillofacialSurgery in Children and Young Adults. Philadelphia, PA:W.B. Saunders, 2000:22–35

Cranial Distraction forPlagiocephaly: QuantitativeMorphologic Analysesof Cranium UsingThree-Dimensional ComputedTomography and aLife-Size Model

Akira Yamada, MD, Keisuke Imai, MD, Takeru Nomachi, MD,Takuya Fujimoto, MD, Hiroaki Sakamoto, MD, Syohei Kitano, MD

Osaka, Japan

Abstract: The aim of this study was to analyze the morphologicchanges of the craniofacial skeleton in synostotic plagiocephalyafter cranial distraction. Preoperative and postoperative computedtomography scans were taken, and three-dimensional reconstruc-tion was then performed. Three measurements were obtained: in-tracranial volume, volume of the middle cranial fossa, and lengthof the zygomatic arch. Intracranial volume reached values thatwere normal for a 2-year-old child postoperatively, and the discrep-ancy between left and right half volumes decreased over time. Thediscrepancy of the volume of the middle cranial fossa also de-creased after surgery, and the asymmetry of the zygomatic arch im-proved significantly. Based on these objective findings, the authorsbelieve that cranial distraction could favorably affect craniofacialdysmorphology of synostotic frontal plagiocephaly.

R ecently, cranial distraction osteogenesis wasintroduced to correct dysmorphology of synos-

totic craniosynostosis; however, objective, quantita-tive evaluation of the results has not been published.Therefore, we evaluated the changes of morphologyafter cranial remodeling with gradual cranial distrac-tion. We measured intracranial volume, volume ofthe middle cranial fossa, and length of the zygomaticarch preoperatively and postoperatively to evaluate

the morphologic changes of the craniofacial skeletonsof patients with craniosynostosis.


Four patients who had plagiocephaly with right uni-lateral coronal suture closure were examined. Theirages at the time of surgery ranged from 7 to 28months (average, 14.5 months).

The cranial distraction protocol was as follows.Figure 1 shows the way in which the devices wereplaced. Distraction was started after 1 week at a rateof 0.5 to 1.0 mm/d. Cranial distraction continued forapproximately3to4weeks.Thentherewasaconsolida-tion period of 4 to 5 months before the distraction devi-ces were removed during a second surgical procedure.

Preoperative and postoperative computedtomography (CT) scans were taken, and then three-dimensional volumetric reconstructions from preop-erative and postoperative CT data were processedusing the Endoplan� (3 DV-Technology Gmbh, Kiel,Germany) imaging software.

Three types of measurements were performedto examine craniofacial morphologic changes postop-eratively: We measured intracranial volume (Fig 2),volume of the middle cranial fossa (Fig 3), and lengthof the zygomatic arch by using preoperative and post-operative CT scans. The segmentation technique hasbeen used for measuring both intracranial volumeand volume of the middle cranial fossa1,2 (Fig 4).

We measured the volume of the middle cranialfossa below the plane consisting of the lesser sphe-noid wing and pyramidalis on CT scan. In eachmeasurement, left and right half volumes were alsomeasured to evaluate symmetry.

CASE REPORT

A 15-month-old boy was referred to us from a localpediatrician. The patient was evaluated by the cranio-facial team. Physical examination, plain radiography,and CT were performed, and a diagnosis of synostoticfrontal plagiocephaly was made. The patient did nothave ophthalmologic abnormality. His psychosocialdevelopment was normal. Our decision was to surgi-cally correct the plagiocephaly with use of the cranialgradual distraction method. Before surgery, we cre-ated a three-dimensional life-size model made of poly-urethane and performed a surgical simulation. Then,we performed surgery on the patient. Via coronalincision, the anterior two thirds of the skull and theupper half of the orbital rim were exposed. A pediatricneurosurgeon then performed the craniotomy as well

From the Department of Plastic and Reconstructive Surgery,Osaka City General Hospital, Osaka, Japan.

Address correspondence to Dr. Yamada, Department of Plasticand Reconstructive Surgery, Osaka City General Hospital, Osaka,Japan 2-13-22, Miyakojimahondori, Miyakojima-ku, Osaka 5340021,Japan; E-mail: [email protected]


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as minimum dissection of the dura from the skullalong the osteotomy line. Osteotomy of the craniumand orbit was performed as we planned in the simu-lated surgery. Next, distraction devices were placed.

The wound was then closed. A small stab inci-sion was necessary for each device to put throughthe bar of the device. A bulky dressing to protectprotruding devices from impact was placed on thepatient’s head.

Distraction was started 1 week after surgery, ata rate of 0.5 to 1.0 mm/d. The duration of distractionwas 3 weeks. Once per week, plain radiographs

were taken to check both the skull and the devices,and fine adjustment of the distraction was madeaccordingly. Average elongation of the cranium inthe anterior-posterior dimension was achieved.

For this patient, approximately 30 days of hospital-ization was needed to perform daily distraction andwound care. Good bone deposition at the distractedcranium was confirmed 12 weeks after surgery withuse of plain radiographs (Fig 6) at an outpatientclinic. Removal of the distraction device was per-formed under general anesthesia 4 months afterdistraction of the cranium was stopped.

Fig 1 Illustrated osteotomy line andplacement of distraction devices. (A) Case1; (B) cases 2 and 3; (C) case 4.

Fig 2 Measurement of intracranial vol-ume on computed tomography scan. Totalintracranial volume and left and right halfvolumes were measured by using the seg-mentation technique.

Fig 3 Measurement of middle cranialfossa. The volume of the cranial fossa be-low the plane consisting of the lesser sphe-noid wing and pyramidalis was measuredand defined as the volume of the middlecranial fossa by using the segmentationtechnique.

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The patient’s postoperative course was smooth,without serious complications (Fig 7). PostoperativeCT scans were taken after 1, 6, 9, and 18 months (Fig 8).

RESULTS

We were able to quantitate the volume change of thecranial vault. The intracranial volume reached normalvalues in all four cases, and the discrepancy betweenthe left and right decreased in all four cases after cra-nial remodeling by gradual distraction; months aftertermination of the consolidation period, intracranialvolume increased to a value that was normal fora 2-year-old child (Fig 9). The volume of the middlecranial fossa also increased, particularly on the affectedside, and, months after termination of the consoli-dation period, reached values that were normal fora 2-year-old child (Fig 10). The difference in the lengthof the zygoma decreased over time (Fig 11).

DISCUSSION

Treatment of synostotic frontal plagiocephalyevolved from frontoparietal suturectomy (strip cra-niectomy)3–5 to bilateral fronto-orbital remodeling.6,10

Recently, less invasive surgical interventions havebeen introduced into the treatment of craniosynos-tosis, such as cranial distraction1,3,5 or spring-mediated cranial reshaping.18,19

Several authors have recently attempted to estab-lish a reference for craniofacial skeletal measurementsbased on CT data instead of plain radiographs.6,23–26

Among these measurements, intracranial volume hasbeen considered a good indicator of head growth.27

However, little attention has been paid to intracranialvolume changes that occur in the normal state and incraniosynostosis. Several studies previously reportedmeasurements of intracranial volume in childrenwith craniosynostosis. These authors concluded thatintracranial volume in children with craniosynostosisis within normal limits, and children with Apert

Fig 4 Bilateral measurement of length of zygomatic arch.

Fig 5 (A, B) Preoperative view of patient in case 2.

Fig 6 Sequential plain radiographs. (A) One week after starting distraction; (B) 2 weeks after; (C) 4 weeks after; (D) 12weeks after. Plain radiographs were taken regularly until good bone deposition was confirmed.


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syndrome tend to have greater-than-normal intracra-nial volume.28–30 Our study showed that intracranialvolume stayed within the normal range before andafter cranial distraction, and these results support

previous studies. The difference between previousstudies and our study is that we focused on synos-totic frontal plagiocephaly. We compared left andright intracranial volumes, with the coronal suture

Fig 7 (A, B) Twelve-month postop-erative view after removing distrac-tion devices.

Fig 8 Sequential morphologic changes in case 2. (A) Preoperative view; (B) 1 month after surgery; (C) 6 months after; (D)9 months after; (E) 18 months after.

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closed on one side and open on the other. By compar-ing the left and right, we were able to documentchanges of intracranial volume in children with syn-ostotic frontal plagiocephaly more accurately overtime. As for synostotic frontal plagiocephaly, therehas been little accurate documentation of the present-ing dysmorphology and postoperative results.4,20 Forexample, Posnic wrote that ‘‘Reliable quantitativeoutcome studies of the assessment of the morpho-logic improvements achieved for [unilateral coronalsynostosis] have yet to be recorded.’’

In the literature, attention had not been paidto the volume of the middle cranial fossa before andafter surgical correction of frontal plagiocephaly. Inthis study, we proposed CT-determined volume ofthe middle cranial fossa. We believe that it wouldbe worth measuring the volume change of the middlecranial fossa because closure of the unilateral coronalsuture may cause anatomical changes of the middlecranial fossa. Our analyses support this hypothesis,although the number may be too small.

Children with synostotic frontal plagiocephalyexhibit characteristic frontal, orbital, nasal, and malarasymmetry, which persists among untreated patients.7–9

Several reports have addressed the possible spontane-ous autodynamic reshaping of the midface structuresduring course of further growth.14–17 In this study,we were able to provide objective data to support thistheory, although the surgical modalities in previousreports (classic fronto-orbital advancement) and ourstudy (fronto-orbital distraction) are different.

CONCLUSIONS

We were able to document the changes of the cranialvault after cranial advancement/remodeling by cranialdistraction. Although the number of cases includedin this study was small, perioperative measurementof intracranial volume, volume of the middle cranialfossa, and length of the zygomatic arch showed ob-jectively that cranial distraction osteogenesis had afavorable effect on the dysmorphology of synostoticfrontal plagiocephaly.

REFERENCES

1. Davis DN, Natarajan K, Claridge E. Multiple energy functionactive contours applied to CT and MRI images. IEEE Conf1995;410:114

2. Sgouros S, Hockley A, Goldin JH, et al. Intracranial volumechange in craniosynostosis. J Neurosurg 1999;91:617

3. Kreiborg S, Bjork A. Craniofacial asymmetry of a dry skull withplagiocephaly. Eur J Orthod 1981;3:195

4. Marchac D. Radical forehead remodeling for craniostenosis.Plast Reconstr Surg 1978;61:823

5. Faber HK, Towne EB. Early operation in mature cranial synos-tosis for the prevention of blindness and other sequelae: fivecase reports with follow up. J Pediatr 1943; 22:286

6. Hirabayashi S, Sugawara Y, Sakurai A, et al. Frontoorbital ad-vancement by gradual distraction. J Neurosurg 1998;89:1058–1061

Fig 9 Intracranial Volume: Left half minus right half vol-umeDiscrepancy between left and right decreased over time.

Fig 10 Volume of middle cranial fossa: Left half minusright half volume Discrepancy between left and right wasdecreased over time.

Fig 11 Length of Zygoma: Normal minus affected Dis-crepancy between left and right was decreased in all casesover time.


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7. Imai K, Tajima S. The growth patterns of normal skull by usingCT scans and their clinical applications for preoperative plan-ning and postoperative follow up in craniofacial surgery. Eur JPlast Surg 1991;14:80–84

8. Imai K, Komune H, Toda C, et al. Cranial remodeling to treatcraniosynostosis by gradual distraction using new device.J Neurosurg 2002;96:654–659

9. Sakurai A, Hirabayashi S, Sugawara Y, et al. Skeletal analysis ofcraniofacial asymmetries in plagiocephaly (unilateral coronalsynostosis). Scand J Plast Reconstr Hand Surg 1998;32:81–89

10. Kobayashi S, Honda T, Saito A, et al. Unilateral coronal synos-tosis treated by internal forehead distraction. J CraniofacialSurg 1999;10:467–471

11. Salyer KE. Salyer & Bardach’s Atlas of Craniofacial & CleftSurgery. Vol 1: Craniofacial Surgery. Philadelphia: Lippincott–Raven, 1998

12. Meara JG, Burvin R, Bartlett RA, et al. Anthropometric studyof synostotic plagiocephaly: before and after fronto-orbitaladvancement with correction of nasal angulation. Plast Re-constr Surg Sep 2003;112:731–738

13. Bruneteau RJ, Mulliken JB. Frontal plagiocephaly: synostotic,compensational, or deformational. Plast Reconstr Surg 1992;89:21

14. Ingraham FD, Matson DD, Alexander E. Experimental observa-tion in the treatment of craniosynostosis. Surgery 1948;23:252

15. Shillito J, Matson DD. Craniosynostosis: a review of 519 surgi-cal patients. Pediatrics 1968;41:829

16. Marchac D, Renier D. Treatment of craniosynostosis in infancy.Clin Plast Surg 1987;14:61–72

17. Muhlbauer W, Anderl H, Schmidt A, et al. Asymmetricalcranio-orbital facial stenosis. Ann Plast Surg 1991;26:45

18. McCarthy JG, Glasberg SB, Cutting CB, et al. Twenty-yearexperience with early surgery for craniosynostosis: I. Isolatedcraniofacial synostosis: results and unsolved problems. PlastReconstr Surg 1995;96:272

19. Bartlett SP, Whitaker LA, Marchac D. The operative treatmentof isolated craniofacial dysostosis (plagiocephaly): a compari-son of the unilateral and bilateral techniques. Plast ReconstrSurg 1990;85:667

20. Lauritzen C, Sugawara Y, Kocabalkan O, et al. Spring mediateddynamic craniofacial reshaping: case report. Scand J PlastReconstr Surg Hand Surg 1998;32:331

21. David LR, Proffer P, Hurst WJ, et al. Spring-mediated cranialreshaping for craniosynostosis. Scand J Plast Reconstr SurgHand Surg 2004;15:810

22. Posnic JC. Anterior plagiocephaly: unilateral coronal synostosisand skull molding. In: Posnic JC, ed. Craniofacial and Maxillo-facial Surgery in Children and Young Adults. Philadelphia:W.B. Saunders, 2000:127–161

23. Abbott A, Netherway D, Nieman DB, et al. CT-determined in-tracranial volume for a normal population. J Craniofacial Surg2000;11:211–223

24. Waitzmann AA, Posnic JC, Armstrong D, et al. Craniofacialskeletal measurements based on computed tomography: I. Ac-curacy and reproducibility. Cleft Palate Craniofac J 1992;29:112

25. Waitzmann AA, Posnic JC, Armstrong D, et al. Craniofacialskeletal measurements based on computed tomography: II.Normal values and growth trends. Cleft Palate Craniofac J1992;29:118

26. Gault DT, Renier D, Marchac D, et al. Intracranial volume inchildren with craniosynostosis. J Craniofac Surg 1990;1:1

27. Gault DT, Jones BM. Indirect intracranial volume measurementusing CT scan [Letter]. Plast Reconstr Surg 1992;45:1126

28. Gault DT, Renier D, Marchac D, et al. Intracranial pressure andintracranial volume in children with craniosynostosis. PlastReconstr Surg 1992;90:377

29. Gosain AK, McCarthy JG, Glatt P, et al. A study of intracranialvolume in Apert syndrome. Plast Reconstr Surg 1995;95:284

Lymphoma ofMucosa-Associated LymphoidTissue (MALT) Arising in theParotid Gland

Hideyuki Tada, MD, PhD,* Mitsuo Hatoko, MD, PhD,y

Aya Tanaka, MD,y Satoshi Yurugi, MD,y Hiroshi Iioka, MD,y

Katsunori Niitsuma, MDy

Nara, Japan

Abstract: Malignant lymphoma of the parotid gland is a rare con-dition. The authors present the case of a 59-year-old woman withprimary malignant lymphoma arising from the parotid gland, inwhich they resected the tumors and surrounding lymph nodes. His-topathologic examination of the tumor demonstrated a lymphomaof mucosa-associated lymphoid tissue (MALT). Chemotherapy andradiotherapy were performed after surgery. The clinical mani-festation, treatment, and postoperative course are described.

Key Words: Histopathologic examination, mucosa-associated lymphoid tissue (MALT) lymphomaparotid gland

I n our clinic, we have often encountered tumorsarising from the parotid gland. Most of these tu-

mors have been diagnosed as pleomorphic adenoma.However, it was reported that 1.7% to 8.5% of all tu-mors of the parotid gland have been diagnosed aslymphoma.1 Lymphomas of the parotid gland are ex-tranodal lymphomas. The structure and function ofthe intestinal mucosa, lung, thyroid, and salivarygland resemble those of lymphoid tissue, and theywere named mucosa-associated lymphoid tissue(MALT).2 A lymphoma arising from MALT in thegastrointestinal tract was first described by Isaacsonand Wright.3,4 Subsequently, lymphomas were ob-served arising from other sites, such as the salivarygland, lung, and thyroid. Most of these lymphomasare of B-cell origin. Histopathologic examination isessential for a definitive diagnosis of MALT lym-phoma. MALT lymphoma exhibit a greater tendencyto remain localized compared with other low-gradeB-cell lymphomas.5 They usually respond well tothe appropriate therapy and thus are associated with

From the *Department of Dermatology, Division of Plastic Sur-gery, Nara Prefectural Nara Hospital, and the yDivision of PlasticSurgery, Nara Medical University, Nara, Japan.

Address correspondence and reprint requests to Dr. H. Tada, Di-vision of Plastic Surgery, Nara Medical University, 840 Shijo-cho,Kashihara, Nara, 634-8522, Japan. E-mail: [email protected]

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a better prognosis than are other low-grade lympho-mas. In the main, they have been diagnosed by post-operative histopathologic examination. Because ofthe characteristics of the tumor, the best managementfor MALT lymphoma in the parotid gland is reportedto be conservative parotidectomy and postoperativechemotherapy or radiation therapy.2 A review of theliterature indicates that the presentation of MALTlymphomas in the parotid gland is extremely rare.6

In this article, we report a case of MALT lymphomaarising from the parotid gland.

CASE REPORT

A 59-year-old woman with an enlargement of the leftparotid region was admitted to our care. A gross, ten-der and painless mass was detected, and there was noevidence of facial nerve involvement. Computed to-mography (CT) scans detected the multiple, well-circumscribed tumors in the left parotid gland(Fig 1). The tumors were 2 3 3 cm, 1 3 1.5 cm and1 3 1 cm in size. These tumors were located in thesuperficial lobule of the parotid gland (Fig 2). UnderGa35 scintigraphy, a high level of radioactivity wasdetected in the left parotid region. We suspectedthe tumors to be a multiple pleomorphic adenomaor Warthin’s tumor, so we performed tumor resection.

At surgery, an incision was extended inferiorly tothe submandibular region. Three tumors were local-ized in the superficial lobule of the parotid gland. Anentire tumor resection was performed to include thesuperficial lobule but retaining the facial nerve. Toprevent Frey’s syndrome, artificial dermis wasgrafted on the deep lobule of the parotid gland. His-topathologically, small to medium centrocyte-like cellsand plasma cells were proliferated. These tumor cellsinfiltrated the ductal epithelium.

Under immunohistopathology, the tumor cellsexpressed CD20, CD22 B-cell antigen, and LCAleukocyte common antigen (Fig 3). Furthermore,the expression of CD5 T-cell antigen was negative.These findings led to the diagnosis of this tumor aslymphoma of mucosa-associated lymphoid tissue(MALT). No similar lesions were detected in generalCT scans. This case was thought to be a primaryMALT lymphoma arising in the parotid gland.

After the patient’s recovery from surgery, she re-ceived chemotherapy, consisting of cyclophospha-mide, pirarubicin, vincristine, and prednisolone.After four courses of this regimen, radiation therapywas administrated to the neck totaling 40 Gy. Thepatient is currently well.

DISCUSSION

A primary lymphoma arising from the major salivaryglands is a rare condition. Incidences reported in ex-isting literature have varied from 1.7% to 3.1% of sal-ivary gland neoplasms and 0.6% to 5% of all tumorsand tumor-like lesions of the parotid gland.5

In the parotid gland, malignant lymphomas oftenare clinically unsuspected, manifesting as nonspecificmasses indistinguishable from other common tumors.No characteristic finding of malignant lymphoma isobserved under radiologic examination. A definitivediagnosis of malignant lymphoma of the parotidgland is established by histopathologic examination.

Fig 1 (A) Preoperative view of the patient: two large sub-cutaneous tumors are palpable in the left parotid gland re-gion. (B) Preoperative CT: large tumor is seen in the leftparotid gland.


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In 1983, Isaacson and Wright suggested that lym-phomas arising from mucosa-associated lymphoidtissue (MALT) constitute a distinct clinicopathologicentity, exhibiting proliferation of centrocyte-likecells.3 Because mucosal lymphoid cells home prefer-entially to mucosal locations, MALT lymphomasshow the tendency to remain localized, as comparedwith other low-grade B-cell lymphomas.5 It is re-ported that most MALT lymphomas arise in the gas-

trointestinal tract and most of these lymphomas are ofB-cell origin.7 A review of existing literature indicatesthat the presentation of MALT lymphomas in the pa-rotid gland is extremely rare.6 Patients with Sjogren’ssyndrome are at a high risk compared with a con-trol population.8 The characteristic histopatho-logic feature of MALT lymphomas is a populationof tumor cells, consisting of small to medium lym-phoid centrocyte-like cells, and infiltration of tumorcells into the epithelium.9

Even with aspiration biopsy, it is very difficult todiagnose MALT lymphoma definitively. Most casesof MALT lymphoma have been diagnosed postoper-atively under pathologic examination of the surgicalspecimen.10

In our case, it was very hard to achieve the diag-nosis of the parotid gland lesion as malignant lym-phoma. The definitive diagnosis was established byhistopathologic and immunohistologic examinationafter surgery.

The literature advises that the best manage-ment of MALT lymphoma in the parotid gland isconservative parotidectomy,11 which allows the de-tailed histologic and immunohistologic examinationof the complete lesion. After the diagnosis, radiationand/or chemotherapy (from 1 to 4 courses) should beperformed administered.

In conclusion, we report a case of primary MALTlymphoma arising from the parotid gland. Usingclinical symptoms and radiologic findings, it wasvery difficult to diagnose the tumor as malignant lym-phoma. Although malignant lymphoma of the pa-rotid gland is a rare condition, we should considerthis diagnosis in the case of progressive tumor inthe parotid gland.

REFERENCES

1. Glesson MJ, Bennet MH, Cawson RA. Lymphoma of the sali-vary glands. Cancer 1986;58:699–704

2. Harris NL. Extra-nodal lymphoid infiltrates and mucosa-associated lymphoid tissue (MALT). A unifying concept. AmJ Surg Pathol 1991;15:879–884

3. Isaacson PG, Wright DH. Malignant lymphoma of mucosa as-sociated lymphoid tissue. Cancer 1983;52:1410–1416

4. Isaacson PG, Wright DH. Extranodal malignant lymphomaarising from mucosa-associated lymphoid tissue. Cancer 1984;52:2512–2524

5. Barnes L, Myers EN, Prokopakis EP. Primary malignant lym-phoma of the parotid gland. Arch Otolaryngol Head Neck Surg1998;124:573–577

6. Bernhard H, Wolf SR. Bilateral mucosa-associated lymphoidtissue lymphoma of the parotid gland. Arch Otolaryngol HeadNeck Surg 2000;126:224–226

7. Berger F, Felman P, Sonet A. Nonfollicular small B-cell lympho-mas: a heterogeneous group of patients with distinct clinicalfeatures and outcome. Blood 1994;83:2829–2835

Fig 2 Excised specimen showing two separate tumorsarising from the superficial lobule of the parotid gland.

Fig 3 Microscopic appearance of the tumor. (Upper)Lymphoepithelial lesion: small centrocyte-like cells infil-trating ductal epithelium (stain, hematoxylin and eosin;original magnification, 3200). (Below) Immunohistologicstaining: lymphoma cells showing distinct reaction withthe B-cell marker CD20 (original magnification, 3200).

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8. Bone CR, Fox RI, Howell FV, Fantozzi R. Sjogren syndrome:a persistent clinical problem. Laryngoscope 1985;95:295–299

9. Li G, Hansmann ML, Zwinger T, Lennert K. Primary lympho-mas of the lung: immunohistological and clinical features. His-topathology 1990;16:519–533

10. Stewart A, Blenkinsopp PT, Henry K. Bilateral parotid MALTlymphoma and Sjogren’s syndrome. Br J Oral Maxillofac Surg1994;32:318–322

11. Balm AJM, Delaere P, Hilgers FJM, et al. Primary lymphoma ofmucosa associated lymphoid tissue (MALT) in the parotidgland. Clin Otolaryngol 1993;18:528–532

Craniosynostosis Anomaliesin Twins

John A. van Aalst, MD, Geoff Schultz, BS,Barry L. Eppley, MD, DMD

Indianapolis, Indiana

Abstract: This report discusses the incidence of twinningin nonsyndromic and syndromic craniosynostosis. Nonsyndromiccraniosynostosis occurs in approximately 1 in 2,100 live births; theincidence of twinning in patients with nonsyndromic craniosynos-tosis has been reported to be higher than the rate of 2.5% of livebirths in the general population. No definitive explanation for thisincreased risk is known. The incidence of twinning in patients withnonsyndromic craniosynostosis may range between 2.5 and 19 in210,000 live births. Syndromic craniosynostosis occurs in between 1per 10,000 live births (Muenke’s syndrome) to 1 per 80,000 livebirths (Apert’s syndrome). Because of the these low occurrences,the actual incidence of twinning in syndromic craniosynostosisis difficult to predict. A theoretical incidence between 2.5 per mil-lion live births in Muenke’s syndrome and 2.5 per 8 million livebirths in Apert’s syndrome can be estimated.

Key Words: Craniosynostosis, genetic, twins

Twinning occurs in 2.5% of live births. Dizygotictwinning (fertilization of two distinct ova) occurs

once in every 100 live births, whereas monozygotictwinning (division of a single zygote) occurs oncein every 330 live births.1 For unknown reasons, theincidence of twinning in patients with nonsyndromiccraniosynostosis has been reported as higher than inthe general population.2–4 Syndromic craniosynosto-sis is a more rare condition, and the incidence of twin-ning in these anomalies has not been determined. Thefollowing is a review of what is currently knownabout twinning in nonsyndromic and syndromic cra-

niosynostosis. Having had four twin cases with cra-niosynostosis over the past decade spurred an inter-est in this topic.

REVIEW

The frequency of twinning in the general populationis estimated at 1 (2.5%) in 40 live births and is on therise with broader application of artificial reproduc-tive technologies.1 Monozygotic twinning, the resultof the division of a single zygote, occurs at a relativelyconstant rate of 1 in 330 live births throughout theworld. Spontaneous division of the fertilized ovummay be secondary to a defective zona pellucida, re-sulting in monozygotic twinning. Dizygotic twin-ning, the result of the fertilization of two separateova, occurs once in approximately every 100 livebirths. Variations in dizygotic twinning account forthe variability in twinning seen in different popula-tions. The frequency of dizygotic twinning is depen-dent on maternal follicle-stimulating hormone levels.Higher circulating follicle-stimulating hormone lev-els increase the frequency of dizygotic twinning.The follicle-stimulating hormone levels themselvesare dependent on factors such as ethnicity, geogra-phy, increasing age, and parity. Use of fertility agentsand other assisted reproductive technologies has in-creased the frequency of monozygotic and dizygotictwinning.1

As a result of the unique intrauterine environ-ment created by multiple gestations, twins are at in-creased risk for the development of structural defectssecondary to intrauterine overcrowding.5 Anomaliessecondary to mechanical force (deformations), in-cluding plagiocephaly, hemifacial hypoplasia, andmicrognathia, are shared by monozygotic and dizy-gotic twins, although the incidence is higher in mono-zygotic twins.6 Anomalies secondary to disruptionsof normal morphogenesis (malformations), includ-ing hemifacial microsomia, cleft lip and palate, andcraniosynostosis, also occur in dizygotic and mono-zygotic twins, although the rates are higher inmonozygotic twins. Among a sample population of1,114 births, 35 twin pairs (21 monozygotic pairsand 14 dizygotic pairs) were identified in which atleast one member of the twin pair was affected witha craniofacial anomaly. Malformations and deforma-tions were more common in monozygotic pairs thanin dizygotic pairs.6 This study additionally found thatmonozygotic pairs were more often concordant formalformationsanddeformations(33%ofmonozygoticpairs compared with 7% of dizygotic pairs).6 Becausemonochorionic monozygotic twins have a shared vas-culature, these fetuses are also at increased risk for the

From the Division of Plastic Surgery, Indiana University Schoolof Medicine, Indianapolis, Indiana.

Address correspondence to Dr. Eppley, Division of PlasticSurgery, Indiana University School of Medicine, 702 Barnhill Drive,#3540, Indianapolis, IN 46202; E-mail: [email protected]


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development of structural defects because of possiblevariations in blood supply. This risk is not shared by di-zygotic twins. As such, monozygotic twins are atgreater risk for the development of structural anoma-lies than dizygotic twins or singlet gestations.5

NONSYNDROMIC CRANIOSYNOSTOSIS

Nonsyndromic craniosynostosis, the result of prema-ture cranial suture fusion, may involve any of thecranial sutures, including the sagittal, coronal, me-topic, or lambdoid sutures. It occurs as an isolatedfinding without associated structural or developmen-tal anomalies. The most commonly involved suturesare the sagittal and coronal sutures, followed, in de-creasing frequency, by the metopic and lambdoidsutures.3,7,8 Sagittal suture synostosis (scaphoce-phaly) is characterized by an elongated skull withbitemporal narrowing. Coronal suture synostosismay be unilateral (anterior plagiocephaly) or bilateral(brachycephaly). Unilateral cases exhibit elevation ofthe ipsilateral superior orbital ridge and flattening ofthe ipsilateral frontal and parietal bones; bilateralcases exhibit a tall anteroposteriorly shortened cal-varium. Metopic synostosis (trigonocephaly) is char-acterized by a triangular-shaped calvarium witha prominent frontal crest. Lambdoid suture syn-ostosis, the most rare of the synostoses, may be uni-lateral (occipital plagiocephaly) or bilateral (occipitalbrachycephaly). Lambdoid synostosis results in oc-cipital flattening with compensatory contralateralparietal and frontal bossing. An ipsilateral occipito-mastoid bulge is also noted.9

Etiology

The genetic contribution to the development of non-syndromic craniosynostosis is not completely known.Familial cases of nonsyndromic craniosynostosis ac-count for only 10% to 14.4% of coronal synostosis, 4%to 6% of sagittal synostosis, and 4.3% to 5.6% of me-topic synostosis.10–12 Cohen7 reports that 8% of non-syndromic craniosynostoses have a familial pedigree.Penetrance for coronal synostosis in familial cases is61%, and it is 38% for sagittal synostosis.10,11

The facts that dizygotic twins would developconcordant sagittal synostosis13 and that not all con-cordant twins simultaneously develop the problemindicate a role for possible environmental and terato-genic influences in the development of nonsyn-dromic craniosynostosis. Graham et al14 suggestedthat early descent of the fetus and cephalopelvic dis-proportion result in prolonged fetal head constraintand contribute to the premature fusion of the sagittal

suture.15 Disturbances in intrauterine fetal circulationduring twin pregnancies may cause disturbancesin growth ultimately resulting in craniosynostosis.3

Eight cases of metopic synostosis were associatedwith fetal exposure to valproic acid, suggesting thatteratogenicity of certain compounds may affect theclosure of cranial sutures.12 This possible link hasbeen reported by other authors.16

Incidence

The best estimates for the prevalence of craniosynos-tosis are 343 to 476 per 1 million live births.7,17 Esti-mates for nonsyndromic craniosynostosis are 1 in1,250 to 1 in 2,500 live births in the general popula-tion.7,10 Frequencies for the types of synostosis varyfrom 4,200 to 5,000 live births with sagittal synosto-sis,10,11,13 1 in 16,000 female newborns and 1 in8,000 male newborns with coronal synostosis, and1 in every 15,000 live births with metopic synosto-sis.10,12 Among all cases of posterior deformationalplagiocephaly, only 1% are related to true lambdoidsynostosis.8 Lambdoid synostosis accounts for 0.5%of all craniosynostosis.18

In their study of nonsyndromic sagittal syn-ostosis and fetal head constraint, Graham et al14

suggested that male newborns were more often af-fected than female newborns because the head ofthe male fetus undergoes more rapid growth andis larger in comparison to that of the head of the fe-male fetus during the third trimester. They postulatedthat the increased size and rapidity of growth put thecalvaria of the male fetus at greater risk for deforma-tion secondary to constraint.

Twinning

There is significant variability in the estimated inci-dence of twinning in patients with nonsyndromic cra-niosynostosis, ranging from 3.3% to 19.0%. Cohen3

reported the rate of twinning in individuals with non-syndromic craniosynostoses as being 10 times greaterthan that in the general population.2,4 Other authorshave reported significantly lower frequencies. In astudy of 373 probands with scaphocephaly, 13(4.8%) were members of twin pairs, nine of whichwere dizygotic and four monozygotic (Fig 1). All ninedizygotic twin pairs were discordant for the anomaly;one of the four monozygotic twin pairs was concor-dant for sagittal synostosis.11 Another study examin-ing sagittal synostosis, found 7 (3.3%) of 214 patientsto be part of a twin pair; one dizygotic pair was con-cordant for the anomaly.13 In a study examining 237patients with trigonocephaly, 14 (7.8%) were twinsand two monozygotic twin pairs were concordant

BRIEF CLINICAL NOTES / van Aalst et al

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for the craniofacial anomaly.12 A single twin pair ofunknown zygosity has been reported in patients withlambdoid synostosis.2 In summary, reported twin-ning rates among patients with nonsyndromic cra-niosynostosis vary significantly, although the trendseems to be a rate that is higher than that in the gen-eral population. Concordance rates for the finding ofsynostosis vary from 7.7% to 14% of twin pairs (Fig 2).

SYNDROMIC CRANIOSYNOSTOSIS

Incidence

In contrast to isolated craniosynostoses, syndromiccraniosynostosis typically occurs in association withvarious other structural anomalies. The incidence ofCrouzon’s and Apert’s syndromes is approximately15.5 per 1 million live births, with each accountingfor approximately 4.5% of all cases of craniosynosto-ses.19,20 The occurrence of Muenke’s syndrome (oftenconfused with Crouzon’s syndrome) is much morecommon at 0.8 to 1 per 10,000 live births.21

Etiology

Mutations in fibroblast growth factor receptor genes,tyrosine kinases that are essential for intracellular sig-naling, have been pinpointed as the cause of manycases of syndromic craniosynostosis.7 Fibroblastgrowth factor receptor 2 mutations are most com-monly responsible for many craniosynostosis syn-dromes, including Apert’s, Crouzon’s, and Pfeiffer’ssyndromes, and are inherited in autosomal dominantfashion. Muenke’s syndrome is the result of aPro250Arg mutation in the fibroblast growth factorreceptor 3 gene and is also inherited in an autosomaldominant fashion. Mutations in TWIST genes havebeen associated with a Saethre-Chotzen syndrome,and homeobox mutations have been associated withBoston-type craniosynostosis.

Twinning

The actual incidence of twinning in patients with syn-dromic craniosynostosis has not been definedbecause of its rarity. Only a couple of articles describetwinning in syndromic craniosynostosis, both ofwhich involved patients with Crouzon’s syn-drome.22,23 There have not been enough reportedcases to determine whether one suture is more in-volved more frequently than others (Fig 3).

SUMMARY

Monozygotic twinning occurs in 1 per 330 live births,whereas scaphocephaly occurs in 1 per 5,000 livebirths, suggesting that the occurrence of monozygotictwins with scaphocephaly occurs in 1 per 15,500live births. The sagittal twins with scaphocephaly re-ported in this article are monozygotic twins with

Fig 1 Monozygotic twins with sagittal craniosynostosis,concordant for the anomaly.

Fig 2 Dizygotic twins with unilateral coronal craniosynos-tosis, with only one having the anomaly.

Fig 3 Monozygotic twins with Muenke’s syndrome, con-cordant for the anomaly, although the degree of expressionof the syndrome is different.


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concordance for the anomaly. The unilateral coronaltwins are dizygotic: one had synostosis but the otherdid not. As these two cases illustrate, monozygotictwins (as opposed to dizygotic twins) have higherconcordance rates for nonsyndromic craniosynosto-sis. The highest rate is approximately 25%, suggestingthat 1 in 60,000 live births results in a set of monozy-gotic twins with concordant craniosynostosis.

The rarity of syndromic craniosynostosis makesany estimates speculative, particularly when com-bined with twinning. In Crouzon’s and Apert’s syn-dromes, an occurrence of 1.5 per 100,000 births(0.000015) merged with twinning rates of 2.5% wouldcreate the rare occurrence of 3 per 10,000,000 births.Such a low occurrence rate accounts for the few casereports of twins with syndromic craniosynostosis.Muenke’s syndrome occurs more commonly in1 per 10,000 live births. If monozygotic births occurin 1 per 330 live births, 1 per 33,000 live births wouldresult in twin births with Muenke’s syndrome. Inmonozygotic births, if 25% are concordant for theanomaly, 1 per 134,000 live births would result ina single concordant pair of monozygotic twinswith Muenke’s syndrome. As a result, twins withMuenke’s syndrome should be seen more com-monly than twins with any other form of syndromiccraniosynostoses.

REFERENCES

1. Hall JG. Developmental biology. IV: twinning. Lancet 2003;362:735–743

2. Bertelson TI. The premature synostosis of the cranial sutures.Acta Ophthalmol (Copenh) 1958;36(Suppl 51):1–76

3. Cohen MM, Jr. Craniosynostosis and syndromes with craniosy-nostosis: incidence, genetics, penetrance, variability, and newsyndrome updating. Birth Defects 1979;15(5B):13–63

4. Franceschetti A, Klein D. Oxycephalie chez trios paires de ju-meaux univitellins, associee dans un des cas a une cutis frontisgyrata (Oxycephaly in three pairs of monozygotic twins, asso-ciated in one case with cutis frontis gyrata). Acta Genet MedGemellol 1952;1:48–65

5. Schnizel AAGL, Smith DW, Miller JR. Monozygotic twinningand structural defects. J Pediatr 1979;95:921–930

6. Keusch CF, Mulliken JB, Kaplan LC. Craniofacial anomaliesin twins. Plast Reconstr Surg 1991;87:16–23

7. Cohen MM, Jr. Craniosynostosis: Diagnosis, Evaluation, andManagement. New York: Oxford University Press, 1986

8. Vander Kolk C, Carson B. Lambdoid synostosis. Clin PlastSurg 1994;21:575

9. Williams JK, Cohen SR, Burstein FD, et al. Non-syndromiccraniosynostosis. In: Vander Kolk CA (ed). Plastic Surgery:Indications, Operations, and Outcomes, vol 2. St. Louis: Mosby,2000

10. Lajeunie E, Le Merrer M, Bonaiti-Pellie C, et al. Genetic studyof nonsyndromic coronal craniosynostosis. Am J Med Genet1995;55:500–504

11. Lajeunie E, Le Merrer M, Bonaiti-Pellie C, et al. Genetic studyof scaphocephaly. Am J Med Genet 1996;62:282–285

12. Lajeunie E, Le Merrer M, Marchac D, et al. Syndromal andnonsyndromal primary trigonocephaly: analysis of a series237 patients. Am J Med Genet 1998;75:211–215

13. Hunter AGW, Rudd NL. Craniosynostosis. I. Sagittal synosto-sis; its genetics and associated clinical findings in 214 patientswho lacked involvement of the coronal suture(s). Teratology1976;14:185–193

14. Graham JM, Jr, deSaxe M, Smith DW. Sagittal craniostenosis:fetal head constraint as one possible cause. J Pediatr 1979;95:747–750

15. Higginbottom MC, Jones KL, James HE. Intrauterine constraintand craniosynostosis. Neurosurgery 1980;6:39–44

16. Chabrolle JP, Bensouda B, Bruel H, et al. Metopic craniosynos-tosis, probable effect of intrauterine exposure to maternal val-proate treatment. Arch Pediatr 2001;8:1333–1336

17. Lammer EJ, Cordero JF, Wilson MJ, et al. Document EPI-83–56–2. Atlanta, Public Health Service, Centers for DiseaseControl and Prevention. April 8, 1987

18. Anderson FM, Geiger L., Craniosynostosis: a survey of 204cases. J Neurosurg 1965;22:229–240

19. Cohen MM, Jr, Kreiborg S, Lammer EJ, et al. Birth prevalencestudy of the Apert syndrome. Am J Med Genet 1992;42:655–659

20. Martinez-Friaz ML, Cereijo A, Bermejo E, et al. Epidemiologicalaspects of Mendelian syndromes in a Spanish population sam-ple: I. Autosomal dominant malformation syndromes. Am JMed Genet 1991;38:662–675

21. Moloney DM, Wall SA, Ashworth GJ, et al. Prevalence ofPro250Arg mutation of fibroblast growth factor receptor 3 incoronal craniosynostosis. Lancet 1997;349:1059–1062

22. Yasuda S, Enomoto T, Yamada Y, et al. Crouzon disease asso-ciated with sinus pericranii: a report on identical twin sisters.Childs Nerv Syst 1993;9:119–122

23. Lajeunie E, Bonaventure J, El-Ghouzzi V, et al. Monozygotictwins with Crouzon syndrome: concordance for craniosynosto-sis and discordance for thumb duplication. Am J Med Genet2000;91:159–160

Head and Neck AngiosarcomasBryan C. McIntosh, MD,*Deepak Narayan, MD, FRCS (ENG), FRCS (EDIN)y

New Haven, Connecticut

Abstract: Angiosarcoma of the head and neck is a rare and lethalneoplasm. Data from the Connecticut Tumor Registry was ana-lyzed for all head and neck angiosarcomas between 1980 and2001. The authors also present the first reported case of familial an-giosarcoma of the head and neck region. The 1-year mortality was48% for all patients with angiosarcomas (38% for males, 69% for

From the *Hospital of St. Raphael and yYale-New Haven Hos-pital, New Haven, Connecticut.

Previously presented at the Connecticut Chapter of the Ameri-can College of Surgeons Scientific and Annual Meeting, November5, 2003. Certain data used in this study were obtained from the Con-necticut Tumor Registry, located in the Connecticut Department ofPublic Health. The authors assume full responsibility for analysesand interpretation of these data.

Address correspondence and reprint requests to Dr. DeepakNarayan, Yale University, 330BB, PO Box 208041, 330 Cedar Street,New Haven, CT 06528; E-mail: [email protected]

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females). The 5-year survival was 28% for all patients (38% formales, 15% for females). The combination of surgery and radiother-apy resulted in improved survival compared with either treatmentalone.

Key Words: Angiosarcoma, familial angiosarcoma,head and neck angiosarcoma

A ngiosarcoma is a rare malignancy of mesenchy-mal origin, comprising ,10% of all soft tissue

sarcomas. Although half of all angiosarcomas occurin the head and neck region, these tumors still consti-tute ,1% of all malignant head and neck neoplasms.Angiosarcomas are often multifocal and histologi-cally have high-grade features. The three clinical pat-terns described are superficial spreading, nodular,and ulcerating.1

A wide variety of predisposing causes have beennoted in the literature. These include exposure toThorotrast, vinyl chloride, arsenic, and radiation.Wilms’ tumor, retinoblastoma, and von Recklinghau-sen disease have been documented to result in sub-sequent angiosarcoma even without prior radiationexposure.2 Mutations of the p53 gene have beenassociated with head and neck angiosarcoma.3

Familial angiosarcoma has been reported onlytwice in the literature. Primary renal angiosarcomain two brothers was reported in 1995.4 In 2002, Cashaet al5 described familial angiosarcoma of the heart.We present the first case of familial head and neckangiosarcoma to be reported in the literature, alongwith a 20-year survey of head and neck angiosarco-mas in Connecticut.

CASE REPORTS

A 64-year-old woman presented with a 3-cm ery-thematous plaque on her left forehead. A punch bi-opsy diagnosed the lesion as an angiosarcoma. Sheunderwent wide local excision with a skin graft fol-lowed by 6 weeks of radiation therapy. Eight yearslater she remains disease free.

The woman’s brother, a 76-year-old Navy veteran,presented with a rapidly growing violaceous lesionon his right parietal scalp. Clinical examination wasalso significant for multiple, palpable, ipsilateral necknodes. A metastatic workup was negative. A biopsyof the lesion confirmed it to be an angiosarcoma. Hereceived preoperative chemotherapy, followed bywide local resection, a radical neck dissection, paro-tidectomy, and skin grafting of the affected area. Healso received 6 weeks of radiation therapy after sur-

gery. He died 6 months later with local recurrenceand metastatic disease.


A retrospective analysis of data was performed on allangiosarcomas occurring between 1980 and 2001 thatwere reported to the Connecticut Tumor Registry. Therecords were examined for race, gender differences,age of diagnosis, life expectancy, history of other tu-mors, and treatments used. Standard methods wereused for statistical analysis.

RESULTS

There were 54 cases of angiosarcoma reported in 53patients during the 21-year study period. All patientswere white. Sixteen patients were male and 37 werefemale (male-to-female ratio [M:F], 1:2.3). Twenty-nine (54%) angiosarcomas occurred in the headand neck; the remaining 25 (46%) occurred solely inwomen in the breast. Using population data fromthe Connecticut Census Bureau, head and neckangiosarcomas occur with an incidence of �4cases/10 million/year.

Of the 29 head and neck angiosarcomas, 16occurred in men and 13 occurred in women (M:F,1:1.2). The mean age at diagnosis was 71 years (range,41–92 years). Males presented with the disease ata mean age of 73 years (range, 55–92 years), whereasfemales presented at a mean age of 69 years (range,41–91 years). Figure 1 shows the distribution of casesby age of diagnosis and gender.

Fig 1 Number of head and neck angiosarcomas versuspatient age at diagnosis.


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Fifteen (52%) patients were reported to have diedof angiosarcoma of the head and neck. Seven of thesepatients were male, nine were female (M:F, 1:1.3).Eight (28%) patients died of other causes, which werereported to be myocardial infarction, coronary arterydisease, squamous cell carcinoma, prostate carcinoma,breast carcinoma, lung carcinoma, and brain hemor-rhage. Six (21%) patients were alive at last follow-up.

The mean survival for all patients with head andneck angiosarcoma who died was 37 months, witha median of 8 months, and a range of 0 to 165 months.Males lived a mean of 56 months (range, 2–165months), whereas females lived an average of9.4 months (range, 0–30 months). The 1-year mortal-ity was 48% for all patients (38% for males, 69% forfemales). The 5-year survival rate was 28% for allpatients (38% for males, 15% for females).

There were 20 patients whose only reported tu-mor was angiosarcoma of the head and neck; 8 weremale, 12 were female. Their mean survival was40 months. Nine patients (7 males, 2 females) werereported to have another tumor in addition to a headand neck angiosarcoma. Three males (no females)with an angiosarcoma had a diagnosis of a secondmalignancy. The tumors reported after angiosarcomawas diagnosed were lung carcinosarcoma, prostateepithelial tumor, and small bowel carcinoid. Themean age of diagnosis of the angiosarcoma was73 years; the second tumor was diagnosed at a meanage of 78 years. These patients lived an average of 138months (range, 119–156 months) after the diagnosisof angiosarcoma. Three males and two females haddiagnoses of another malignancy before the diagno-sis of angiosarcoma. These patients’ first neoplasmswere reported to be laryngeal squamous cell carci-noma, prostate adenocarcinoma, skin carcinoma,and uterine papilloma. These patients lived a meanof 13 months (range, 4–22 months).

Of all patients with head and neck angiosar-coma, 21 were reported to have had surgical treat-ment (with or without chemotherapy or radiationtherapy), whereas the remaining 8 patients eitherhad no surgery or surgical therapy was not reported.The mean survival of these 21 patients (13 males,8 females) was 41 months (55 months for males,9.5 months for females; range, 2–165 months). Ofthese 21 patients (7 males, 4 females), 11 also hadradiation therapy, and they lived an average of66 months (98 months for males, 12 months for fe-males; range, 3–165 months) after diagnosis of angio-sarcoma. Of the 3 patients who were reported to havehad no surgical treatment, 1 was male and the other2 were female; their average survival was 7 months(range, 5–10 months).

DISCUSSION

This tumor typically presents as described in the twoprevious cases, namely as a purplish plaque withpoorly defined borders (associated with well- ormoderately differentiated tumor); as diffuse, indu-rated nodules (associated with poorly differentiatedtumor); or as an ulcerated lesion.6 They can present asbenignly as alopecia, scarring, or edema alone. Ten-derness, ulceration, and bleeding are signs of moreadvanced disease.7 Because of the insidious natureof onset and growth, delays in presentation anddiagnosis are common. These tumors most com-monly occur in the upper face or scalp, followedby the central face, and least commonly in the man-dibular area.6

Angiosarcomas occurred about twice as oftenin women as in men. This finding was also seen byDaugaard et al.8 When angiosarcomas that occurredin the head and neck were isolated, they were seento occur slightly more frequently in men than inwomen, although we did not see a male:female pre-ponderance of 2:1 or 3:1, as reported previously.9,10

The mean age of diagnosis of head and neckangiosarcomas in this group of patients is similarto that reported in the literature, and it is essentiallythe same for men and women.9 However, there seemsto be a bimodal distribution for when these tumorsoccur in women, whereas a more bell-shaped curveis seen for men (Figure). The significance of this phe-nomenon is unclear.

There is a striking absence of African Americansin this study. This absence is mirrored in other studiesof angiosarcomas of the head and neck region inwhich race was one of the parameters studied.

Angiosarcoma of the head and neck portendsa poor prognosis when compared with those arisingin other locations.11 Complete spontaneous regres-sion has been reported only twice.12,13 Other prog-nostic factors have been reported for head and neckangiosarcomas. Smaller size of the tumor is a favor-able factor: ,10 cm, as reported by Holden et al,6 or,5 cm, as reported by Maddox and Evans.14 Tumorswith nodular macroscopic characteristics were asso-ciated with a more favorable prognosis than werethose with an endophytic appearance.15 Younger pa-tients and those with a T1 disease stage have beenshown to have improved survival.16 Tumor gradewas noted to be the most important prognostic factorin one review of sarcomas of all sites, but this has notbeen reported for head and neck angiosarcomas spe-cifically.17 Increased lymphocytic admixture with thetumor has been associated with increased survivaland decreased rates of metastases.14

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Head and neck angiosarcomas are quite lethal,with a 48% 1-year mortality. This figure is similarto that reported by Daugaard et al.8 However, the5-year survival rate of 28% in our study is more thandouble that reported by Holden et al.6 This figure isconsistent with that reported by Mark et al,10 whonoted a 26% 5-year survival. We also note thatfemales fared worse than did males with respectto mortality, a finding not previously reported.

Although multimodality treatment often is re-quired, surgery has been the mainstay of treatmentof all angiosarcomas. Very wide margins of severalcentimeters as determined by multiple biopsies arerequired for clearance because these lesions oftenare known to be multifocal and extend well beyondmargins visible on the surface of the skin.18 It hasbeen reported that about half of the patients presentwith metastatic disease, and this occurs both throughvascular and lymphatic channels most commonly tocervical lymph nodes, lungs, and liver.19 Hodgkinsonet al1 recommended lymph node dissection for pa-tients in whom lymphadenopathy is apparent or ifthe tumor is confined to one side of the body. Inour study, patients who had surgical therapy sur-vived longer than did those who did not. The datareported do not indicate thoroughly how extensivethe surgical resection was for these patients. In addi-tion, it is unclear from these data whether the pa-tients who did not receive surgical treatment hadadvanced disease that was thought to be unresect-able. Such patients would not have an opportunityfor curative resection. It is also possible that someof the patients who had surgery merely had a pallia-tive procedure.

Radiation has also been used as a treatment mo-dality. Mark et al10 demonstrated improved survivalin patients who received postoperative radiationtherapy compared with those who had only surgicaltreatment. Morrison et al20 showed that radiotherapyof head and neck angiosarcomas that were clinicallynot evident showed significant improvement in sur-vival compared with treatment of clinically evidenttumors. Our results indicate a clear role for radiationtherapy in the treatment of head and neck angiosar-coma. It appears that radiotherapy extending well be-yond the visible margins of the tumor is required, butmetastases can occur even after apparent cure.6

Chemotherapy is a third treatment modality forangiosarcoma. Only four of our patients were re-ported to have received chemotherapy. Because thesepatients also had a mixture of surgical or radiationtherapies, it is difficult to comment on the effect ofchemotherapy on the survival of these patients.Adriamycin and paclitaxel are the drugs currently

used. Immunotherapy involving recombinant inter-leukin-2 has been reported to suppress the occur-rence of distant metastases.15

Most authors report that head and neck angio-sarcomas often recur locally and metastasize readilydespite multimodality therapy. There were obviousgender differences seen among our cohort of angio-sarcomas. Across the board, males fared better thandid females: males had disease diagnosed at a laterage and had better survival than did females whencompared with no treatment or any treatment. It isunknown whether estrogen or testosterone plays anyrole in the striking differences seen between menand women with head and neck angiosarcoma. Todate there are no published studies that discuss headand neck angiosarcoma in relation to sex steroids.

REFERENCES

1. Hodgkinson DJ, Soule EH, Woods JE. Cutaneous angiosarcomaof the head and neck. Cancer 1979;44:1106–1113

2. Meadows AT, Baum E, Fossati-Bellani F, et al. Second malignantneoplasms in children: an update from the late effects studygroup. J Clin Oncol 1985;3:532–538

3. Naka N, Tomita Y, Nakanishi H, et al. Mutations of p53 tumor-suppressor gene in angiosarcoma. Int J Cancer 1997;71:952–955

4. Kern SB, Gott L, Faulkner J II. Occurrence of primary renalangiosarcoma in brothers. Arch Pathol Lab Med 1995;119:75–78

5. Casha AR, Davidson LA, Roberts P, Nair RU. Familial angiosar-coma of the heart. J Thorac Cardiovasc Surg 2002;124:392–394

6. Holden CA, Spittle MF, Jones EW. Angiosarcoma of the faceand scalp, prognosis and treatment. Cancer 1987;59:1046–1057

7. Panje W, Moran W, Bostwick D, Kitt V. Angiosarcoma of thehead and neck: review of 11 cases. Laryngoscope 1986;96:1381–1384

8. Daugaard S, Hultberg BM, Hou-Jensen K, Mouridsen HT.Clinical features of malignant haemangiopericytomas andhaemangioendotheliosarcomas. Acta Oncol 1988;27:209–213

9. Liu AC, Kapp DS, Egbert B, et al. Angiosarcoma of the face andscalp. Ann Plast Surg 1990;24:68–74

10. Mark RJ, Tran LM, Sercarz J, et al. Angiosarcoma of the headand neck: the UCLA experience 1955 through 1990. Arch Oto-laryngol Head Neck Surg 1993;119:973–978

11. Ward JR, Feigenberg SJ, Mendenhall NP, et al. Radiation ther-apy for angiosarcoma. Head Neck 2003;25:873–878

12. Wilson Jones E. Malignant vascular tumors. Clin Exp Dermatol1976;1:287–312

13. Cerroni L, Peris K, Legge A, Chimenti S. Angiosarcoma of theface and scalp. A case report with complete spontaneousregression. J Dermatol Surg Oncol 1991;17:539–542

14. Maddox J, Evans H. Angiosarcoma of skin and soft tissue:a study of 44 cases. Cancer 1981;48:1907–1921

15. Sasaki R, Soejima T, Kishi K, et al. Angiosarcoma treated withradiotherapy: impact of tumor type and size on outcome. Int JRadiat Oncol Biol Phys 2002;52:1032–1040


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16. Pawlik TM, Paulino AF, McGinn CJ, et al. Cutaneous angiosar-coma of the scalp: a multidisciplinary approach. Cancer 2003;98:1716–1726

17. Russell WD, Cohen J, Enzinger FM, et al. A clinical and path-ologic staging system for soft tissue sarcomas. Cancer 1977;40:1562–1570

18. Barttelbort SW, Stahl R, Ariyan S. Cutaneous angiosarcoma ofthe face and scalp. Plast Reconstr Surg 1989;84:55–59

19. Knight T, Robinson H, Sina B. Angiosarcoma (angioendothelioma)of the scalp: an unusual case of scarring alopecia. Arch Derma-tol 1980;116:683–686

20. Morrison WH, Byers RM, Garden AS, et al. Cutaneous angio-sarcoma of the head and neck: a therapeutic dilemma. Cancer1995;76:319–327

Intraosseous Hemangioma ofthe Infraorbital Rim

Tracey H. Stokes, MD, Tristan L. Hartzell, BS,Laura A. Gunn, MD, Jeffrey R. Marcus, MD

Durham, North Carolina

I ntraosseous hemangiomas are benign vascular tu-mors accounting for 0.5% to 1% of all intraosseous

tumors.1,2 Of these, primary orbital intraosseous he-mangiomas are even more rare. The first intraosseoushemangioma was described in 1845 by Toynbee,3 andthe first zygomatic intraosseous hemangioma was re-ported by Schofield in 1950.4 We report on a 51-year-old patient who experienced a slow-growing bonymass in the infraorbital region.

CASE REPORT

A 51-year-old woman presented with a mass in thearea of the left infraorbital rim. The patient notedan irregularity in the area after a traumatic event15 years earlier. This mass grew gradually to becomea notable prominence in the upper left cheek. At thetime of presentation, she noted visual obstruction ondownward gaze. She had a remote 14 pack-year his-tory of tobacco use with an otherwise unremarkablepast medical history.

On examination, the hard mass was about 1.5 cmin diameter, well-circumscribed, nonmobile, andfixed, without adhesions to the overlying skin (Fig 1).It extended over the left infraorbital rim and onto

the floor of the orbit. It was slightly tender to palpa-tion. Extraocular motility was intact, as was sensationto the cheeks. Visual acuity was grossly within nor-mal limits. The neck examination was negative for cer-vical lymphadenopathy. The differential diagnosisbased on the history and physical examination includedosteochondroma, vascular tumor, exostosis, and bonyor cartilaginous malignancy. A craniofacial computedtomography (CT) scan with three-dimensionalreconstructions was performed (Fig 2), revealing a1.0 3 1.3 cm exophytic lesion extending from theanterior wall of the left maxillary antrum, with inter-nal density not typical for an osteoma or fibrousdysplasia. Radiographic diagnostic considerationsincluded cartilaginous lesions, such as a juxtacorti-cal chondroma or even possibly a small chondrosar-coma.

The localized bony lesion did not demonstrateinvasion into the maxillary sinus, erosion of adjacentbones, or soft tissue involvement. Based on the CTresults, symptoms, and aesthetic concerns, a decisionwas made to excise the lesion for both pathologicaldiagnosis and treatment.

The patient was brought to the operating room,and with the patient under general anesthesia, an ex-cisional biopsy of the bony mass was performed. Asubciliary incision overlying the mass was made,with standard elevation of a skin/muscle flap. Sub-periosteal dissection revealed the bony mass to beround and variegated in color with displacementof the infraorbital rim in a medial and inferior direc-tion (Fig 3). The infraorbital foramen was also dis-placed, suggesting a mass effect rather than bonydestruction. The bony mass was excised en toto bycircumscribing with a small rotary burr. The sur-rounding bony ledge along the infraorbital rim wasthen contoured to a smooth surface. Hemostasiswas achieved with bone wax and Surgicel. Thewound was closed in layers, first reapproximatingthe orbicularis oculi muscle and then the skin. A tem-porary tarsorrhaphy stitch was placed. The patienttolerated the procedure well without any intra-operative complications. A forced duction test wasnormal at the conclusion of the procedure.

Histopathologic analysis revealed trabeculaesurrounding cavernous vascular spaces filled witherythrocytes and lined by a single layer of endothelialcells (Fig 4). The pathological diagnosis was a primaryintramedullary cavernous hemangioma, without anyevidence of malignancy.

The patient recovered with no postoperativecomplications, and a satisfactory aesthetic resultwas achieved. At 1-year follow-up, the patient hasno evidence of recurrence.

From the Division of Plastic, Reconstructive, and MaxillofacialSurgery, Duke University Medical Center, Durham, North Carolina.

Address correspondence and reprint requests to Dr. Jeffrey R.Marcus, Pediatric Plastic Surgery and Craniofacial Surgery, DukeUniversity, DUMC 3974, Durham, NC 27710; E-mail: [email protected]

BRIEF CLINICAL NOTES / Stokes et al

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DISCUSSION

The first intraosseous hemangioma was described in1845 by Toynbee,3 and the first zygomatic intra-osseous hemangioma was reported by Schofield in1950.4 Relf et al1 reported only 16 published casesof orbital intraosseous hemangiomas in his compre-hensive review, whereas Koybasi et al5 found 22 casesof zygomatic intraosseous hemangiomas, includingtheir own case. The most extensive review, publishedby Barnes,6 found 123 intraosseous hemangiomas ofthe skull and 74 hemangiomas of the jaws reportedin the literature from 1845 to 1981. However, manyof the diagnoses were questionable and were not his-tologically proven. To date, the most accurate andcomprehensive literature review performed comesfrom Heckl et al,2 who disclosed only 14 histopatho-logically proven zygomatic intraosseous cavernoushemangiomas in the literature since 1975. Interest-ingly, most reports of osseous hemangiomas typicallyoccur in patients in the fourth or fifth decade of life.1

Fig 1 Preoperative appearance of patient with left infraor-bital mass. The lesion was fixed, hard, well-circumscribed,and mildly tender.

Fig 2 (A) Axial computed tomography illustrating massemanating from the bony surface of the left infraorbitalrim. (B) Three-dimensional computed tomography recon-structions confirming mass of the left infraorbital rim.

Fig 3 Intraoperative view of the mass showing trabecu-lated appearance.


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Primary orbital intraosseous hemangiomas pre-sent as nontender, slow-growing bony masses thatcan lead to proptosis, globe displacement, diplopia,neuralgia, epistaxis, and loss of vision, dependingon location.1,7 Trauma to the area in question distin-guishes this report, but the cause and effect of thisobservation is questionable and cannot be estab-lished. The clinical differential diagnosis for intra-osseous hemangioma includes metastatic disease,multiple myeloma, fibrous dysplasia, histiocytosisX, dermoid cyst, osteoma, chondroma, and osteo-genic sarcoma. Plain films may reveal a sunburstor honeycomb pattern radiating from the center ofthe lesion. However, these findings are not alwayspresent.1,7,8 CT can also be an excellent diagnostictool, revealing a characteristic crosshatched patternwith radiating trabeculae. Any sign of destructionof surrounding tissue should lead to concerns regard-ing malignancy.2 Histopathologically, the tumor con-sists of large, erythrocyte-filled, endothelial-lined cav-ernous vessels surrounded by osseous trabeculae.9

Asymptomatic lesions may be followed clini-cally if there is no suspicion of malignancy. Indica-tions for treatment include visual field defects, masseffect on the adjacent eye, diagnostic concerns, andcosmetic reasons. Complete resection is the treatmentof choice,1,5,8 when possible. Of note, bleeding duringexcision may be problematic. However, this was notencountered in the case described here.10 Resection ofthese benign lesions usually results in complete curewith very rare instances of recurrence.8

Resection can result in a bony contour defect forlesions of the zygoma/infraorbital region. Providedthat no diagnostic ambiguity exists, reconstructioncan be performed at the time of resection. A numberof options exist for reconstruction, with autologousbone grafting providing a reliable modality and beingthe authors’ preference. The use of alloplastic materi-als, such as Medpore, also carries advantages.

SUMMARY

In summary, we present a case of a primary heman-gioma of the infraorbital rim causing symptoms of vi-sual field obstruction and diagnostic concerns. Enbloc resection was performed for diagnosis and treat-ment with satisfactory operative and aesthetic out-comes. Intraosseous hemangioma is a diagnosis thatshould be entertained for slow-growing bony massesof the craniofacial skeleton.

REFERENCES

1. Relf SJ, Bartley GB, Unni KK. Primary orbital intraosseoushemangioma. Ophthalmology 1991;98:541–546; discussion547

2. Heckl S, Aschoff A, Kunze S. Cavernomas of the skull: reviewof the literature 1975–2000. Neurosurg Rev 2002;25:56–62;discussion 66–57

3. Toynbee J. An account of two vascular tumors developed in thesubstance of bone. Lancet 1845;2:676

4. Schofield A. Primary hemangioma of the malar bone. Br J PlastSurg 1950;3:136–140

5. Koybasi S, Saydam L, Kutluay L. Intraosseous hemangioma ofthe zygoma. Am J Otolaryngol 2003;24:194–197

6. Barnes L. Surgical pathology of the head and neck. New York:Dekker, 1985:932–936

7. Sharma RR, Pawar SJ, Lad SD, et al. Frontal intraosseous cryptichemangioma presenting with supraorbital neuralgia. ClinNeurol Neurosurg 1999;101:215–219

8. Zucker JJ, Levine MR, Chu A. Primary intraosseous hemangi-oma of the orbit. Report of a case and review of literature.Ophthal Plast Reconstr Surg 1989;5:247–255

9. Heckl S, Aschoff A, Kunze S. Cavernomas of the skull: reviewof the literature 1975–2000. Neurosurg Rev 2002;25:56–62;discussion 66–57

10. Okada AA, Shore JW, Rubin PA. Periorbital intraosseoushemangiomas. Int Ophthalmol Clin 1992;32:111–121

Fig 4 Microscopic pathology demonstrating thin-walledvascular channels filled with erythrocytes, replacing normalmarrow hematopoietic elements, which are typical of intraoss-eous hemangioma (H & E stain, original magnification 340).

BRIEF CLINICAL NOTES / Stokes et al

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Surgical Technique for PrimaryAlveolar Bone Grafting

John A. van Aalst, MD, Barry L. Eppley, MD, DMD,Ronald R. Hathaway, DDS, A. Michael. Sadove, MD

Indianapolis, Indiana

Abstract: Bone grafting of the alveolar cleft is an important com-ponent of the comprehensive care of the cleft lip and palate patient.Although debate exists regarding the optimal timing of the proce-dure, one approach is the placement of a rib graft in the alveolarcleft before eruption of the deciduous canine (primary alveolarbone graft). Since 1982, primary alveolar bone grafting has beenperformed at our institution in more than 300 patients. Becauselittle is reported in the literature on primary alveolar bone graftingin general, an experienced surgical technique and protocol are lack-ing. Our surgical technique for rib graft harvest as well as alveolarbone graft placement in the infant cleft alveolus is described.

Key Words: Primary alveolar bone grafting, alveolarcleft, bone grafting, rib

B efore 1900, no surgical intervention was per-formed for the alveolar cleft, although tech-

niques for repair of the cleft lip and cleft palate werehundreds of years old. By the early 1900s, surgeonsbegan describing techniques to fill the alveolar defectwith autogenous bone1–3; sources of bone includedthe tibia, cranium, iliac crest, and rib. By the 1950s,alveolar bone grafting had become standard of carefor patients with an alveolar cleft and was routinelyperformed when the infant was less than 1 year ofage. In preparation for the bone graft, infants werefitted with a passive palatal molding appliance thatguided the abutment of the maxillary segments inpreparation for bone grafting.4,5 A segment of ribwas harvested for filling the alveolar cleft.

By the 1960s, concerns were raised that midfacialgrowth may be restricted because of the primary al-veolar bone grafting (PABG) technique.6–9 This wasbelieved to be related to dissection in the vicinityof the vomeromaxillary suture (a primary midfacialgrowth center) to perform PABG.10–12 Subsequent tothis concern, an increasing number of centers beganto delay alveolar bone grafting until patients attainedmixed dentition between the ages of 6 and 9 years (be-

fore eruption of the permanent canine). Delayed bonegrafting, referred to as secondary alveolar bone graft-ing, theoretically avoids interference with midfacialgrowth yet still provides bony stock for the eruptionof permanent teeth (principally the lateral incisor andcuspid) at the alveolar cleft. The preferred source ofbone for secondary alveolar bone grafting has be-come cancellous bone taken from the iliac crest.

Proponents of PABG, however, claim that be-cause of a lack of standardized techniques, it wasnot the timing of the technique but an overly—andunnecessarily—aggressive dissection technique thatresulted in midface growth restriction.13–15 Thesetechniques ranged from wedging the rib graft intothe entire cleft (Fig 1) to resection of segments of vo-mer for grafting the cleft alveolus (Fig 2) and to a widedissection along the cleft alveolus, vomer, and hardpalate to inlay tibial bone (Fig 3). Our technique forPABG is much more minimal (Figs 4 and 5); when ap-propriately performed, it does not restrict midfacegrowth and has multiple benefits.16,17 In this report,we present our technique used to perform PABG andthe benefits that accrue from this technique.

TECHNIQUE

Decision for Grafting

All patients with complete clefts of the lip and palate(unilateral and bilateral) are fitted with a palatal

Fig 1 Previously described technique for primary alveolarbone grafting, where a rib graft was wedged into the entirealveolar cleft.6

From the Division of Plastic Surgery, Indiana University Schoolof Medicine, Indianapolis, Indiana.

Address correspondence to Dr Eppley, Division of Plastic Sur-gery, Indiana University School of Medicine, 702 Barnhill Drive,#3540, Indianapolis, IN 46202; E-mail: [email protected]

Fig 2 Previously described technique for primary alveolarbone grafting, where portions of the vomer were resectedand placed into the alveolar cleft.


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obturator shortly after birth. The purpose of the ob-turator is to align the greater and lesser maxillary seg-ments passively. Lip closure, which is completed at 10weeks of age, aids in actively aligning the maxillarysegments. This combination of active and passiveforces leads to an approximation of the greater andlesser maxillary segments (Figs 6 and 7).

Between the ages of 7 and 9 months of age, the prox-imity of the greater and lesser segments determineswhether PABG can be undertaken effectively. If thesoft tissue of the segments abuts or is at a distanceof up to 1 mm apart, PABG can be undertaken. Evenin cases in which the soft tissue abuts, the alveolar cleftgap may still be 5 to 7 mm. If the soft tissue distance isgreater, adequate soft tissue coverage after bone graft-

ing may be compromised, leading to rib graft extru-sion and failure of the procedure. Rarely, maxillaryarch alignment may be supplemented with an activeobturator to move the arch segments into alignment.18

Harvesting the Rib Graft

If there is good alignment of the maxillary segmentswhen the patient is between the ages of 7 and 9 monthsof age, the patient is taken to the operating room for

Fig 3 Previously described technique for dissection alongthe cleft alveolus and vomeromaxillary suture and onto thehard palate to place the tibial bone graft.5

Fig 4 Our technique for primary alveolar bone grafting fora unilateral cleft lip and palate patient.

Fig 5 Our technique for primary alveolar bone grafting fora bilateral cleft lip and palate patient.

Fig 6 Model of a bilateral cleft lip and palate patient beforepalatal molding and lip closure.

Fig 7 Abutment of the maxillary segments after passivepalatal molding and lip closure.


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PABG. The patient is placed in the supine position,and general endotracheal intubation is administered.The head is placed in a Mayfield headrest, the chestis prepared with Hibiclens, and the oral cavity isprepared with an Ivory soap mixture. For rib harvest,the fifth through seventh ribs may be used. In a malepatient, at the intersection of the inframammary foldand the lateral border of the pectoralis major muscle(usually the fifth rib), a 1.5-cm incision is marked run-ning medial to lateral along the central axis of the rib.In a female patient, the seventh rib is chosen to avoidany potential damage to the future breast mound. Amixture of 1% lidocaine with 1:100,000 epinephrine isinfiltrated at the site (Fig 8). The incision follows thecurve of the rib and is directed toward the rib’s center.The serratus anterior is divided by needle tip electro-cautery, and the periosteum is incised at the center ofthe rib along the entire length to be harvested (Fig 9).After this, a freer is used to lift the periosteum gentlyfrom the visible portion of the rib. A doyen elevator isthen used to strip periosteum along the posterior partof the rib. When the length of rib intended for harvest

Fig 8 Markings for rib harvest during primary alveolarbone grafting.

Fig 9 A 1.5-cm incision is made along the central axis ofthe rib.

Fig 10 A 2.5-cm section of rib is harvested for unilateralcleft lip and palate cases; a 3.5-cm section is harvestedfor grafting of bilateral cleft lip and palate cases.


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is stripped of periosteum, the rib is disarticulatedwith the doyen elevator medially at the osseous-car-tilaginous junction and then cut at its lateral extentwith small rib cutters (Fig 10). A 2.5-cm segment ofrib is harvested for unilateral clefts, and a 3.5-cmsegment is harvested for bilateral clefts.18 The harvestsite is then filled with fluid, and the anesthesiologistgenerates a 40-mm Hg Valsalva maneuver to deter-

mine whether the pleura has been violated. If thepleura has not been violated, a layered closure is per-formed (Fig 11). The periosteum is closed with 3-0Vicryl; 4-0 Vicryl is used to reapproximate the serra-tus anterior and the dermis in separate layers. A run-ning subcuticular stitch of 5-0 PDS is used for skinclosure. A Steri-Strip is applied and covered witha dry sterile dressing. If the pleura has been violated,a small red rubber catheter is placed into the defect;the pleura is closed around the catheter with 4-0Vicryl, and a layered closure of periosteum, muscle,dermis, and skin is then performed over the tube. Atthe end of the case, during a positive-pressure breath,the catheter is removed and the wound is dressed.

Preparing the Alveolar Cleft for Grafting

The mucosa around the alveolar cleft is infiltratedwith a mixture of 1% lidocaine and 1:100,000 epi-nephrine. A trapezoidal flap is raised, with the bloodsupply based in the upper vestibule (Fig 12). The

Fig 11 The rib periosteum is the first layer of closure afterrib harvest.

Fig 12 A trapezoidal flap is raised with blood supply fromthe upper vestibule of the lip.

Fig 13 An anterior subperiosteal pocket is created forplacement of the rib graft.


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mucosal edges of the cleft are raised and folded backto be approximated as the nasal surface lining. A sub-periosteal dissection is performed along the anterior(labial) surface of the upper alveolus (Fig 13). Dissec-tion is not extended onto the palate or the piriformaperture. The nasal mucosal layer is then closed withinterrupted 4-0 Vicryl for watertight closure.18

Once the subperiosteal pocket has been created,the rib segment is bisected longitudinally (Fig 14),and the half with the appropriate curvature for thealveolus is placed into the pocket as an onlay graft(Fig 15). The remaining half of the rib is morselized,with the fragments placed between the rib and theposterior mucosal closure. After this, the previouslyraised upper vestibular flap is used to cover theanterior surface of the rib graft using interrupted4-0 PDS (Fig 16). The soft tissue covering of the ribmust be watertight to protect the bone graft.

After surgery, the chest site is covered for 3 to5 days and the dressing is then removed. Early inour use of this technique, we routinely performedchest radiographs in the recovery room; however,

Fig 14 The rib graft is split longitudinally.

Fig 15 The split rib is placed in onlay fashion into thesubperiosteal pocket on the anterior alveolar surface.

Fig 16 Closure of the anterior trapezoidal flap over theonly rib graft.

Fig 17 Occlusal film of a patient with bilateral cleft alve-olus before primary alveolar bone grafting. Note the ab-sence of bone in the alveolar clefts.


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no pneumothorax was noted. At present, only in se-lected cases is a chest radiograph performed. At 7 to 9months of age, most children are still bottle-feeding,and this feeding regimen is continued. Parents areencouraged to place the nipple of the bottle on theside opposite the cleft.

DISCUSSION

The major concerns raised about PABG have beenmidface growth attenuation, poor arch forms, and in-adequate alveolar bone formation at the cleft site.18

Early versions of the technique (from which subse-quent concerns arose) placed inlay bone grafts aftera more extensive dissection onto the palate, crossingthe vomerine-premaxillary suture (see Figs 1–3). Thecurrent technique for PABG abandons the extensivedissection of earlier techniques precisely because ofthis growth disturbance in favor of a limited dissec-tion for onlay grafting (see Figs 4 and 5). More recentstudies of patients after PABG do not show thegrowth disturbances previously cited16,19,20 and sug-gest that benefits accrue from this technique, includ-ing restoration of continuity to the maxilla (Figs 17and 18), with attendant improvement of maxillaryarch form, including stabilization of previously mo-bile bony segments. Primary alveolar bone graftingalso facilitates the development and favorable migra-tion of adjacent dental units into the cleft, enhancingdental function and esthetics, and decreases oronasalfistula rates.14,15,17

CONCLUSIONS

The technique for PABG is relatively simple and hasminimal morbidity. The procedure abandons the ex-

tensive dissection originally performed for PABG infavor of a minimal dissection along the labial surfaceof the alveolar cleft for placement of the rib graft.

REFERENCES

1. von Eiselsberg FW. Zur technic der uranoplastic. Arch KlinChirn 1901;64:509

2. Lexer E. Die verwendung de Freien knochenplastik nebst ver-suchen uber glenkversteinfung und gelenktransplantation lan-genbecks. Arch Klin Chir 1908;86:939

3. Drachter R. Die gaumenspalte und deten operative behand-lurg. Dtsch Zschr Chir 1914;131:2

4. Schruddle J, Stellmach R. Die primare osteoplastic der defektedes kieferbogens bei lippen-kiefer-gaumenspalten am sau-gling. Zbl Chir 1958;83:849

5. Johanson B, Ohlsson A. Bone grafting and dental orthopedicsin primary and secondary cases of cleft lip and palate. Acta ChirScand 1961;122:112–124

6. Pickrell K, Quinn G, Massengill R. Primary bone grafting of themaxilla in cleft of the lip and palate. A four year study. PlastReconstr Surg 1968;41:438

7. Rehrmann A, Koberg WR, Koch H. Long term postoperativeresults of primary and secondary bone grafting in completeclefts of lip and palate. Cleft Palate J 1970;7:206

8. Robertson NRE, Jolleys A. Effects of early bone grafting in com-plete clefts of the lip and palate. Plast Reconstr Surg 1968;42:414–420

9. Jolley A, Robertson NRE. A study of the effects of early bonegrafting in complete clefts of the lip and palate: five-year study.Br J Plast Surg 1972;25:229–237

10. Friede H, Johanson B. Adolescent facial morphology of earlybone-grafted cleft lip and palate patients. Scand J Plast Surg1982;16:41–53

11. Friede H, Johanson B. A follow-up study of cleft children trea-ted with primary bone grafting. Scand J Plast Reconstr Surg1974;8:88–104

12. Friede H. The vomeropremaxillary suture: a neglected growthsite in midfacial development of unilateral cleft lip and palatepatients. Cleft Palate J 1978;15:398–403

13. Rosenstein SW, Jacobson BN, Monroe CW, et al. A series ofcleft lip and palate children five years after undergoing ortho-pedic and bone grafting procedures. Angle Orthod 1972;42:1–8

14. Rosenstein S, Monroe CW, Kernahan DA, et al. The case forearly bone grafting in cleft lip and cleft palate patients. PlastReconstr Surg 1982;70:297

15. Rosenstein SW, Dado D, Kernahan DA, et al. The case for earlybone grafting in cleft lip and palate: a second report. Plast Re-constr Surg 1991;87:644

16. Sadove AM, Eppley BL. Timing of alveolar bone grafting: a sur-geon’s viewpoint. Plast Reconstr Surg 1992;2:39

17. Rosenstein S, Grasseschi M, Dado D. A long term retrospectiveoutcome assessment of facial growth, secondary surgical needand lateral incisor status in a surgical-orthodontic protocol forcomplete clefts. Plast Reconstr Surg 2003;1:111

18. Eppley BL, Sadove AM. Alveolar cleft management. In: VanderKolk CA (ed). Plastic Surgery, Indications, Operations and Out-comes. St. Louis: Mosby, 2000. p. 809–817

19. Hathaway RR, Eppley BL, Nelson CL, et al. Primary alveolarcleft bone grafting in unilateral cleft lip and palate: craniofacialform at age 8. J Craniofac Surg 1999;10:68–72

20. Hathaway RR, Eppley BL, Hennon DK, et al. Primary alveolarcleft bone grafting in unilateral cleft lip and palate: arch dimen-sions at age 8. J Craniofac Surg 1999;10:58–67

Fig 18 Occlusal film of the same patient after primary alveolarbone grafting. Note the bone bridges across the alveolar clefts.


711

Pigmented VillonodularSynovitis of theTemporomandibular Joint

Piero Cascone, PhD, Claudio Rinna, MD, Claudio Ungari, MD,Poladas Giulio, MD, Fabio Filiaci, MD

Rome, Italy

Abstract: Pigmented villonodular synovitis (PVNS) is a relativelyrare disease, affecting the synovial-lined joints. PVNS was firstfully described by Jaffe in 1941, who considered it to be a benigninflammatory state of the synovium of an uncertain etiology.Reports of this disease in the temporomandibular joint (TMJ) areextremely rare. The authors report a case of a 38-year-old womanaffected by pigmented villonodular synovitis of the TMJ. Clinicalexamination revealed the presence of a preauricular mass in the leftside; a computed tomography scan showed a 3.0 3 2.0 cm preau-ricular mass in close continuity to the TMJ capsule. The purpose ofthis report is to describe the clinicopathological features of a caseinvolving the TMJ. The previously reported cases in the literatureare also reviewed.

KeyWords: Pigmented villonodular synovitis, synovi-tis pigmented villonodular, temporomandibular jointdiseases

P igmented villonodular synovitis (PVNS) is a rela-tively rare disease, affecting the synovial-lined

joints, with an incidence of 1.8 cases per millionpopulation.1 PVNS is a benign condition of unknownetiology featuring synovial hypertrophy with hemo-siderin deposition, inflammation, and rarely, bonyerosions. PVNS usually affects the foot, ankle, finger,hip, elbow, wrist, and shoulder; temporomandibularjoint (TMJ) involvement is extremely rare.2 Chassai-gnac3 was the first to describe PVNS in 1852;Lapayowker et al,4 in 1973, described a case of PVNSof the TMJ. Young and Judacek5 reproduced PVNS-like lesions in a canine model by repeated hemarth-rosis. McCollin et al6 repeated the experiment byintraarticular injections of blood and saline solutionin a dog.

The current work describes a case report and acomprehensive review of the literature on pigmentedvillonodular synovitis of the TMJ (Table 1).

CASE REPORT

A 38-year-old woman presented at the Unit of Max-illofacial Surgery of the University ‘‘La Sapienza’’ inRome, experiencing TMJ pain. Clinical examinationrevealed the presence of a preauricular mass in theleft side and episodes of rheumatism at 5 years ofage, when she had undergone a tonsillectomy.

A computed tomography (CT) scan showeda 3.0 3 2.0 cm preauricular mass in close continuityto the TMJ capsule (Fig 1). Fine-needle aspirationand cytological examination disclosed nonspecificchronic inflammation of the TMJ capsule. Functionalsuperficial parotidectomy was performed, and a4.5 3 3.0 3 2.0 cm yellowish mass of the deeplobe was found, firmly adhering to the TMJ capsule(Figs 2 and 3). The articular disc showed signs ofdegeneration and was also removed.

Definitive histological examination disclosed anodular pattern of high-density multinucleated giantcells and ovoid cells, confirming the diagnosis ofPVNS. The postoperative course was uneventful.Radiological follow-up at 12 months showed norecurrence of PVNS-associated lesions (Fig 4).

DISCUSSION

PVNS is a benign proliferative disorder of the syno-vium. The term PVNS was introduced by Jaffe et al7

in 1941 to describe an idiopathic proliferative lesioninvolving tendon sheaths, bursae, and diarthrodialjoints. Such an entity belongs to a common lesion,such as pigmented villonodular synovitis, pigmentedvillonodular tenosynovitis, or pigmented villonodu-lar bursitis, depending on the area involved.

PVNS features a nodular and diffuse pattern.8

The most common nodular variants include giant celltumor, xanthoma, xanthogranuloma, and myeloplax-oma, generally affecting a discrete part of the syno-vium. The diffuse variant can affect the entire syno-vial membrane and be referred to as giant cellfibrohemangioma, chronic hemorrhagic villous syno-vitis, and benign polymorphocellular tumor of thesynovium.

Microscopic features of villonodular synovitis in-dicate a nonspecific inflammatory process,10 resultingin a papillary, villous, and nodular expansion of thesynovial membrane; the overlying cells of the syno-vium appear in a lobular and sheet-like arrangementof mononuclear, round, epithelioid cells and lipid-rich cells.

Cellular immunohistochemical features ofPVNS are CD 68, HAM 56, MAC 387, and PG-M1expression.11 Some authors take this as evidence of

From the Department of Maxillo-Facial Surgery, University ofRome La Sapienza, Rome, Italy Head Department. G. Ianetti, PhD.

Address correspondence and reprint requests to Dr. Fabio Filiaci,Via Romano Calo n. 26 CAP 00139, Rome, Italy; E-mail: [email protected]


712

Table 1. Literature on Pigmented Villonodular Synovitis of the Temporomandibular Joint

Case Year Authors Sex Age Clinical Localization Clinical Features Radiographic Features

1 1973 Lapayowker et al4

M 22 Preauricular mass 3 cm

in diameter

Mass was hard, relatively immobile,

nontender, pain on mastication

Erosion of the articular tubercle

2 1973 Lapayowker et al4

F 58 Preauricular swelling TMJ clicking, tinnitus, decreased

hearing

Destruction of the TMJ roof

3 1975 Barnard13

M 37 Preauricular swelling

1 cm in diameter

Tender swelling, pain, trismus Destruction of glenoid fossa

4 1977 Miyamoto et al14

M 34 Parotid mass 2.5 cm

in diameter

Deviation of mandible when

opening

Normal form of TMJ

5 1977 Raibley15

F 62 Parotid mass Mass was hard and firm Lytic lesion of condyle

6 1980 Geiger and Pesch16

F 50 Preauricular mass Diplopia, vomiting, trismus, pain Lytic lesion in the glenoid

fossa

7 1981 Takagi and Ishikawa17

M 36 Preauricular swelling

434 cm

TMJ clicking, pain, trismus /

8 1983 Curtin et al18

Gallia et al19

F 47 Preauricular swelling 335 Swelling was immobile and mildly

tender, deviation of

mandible when opening,

pain on mastication, trismus

Erosion of condyle with lateral

displacement of soft tissue

mass

9 1982 Rickert and Shapiro20

F 39 Parotid mass 2.533 / Defect in head of condyle

10 1984 O’Sullivan et al21

F 61 Preauricular mass 4 cm Mass, smooth, firm, partly fixed ?

11 1989 Dwaiskiba et al22

M 32 Mass lateral to TMJ Mass, pain, trismus Soft tissue tumor lateral and

posterior to TMJ

12 1992 Eisig et al23

F 50 Mass in the ear canal Hearing loss Destruction of glenoid fossa,

extension into the middle and

infratemporal fossa

13 1993 Syed et al24

F 10 Preauricular swelling

1.531.5

/ On ultrasonogram, hypoechoic

lesion within the parotid gland

14 1994 Franchi et al25

F 59 Swelling in parotid Swelling painful, firm area 332 /

15 1996 Shapiro et al26

M 36 / TMJ clicking, hearing loss Extension into the right

infratemporal fossa

16 1996 Youssef et al27

F 41 Swelling in TMJ Swelling, pain On CT preauricular lesion

continuous with TMJ

capsule, eroding of

the auditory canal

17 1997 Renega Rubin et al28

F 70 / Swelling 1 cm in diameter, firm,

hard, TMJ clicking, pain, trismus

On CT increased joint space

of TMJ

18 1997 Tanaka et al29

M 47 Preauricular swelling Deviation of mandible when

opening, pain on mastication,

trismus

Destruction of condyle

19 1997 Yu et al30

M 48 Preauricular mass 232 Mass fixed, solid, non-fluctuant /

20 1998 Chow et al31

F 42 Swelling in zigomatic

region 533 cm across

Soft tissue swelling Erosion into temporal bone

21 1998 Stojadinovic et al32

M 63 / Otalgia, trismus Intracranial extension

22 1999 Bemporad et al33

M 37 Preauricular mass Pain, hearing loss, trismus Destruction of skull base and

upper mandible

23 1999 Song et al34

F 57 Swelling in TMJ

4.533.533 cm

/ Erosion the plate of the condyle

and extension into the

medullary space

24 2000 Lee et al35

F 58 Preauricular mass 434 cm Decreased salivary flow Enlargement of the condylar

process

25 2001 Kisnisci et al36

F 45 Swelling in preauricular

region 432

Swelling was painful diffuse MRI: grossly move anterior of

the TMJ disc

M = male; F = female; not signaled TMJ = temporomandibular joint; MRI = magnetic resonance imaging; CT = computerized tomography.

BRIEF CLINICAL NOTES / Cascone et al

713

monocyte/macrophage differentiation, similar to theimmunophenotypic features of non-neoplastic syno-vium. Recently, Sciot et al12 found that rearrangementof 1p 11–13 is the most common chromosomic aber-ration of the localized and diffuse form of giant cell

tumor. The real cause is unknown; some authors havespeculated causes such as hemarthrosis, inflammatoryreaction, neoplasm, and repeated trauma.9

Twenty-five cases of PVNS of the TMJ are re-ported in the literature. A review of these casesreveals that PVNS can occur at any age, with a pre-vious incidence peak in the fourth and fifth decadesof life. No gender difference is seen. Clinical featuresof PVNS are preauricular swelling, parotid mass, orsoft mass in the TMJ area.

Fig 1 CT image of the patient showing the lesion in itsmedial and lateral aspects.

Fig 2 Follow-up magnetic resonance imaging scan 1 yearafter surgery; no recurrence was demonstrated.

Fig 4 After functional superficial parotidectomy was per-formed, a yellowish mass of the deep lobe was found.

Fig 3 Intraoperative photograph shows the neoformationcompression on the deep pole of the parotid and on the sev-enth pair of cranial nerves.


714

Diagnosis of PVNS may be delayed because ofthe nonspecificity of the clinical picture.13 From ourreview of the literature, only seven patients hadsymptoms related to the TMJ, including pain on mas-tication, deviation of the mandible during opening,decreased joint movement, clicking, joint swelling,tinnitus, heat, and tenderness. Otalgia, trismus,diplopia, vomiting, and hearing loss were seen inthree patients; in one case, a mass in the skull basewas present.14 In the remaining two cases,15,16 a massin the infratemporal fossa was present.

Diagnosis of PVNS should be considered in theradiological evaluation of patients with preauricularswelling and bone cysts of the TMJ. The radiologicalaspect of TMJ PVNS is usually normal in the earlystage; bony erosion may be seen in the articular fossa,particularly in the advanced stage4–17; CT scan andmagnetic resonance imaging may be useful in defin-ing localization and extent of the lesion.18 Arthros-copy appears to be useful for the diagnosis of PVNS.Fine-needle aspiration cytology is mandatory to con-firm the preoperative diagnosis.

Treatment of PVNS is exclusively surgical andconsists of complete excision of the involved boneand total synovectomy because synovial lesions havea high rate of local recurrence (.50%). Some authorshave proposed postoperative radiotherapy (35 Gy in15 fractions)19 in cases of high risk of recurrence. TMJrecurrence of PVNS has been reported in only two pa-tients by Takagi and Ishikawa20 5 years after surgicalexcision. O’Sullivan et al21 reported three cases ofrecurrence, treated by radiation therapy, in a 7-yearfollow-up period. The international literature reportsfacial paralysis as a common postoperative complica-tion in three cases22–24; in another patient, weaknessof the face was noticed.25

CONCLUSION

Despite the benign nature of the lesion, preopera-tive planning is mandatory for the successful treat-ment of PVNS caused by the possible extensionof the lesion into the middle and infratemporalfossa, the external and middle ear, near the carotidcanal, and because of possible postoperativecomplications.26–38

REFERENCES

1. Myers BW, Masi AT. Pigmented villonodular synovitis andtenosinovitis: a clinical epidemiologic study of 166 cases ofliterature review. Medicine 1980;59:223–38

2. Greenfield MM, Wallace KM. Pigmented villonodular synovi-tis. Radiology 1950;54:350

3. Chassaignac EPM. Cancer de la gaine des tendons. Gazzette desHopitaux Civils et Militaire 1852;25:185–186

4. Lapayowker MS, Miller WT, Levy WM, Harwich RD. Pig-mented villonodular synovitis of the temporomandibular joint.Radiology 1973;108:313–6

5. Young JM, Judacek AG. Experimental production of Pigmentedvillonodular synovitis in dog. Am J Pathol 1954;30:799–812

6. McCollin DE, Musser AW, Rhangos WC. Experimental villo-nodular synovitis, South. Med J 1966;59:966–70

7. Jaffe HL, Lichtenstein L, Sutro CJ. Pigmented villonodularsynovitis, bursitis and tenosynoviti. Arch Pathol 1941;31:731–65

8. Docken WP. Pigmented villonodular synovitis a review withillustrative case report. Semin Arthritis Rheum 1979;9:1–22

9. Alguacil-Garcia A, Unni KK, Goellner JR. Giant cell tumor oftendon sheath and pigmented villonodular synovitis: an ultra-structural study. Am J Clin Pathol 1978;69:6

10. Cavaliere A, Sidoni A, Bucciarelli E. Giant cell tumor of tendonsheath: immunohistochemical study of 20 cases Tumori 1997;83:841

11. Sciot R, Rosai J, Dal Cin P, et al. Analysis of 35 cases of localizedand diffuse tenosynovial giant cell tumor: a report from theChromosomes and Morphology (CHAMP) Study Group.Mod Pathol 1999;12:576

12. Van Meter CD, Rowdon GA. Localized pigmented villonodularsynovitis presenting as a locked lateral meniscal bucket handletear: a case report and review of the literature. Arthroscopy1994;10:309–12

13. Barnard JDW. Pigmented villonodular synovitis in the tempo-romandibular joint: a case report. Br J Oral Surg 1975;13:183–7

14. Miyamoto Y, Tani T, Hamaya K. Pigmented villonodular syno-vitis of the temporomandibular joint: a case report. Plast Re-constr Surg 1977;59:283–6

15. Raibley SO. Villonodular synovitis with chondromatosis. OralSurg Oral Med Oral Pathol 1977;44:279–84

16. Geiger S, Pesch HJ. Synovitis pigmentosa villonodularis. eineseltene Kiefergelenkerkrankung Fortschr Kiefer Gesichtschir1980;25:29–31

17. Takagi M, Ishikawa G. Simultaneous villonodular synovitisand synovial chondromatosis of the temporomandibular joint:report of a case. J Oral Surg 1981;39:699–701

18. Curtin HD, Williams R, Galla L, Meyers EN. Pigmented villo-nodular synovitis of the temporomandibular joint. ComputRadiol 1983;7:257–60

19. Gallia LJ, Jhonson JT, Myers EN. Pigmented villonodular syno-vitis of the temporomandibular joint: a case report. OtolaryngolHead Neck Surg 1982;90:691–5

20. Rickert RR, Shapiro MJ. Pigmented villonodular synovitis ofthe temporomandibular joint: Otolaryngol Head Neck Surg1982;90:668–70

21. O’Sullivan TJ, Alport EC, Whiston HG. Pigmented villonodularsynovitis of the temporomandibular joint. J Otolaryngol 1984;13:123–6

22. Dwaiskiba S, Eriksson L, Elner A, Johansen CC, Hansson LG,Westesson PL. Diffuse pigmented villonodular synovitis of thetemporomandibular joint diagnosed by fine needle aspirationcytology. Diagn Cytol 1989;5:301–4

23. Eisig S, Dorfman HD, Cusamano RJ, Kantrowitz AB. Pig-mented villonodular synovitis of the temporomandibular joint:case report and review of the literature. Oral Surg Oral MedOral Pathol 1992;73:328–33

24. Syed A, Van Hasselt CA, To KF. Pigmented villonodular synovi-tis of the temporomandibular joint. J Laryngol Otol 1993;16:47–50

25. Franchi A, Frosini P, Santoro R. Pigmented villonodular syno-vitis of the temporomandibular joint: report of a case. J LaryngolOtol 1994;108:166–7

26. Shapiro S, Kessler S, McMenomey SO. Pathologic quizcase 1. Temporomandibular pigmented villonodular synovi-tis. Arch Otolaryngol Head Neck Surgery 1996;122:194–96

BRIEF CLINICAL NOTES / Cascone et al

715

27. Youssef RE, Roszkowski MJ, Richter KJ. Pigmented villonodu-lar synovitis of the temporomandibular joint. J Oral MaxillofacSurg 1996;54:224–7

28. Renaga Rubin I, Salaver Girona A, Vasquez Rodriguez A,Anmella Valmanya J. Pigmented villonodular synovitis of thetemporomandibular joint. Oral Surg Oral Med Oral PatholOral Radiol Endod 1997;84:459–60

29. Tanaka K, Suzuki M, Nameki H, Sugiyama H. Pigmentedvillonodular synovitis of the temporomandibular joint. ArchOtolarygol Head Neck Surg 1997;123:536–9

30. Yu GH, Staerkel GA, Kershisnik MM, Varma DG. Fine-needleaspiration of pigmented villonodular synovitis of the temporo-mandibular joint masquerading as a primary parotid glandlesion. Diagn Cytopathol 1997;16:47–50

31. Chow LT, Kumta SM, King WW. Extra-articular pigmented vil-lonodular synovitis of the temporomandibular joint. J LaryngolOtol 1998;112:182–5

32. Stojadinovic S, Reinert S, Wildforster U, Jundt G, Machtens E.Pigmentierte villonodulare Synovitis des Kiefergelenks mitEinbruch in die mittlere Schadelgrube. Mund Kiefer Gesicht-schu 1998;2:279–81

33. Bemporad JA, Chaloupka JC, Putman CM, et al. Pigmentedvillonodular synovitis of the temporomandibular joint: diag-nostic imaging and endovascular therapeutic embolization ofa rare head and neck tumor. AJNR Am J NeUroradiol 1999;20:159–62

34. Song MY, Heo MS, Choi SC, Park TW, Lim CY, Lim JJ. Diag-nostic imaging of pigmented villonodular synovitis of the tem-poromandibular joint associated with condylar expansion.Dentomaxillofacial Radiology 1999;28:386–90

35. Lee JH, Kim YY, Seo BM, Baeck SH, Choi JY, Choung PH,Kim MJ. Extra-articular pigmented villonodular synovitisof the temporomandibular joint: case report and reviewof the literature. Int J Oral Maxillofac Surg 2000;29:408–15

36. Kisnisci RS, Tuz HH, Gunhan O, Onder O. Villonodular syno-vitis of the temporomandibular joint: case report. J Oral Max-illofac Surg 2001;59:1482–4

37. Dorwart RH, Genant HK, Johnson WH, Morris JM. Pigmentedvillonodular synovitis of synovial joints: clinical, phatologic,and radiologic features. A J R 1984;143:877–85

38. O’Sullivan B, Catton C, Bell R, Davis A, Fornasier V, GoldbergerR. Outcome following radiation treatment for high-risk pig-mented villonodular synovitis. Int J Radiat Oncol Biol Phys1995;32:777–86

Variations of the Gonial Angle inVertical Surgical Reduction of theMaxillary-Mandibular Complex

Rinna Claudio, MD, Ungari Claudio, MD, Agrillo Alessandro, MDTedaldi Massimiliano, MD, Moles Angelo, MD

Rome, Italy

Abstract: From an esthetic point of view, the mandibular or gonialangle plays an important role in ensuring a harmonious facial profile.The purpose of this study was to analyze the values of the mandib-ular angle in relation to variations of the vertical and sagittal posi-

tions of the jaw. The study was carried out on a sample of 42 patientswho underwent surgery for maxillary-mandibular repositioning, ad-vancing or raising the jaw, after a Le Fort I osteotomy and bilateralsagittal osteotomy of the jaw according to the technique described byObwegeser-Dal Pont. In all patients, the maxillary bone was raisedwith a discrepancy of at least 3 mm between the anterior and pos-terior portions (favoring the latter) as a result of (clockwise) rotationof the bispinal plane. The data, obtained by means of cephalometricanalysis according to the method of Ricketts, showed how changes inthe gonial angle differed between patients who require correction ofan open-bite and those who do not. In particular, in patients withoutan open-bite, advancement of the maxilla limited the opening of theangle. Similarly, in patients with an open-bite, the tendency to forman open gonial angle can usually be corrected by the association ofadvancement of the maxilla whenever possible.

Key Words: Goniac angle, mandibular angle, mandib-ular repositioning

T he aims of surgical treatment of maxillary-mandibular malformations are functional and

esthetic facial correction. A correct diagnosis ofthe malformation and surgical planning representfundamental steps in the achievement of thesegoals.

In the last few years, esthetic studies have be-come more and more important in the planning ofpatients undergoing orthognathic surgery.1,2 Thesestudies have focused on the mandibular or gonialangle, which is important in terms of estheticresults because it ensures a harmonious profile. Ac-cording to anthropometric values, the mandibularangle must be between the mean values of 123.5�(with a 5.9� standard deviation in male patients)and 122.2� (with a 4.2� standard deviation in femalepatients).

An increased gonial angle is often caused by the(clockwise) rotation of the mandibular body with atendency to class III. Alternately, it may be associatedwith class II with a short mandibular ramus.3

Modification of gonial angle values results ina variation of posterior and vertical facial diameters.Furthermore, the variations of the gonial angle can beassociated with physiological alterations caused bydifferent factors such as age, gender, and the degreeof tooth retention.4

The purpose of our research was to study man-dibular angle changes subsequent to surgical man-dibular-maxillary repositioning after a Le Fort Iosteotomy and sagittal osteotomy of the jaw accord-ing to the technique described by Obgeweser-Dal Pont. In particular, the values of the mandibularangle in relation to the variations of the sagittaland vertical positions of the maxillary bone wereanalyzed.

From the Department of Maxillofacial Surgery, Faculty of Med-icine, University ‘‘La Sapienza,’’ Rome, Italy.

Address correspondence to Dr Angelo, via Catania, 21, 00161,Rome, Italy; E-mail: [email protected]


716


Among all the orthognathic operations carried out atthe Department of Maxilla-Facial Surgery at the Po-liclinic Umber to I in Rome from 1977 to date, 540patients were considered for inclusion in this study.

Among these patients, those in whom the max-illary bone had been raised with a discrepancy ofat least 3 mm between the anterior and posteriorportions (in favor of the latter) as a result of (clock-wise) rotation of the basinal plane (ANS-PNS) wereselected for the study. In addition to the type of max-illa displacement, the following requirements weretaken into consideration:

• Patients were no longer in the growing phase• No previous traumas or operations involving the

maxillary-mandibular bones• No patients with congenital malformations (e.g.,

patients with LPS)• No surgical segmentary procedures• No previous operations on the facial skeleton• No bone remodeling procedures (e.g., remodeling

of the ramus or mandibular angle)• Use of screw fixation• Use of plates for repositioning of the mandibular

ramus• At least 6 months of follow-up• Identification of the anthropometric values by means

of cephalometric analysis according to the methodof Ricketts5 and use of the cephalometric values forteleradiographs

The sample consisted of 42 patients (28 femaleand 14 male), whose ages ranged from 17 to 44 years,with mean age of 25 years.

The sample was divided into two groups:

1. Patients with an anterior open-bite, with overbitevalues between 1.5 mm and 10 mm (20 patients)

2. Patients without an anterior open-bite, with over-bite values between 0 mm and 4 mm (22 patients)

Each group was made up of two subgroups:

• Patients with an anterior open-bite with clockwiserotation of the basinal plane and with at least a3-mm elevation discrepancy between the anteriorand posterior maxillae (9 patients; subgroup 1a)

• Patients with an anterior open-bite who presentedwith clockwise rotation of the basinal plane and alsounderwent advancement of the maxilla on the sagit-tal plane, with values between 2 mm and 5 mm, withan average of 2.76 mm (13 patients; subgroup 1b)

• Patients without an anterior open-bite who pre-sented with clockwise rotation of the basinal planeand with at least a 3-mm elevation discrepancy be-tween the anterior and posterior maxillae (11 pa-tients; subgroup 2a)

• Patients without an anterior open-bite who presentedwith clockwise rotation of the basinal plane and

Table 1. Group of Patients With Open-Bite

Patients Open-Bite

Persurgical

Gonial Angle

Postsurgical

Gonial Angle Displacement

CP �6 mm 137� 133� [2 mm ant and

5 mm post

MA �4 mm 126� 125� [2 mm ant and

5 mm post

DA �6 mm 135� 137� [2 mm ant and

6 mm post

TF �5 mm 126.5� 125� [0 mm ant and

6 mm post/3 mm

VD �4 mm 118� 117.5� [2 mm ant and

6 mm post/4 mm

SD �4 mm 121� 118.5� [2 mm ant and

5 mm post

CM �7 mm 134.5� 136� [0 mm ant and

4 mm post/2 mm

RF �10 mm 137� 138� [4 mm ant and

9 mm post

VM �3.5 mm 133.5� 132.5� [0 mm ant and

3 mm post/ 5 mm

CD �5 mm 138.5� 132� [0 mm ant and

3 mm post /2 mm

SG �3 mm 123� 115� [2 mm ant and

5 mm post /3 mm

PMC �5 mm 118� 120� [ant 2 mm and

post 5 mm

IM �1.5 mm 125� 125.5� [3 mm ant and

6 mm post

MS �2 mm 137� 136� [2 mm ant and

5 mm post/2 mm

SS �3 mm 136� 136� [1 mm ant and

4 mm post

MR �3 mm 134.5� 132� [2 mm ant and

5 mm post/3 mm

MD �8 mm 133.5� 132� [1 mm ant and

4 mm post /2 mm

DRL �6 mm 139� 132� [3 mm ant and

7 mm post /2 mm

GL �3 mm 120� 121.5� [0 mm ant and

3 mm post /4 mm

GM �1 mm 124.5� 121.5� [2 mm ant and

5 mm post /2 mm

VT �5 mm 120.5� 113� [3 mm ant and

6 mm post /2 mm

TA �4 mm 142� 141.5� [0 mm ant and

3 mm post

ant = anterior; post = posterior.

BRIEF CLINICAL NOTES / Claudio et al

717

also underwent advancement of the maxilla onthe sagittal plane with values between 2 mmand 4 mm, with an average of 2.88 mm (9 patients;subgroup 2b)

The following parameters were evaluated beforeand after surgery:

1. Distance between the occlusal margin of the infe-rior incisor and the margin of the superior incisor,which is measured on the basis of their projection

on the perpendicular-to-Frankfurt plane, describ-ing the distance between superior and inferior in-cisors on the vertical plane (VM ± 2 mm, witha standard deviation ± 1.8 mm)

2. Angle (AR-MP/MI-ME) formed by the segmentscrossing through the point of intersection of theinferior spheno-occipital profile with the posteriormandibular border at the condyle level (AR) andthe posterior point of the gonial angle (MP), withthe segment crossing through the inferior pointof the gonial angle (MI) and the lower point ofthe prominence of the chin (MI)

The study of these parameters allowed us to as-sess the gonial angle changes in relation to those ofthe occlusal plane.

RESULTS

The cephalometric studies carried out on the postop-erative control radiographs revealed normalizationof the overbite (+2 mm) with a standard deviationof ±0.7 mm in all patients included in the sample.As regards the mandibular angle values, results weredifferent in the two groups.

In group 1a, we observed a mean increase of theangle values of 0.33� in all patients in whom the de-gree of maxilla rotation was equivalent to the degreeof the open-bite. In all patients who had also under-gone superior advancement of the maxilla (group 2b),the mandibular angle value decreased by a meanof 2.77� at the 6-month follow-up.

In group 2a, we observed a mean increase of themandibular angle of 2.81�. In group 2b, we observedan increase of the gonial angle of 0.27� (Tables 1 and 2).

CONCLUSIONS

The advances made in planning methods havebrought about an increasing predictability of resultsin terms of functional and esthetic correction. Thishas also been made possible by the use of T-shapedplates for repositioning of the mandibular ramuson the sagittal and transversal planes in exactly thesame preoperative spatial position. This avoidsgonial angle alterations caused by incorrect position-ing of the ramus. In fact, the mandibular angle playsa significant role in ensuring a harmonious profile,favoring the achievement of facial eurhythmy.

This study demonstrated how the behavior ofthe gonial angle differs between patients who requirecorrection of an open-bite and patients who do not.In particular, when the degree of rotation of themaxilla is equal to that of the open-bite, there are

Table 2. Group of Patients Without Open-Bite

Patients Over bite

Presurgical

Gonial Angle

Postsurgical

Gonial Angle Displacement

BS 1 mm 127� 128� [3 mm ant and

6 mm post /3 mm

BM 0 mm 124.5� 128� [2 mm ant and

5 mm post

SA 2 mm 123� 114� [0 mm ant and

4 mm post /4 mm

PD 1 mm 138� 141� [0 mm ant and

4 mm post /2 mm

UN 1 mm 125� 127� [3 mm ant and

6 mm post

CF 0 mm 128� 137� [0 mm ant and

4 mm post

VS 1 mm 134� 132� [0 mm ant and

5 mm post

SS 3 mm 130� 132.5� [1 mm ant and

5 mm post /3 mm

DR 2 mm 134� 137� [2 mm ant and

5 mm post /3 mm

PM 4 mm 131� 136� [3 mm ant and

6 mm post

VG 4 mm 122� 128� [3 mm ant and

6 mm post

ZM 4 mm 112� 117.5� [3 mm ant and

6 mm post

LV 3.5 mm 135� 136� [2 mm ant and

5 mm post

CF 0 mm 124� 125� [2 mm ant and

5 mm post /3 mm

AA 1 mm 129.5� 132� [1 ant and

4 mm post

TI 1 mm 137.5� 131� [1 ant and

4 mm post /4 mm

RE 0 mm 130� 133.5� [2 mm ant and

5 mm post /3 mm

PA 1 mm 120� 121� [1 mm ant and

4 mm post /2 mm

MA 2 mm 125� 128� [0 mm ant and

3 mm post /4 mm

GD 1 mm 129� 132� [1 mm ant and

4 mm post

ant = anterior; post = posterior.


718

not considerable changes in the mandibular angle.When rotation of the maxilla is associated with an-terior displacement, however, the mandibular angle isreduced.

In patients without an open-bite, modificationsof the occlusal plane produce an increase of the anglethat is inversely proportional to the degree of maxil-lary bone advancement.

These results demonstrate that elevation of themaxilla alone increases the mandibular angle in pa-tients without an open-bite. The association of max-illa advancement suffices to limit opening of theangle. Similarly, in patients with an open-bite, the ten-dency to form an open gonial angle can be correctedby the association of maxilla advancement wheneverpossible.

REFERENCES

1. Arnott GW, Bergman RT. Facial keys to orthodontic diagnosisand treatment planning. Part I. Am J Ortho Dentofacial Ortho1993;103:4

2. Arnott GW, Bergman RT. Facial keys to orthodontic diagnosisand treatment planning. Part II. Am J Ortho Dentofacial Ortho1993;103:5

3. Castone P, De Ponte F, Schaer M. Analis cephalometric in chir-urgiae maxillofacial. Monod Ortodontico 1987;12:97–106

4. Ohm E, Silness J. Size of the mandibular jaw angle related to age,tooth retention and gender. J Oral Rehabil 1999;26;883–891

5. Ricketts RM. Foundation for cephalometric communication.Am J Orthod 1960;46:330

Use of Adhesives in CleftPalate Surgery:A New FlapFixation Technique

Tayfun Turkaslan, MD, Hakan Ozcan, MD,Deniz Dayicioglu, MD, Zafer Ozsoy, MD

Istanbul, Turkey

Abstract: N-Butyl-2-cyanoacrylate (NB2C) is a synthetic tissueadhesive, and it has been used in many surgical procedures. Thisstudy was aimed at evaluating its efficacy in cleft palate repairs.Fifteen patients with soft palate and hard palate clefts had cleftsrepaired with the use of this synthetic adhesive. The ages at repairranged from 11 months to 17 years, with an average of 2.4 years.Follow-up time ranged from 14 months to 24 months. No compli-

cations were found. The advantages of using NB2C were shorteroperative time, more pushback gained, better hemostasis, earlyand well-tolerated feeding, tension-free closure, and better patientcomfort. This study shows NB2C as a useful adjunctive material foraffixing the mucoperiosteal flaps to the hard palate.

KeyWords:Cleft palate, cyanoacrylate, tissue adhesive

S ince their adhesive properties were realized in1959, there has been interest in the use

of cyanoacrylates for surgical procedures,1 includingtraumatic laceration repair,2,3 bronchopleural fistularepair,4 pulmonary resection,5 repair of myocardialtears,6 mesh fixation for inguinal hernia repair,7 cos-metic rhinoplasty,8,9 and embolization of intracranialarteriovenous malformations.8

Cyanoacrylate adhesives are available in short-chain (methyl and ethyl cyanoacrylates) and longer-chain (butyl, isobutyl and octyl cyanoacrylates)derivatives. The longer-chain derivatives, such asN-butyl-2-cyanoacrylate (NB2C), are least histotoxic.8

Toriumi et al10 were the first to state that cyano-acrylate could be used for hard palate repairs;however, there have not been any studies. Someauthors have reported the clinical use of cyanoacry-lates with several advantages, including ease of usewith shorter operative time, formation of its protec-tive barrier, no suture removal anxiety, and excellentcosmetic outcome. Moreover, some additional advan-tages of using cyanoacrylates in cleft palate surgerythat the authors have found are tension-free closure,more pushback, early and well-tolerated feeding, andrapid recovery.

METHODS

Fifteen patients were the subject of this study.Informed consent was obtained by explaining thatthis was a new technique. The cases with wide clefts(larger than 10 mm) were included in the study.Complicated alveolar clefts, such as collapsed orwide alveolar arch requiring preoperative orthodon-tics, were excluded from the study. The features ofcases are explained in the Table. For the cleft palaterepair, two-flap palatoplasty, V-Y pushback palato-plasty, and ‘‘Dorrance techniques’’11 were used.The palatal clefts were repaired as a one-stage proce-dure by the same surgeon. Two-layer closure nasaland oral sides of the hard palate cleft and three-layerclosure nasal, muscle, and oral mucosa of the softpalate were the basis for the surgical procedure. Incases with bilateral hard palate cleft, vomer flapswere used to reconstruct the nasal side of the

From the Department of Plastic and Reconstructive Surgery,SSK Vakif Gureba Research Hospital, Istanbul, Turkey.

Address correspondence and reprint requests to Dr. TayfunTurkaslan, Fahrettin Kerim Gokay cd Dagdelen, Apt. 240/39Goztepe/Kadikoy, Istanbul, Turkey; E-mail: [email protected]

BRIEF CLINICAL NOTES / Turkaslan et al

719

nasopharyngeal floor. To create palatal muscle sling,levator muscle was dissected from the hard palateposterior edge and nasal side periosteum. The nasalmucosal layer of the cleft palate was closed with in-verted 4/0 Vicryl sutures, beginning from the uvulato the alveolar ridges. Dissected and widely freedmuscle was redirected downward and mediallyand then sutured with 4/0 Vicryl. The oral mucosallayer was then closed, starting at the uvula and pro-ceeding anteriorly. Vertical mattress sutures wereplaced through both the oral mucosa and musclelayer on either side. In the region of the hard palate,the sutures went through only the oral side. Nosuture was passed from the nasal mucosal layer forfixing the midline-sutured flap to the hard palate.After that, the NB2C (Loctite, Dublin, Ireland; TycoHealthcare, Mansfield, Mass), which is presentedas a topical solution of low viscosity in a polyethylenebottle and is gamma-radiation sterile, was spread onthe posterior surface of the mucoperiosteal flap asa flimsy layer with a wooden applicator (Figs 1 and2) Gentle finger pressure for 5 or 6 minutes towardthe hard palate was applied to affix the flap. Afterthe palatal cleft was closed, no suture was used toclose the gap or for margin approximation. No ma-terials that control the bleeding or close the bonysurfaces were used, such as gel foam or Vaselinegauze at the final stage of operation. Arm restraintswere routinely fastened in the operating room. Earlyclear-liquid feeding was given orally as tolerated for48 hours. After that time, the children ate a soft-fooddiet for 3 weeks. When oral intake was sufficient,fluids were discontinued. First-generation cephalo-sporins were used for prophylactic purposes before

the operations, and no antibiotics were given duringthe postoperative period. When possible, patientswere seen 2 weeks after the procedure and followedup at 3- to 6-month intervals for as long as 24 months.

RESULTS

This study consisted of 15 cases treated between 2001and 2004. Cleft type and repair technique were ex-plained, as shown in the Table. Ages at repair rangedfrom 11 months to 17 years, with an average age of 2.4years. No patient with ,14 months of follow-up wasincluded. In this series, no complications such as de-hiscence, hemorrhage, infection, or airway problemsand late oronasal fistula formation were seen (Fig 3).No allergic reactions or adverse effects of NB2C wereencountered. No revision or secondary interventionswere needed.

DISCUSSION

Cyanoacrylate adhesives were first described in 1949,and their first reported use as a clinical adhesive was10 years later.12 They polymerize in an exothermic re-action on contact with a fluid or basic medium, form-ing a strong bond. Shorter-chain cyanoacrylates werefound to cause inflammatory reactions, and the lon-ger-chain NB2C was believed to be a better alterna-tive as a tissue adhesive.13,14 There have been numer-ous clinical reports of its successful use for topicalskin closure, and it has been approved for this indi-cation in Canada since 1980, with no adverse effectsreported. There have been no reports of toxic effectsor carcinogenicity.2,15–20

Table 1. n

Case No. Age Cleft Type Procedure Follow-up Complication

1 12 mo Incomplete V-Y pushback 6 mo Ø


3 24 mo Complete V-Y pushback 8 mo Ø

4 28 mo Complete Dorrance repair 14 mo Ø



7 12 mo Incomplete Dorrance repair 8 mo Ø


9 17 y Complete Dorrance repair 7 mo Ø


11 30 mo Unilateral prepalatal Two-flap palatoplasty 6 mo Ø


13 18 mo Bilateral prepalatal Two-Flap palatoplasty 13 mo Ø

14 13 mo Incomplete Dorrance repair 8 mo Ø

15 11 mo Complete V-Y pushback 7 mo Ø


720

Toriumi et al8 showed in a rabbit model that bonegrafts affixed to cartilage with butyl-2-cyanoacrylatewill develop minimal to no acute inflammation ifthe adhesive is placed at the interface of bone andcartilage or in areas of poor vascularity, for instance,subcutaneous pockets and the avascular planedevoid of vessels and lymphatics. Considering thisexperimental research, we also did not encounterclinical findings of excessive inflammatory reactionsduring the postoperative period.

From the surgical point of view, the most impor-tant rules are to gain more pushback and to achievetension-free closure of the palate, which are the aimsof all repair techniques. High tension and excesssuture material can scar the palate, which leads tomaxillary growth retardation, orthognathic prob-lems, and fistula formation.21,22

It has been asserted that in most cases, gapclosure or margin approximation is possible to facili-tate healing in those sites22; however, in many instances,direct tension-free closure is not possible, secondaryto the necessary medial mobilization of oral flaps andfixation of flaps to palate margins or nasal mucosa forcleft palate repair, especially in wider clefts. Only su-ture fixation causes tense flaps that lead to inadequate

pushback position in the wide cleft palate. Usingtissue adhesive such as NB2C to attach the oral flapsto the hard palate enables tension-free closure andprovides tensionless flaps, permitting an easy push-back procedure. With this technique, mucoperiostealflaps are totally and three-dimensionally adhered tohard palate without leaving dead space (Figs 1 and 2)so that secondary flap and scar contracture are min-imized, resulting from tense closure and dead space.Adhesion with the glue in the pushback position isthe major advantage of the technique. Thus, an im-portant problem can be solved in cleft palate surgeryby using NB2C with tension-free closure and lesssuture application.

Cyanoacrylates have been shown to speedwound healing in partial-thickness wounds in ananimal model.23 This may explain the absence ofearly wound dehiscence and late fistula formationin our cases. Cyanoacrylate adhesives also have anti-microbial properties against gram-positive organisms

Fig 1 Lateral cross-cut view of schematic drawing shows(upper) application of N-butyl-2-cyanoacrylates (NB2C)and (lower) fixation of the flap to the pushback position.

Fig 2 Frontal cross-cut view of schematic drawing shows(left) normal cleft anatomy, (upper right) repaired cleft andtense flaps, and (lower right) fastened and settled flaps.

Fig 3 (A) Preoperative view of thepatient with a wide complete pala-tal cleft. (b) Dorrance planning.(C) Early postoperative view. (D)Postoperative view 1 y later.

BRIEF CLINICAL NOTES / Turkaslan et al

721

and may decrease wound infections.18,19 This effectmay have a possible reason. Cyanoacrylates havean antibacterial effect, possibly caused by strong elec-tronegative charge of the polymer.24 This is very im-portant for the oral cavity, where it may provide bet-ter oral hygiene and prevent early wound dehiscenceand late fistula formation secondary to oral flora infec-tions. As a waterproof material, cyanoacrylates may bebeneficial in the repair of cleft palate surgery becausethey do not permit passage of particles between theoral and nasal sides of the flap. Oral feeding and liquidintake are important in surgically treated neonates andinfants, who should begin receiving food and liquidsin the early postoperative period. Such immediateintake makes the recovery period short so that suchpatients can be rapidly discharged.

There is no doubt that other adhesives, such asfibrin glue, can be used for palatal surgery in thismanner; however, most of them are produced fromdonor-pooled plasma and possess the potential riskof blood-borne infections.25 Alternatively, the useof long-chain cyanoacrylate is safer, faster, and lessexpensive than the use of fibrin glue-based adhesives.

In the present study, we did not need a controlgroup to compare results because our aim was tointroduce our novel method as a surgical technique.The next step, in the near future, will be to increasethe number of cases and to evaluate soft palate func-tion, comparing cyanoacrylates with a control group.

Synthetic tissue adhesive can be used to glueflaps or skin grafts into a site where suturing wouldbe difficult, such as the hard palate, orbital defect,and intranasal area in craniofacial surgery. Addi-tional investigation must be done to elucidate whichtypes of tissue adhesive can be used without inflam-mation and histotoxicity. Because of many practicaladvantages, the authors believe that the adhesivematerial can replace many surgical suturing pro-cesses in the near future.

CONCLUSION

Longer-chain cyanoacrylates provide a better andtension-free method of palatal closure than doesthe use of sutures. This synthetic tissue adhesive isalso completely removed from the tissues by enzy-matic action. These properties make it ideally suitedto the closure of cleft palate defects.

REFERENCES

1. Coover HN, Joyner FB, Sheerer NH, et al. Chemistry and per-formance of cyanoacrylate adhesive. Special Technical Papers1959;5:413–417

2. Quinn JV, Drzewiecki A, Li MM, et al. A randomized, controlledtrial comparing tissue adhesive with suturing in the repair ofpediatric facial lacerations. Ann Emerg Med 1993;22:1130–1135

3. Applebaum JS, Zalut T, Applebaum D. The use of tissue adhe-sion for traumatic laceration repair in the emergency depart-ment. Ann Emerg Med 1993;22:1190–1192

4. Scappattici E, Ardissone F, Ruffini E, et al. Postoperative bron-chopleural fistula: endoscopic closure in 12 patients. Ann ThoracSurg 1994;57:119–122

5. Sabanathan S, Eng J, Richardson J. The use of tissue adhesive inpulmonary resections. Eur J Cardiothorac Surg 1993;7:657–660

6. Padro JM, Mesa JM, Silvestre J, et al. Subacute cardiac rupture:repair with a sutureless technique. Ann Thorac Surg 1993;55:20–24

7. Farouk R, Drew PJ, Qureshi A, et al. Preliminary experiencewith buthyl-2-cyanoacrylate adhesive in tension free hernia re-pair. Br J Surg 1996;83:1100

8. Toriumi DM, Raslan WF, Friedman M, et al. Histotoxicity ofcyanoacrylate tissue adhesives. A comparative study. ArchOtolaryngol Head Neck Surg 1990;116:546–550

9. Sachs ME. Enbucrilate as cartilage adhesive in augmentationrhinoplasty. Arch Otolaryngol 1985;111:389–393

10. Toriumi DM, Raslan WF, Friedman M, et al. Variable histotox-icity of histoacryl when used in a subcutaneous site: an exper-imental study. Laryngoscope 1991;101:339–343

11. McDowell F. The classic reprint: lengthening the soft palatein cleft palate operations by George M. Dorrance, M.D. PlastReconstr Surg 1972;50:275–279

12. Storrow AB, Stack LB, Peterson P. An approach to emergencydepartment photography. Acad Emerg Med 1994;1:454–462

13. Quin JV, Drzewiecki AE, Stiell IG, et al. Appearance scales tomeasure the cosmetic outcomes of healed lacerations. Am JEmerg Med 1995;13:229–231

14. Ronis ML, Harwick JD, Fung R, et al. Review of cyanoacrylateglues with emphasis on their otorhinolaryngological applica-tions. Laryngoscope 1984;94:210–213

15. Maw JL, Quinn V, Wells GA, et al. A prospective comparison ofoctylcyanoacrylate tissue adhesive and suture for the closure ofhead and neck incision. J Otolaryngol 1997;26:26–30

16. Tse DT, Panje WR, Anderson RL, et al. Cyanoacrylate adhesiveused to stop CSF leaks during orbital surgery. Arch Ophthalmol1984;102:1337–1339

17. Kung H. Evaluation of the undesirable side-effects of the sur-gical use of histoacryl glue with special regard to possible car-cinogenicity. RCC Institute for Contract Research in Toxicologyand Ecology Project 064315, March 1986

18. Quin JV, Osmond MH, Yurach J, et al. Histoacryl: risk of con-tamination with an appraisal of its antibacterial effects. J EmergMed 1995;13:581–585

19. Quin JV, Ramotar K, Osmond MH. The antimicrobial effects ofa new tissue adhesive. Acad Emerg Med 1996;3:536–537

20. Samuel PR, Roberts AC, Phil M, et al. The use of Indermil(n-butyl cyanoacrylate) in otorhinolaryngology and head andneck surgery. A preliminary report on the first 33 patients.J Laryngol Otology 1997;111:536–540

21. Randal P, LaRossa D. Cleft palate. In: McCarthy J, ed. PlasticSurgery. Philadelphia: WB Saunders Company; 1990:2723–2752

22. Afifi GY, Kaidi AA, Hardesty RA. Cleft palate repair. In: EvansGR, ed. Operative Plastic Surgery. New York: McGraw-Hill Pro-fessional; 2000:479–503

23. Davis SC, Eaglestein WH, Cazzaniga AL, et al. An octyl-2-cyanoacrylate formulation speeds healing of partial-thicknesswounds. Dermatol Surg 2001;27:783–788

24. Quin J, Maw J, Ramotar K, et al. Octylcyanoacrylate tissue ad-hesive versus suture wound repair in a contaminated woundmodel. Surgery 1997;122:69–72

25. Quatela VC, Futran ND, Frisina RD. Effects of cyanoacrylatetissue adhesives on cartilage graft viability. Laryngoscope1993;103:798–803


722

Bilateral Hyperplasia of theCoronoid Processes: ClinicalReport

Riccardo Tieghi, MD, Manlio Galie, MD, DMD,Luigi Piersanti, MD, Luigi Clauser, MD, DMD, PhD

Ferrara, Italy

Abstract: Monolateral or bilateral hyperplasia of the coronoid pro-cesses of the mandible is a rare disorder resulting in reduction ofmouth opening because of the unnatural contact of the coronoidprocess with the zygomatic bones. The authors describe two casesof bilateral hyperplasia of the coronoid processes that were success-fully treated.

Key Words:

M onolateral or bilateral hyperplasia of the coro-noid processes of the mandible is a rare disor-

der resulting in reduction of mouth opening becauseof the unnatural contact of the coronoid process withthe zygomatic bones.

The first case described in the literature was byHolmes in 1965; since then, many cases have been de-scribed, even though it is still considered an uncom-mon condition.1,2 The authors describe two cases ofbilateral hyperplasia of the coronoid processes thatwere successfully treated.

CASE 1

A 15-year old male patient presented to the Maxillo-facial Unit of the University of Ferrara for evaluationof a severely limited mouth opening. The patient�shistory revealed mandibular trauma when he was4 years old, with no fractures and apparently no se-quelae. From the age of 13 years, the patient reportedpain in the temporomandibular joint region witha progressive reduction in the mouth opening.

When first seen, clinical evaluation revealed noanomalies in the occlusion except for reduced mouthopening with a maximal interincisal distance of 25mm and no lateral deviation. A limited protrusionof the mandible was also present, and pain was

caused by forced manual opening. No other signsof muscle pain or contracture were present.

A computed tomography (CT) scan showed bi-lateral symmetrical enlargement of the coronoid pro-cesses that extended above the zygomatic arch. Axialand coronal CT scans, combined with a three-dimen-sional reconstruction, revealed the thickened andwidened coronoid processes in all directions of thespace, and in the mouth opening, the anterior surfaceof the processes touched the posterior surface of thezygoma. On the basis of the clinical examination andCT scans, a diagnosis of bilateral coronoid processeshyperplasia was made.

The patient underwent a bilateral coronoidec-tomy under general anesthesia. With an intraoral ap-proach, the ascending right ramus of the mandiblewas exposed as far as the top of the coronoid process.

The temporalis muscle was then detached fromthe coronoid with a dissector, and a horizontal osteot-omy was made with a reciprocating saw from the sig-moid incision to the anterior aspect of the ascendingramus. The entire right coronoid process was then re-moved. The same procedure was carried out for theleft process. At the end of the operation, the interin-cisal distance achieved was 40 mm with a mechanicalforced opening.

Fig 1 Maximum mouth opening before surgery.

From the Unit of Cranio-Maxillofacial Surgery, Center for OrbitalPathology, St. Anna Hospital and University, Ferrara, Italy.

Address correspondence to Dr Clauser, Unit of Cranio-Maxillo-facial Surgery, Center for Orbital Pathology, St. Anna Hospital andUniversity, Corso Giovecca 203, 44100 Ferrara, Italy; E-mail: [email protected]

BRIEF CLINICAL NOTES / Tieghi et al

723

In the days after surgery, a bite block was placedin the patient�s mouth to maintain and even improvethe opening movements. After his recovery, hestarted physiokinesitherapy rehabilitation with a me-chanical device (Butterfly Jaw Exerciser, Darcissactype) for 10 hours a day. After 30 days of therapy,the mouth opening was 34 mm, and after 60 days,

it was 43 mm. At the 3 month follow-up examination,the patient was able to open his mouth up to 46 mmspontaneously, with good protrusion and lateral ex-cursion of the mandible. After 6 months, the authorsdecided to suspend the rehabilitation with the Butter-fly Jaw Exciser.

Fig 4 Postoperative tridimensional computed tomogra-phy scan. Note the resection of the coronoids.

Fig 2 Maximum mouth opening 5 months after surgery.

Fig 3 Computed tomography scan showing hyperplasiaof the coronoid processes.

Fig 5 Trismus and limited interincisal distance beforesurgery.


724

Case 2

A 17-year-old female patient presented to our clinicafter a long history of mandibular opening reductionand pain in the temporomandibular jointregion as well as frequent headaches with no recenttrauma. She also reported limitation in the lateralmovements and protrusion of the mandible and signsof muscular contracture in the forced opening. Noother signs of lateral deviation or occlusal impair-ment were present.

Before she presented to our clinic, she had con-sulted a dentist, who performed 1.8, 2.8, and 3.8 ex-tractions and suggested the use of a splint to try andreduce pain and limitations of mouth opening. After1 year, the patient had a slight improvement, but themain symptoms were still present, with an interin-cisal distance of 25 mm.

The CT scan and panoramic radiography re-vealed asymmetry of the coronoid processes, withright hyperplasia and bony sclerosis. Clinical evalu-ation and radiological evidence led to a diagnosis ofbilateral asymmetrical coronoid hyperplasia.

The patient underwent a bilateral coronoidec-tomy under general anesthesia. With the same intrao-ral approach as in the first case, the coronoid processes

were exposed, a horizontal osteotomy was made, andboth processes were removed. At the end of the op-eration, the forced interincisal distance was 40 mm,and immediately after surgery, a static bite-blockwas placed to maintain the opening.

Approximately 15 days after surgical treatment,despite the use of a mechanical opener, the maximummouth opening had decreased to 28 mm. The authorsdecided to use a dynamic device (Darcissac type) for10 hours per day for 30 days.

After this, the mouth opening increased to35 mm, and at the 3-month follow-up, it had stabilized

Fig 7 Interincisal distance 12 months after surgery.Fig 6 Postoperative physiokinesitherapy with the help ofan intraoral device (Darcissac type exerciser).

Fig 8 Preoperative panoramic radiograph showing bilat-eral coronoid hypertrophy.

BRIEF CLINICAL NOTES / Tieghi et al

725

at 40 mm. Mechanical therapy was then interrupted.At the 20-month follow-up, the maximum spontane-ous mouth opening was still 40 mm, with goodprotrusion and lateral mandibular excursion andno pain in the temporomandibular joint region.

DISCUSSION

Bilateral hypertrophy of the coronoid processes is fre-quently misdiagnosed because it is relatively rare andusually has a slow and progressive clinical evolu-tion.3 If bilateral hyperplasia is suspected, panoramicradiography can confirm the diagnosis, even thoughit does not reveal the tridimensional relation betweenthe coronoid process and the zygomatic bone.

Improved technology, such as CT with axial andcoronal scans, combined with tridimensional recon-struction, has allowed us to reveal the exact coronoidand surrounding tissue shapes and structure. Thepathogenesis of coronal hypertrophy is still unknown.Although different hypotheses have been put for-ward, none of them has been completely accepted.

Some authors have suggested increased activityof the temporalis muscles as a causative factor. Sev-eral studies on the relation between growth of thecoronoid processes and activity of the temporalismuscle seem to support this theory.2,3

Other authors have reported that in bilateralcases, the tissue of the coronoid process always con-sists of normal mature bone, occasionally with an os-teosclerotic or fibrocartilaginous component.3

In conclusion, in bilateral coronoid hyperplasia,limitation of mandibular movement is caused by themechanical impingement of the enlarged coronoidprocesses on the posterior surface of the zygomaticbone. Therefore, surgical treatment is always recom-mended. In the authors� experience an intraoral coro-noidectomy in association with early postoperativerehabilitation allows satisfactory long-term results.4

(Figs 1–9).

REFERENCES

1. Fabie L, Boutault F, Gas C, et al. Neonatal bilateral idiopathichyperplasia of the coronoid processes: case report. J Oral Max-illofac Surg 2002;60:459–462

2. Totsuka Y, Fukuda H. Bilateral coronoid hyperplasia. J Cranio-maxillofac Surg 1991;19:172–177

3. Gerbino G, Bianchi SD, Bernardi M, et al. Hyperplasia ofthe mandibular coronoid process: long-term follow-upafter coronoidotomy. J Craniomaxillofac Surg 1997;25:169–173

4. Taylor Monks FT. Bilateral hyperplasia of the mandibularcoronoid processes: a case report. Br J Oral Surg 1978;16:31–37

Modern Surgical Treatment ofComplex Facial Fractures: A6-Year Review

Jason M. Jack, MD, Daniel H. Stewart, MD, Brian D. Rinker, MD,Henry C. Vasconez, MD, Lee L. Q. Pu, MD, PhD

Lexington, Kentucky

Abstract: Open reduction and internal fixation (ORIF) with refinedtitanium plates and screws have revolutionized the surgical treat-ment of complex facial fractures, but its outcome remains relativelyunknown. The purpose of this study is to review the results of mod-ern surgical treatment of complex facial fractures involving at leasttwo of the three zones of the face. From 1996 to 2002, 54 consecutivepatients (41 males, 13 females; age 5–74 years) with complex facialfractures were treated by the plastic surgery service in our institu-tion. All patients had their fractures repaired within 10 days afterinjury. ORIF of the fractures was performed through standard sur-gical approaches for the upper, middle, or lower face in a preferredsequence. Multiple rigid fixations were completed with currenttitanium plates and screws. Postoperative functional problems,residual cosmetic deformities, and reoperations for any complicationsin each patient were evaluated. All patients have been followed forup to 5 years. Overall, of the 54 consecutive patients in this study,8 (14.8%) patients had postoperative functional problems, and7 (13.0%) developed variable postoperative cosmetic deformities.Only nine (16.7%) patients underwent a subsequent reoperationfor correction of the functional or cosmetic concern. Thus, we be-lieve that modern surgical treatment of complex facial fractures byway of open treatment and rigid fixation with refined titaniumplates and screws has resulted in overall good to excellent out-comes and a relatively low postoperative complication rate.

Key Words: Complex facial fractures, rigid fixationtitanium plates, outcome study

Fig 9 Postoperative panoramic radiograph showing theostectomy of both coronoid processes.

From the Division of Plastic Surgery, University of KentuckyCollege of Medicine, Lexington, Kentucky.

Address correspondence ti Dr. Lee L.Q. Pu, Division of PlasticSurgery, University of Kentucky, Kentucky Clinic, K454 Lexington,KY 40536-0284, USA; E-mail: [email protected]

Presented at the Thirteenth International Congress of the Inter-national Confederation for Plastic, Reconstructive and AestheticSurgery, Sydney, Australia, August 10 to 15, 2003.


726

C omplex facial fractures often involve at least twoof the three zones of the face and may result in

significant functional problems and cosmetic deform-ities. In the past decade, the management of complexfacial fractures has evolved from limited internal fix-ation using intraosseous wiring and stainless-steelplating to open reduction and internal fixation (ORIF)with newly developed or refined titanium plates andscrews.1 This advancement has led to improvementsin the contouring and placement of this refined sys-tem compared with past modalities, the ability touse modern imaging techniques such as magneticresonance imaging and computerized tomography(CT) scanning without interference, and a decreasein the long-term morbidity of facial fractures.2–4 Al-though much has been written about surgical tech-niques of the modern management of complex facialfractures,1,5–8 the long-term outcomes of this modal-ity still remain relatively unknown. The purpose ofthis study was to retrospectively review the resultsof modern surgical treatment of complex facial frac-tures in a busy university plastic surgery practice.The study would represent a long-term outcomestudy of the modern surgical treatment of complexfacial fractures.

PATIENTS AND METHODS

From 1996 to 2002, a total of 54 consecutive patientswho sustained complex facial fractures were treated

by the plastic surgery service in our institution. Themedical records of all 54 patients were reviewed bythe authors and appropriate information regardingpattern of facial fracture, surgical treatment, postop-erative complications, any functional and cosmeticproblems, and reoperations were recorded. Complexfacial fractures were defined in our study as thosefractures that involved at least two of the three zonesof the face (upper, middle, and lower facial units).The upper facial unit extends from the top of the skullto the superior orbital rims and includes the frontalsinus and orbital roof. The midface extends fromthe superior orbital rims to the Lefort I level andincludes the naso-orbital ethmoidal area, zygoma,and the upper maxilla. The lower facial unit extendsfrom the Lefort I level to the menton and includes theocclusal subunit of the palate, alveolar maxilla anddentition, and mandible.8

Of the 54 patients, there were 41 males and13 females ranging in ages from 5 to 74 years old.Twenty-one patients had both upper- and middle-third facial fractures, 25 had both middle- and lower-third facial fractures, and 8 patients had true panfa-cial fractures involving all three zones of the face.1,7

The facial fracture pattern was diagnosed by CT scan-ning, and Panorex films were also obtained for allsuspected mandibular fractures. A three-dimensionalreconstruction imaging of CT scan was obtained inselected patients with complicated complex facialfractures or panfacial fractures. All patients had their

Fig 1 Current Leibinger craniomaxil-lofacial titanium plates and screws usedin this study.

BRIEF CLINICAL NOTES / Jack et al

727

complex facial fractures repaired within 10 days afterinitial injury. ORIF was performed through standardsurgical approaches for the upper, middle, and lowerfacial fractures in a preferred sequence by attendingsurgeons. This sequence often began with palatal re-duction and fixation followed by obtaining properocclusion and maintaining this relationship withintermaxillary fixation. The upper face was then ad-dressed if fractures existed within this unit. This in-cluded repair of supraorbital rim and roof fractures aswell as frontal sinus fractures. Attention was thenturned to reduction and fixation of any mandibularfractures that existed. Once a superior and inferiorframework was developed, restoration of the midfacewas accomplished. Within this unit, sequencing of re-duction and fixation began with the naso-orbital eth-moidal area followed by the zygomatic arch, orbitalrims, and maxillary buttresses, in that order. Inter-maxillary fixation was released, occlusion checked,and placement back into intermaxillary fixationwas performed if indicated. All rigid fixations wereaccomplished with current Leibinger or Synthes cra-niofacial titanium mini- and microplating systems(Figs 1 and 2). Primary bone grafting with a split cal-varial bone graft was implemented as necessary ifthere was a bony gap greater than 1 cm.

Postoperative functional problems (malocclusion,severe ectropion, epiphora, etc.) residual cosmetic de-formities (saddle-nose deformity, enophthalmos, tel-ecanthus, poor scarring, etc.), and reoperations forany complications (exposed plates, bony deformity,

etc.) in each patient were evaluated and reportedthrough up to a 5 year follow-up period.

RESULTS

Overall, of the 54 consecutive patients in our studywho sustained complex facial fractures and underwentORIF with refined titanium mini- and micro-platingsystems, 8 patients had postoperative functionalproblems, and 7 developed variable postoperativecosmetic deformities. Nine patients underwent a sub-sequent operation for correction of the functional orcosmetic concern. All patients were further dividedinto subgroups based on their pattern of complex fa-cial fracture, and the results are summarized in Table 1.

In analyzing our data in this patient population,we found that three patients with fractures involvingthe upper two-thirds developed a functional prob-lem. These included one patient with severe ectropion,one with malocclusion, and one with epiphora. Cos-metic deformities were noted in two patients, one

Fig 2 Current Synthes craniomaxillo-facial titanium plates and screws usedin this study.

Table 1. Summary of Results

Fracture

Pattern

Functional

Problem (%)

Cosmetic

Deformity (%)

Reoperation (%)

Upper 2/3 rd (n=21) 3 (14.3) 2 (9.5) 3 (14.3)

Lower 2/3 rd (n=25) 3 (12.0) 3 (12.0) 3 (12.0)

Panfacial (n=8) 2 (25.0) 2 (25.0) 3 (37.5)

Overall (n=54) 8 (14.8) 7 (13.0) 9 (16.7)


728

with a saddle-nose deformity and one with enoph-thalmos. Re-operation was required in three patients,one to correct severe ectropion, one to correct maloc-clusion, and one to correct the saddle-nose deformity.

Of the patients who sustained fractures of thelower two-thirds of the face, three developed func-tional problems, and three developed cosmetic de-formities. These complications included malocclusion,severe ectropion, and plate exposure. Three subse-quent operations were needed in this subgroup. Oneoperation again was needed to correct severe ectro-pion, another was needed to remove loose, exposedhardware, and a final surgical procedure was per-formed for scar revision on a hypertrophic subman-dibular incision.

Patients with panfacial fractures represented15% of our study population, which is in accordancewith other published reports of their incidence.6

Despite the relatively low number of patients in thissubgroup, a high incidence of postoperative compli-cations and subsequent reoperations resulted, in com-parison with the other two subgroups (Table 1). Of theeight patients with panfacial fractures, two patientsdeveloped functional problems (1 with malocclusion,1 with ectropion) and two developed cosmetic de-formities (1 with telecanthus and 1 with enophthalmos).A total of three patients underwent reoperations inthis subgroup. Among them, one had reoperation forsevere ectropion, one for malocclusion, and one forexposed hardware requiring an additional operationfor soft tissue coverage.

DISCUSSION

The role of ORIF using the latest advances in titaniumplate and screw technology is now the gold standardof treatment of complex facial fractures. To maximizegood to excellent postoperative results using this im-proved plating system, a thorough understanding ofboth the normal craniofacial anatomy and the ad-verse changes that take place with complex facial frac-tures is still warranted. There are various sequencesof complex facial fracture repair.1,5–10 Some of themost commonly described surgical approaches havebeen from superior to inferior, inferior to superior, pe-ripheral to central, and central to peripheral. Morerecently, the trend has become to repair the upperand lower facial components to provide a stableframework for midface restoration.8 This has becomeour standard approach to complex facial fracturesand has served us well over the past several years.Ultimately though, the key to success in sequencingthese fractures is to have a system in place, which

avoids haphazard organization of the restorationof the proper bony craniofacial anatomy.

When bony loss is observed because of severecomminution, especially in the relatively thin andweakly reinforced bones of the midface, bone graft-ing is implemented. This technique used in the acutesetting is now standard of care to ‘‘bridge the gap’’ ofbony nonunion found between the spanning platefixed to solid bone on either side of the defect. Bonegrafts can easily be harvested from parietal outer-tablecortex, especially in patients who already require abicoronal incision to expose their fractures. Adequateexposure of all fracture sites is very important inthe ultimate outcome of complex facial fracturemanagement. The surgeon must take into accountnot only the bony anatomy but also the soft tissuesof the face. Proper placement of inconspicuous inci-sions, delicate handling of the facial soft tissue, andresuspension of this soft tissue envelope after bonyreduction and fixation are major determinants ofthe overall functional and cosmetic outcome in thispatient population.1,4–7 Our standard surgical ap-proaches for the upper, middle, and lower face includebicoronal, lateral brow, subciliary, upper and lower gin-givobuccal, and submandibular (Risdon) incisionsdepending on the pattern of complex facial fractures.

In a majority of our cases, ORIF using titaniumplating systems, inconspicuous incisions, careful soft-tissue handling, bone-grafting when necessary, anda systematic approach to the bony facial skeleton re-sulted in good to excellent functional and cosmeticresults. In this study, the results have been arbitrarilysummarized in three categories: functional problems,cosmetic deformity, and reoperation. Although thesize of our series in this study was somehow moder-ate, the complication rate reported here was relativelylow (less than 15%), and reoperation was very oftenfor less complicated problems (i.e., ectropion, scar re-vision, or exposed plates). Our series represents amodern experience reviewing exclusively surgicalmanagement of complex facial fractures because nosimilar outcome study has been reported in the recentliterature. In analyzing our complications in the man-agement of complex facial fractures, a few key pointsshould be mentioned.

First, there were no failures of the titanium plat-ing systems in the restoration of bony width, projec-tion, and height of the facial skeleton. There were twocomplications of plate exposure, both involving de-hiscence of upper gingivobuccal incisions. One dehis-cence was in the acute setting, and the patient wasreturned to the operating room for irrigation and sub-sequent reclosure of the upper gingivobuccal inci-sion. The other hardware complication was extrusion

BRIEF CLINICAL NOTES / Jack et al

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of a loose maxillary buttress L-plate through an up-per gingivobuccal incision. These two complicationsoccurred relatively early in our study, and adjust-ments were made as to the closure technique of thegingivobuccal incisions. Initially, a single 4–0 chromicgut suture placed in a running fashion was used. Thetwo above complications occurred using this closure.We then changed to a two-layer closure using 4–0 Vicryldeep, interrupted suture for approximation of themuscle followed by a running 4–0 chromic gut suturefor mucosal closure. No further gingivobuccal inci-sion dehiscence has occurred since the change inclosure technique.

Three patients required reoperations for severeectropion in this series. All three patients had asubciliary skin-muscle flap approach for repair of in-fraorbital rim or orbital floor fractures. Although tem-porary postoperative ectropion and scleral show canbe corrected with early frequent scar massage, severeectropion with any exposure of the cornea caused bysignificant lower eyelid scar contracture warrants sur-gical intervention. In reviewing the literature on thisissue, the subciliary approach has, in general, a highercomplication rate than a transconjunctival approach,especially in postoperative eyelid retraction and ec-tropion.11,12 Therefore, a transconjunctival approachto the infraorbital rim or orbital fractures could beconsidered to replace a subciliary approach to mini-mize these low eyelid complications.

Severe naso-orbital ethmoidal fractures can re-sult in cosmetic and functional problems if not prop-erly treated. Three postoperative complications werethe result of these fractures. Saddle-nose deformitywas corrected with subsequent bone grafting result-ing in a good cosmetic outcome. An ophthalmologyconsult was obtained for severe epiphora with scar-ing of the lacrimal system. Dacryocystorhinostomywas performed with eventual resolution of epiphora.Telecanthus was observed in one patient from failureof the transnasal wiring of the medial canthal bonyfragments. The fracture site was re-explored with re-placement of the transnasal wiring, and the telecan-thus was improved.

One patient developed hypertrophic scarring ofa submandibular incision requiring scar revision. Thesubsequent incision healed well without hypertro-phic scarring or keloidal formation. One patient de-veloped enophthalmos requiring reconstruction ofthe bony orbit. On re-exploration, it was found thatthe inferior orbital rim was displaced inferiorlyand posteriorly, resulting in an increase in orbital vol-ume. This complication was felt to be from failure ofthe initial anatomic reduction of the infraorbital rimfracture and was not from subsequent weakness and

deformity of the plate itself. On review of the litera-ture, the position of the displaced inferior orbital rimwas found to be the most common, and a surgicalapproach to its correction was reported previously.1

After re-establishing the correct alignment of theinferior orbital rim, the patient’s enophthalmoswas significantly improved.

Finally, three patients in our study developedpostoperative malocclusion. On review of our resultswith the current literature, all three complications ofmalocclusion occurred because of inadequate reductionof the fractures with the bony fragments rigidly fixedin an unreduced position.13–15 Improvements in ex-posure and reduction of the bony fragments of den-tition should continually be sought because this is theinitial step in the management of complex facial frac-tures and constitutes the framework on which the restof the operation is dependent.

The use of refined titanium plating system has alsobeen reported by others for various facial fractureswith good success and low complication rates.2,16–18

As the management of complex facial fractures con-tinues to evolve, new directions will be sought to im-prove on the current techniques and technology. Futureinnovations, such as the refinement of absorbable plat-ing systems to use in the management of pediatric andcertain adult facial fracture populations19,20 and thecontinued development of alloplastic materials,21,22

will no doubt improve the outcome in surgicalmanagement of complex facial fractures and serveas a new gold standard in craniomaxillofacial surgery.

In conclusion, the management of complex facialfractures has made great progress in the last decadewith the development of an improved understandingof the current concepts in bony facial restoration fol-lowed by the advances of plate and screw technology.Our study reveals that modern surgical treatment ofcomplex facial fractures by way of open treatment andrigid fixation with refined titanium plates and screwshas resulted in overall good to excellent outcomes(i.e., normal facial appearance and function) and arelatively low postoperative complication rate. How-ever, a multicenter study may still be warranted toconfirm our results.

The authors thank Mrs. Rita Morrissey for her secretarial support inpreparation of the manuscript.

REFERENCES

1. Manson PN, Clark N, Robertson B, et al. Comprehensive man-agement of pan-facial fractures. J Craniomaxillofac Trauma1995;1:43–56


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2. Haug RH, Jenskins WS, Brandt MT. Advances in plate andscrew technology: thoughts on design and clinical applications.Semin Plast Surg 2002;16:219–227

3. Rohrich RJ, Watumull D. Comparison of rigid plate versuswire fixation in the management of zygoma fractures: a long-term follow-up clinical study. Plast Reconstr Surg 1995;96:570–575

4. Evans GR, Clark N, Manson PN, et al. Role of mini- and micro-plate fixation in fractures of the midface and mandible. AnnPlast Surg 1995;34:453–456

5. Crawley WA, Vasconez HC. Midface, upper face, and panfacialfractures. In: Ferrero J, ed. Fundamentals of Maxillofacial Sur-gery. New York: Springer-Velag, 1997;203–214

6. Wenig BL. Management of panfacial fractures. OtolaryngolClin North Am 1991;24:93–101

7. Markowitz BL, Manson PN. Panfacial fractures: organization oftreatment. Clinics Plast Surg 1989;16:105–114

8. Kelly KJ, Manson PN, VanderKolk CA, et al. Sequencing Lefortfracture treatment (organization of treatment for a panfacialfracture). J Craniofac Surg 1990;1:168–178

9. Rohner D, Tay A, Meng CS, Hutmacher DW, et al. The spheno-zygomatic suture as a key site for osteosynthesis of the orbito-zygomatic complex in pan facial fractures: a biomechanicalstudy in human cadavers based on clinical practice. Plast Re-constr Surg 2002;110:1463–1475

10. Tullio A, Sesenna E. Role of surgical reduction of condylar frac-tures in the management of panfacial fractures. Br J Oral Max-illofac Surg 2000;38:472–476

11. Patel PC, Sobota VT, Patel NM, et al. Comparison of trans-conjunctival versus subciliary approaches for orbital fractures:a review of 60 cases. J Craniomaxillofac Trauma 1998;4:17–21

12. Appling WD, Patrinely JR, Salzer TA. Transconjunctival ap-proach vs subciliary skin-muscle flap approach for orbital frac-ture repair. Arch Otolaryngol Head Neck Surg 1993;119:1000–1007

13. Gruss JS, Whelan MF, Rand RP, et al. Lessons learnt from themanagement of 1500 complex facial fractures. Ann Acad MedSingapore 1999;28:677–686

14. Zachariades N, Papademetriou I, Rallis G. Complications asso-ciated with rigid internal fixation of facial bone fractures. J OralMaxillofac Surg 1993;51:275–278

15. Mason PN, Clark N, Robertson B, et al. Subunit principlesin midface fractures: the importance of sagittal buttresses,soft-tissue reductions, and sequencing treatment of segmentalfractures. Plast Reconstr Surg 1999;103:1287–1306

16. Francel TJ, Birely BC, Ringelman PR, et al. The fate of plates andscrews after facial fracture reconstruction. Plast Reconstr Surg1992;90:568–573

17. Schortinghuis J, Bos RR, Vissink A. Complications of internalfixation of maxillofacial fractures with microplates. J Oral Max-illofac Surg 1999;57:130–134

18. O’Sullivan ST, Snyder BJ, Moore MH, et al. Outcome mea-surement of the treatment of maxillary fractures: a prospectiveanalysis of 100 consecutive cases. Br J Plast Surg 1992;52:519–523

19. Eppley BL, Sadove AM, Havlik RJ. Resorbable plate fixationin pediatric craniofacial surgery. Plast Reconstr Surg 1997;100:1–7

20. Eppley BL. Zygomaticomaxillary fracture repair with resorb-able plates and screws. J Craniofac Surg 2000;11:377–385

21. Amarante MTJ, Constantinescu MA, et al. Cyanoacrylate fixa-tion of the craniofacial skeleton: An experimental study. PlastReconstr Surg 1995;95:639–646

22. Gosain AK, Song L, Corrao MA, et al. Biomechanical evaluationof titanium, biodegradable plate and screw, and cyanoacrylateglue fixation systems in craniofacial surgery. Plast ReconstrSurg 1998;101:582–591

Frontonasal Osteotomy toFacilitate Removal of anIntracranial Nasal Dermoid

Gary F. Rogers, MD, Mark R. Proctor, MD, Arin K. Greene, MD,John B. Mulliken, MD

Boston, Massachusetts

Abstract: Dermoid sinus is the most common midline nasal anom-aly. Intracranial extension has been reported in up to 45% of casesand requires a combined intracranial-extracranial besection. Wepresent a technique of en-bloc ostectomy of the central bandeauand nasal bones to approach this malformation. This allows unob-structed visualization of the entire cyst and sinus tract, includingportions that traverse or lie under the nasal bones, and ensurescomplete excision. Additionally unnecessary disruption of the na-sal bones and facial scarring are avoided.

Key Words: Dermoid sinus, frontonasal osteotomy,intracranial

C ongenital anomalies in the nasal midline arerare, occurring in an estimated 1 per 20,000 to

40,000 live births.1–3 Nasal dermoid sinus cyst andepidermoid cyst are the most common, followed byencephalocele, hemangioma, and glioma.4–10 Nasaldermoids represent 6–7% of dermoids in the headand neck and 1–3% of all dermoid cysts.11–13 A 2:1 malepreponderance has been reported.4–6,12–14 Althoughthe majority of nasal dermoids are sporadic, thereare a number of familial examples.15–21 An associa-tion with other malformations has been noted in5% to 41% of patients.12–15,19,22,23

Nasal dermoid presents as either a cyst, a sinus,or a fistula.14 There is usually a firm, subcutaneousmass, midline or slightly off center, although a cystcan lie between or under the nasal bones, in the nasalseptum, or intracranially. A cutaneous punctum ispresent in up to 91% of nasal dermoids and maybe located at any point on the mid-dorsal line fromthe glabela to the columella.10,15,24 Hair protrudingfrom a nasal pit is pathognomonic for nasal dermoidsinus, but is present in less than 50% of patients.8–14,15

Several operative approaches have been advo-cated for removal of intracranial nasal dermoids.

From the Craniofacial Centre, Division of Plastic Surgeryand Department of Neurosurgery, Children’s Hospital, HarvardMedical School, Boston, Massachusetts.

Address correspondence to Gary Rogers, MD, Children’sHospital, Division of Plastic Surgery, 300 Longwood Ave., Boston,MA 02115; E-mail: [email protected]

BRIEF CLINICAL NOTES / Rogers et al

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Pollock proposed that any excisional strategy shouldmeet four criteria: 1) permit access to all midline cystsand permit medial and lateral osteotomies; 2) allowrepair of a defect in the cribriform plate and possiblecerebrospinal fluid leak; 3) facilitate reconstruction ofthe nasal dorsum, if necessary, and 4) leave an accept-able scar.25 Raveh described a subcranial approachfor treatment of traumatic injuries in the anteriorskull base26–27, and subsequently for congenitalanomalies28 and cranial base tumors.29–30 We proposea modification of this technique for removal of intra-cranial nasal dermoid, which provides complete ex-posure of the cyst and sinus tract and fulfills Pollock’sother three criteria.

TECHNIQUE

The nasal component of the dermoid cyst isapproached through either bilateral rim incisions, in-tercartilaginous incisions or, if a punctum is present,through a small vertical cutaneous excision. The cyst

is freed from the surrounding subcutaneous tissueand its tract is traced cephalad until it passes beneaththe nasal bones. The tract can penetrate deeply at anypoint between the upper lateral cartilages and thenasofrontal suture. The sinus is tagged with a 5-0 silksuture, thus completing the extracranial dissection.

A coronal incision, subperiosteal dissection, andthe superior orbital release provides sufficient laxityto expose the nasal bones down to the caudal margin.The periosteum over the nasofrontal region can be cutvertically using electrocautery to facilitate this expo-sure. A forked Langenbeck retractor is placed as thedissection extends over the nasal pyramid. Careshould be taken to avoid disruption of the medialcanthal tendons as the periosteum is elevated fromthe nasal process of the maxilla. The tagged distalend of the sinus is visualized (Fig 1).

A small frontal craniotomy is made in a demiluneshape, no more than 3 cm wide, and medial to the

Fig 1 Sinus tract (arrow) and nasal bones exposed.Fig 2 Demilune craniotomy and frontonasal segments.Note dermoid sinus (arrow) traversing nasal bones.


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supra-orbital foramen or notch. The central bandeauand the nasal bones are removed in continuity (Fig 2).Unlike the subcranial approach described by Ravehet al,26–30 the nasal process of the maxilla and medialorbital walls is not included in the removed segmentand, therefore, the sphenoidal and ethmoidal sinusesare not exposed. The nasofrontal suture can be sepa-rated and, therefore, it is important not to applyundue force across this junction as the frontonasalsegment is elevated. The sinus tract lies on the upperlateral cartilages, which are apposed to the ethmoidsat the level of the nasofrontal junction in an infant.28

Occasionally, the sinus tract passes under the upperlateral cartilages; they can be incised vertically for ex-posure, so that the entire specimen can be removed(Fig 3). Any dural defects are sealed. The nasofrontalsegment and craniotomy graft are replaced and fixedin anatomic position using absorbable plates andscrews in an infant or titanium plates in an older child(Fig 4). The wound is irrigated and closed.

CLINICAL EXPERIENCE

Between April 2001 and April 2004, we evaluated 18patients with midline nasal dermoids: 8 were boysand 10 were girls with a mean age of 2.3 years atpresentation. All patients had CT and/or MRI imag-ing, and 5 patients had radiographic evidence ofintracranial communication. In these 5 patients, thedermoid tract was removed through a combinedintracranial-extracranial approach, as describedabove. There were no complications. Four patientshad a minimum follow-up of one year and there wereno recurrences. In all cases, the small dorsal nasalscar and the forehead contour were aestheticallyacceptable (Fig 5).

In one patient, the dermoid sinus lay under thenasal bones but appeared to terminate in a fibrousstalk. After intracranial exposure, we found an intra-cranial cyst, but there was no communication withthe nasal dermoid. In the remaining four patients,the sinus was found lying either above the upper lat-eral cartilages (n = 1), between the upper lateral carti-lages (n = 2), or under the upper lateral cartilages(n = 1). One of these patients had an unusually large

Fig 3 (Above) Nasal dermoid sinus seen under intact up-per lateral cartilages and extending to dura; (Below) sinusexposed by incision between the upper lateral cartilages.

Fig 4 After stabilization of the craniotomy and frontonasalelements.


733

intracranial cyst and a large dural defect after re-moval that had to be repaired with a free pericranialgraft.

DISCUSSION

Two essential and often conflicting goals guide exci-sion of an intracranial nasal dermoid: the cyst andsinus must be completely excised but with minimalexternal scarring.31 Preoperative evaluation and plan-ning are essential in balancing these goals. Determi-nation of possible intracranial extension is the criticalpiece of preoperative information.14,22,32–33 Incompleteexcision of a nasal dermoid portends a high recur-rence rate.5,6,9,10,11–13,34 Previous authors have sug-gested that the incidence of intracranial extensionwas 1–2%11–13,35–36; however, more recent studies re-port rates as high as 45%.15,33,37 Failure to appreciatean intracranial extension can lead to life-threateningcomplications from infection, such as meningitis, in-tracranial abscess, and osteomyelitis.15,38–40 CT andMRI are complimentary studies to determine whetheror not there is an intracranial component, i.e.,each provides important information.14,25,33,37,41 CTfindings that suggest intracranial extension are a pat-ent foramen cecum and a bifid crista galli.23,33,42

However, these are not specific signs, and both falsepositive and negative CT examinations have been re-ported. MRI shows soft tissues better than CT and ismore specific.22,32–33,42,44,45 Some authors have rec-ommended MRI if the initial CT is suspicious.14,22,41

If it is unclear whether or not there is intracra-nial extension, several authors have proposed a two-

stage procedure, i.e. extracranial dissection beforeembarking on a craniotomy.14,23,37,43 Sessions advo-cated extracranial dissection and biopsy at the upperend of the sinus; absence of a dermal element wasconsidered sufficient indication to abort craniot-omy.23 This recommendation is dated because itwas based on an equivocal preoperative CT beforethe general use of MRI. This strategy would have re-sulted in an incomplete resection in one of our pa-tients in whom there was no obvious connection be-tween separate extracranial and intracranial cysts.Fortunately, preoperative MRI revealed the intra-cranial cyst and a combined intracranial-extracranialexposure was planned. A similar situation was de-scribed by Posnick and colleagues.46

Whenever the sinus tract extends to dura, a com-bined intracranial-extracranial procedure is neces-sary to ensure complete resection.33 Intracranialexposure requires the standard coronal incisionand a frontal craniotomy.14,31,33,37,39,43 If there is a cu-taneous punctum, it must be excised, using a smallvertical external incision.10,15,24 However, in the pastmore extensive incisions, usually combined with nasalosteotomies, were advocated to expose the sinus underthe nasal bones. Proposed external incisions include:vertical midline,4,7,13,25,37 lateral rhinotomy,1,25

transverse linear or curvilinear,13,31 U-shaped dorsalrhinotomy,25,31 vertical zig-zag,25 inter-cartilaginousincisions (‘‘closed’’ rhinoplasty),47 open rhinoplastywith or without lateral rhinotomy,22,42,48–50 paracan-thal,22 and wide T-shaped glabelar.38 Transnasal en-doscopic approach has also been described to avoidfrontal craniotomy when the sinus tract extends to the

Fig 5 (Left) Preoperative-3 year-oldmale with intracranial nasal dermoidsinus. Note cutaneous punctum withprotruding hair. (Right) Postopera-tive-6 month follow-up.


734

dura but not between the leaves of the falx cerebri.41

Whatever the cutaneous incisions, the nasal bonesmust be replaced and stabilized;10,31 subsequentnasal deformity has been reported.10

Our frontonasal craniotomy technique, a varia-tion of that described by Raveh,26–30 allows full expo-sure and complete removal of any intracranial nasalcyst or sinus. External cutaneous incision is unneces-sary unless there is a punctum. The nasal bones areremoved in continuity with the central bandeau and,therefore, nasal reconstruction is not required. In pa-tients with nasal deformity secondary to expansion ofthe cyst, the nasal bones can be replaced and stabi-lized to the bandeau ex vivo. Furthermore, the upperlateral cartilages are easily manipulated through thecoronal exposure. The senior author has used thesame approach, i.e., removal of the nasal bones incontinuity with the bandeau, for correction of nasalangulation in unilateral coronal synostosis for over20 years.51–53 We have not observed any alterationsof normal nasal growth in these patients who haveundergone frontonasal osteotomy and closing wedgenasal ostectomy for synostotic frontal plagiocephaly.

SUMMARY

The frontonasal osteotomy technique offers unparal-leled exposure and assures complete removal of anintracranial nasal dermoid sinus. Nasal incisionand dissection are minimized. The access to the nasalbones and upper lateral cartilages also facilitatesreconstruction of the bony-cartilaginous pyramid,if necessary.

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40. Fujisawa K, Suzuki N, Hoshino M, et al. Congenital dermal si-nus of the nose complicated with a brain abscess– a case report(author’s transl). Neurol Med Chir (Tokyo) 1981;21:521–524

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45. Barkovich AJ, Vandermarck P, Edwards MS, Cogen PH. Con-genital nasal masses: CT and MR imaging features in 16 cases.AJNR Am J Neuroradiol 1991;12:105–116

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48. Bilkay U, Gundogan H, Ozek C, et al. Nasal dermoid sinuscysts and the role of open rhinoplasty. Ann Plast Surg 2001;47:8–14

49. Koltai PJ, Hoehn J, Bailey CM. The external rhinoplasty ap-proach for rhinologic surgery in children. Arch OtolaryngolHead Neck Surg 1992;118:401–405

50. Mankarious LA, Smith RJ. External rhinoplasty approach forextirpation and immediate reconstruction of congenital midlinenasal dermoids. Ann Otol Rhinol Laryngol 1998;107:786–789

51. Hansen M, Padwa BL, Scott RM, Stieg PE, Mulliken JB. Synos-totic frontal plagiocephaly: Anthropometric comparison ofthree techniques for surgical correction. Plast Reconstr Surg1997;100:1387–1395

52. Hansen M, Mulliken JB. Frontal plagiocephaly: Diagnosis andtreatment. Clin Plast Surg 1994;21:543–553

53. Meara JG, Burvin R, Bartlett RA, Mulliken JB. Anthropometricstudy of synostotic frontal plagiocephaly: Before and after fronto-orbital advancement with correction of nasal angulation. PlastReconstr Surg 2003;112:731–738

Overgrowth of a CostochondralGraft in Nasal Reconstruction

Rong-Min Baek, MD, Yoonho Lee, MD,* Yong-Tai Song, MD**

Seoul, Korea

Abstract: Nasal reconstruction with autogenous costochondralgraft has been one of the surgical managements in craniofacialclefts. We have recently seen one patient who underwent this op-eration and the graft had overgrown like as in Pinocchio’s nose-

overly projecting nasal tip for almost 7 years. This case supportsthe notion that the costochondral graft itself carries intrinsic factorsand the growth of the graft depends mainly on the costochondralgraft itself. Therefore we acknowledge ‘‘the primary growth centertheory’’ rather than ‘‘the functional matrix theory.’’

Key Words: Costochondral graft, nasal reconstruction,craniofacial cleft, Pinocchio’s nose

C raniofacial clefts have required diverse proce-dures of surgical managements. Nasal recon-

struction, as one of these operations, is indispensableto proportionate facial configuration, and especiallycantilevered autogenous costochondral graft recon-struction of the nasal dorsum and the tip is an effec-tive means of reconstruction for the hypoplastic nosein childhood. However, the growth pattern of the

Fig 1 A baby presented number 3 facial cleft with hyper-telorism at birth.

*From the Department of Plastic Surgery, Seoul National Univer-sity Bundang Hospital, Kyongki-do, Korea; **Department of PlasticSurgery, Inje University Medical Center, Seoul, Korea.

Address correspondence to Rong-Min Baek, MD, Departmentof Plastic and Reconstructive Surgery, Seoul National UniversityBundang Hospital, Gumi-dong 300, Bundang-gu, Sungnam-si,Kyungki-do, Korea; E-mail: [email protected]


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costochondral graft is extremely unpredictable and inour case the growth of the graft extremely exceededthe general growth rate of the young patient. There-fore we are of the opinion that the costochondral graftitself carries intrinsic growth factors and the growthof the graft depends mainly on the costochondralgraft itself and acknowledge ‘‘the primary growth

center theory’’ rather than ‘‘the functional matrixtheory.’’

We have recently seen one patient who under-went autogenous costochondral graft to reconstructthe nose. The graft had overgrown like as in Pino-cchio’s nose - overly projecting nasal tip for almost7 years.

Fig 2 At the age of 3 years and 9months. Postoperative views afterorbital osteotomy and calvarial on-lay graft at both lateral orbital rimand both medial canthopexy. (2A)frontal and (2B) left three quartersviews.

Fig 3 At the age of 7 years. Postop-erative views after augmentationrhinoplasty with costochondral graftby cantilever method and V-Y ad-vancement flap on the dorsum ofnose and correction of the left enoph-thalmos with rib bone graft and lat-eral canthopexy. (3A) frontal and(3B) left three quarters views demon-strate improved nasal augmentation.

BRIEF CLINICAL NOTES / Baek et al

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CASE REPORT

A 12-year-old girl revisited us 7 years after the lastsurgical procedure with the complaint of Pinocchio’snose-like deformity. She had number 3 facial cleft(Tessier classification) with hypertelorism at birth(Fig 1), and examination by the pediatrician revealedno deformity other than that of the face. She under-went closure of facial cleft at the age of 20 days,reconstruction of the left eyelid at 8 months, andcheiloplasty at 20 months after birth, successively.

When she was 3 years 9 months old, the intercan-thal distance was 44mm, and therefore we planned tocorrect hypertelorism. We first performed orbital os-teotomy through the craniotomy of frontal bone, andthen a calvarial bone onlay graft at both lateral orbitalrims, which resulted from reposition of orbital bone,and finally both medial canthopexy was performed(Fig 2). By this operation, the intercanthal distancedecreased as 31 mm (immediate postoperative data).

At 6 years 4 months of age, the final reconstructionwas performed including augmentation rhinoplasty

Fig 4 Follow-up view of the patientat age 12. She presents Pinocchio’snose deformity, prominently project-ing nose owing to overgrowth of thecostochondral graft. (4A) frontal,(4B) worm’s eye, (4C) left three quar-ters views, and (4D) left lateral view.


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with costochondral graft by cantilever method and V-Y advancement flap on the dorsum of nose. At thesame time, the left side enophthalmos was correctedsurgically with rib bone graft and left lateral cantho-pexy was performed. She was discharged after 17days without any problem (Fig 3).

When she was 12 years old, she came to our clinicpresenting Pinocchio’s nose-like deformity (Fig 4).We thought that overgrowth of the costochondralgraft made her nose project forward. Five monthslater, she underwent excision of overgrown rib boneand cartilage, and nasal tip plasty (Fig 5).

DISCUSSION

Craniofacial clefts exist in a multitude of patterns,varying in degrees of severity and manifestation. Thenumber 3 facial cleft begins at the Cupid’s bowand undermines the nasal alar base and continues

cephalad to end just medial to the inferior punctumof the lower eyelid. The skeletal disruption can beextensive. The frontal process of the maxilla is inter-rupted as the cleft terminates in the lacrimal groove.Thus, a confluent cavity is formed composed of themouth, nose, maxillary sinus, and orbit. 1

We had performed surgical operations on this pa-tient step by step, and it was found that the costochon-dral graft in nasal reconstruction had overgrowntherefore letting us deal with no more than costochon-dral graft in nasal reconstruction and aim to surmisethe cause of overgrowth in this report.

It is reasonable and cogent that missing tissueshould be replaced by similar tissue in kind. So nasalskeletal abnormalities often involve both the bonyand cartilaginous parts, and the composite osteo-chondral costal graft is ideally suited for their re-construction.2 There are several advantages of thecostochondral graft, including biologic and anatomicsimilarity, low morbidity of the donor site, ease inobtaining and adapting the graft, and its regenerativeand growth potential.3

Judith M. Gurley 4 (2001) reported that cantileveredautogenous chondro-osseous rib graft reconstructionof the nasal dorsum is an effective means of recon-struction for the hypoplastic nose in childhood withrespect to morphometric measurements, patient self-perception, and the assessment of nasal appearanceby others. Utilization of cartilage is the first choicewhen autologous tissue is required for improvementof the nose.5

The cartilage is thought by some to act as a pri-mary growth center, whereas others believe it to bean adaptive growth site secondary to the transitionalmovement of the adjacent bony structure accordingto the functional matrix theory. This functional matrixtheory of Moss6,7 can explain the continuing growthin most patients but not the overgrowth in our patient.

There are some reports supporting this func-tional matrix theory. Ian R. Munro8 (1989) reportedthat free costochondral grafts were used to constructthe absent ascending ramus and condylar head of22 children with hemifacial microsomia who wereless than 14 years of age, and evidence of continuedgrowth was demonstrated in all patients; however,in two patients, considerable overgrowth occurred.R. Bruce Ross9(1999) reported that when costochon-dral grafts replacing the mandibular condyle wereperformed, growth of the graft did not always equalthe growth of the normal side, but in most cases a sat-isfactory symmetry was achieved, and several casesexhibited excessive overgrowth.

The growth potential of costchondral grafts hasbeen documented in children; however, Bahman

Fig 5 Five months after the correction of Pinocchio’s nose –excision of overgrown rib bone and cartilage, and nasal tipplasty.

BRIEF CLINICAL NOTES / Baek et al

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Guyuron10 (1992) reported that the growth pattern ofthe costochondral graft is extremely unpredictableand only the functional matrix theory cannot give ex-planation for possible causes. It still remains contro-versial whether the costochondral graft exhibits aprimary (inherent) growth potential or a secondary(adaptive) growth potential in response to functionalmatrix.11

Costochondral grafts do not adapt to the growthvelocity of the transplantation site so costochondralgrafts grow unrelated to their functional environ-ments, and that by varying amounts of cartilage inthe graft, subsequent growth can also be varied.12

N. Samman13(1995) assumed the possible inher-ent growth potential of a costochondral graft andreported that the effect of humoral factors on germi-native cartilage cells was partly responsible for celldifferentiation and proliferation.

William H. Ware14 (1981) reported that the trans-planted bony growth center had intrinsic growthproperties so intrinsic growth of the transplant wouldappear to be a more rational explanation than thefunctional matrix concept.

The costochondral junction can be considered tobe a secondary growth site responding to enlarge-ment of the surrounding functional matrix; however,our case of excessive growth of the graft supports theview that the costochondral junction carries intrinsicgrowth properties and hyperplasia of a graft canoccur due to intrinsic factors.

Now we can, therefore, accept the scientific factthat the costochondral grafts transplanted in activelygrowing children have their own growth potential,and we can also find that all of the above reportson the growth of the costochondral graft were limitedto the cases presented in temporomandibular joint re-construction. In this case report, we report on the un-usual extreme overgrowth of the costochondral graftin the nasal reconstruction. The growth of the graftextremely exceeded the general growth rate of theyoung patient. So from this unusual case we can de-duce that the growth of the grafted unit dependsmainly on the costochondral graft itself and highlight‘‘the primary growth center theory.’’14

The authors thank professor Se-Min Baek, MD, who has advisedand instructed us in a profound and comprehensive knowledgeof the craniofacial clefts.

REFERENCES

1. Kawamoto HK Jr. Craniofacial Clefts. In: SJ Aston, RW Beasley,CHM Thorne (eds). Grabb and Smith’s plastic surgery, 5th ed.Philadephia: Lippincott-Raven; 1997:349–364

2. Laurence AC, Hilton B, Alex C. The versatile costal osteochondralgraft in nasal reconstruction. Br J Plast Surg 1980;33:179–184

3. Ellen WCK, Chiung SH, Yu RC. Temporomandibular joint re-construction in children using constochondral grafts. J OralMaxillofac Surg 1999;57:789–798

4. Judith MG, Thomas P, Chad AP, Jeffrey LM. Long-termoutcome of autogenous rib graft nasal reconstruction. PlastReconstr Surg 2001;108:1895–1907

5. Lazaro CC, Maria TG. Use of cartilaginous autografts in nasalsurgery: 8 years of experience. Plast Reconstr Surg 1999;103:1003–1014

6. Melvin LM, Robin MR. The role of the functional matrix inmandibular growth. Angle Orthod 1968;38:95–103

7. Melvin LM, Letty S. The primary role of functional matrices infacial growth. Am J Orthod 1969;55:566–577

8. Munro IR, Phillips JH, Griffin G. Growth after constructionof the temporomandibular joint in children with hemifacialmicrosomia. Cleft Palate J 1989;26:303–311

9. Bruce R. Costochondral grafts replacing the mandibularcondyle. Cleft Palate Craniofac J 1999;36:334–339

10. Bahman G, Carlos IL, Jr. Unpredictable growth pattern ofcostochondral graft. Plast Reconstr Surg 1992;90:880–886

11. Lata J, Kapila BK. Overgrowth of a costochondral graft in tem-poromandibular joint reconstructive surgery: An uncommoncomplication. Quintessence Int 2000;31:412–414

12. Hossein B, Mohammad HKM, Azita T. Use of activator appli-ances in pediatric patients treated with costochondral grafts fortemporomandibular joint ankylosis: Analysis of 13 cases. J OralMaxillofac Surg 1997;55:1408–1414

13. Samman N, Cheung LK, Tideman H. Overgrowth of a costo-chondral graft in an adult male. Int J Oral Maxillofac Surg1995;24:333–335

14. William HW, Steven LB. Growth center transplantation toreplace mandibular condyles. J Maxillofac Surg 1981;9:50–58

Neuromuscular Hamartomaof the Occipital Nerve:Clinical Report

Afsxin Uysal,* Nezih Sungur,* Ugur Kocxer,* Harun Cxologlu,*Melike Orucx,* Tulin Yaltay

Ankara, Turkiye

Abstract: Neuromuscular hamartoma is a rare benign tumor con-sisted of well-differentiated striated muscle fibers within matureneural elements. Though most cases are composed of solitarymasses associated with major peripheral nerves, multiple tumorsor subcutaneous tumors without any association with peripheralnerves have also been reported. Twenty-three neuromuscular ha-martomas of various anatomical locations have been reported inEnglish literature. There have been no reports of this tumor in

From the *Ankara Training and Research Hospital, Plastic andReconstructive Surgery Clinic, Ankara, Turkiye; yAnkara Trainingand Research Hospital, Pathology Department, Ankara, Turkiye.

Address correspondence to Dr. Afsxin Uysal, Cx. Emecx Bulvarı,C. Atıf Kansu Cad., 11. Sok., Balgat, Ankara, Turkiye.


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association with the occipital nerve. An interesting case of neuro-muscular hamartoma of the occipital nerve is presented and pre-vious reports about this rare pathology is reviewed.

Key Words: Neuromuscular hamartoma, occipitalnerve

‘‘N euromuscular hamartoma,’’ also known as‘‘neuromuscular choristoma’’ or ‘‘benign tri-

ton tumor’’ is a rare benign tumor characterized bythe presence of well-differentiated striated musclefibers within myelinated or nonmyelinated neuralelements.1 It is a developmental or hamartamatouslesion rather than a true neoplasia.2 Diagnosis ismostly in infancy or in early childhood. It can occurin any part of the body but mostly seen as a solitarymass associated with a major peripheral nerve.Cases of neuromuscular hamartoma not associatedwith peripheral nerves have been reported as rareentities.1,3,4,5,6

Twenty-three cases of neuromuscular hamar-toma have been reported in English literature; 8 ofthem were associated with brachial plexus, 6 with sci-atic nerve, 3 with trigeminal nerve, 2 on spinal chord,1 with cochlear nerve, 1 with facial nerve, 1 on opticnerve, and 1 on median nerve. No sex difference ofthe patients was noted.

A case of neuromuscular hamartoma associatedwith occipital nerve is presented with the review ofthe literature on this issue. This case is the first tobe reported on occipital nerve.

CASE REPORT

Eight-year-old girl with a fixed, painless, soft subcu-taneous mass beneath the occipital scalp measuring6 3 5 cm applied to our clinic in 2002. Her parents�history revealed a quite slow enlargement for the last5 years since they had noticed. The patient was oper-ated under general anesthesia with a preliminary di-agnosis of benign soft tissue tumor. Scalp flaps wereelevated for the full exposure of the mass. The masscovered the entire occipital region and could hardlybe distinguished from the surrounding tissues, hada fibrotic nature, and was composed of nodules andchords. As the dissection was carried out, it wasnoticed that the tumor was associated with the occip-ital nerve. The tumor was totally excised by the trans-action of the occipital nerve. After the hemostasis, thescalp flaps were sutured back.

The histopathological evaluation of the tumor re-vealed intense fibrotic tissues composed of collagenfibers among well-differentiated striated muscle fi-

bers and both proliferating and some mature nervefibers. The conclusive diagnosis was ‘‘neuromuscularhamartoma.’’

Complication or recurrence did not occur on the1 year follow-up of the patient.

DISCUSSION

The first report on neuromuscular hamartomabelongs to Orlandi in 1895. He defined a mass thatconsisted of muscle and nerve fibers originating formthe sciatic nerve, but named it as ‘‘rhabdomyoma.’’7

Louhimo and Rapola were the first to define a similartumor as ‘‘intraneural muscular hamartoma.’’8

Neuromuscular hamartomas appear as childhoodtumors,3,5,9 with only a few exceptional reports.7,8

Fig 1 Intraoperative appearance of the lesion.

Fig 2 Histopathological view (H&E, 3 40).

BRIEF CLINICAL NOTES / Uysal et al

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Most of the cases were solitary masses associatedwith major peripheral nerves.3,10 Recurrence is rare,even after partial resection, so salvation of the asso-ciated nerve is recommended to prevent loss of func-tion.3,4,10,11,12 On the other hand, reports of recurrenceafter surgical intervention also exist.3,5

Histopathogenesis of neuromuscular hamar-toma is not fully understood yet. They progressslowly and total cure is possible by convenient surgi-cal resection.6

The first case report of neuromuscular hamar-toma associated with occipital nerve is presentedwith the review of the literature on this rare tumor.As the sacrifice of the occipital nerve did not causeany serious morbidity, total resection of the tumorwas performed.

REFERENCES

1. Maher CO, Spinner RJ, Giannini C, Scheithauer BW, Crum BA.Neuromuscular choristoma of the sciatic nerve. Case report.J Neurosurg 2002;96:1123–1126

2. Giannini C, Reynolds C, Leawitt JA, Schultz GA, Garrity JA,Ebersold MJ, Schithauer BW, Salomao DR. Choristoma of theoptic nerve: Case report. Neurosurgery 2002;50:1125–1128

3. Chen KT. Neuromuscular hamartoma. J Surg Oncol 1984;26:

158–1604. O’Connell JX, Rosenberg AE. Multiple cutaneous neuromuscu-

lar choristomas. Am J Surg Pathol 1990;14:93–965. Awasthi D, Kline DG, Beckman EN. Neuromuscular hamarto-

ma (benign ‘‘triton’’ tumor) of the brachial plexus. Case report.

J Neurosurg 1991;75:795–7976. Gersdorff MC, Decat M, Duprez T. Neuromuscular hamartoma

of the internal auditory canal. Eur Arch Otorhinolaryngol 1996;

253:440–4427. Orlandi E. Sopra un caso di rabdomioma del norvo ischiatico.

Arch Sci Med 1895;19:113–1378. Tiffee JC, Barnes EL. Neuromuscular hamartomas of the head

and neck. Arch Otolaryngol Head Neck Surg 1998;124:212–2169. Louhimo I, Rapola J. Intraneural muscular hamartoma: A re-

port of two cases in small children. J Pediatr Surg 1972;7:

696–69910. Demir Y, Uluoglu O, Ozmen S, Boyacıoglu ZM, Atabay K. Neu-

romuscular hamartoma in the mental region. J Oral Maxillofac

Surg 2003;61:397–40011. Van Dorpe J, Sciot R, De Vos R. Neuromuscular choristoma

(hamartoma) with smooth and striated muscle component:

Case report with immunohistochemical and ultrastructural

analysis. Am J Surg Pathol 1997;21:1090–109512. Bonneau R, Brochu P. Neuromuscular choristoma. A clinico-

pathologic study of two cases. Am J Surg Pathol 1983;7:521–

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surgical techniques alveolar bone grafting

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