craniofacial book

7/28/2019 Craniofacial Book

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1997 Erlanger Health System Tennessee Craniofacial Center 1(800) 418-3223

INTRODUCTION

THE TENNESSEE CRANIOFACIAL CENTER, part of the ErlangerHealth System, is located in Chattanooga, Tennessee. TheCenter specializes in the evaluation and treatment of patientsof all ages with craniofacial deformities. Its services cover a

broad range of reconstructive operations for the treatment ofdeformities of the face and skull resulting from birth defects,tumors and trauma.

Dr. Larry A. Sargent, M.D. is the Medical Director of theTennessee Craniofacial Center. He is a plastic and recon-structive surgeon specializing in craniofacial surgery andmaxillofacial trauma. Before joining The Plastic SurgeryGroup, P.C., of Chattanooga, Dr. Sargent was Director ofCraniofacial Surgery at Walter Reed Army Medical Center inWashington, D.C. He is a graduate of the Georgia Institute of

Technology and Emory University School of Medicine. Hecompleted his general surgery and plastic surgery training atThe John Hopkins Hospital in Baltimore, Maryland andstudied Craniofacial Surgery with Dr. Paul Tessier in Paris,France. Dr. Sargent is Professor and Chairman of theDepartment of Plastic Surgery at the University of TennesseeCollege of Medicine in Chattanooga, a Fellow of theAmerican College of Surgeons, a Fellow of the AmericanAcademy of Pediatrics, and board certified in plastic surgery.He is an active member of the American Association of

Plastic Surgeons, the American Society of Plastic andReconstructive Surgeons, the American Society ofMaxillofacial Surgeons, the American Association ofPediatric Plastic Surgeons, the American Cleft Palate-Craniofacial Association, the Plastic Surgery ResearchCouncil, and numerous regional societies.

The primary purpose of this book is to inform health careprofessionals, as well as parents, of the treatment that isavailable at the Tennessee Craniofacial Center for varioustypes of craniofacial deformities. All patient examples shown

in this book are the reconstructive work of Dr. Larry Sargent.


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PREFACE

The Regional Craniofacial Center offers new hope andpromise to many patients with facial deformities. The effec-tive Craniofacial Center is one that has a dedicated, orga-nized team of professionals of which each member has a

genuine interest in evaluating and treating these types ofdeformities. The team approach remains our standard carefor evaluation; however, the ultimate responsibility is withthe Craniofacial Surgeon for choice of operation and the finalsuccess or failure dependent on his skill, experience andjudgement. I feel, to continue to maintain this level of exper-tise, the Craniofacial Surgeon must devote the majority of hispractice to the treatment of these types of problems.Adequate volumes of patients are necessary to maintain thisexpertise, which enhances safety and insures that optimumresults are obtained. The specialized care and support facili-ties of a major medical center and childrens hospital are alsomandatory. I feel these are the criteria by which a centershould be judged.

This book is organized like a monograph, using mypatients to illustrate current surgical techniques that I use ona regular basis to treat various craniofacial deformities. Itstrongly reflects the comprehensive, coordinated treatmentplan used at our center. In putting together this material, Ihad several goals in mind. First was to educate health pro-fessionals about the new and rapidly growing field of

Dr. Larry A. Sargent


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Craniofacial Surgery. Second, was to provide a reference forpatients and their families concerning treatments that areavailable and the type of results that might be expected. Andfinally, to reflect my own experience and philosophy as relat-

ed to the treatment of these complex problems.

Providing informative material on craniofacial deformi-ties, ultimately results in better patient care. As the primarycare physician and the public become aware of what a centersuch as ours has to offer, expectations will and should con-tinue to rise. It is not enough to correct a deformity; the ulti-mate goal should be to create an attractive face. This requiresa thorough appreciation of facial aesthetics and the constantdesire to strive toward perfection.

Craniofacial Surgery is a unique field in Plastic Surgerythat at its best blends technical skill with artistic creativity.This type of facial reconstruction is often very hard and tir-ing work involving long operations, demanding a certaindedication from the surgeon. However, the treatment ofthese complex problems is both challenging and rewarding.Correcting a facial deformity can very often change a per-sons entire outlook on life, giving them a new self-esteemand a second chance. As this new field of surgery grows withthe advances in technology and surgical techniques, thereshould always be that unfatigable desire that pushes one to

continually strive for the best results possible for eachpatient. I feel this is and will continue to be my philosophy.

Larry A. Sargent, M.D., F.A.C.S., F.A.A.P.Medical Director, Tennessee Craniofacial CenterProfessor and ChairmanDepartment of Plastic SurgeryUniversity of TennesseeChattanooga, Tennessee


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THE CRANIOFACIALDEFORMITY

Diagnosis: Craniofacial deformi-ties, or alterations in the natural form

of the face or skull, can becongenital or acquired. For thosepatients born with craniofacialanomalies, the obstetrician or pedia-trician is the initial point of contactfor appropriate medical treatment.Referrals should be made to a cranio-facial center as soon as it is accept-able for the child to be evaluated.Accurate diagnosis at an early age

not only avoids unnecessary emo-tional distress for the parents andfamily, but it also minimizes poten-tial future problems associated withthe deformities by early correction.For those patients with acquireddeformities as the result of trauma ortumor resection, referral to a cranio-facial center may be desirable to helprestore facial function and appear-ance.

Etiology: While the pattern ofembryonic craniofacial developmenthas been well defined through exten-sive research, very little is knownabout the etiology of many craniofa-cial anomalies. Some are known tobe primarily genetic in nature, whileothers are thought to be caused byenvironmental factors. A combina-

tion of both environment and genet-ics may play a role in the etiology;however, most of the time the causeis unknown.

Craniofacial Team: The care ofcraniofacial patients requires theexpertise of super-specialized profes-sionals from many health care fields.

New advanced computer systems enable the surgeon to performcomplex photographic and radiographic analysis that aid intreatment planning.

Computer images of patient with orbital hypertelorism.


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Multidisciplinary teams have been estab-lished at regional centers to provide thecomprehensive care necessary to ade-quately evaluate and treat craniofacialpatients and their families. No singlephysician can possess the expertise toevaluate and treat all the abnormalities of

these patients.The benefits of the team approach are

numerous. Members of the craniofacialteam work together to ensure that thepatient is evaluated and treated in a coor-dinated manner and that all of his needs,both physical and psychosocial, are met.The team combines the expertise of eachspecialist to provide a level of compre-hensive care that cannot be provided by a

single physician, no matter how rep-utable.

These multidisciplinary craniofacialteams are found at a few major medicalcenters across the U.S. where theresources are available to provide thesafest and most advanced treatment forpatients suffering from facial anomalies.Regionalization also ensures that eachteam has a large enough patient load to

maintain the necessary expertise for prop-er treatment. The more procedures theyperform together, the better the teambecomes. Consequently, operative time isdecreased, complications are minimized,and results are improved. Craniofacialprocedures performed on an irregular oroccasional basis invite disaster and arenot in the best interest of the patient.

The craniofacial team is directed by

the craniofacial surgeon, a plastic surgeonwho has received additional trainingextensively in craniofacial techniques andwhose practice is predominantly dedicat-ed to the treatment of facial anomalies.

At the Tennessee Craniofacial Center,we adhere to Dr. Tessiers principles that,Craniofacial surgery should be per-

formed only if it is the main interest of that surgeon,and he has the support facilities of a major medicalcenter.

Dr. Sargent is the medical director of the centerand the leader of the team. Other disciplines repre-sented on the team include:

Neurosurgery

Ophthalmology Pedodontics/Orthodontics Otolaryngology Anesthesiology Speech Pathology Pediatrics Audiology Psychology Social Service Genetics Nursing

Prosthodontics Clinical Coordinator


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Recent Advancements: A number of advances have been made in surgical tech-nique and technology as applied to craniofacialsurgery. Calvarial bone grafts for the most parthave replaced rib and hip grafts. These outertable split grafts are available in an assortmentof sizes and shapes with less painful donorsites and less resorption compared to rib or hip

bone.

Tremendous radiological advances havebeen made in the past ten years that haveimproved preoperative analysis of craniofacialdeformities. The use of two- and three-dimen-sional CT scans has drastically enhanced ourability to analyze these complex deformities.Computer analysis of photographs and radi-ographs is also available and can provide fur-

ther information for preoperative planning.Another major advancement has been the

application of rigid skeletal fixation to cranio-facial surgery. The new techniques of rigidskeletal fixation combined with wide exposurehave allowed the craniofacial surgeon to obtainmuch better stability and eliminate intermaxil-lary fixation in most cases. This techniqueoffers significant advantages, particularly inchildren. It has improved our overall quality of

results as well as decreasing morbidity.Follow-Up Care: Treatment of craniofacial

problems does not end with surgical restora-tion, but continues for many years. Thisfollow-up should be conducted by the cranio-facial team in order to maintain a continuity ofcare that assures the patient the best long termoutcome. As a child grows and develops,asymmetries may result if areas of the face failto develop equally; therefore, a childs growth

and development must be routinely followed.Sometimes it may be necessary to repair theseasymmetries surgically. Often, major craniofa-cial deformities require multiple, staged proce-dures performed at different ages. Oncetreatment is initiated, it is important that fol-low-up care continues.

Each patient referred to the TennesseeCraniofacial Center undergoes an evaluationby the principle members of the team, withadditional team members called in dependingupon the individual needs of that patient. Agroup conference is held following these evalu-ations to discuss each patient. The goal of theteam is to diagnose the physical and psychoso-cial problems and formulate a coordinated,comprehensive treatment plan. This is fol-lowed by execution of the treatment plan at theappropriate time, longitudinal follow-up, andcollection of data on the teams activities andresults.

Surgical Plan Established: WhenCraniofacial Surgery is recommended as treat-ment for a specific anomaly, there are two dis-

tinct goals of this surgery. First, is the attempt torestore the patient to as near normal function aspossible and to prevent future dysfunction.Secondly, surgery may be necessary to correctstructural disfigurement in order to achieve opti-mal appearance. A patient suffering from a facialdeformity may experience problems in dealing

with his disfigurement emotionally or socially.Often, improvements in appearance followingcraniofacial surgery can lead to increases in self-esteem, self-confidence and social acceptance.The psychological benefit of craniofacial surgeryis an extremely important goal of the surgery.

Computer simulated images. Top left shows chin

advancement with rhinoplasty.


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ENOPHTHALMOS

Enophthalmos can be definedas the relative recession (back-ward or downward displacement)of the globe into the bony orbit.The three basic structures thatdetermine globe position are the

bony orbits, the ligament systemand the orbital fat. Displacementof the orbital walls with enlarge-ment of the bony orbit may be themajor components in the produc-tion of enophthalmos in orbitalfractures. Post-traumatic enoph-thalmos is frequently seen and isthe result of disruption of thebony orbit and ligament system

with displacement of the orbitalsoft tissue. This presents clinicallyas a sunken appearance to the eyewith pseudoptosis and deepeningof the supratarsal fold. Treatmentinvolves reconstruction of thebony orbit with restoration ofbony orbital volume and reposi-tioning of the globe. The use ofcraniofacial techniques allows thisto be accomplished with minimalcomplications.

This patient has traumatic enophthalmos of the left orbit.Note sunken-in appearance of the left eye due to unre-paired orbital fracture.

This patient has severe displace-ment of the right eye caused bytumor.

Postoperative result after tumorresection and orbital reconstruc-tion with repositioning of theright eye.

Postoperative result after orbital reconstruction and repo-sitioning of eye.

Enophthalmos

Enophthalmos


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Clefts of the Lip and Palate


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Each year approximately 227,500 or 7percent of births in the United States areaffected by birth defects of the head andface. The most common of these areclefts of the lip and palate which occuronce in every 700 births. Clefts occur ininfants of all races with a 2:1 male tofemale ratio. The incidence of clefts ishighest in the Asian population andlowest in African Americans. Of all oro-facial clefts, 21 percent present as cleftlip only (unilateral and bilateral), 46 per-cent present as cleft lip and palate, whilethe remaining 33 percent have cleftpalate alone.

What is a cleft? A cleft is a divisionor separation of parts of the lip or roof ofthe mouth that is formed during theearly months of development of the

unborn child. All of the parts of the lipor roof of the mouth are present; theysimply failed to fuse in a normal way.Surgical intervention is necessary toalign the parts and join them. Often thebones of upper jaw (maxilla) and/or theupper gum are affected. Acleft lip can beincomplete with a variable degree ofnotching of the lip, or complete, extend-ing through the lip and into the nose.

Preoperative Postoperative

CLEFTS OF THE

LIP AND PALATE

Variations in clefts of the palate.

A). Cleft of soft palate only. B). Incomplete cleftof soft and hard palate. C). Complete unilateralcleft palate. D). Complete bilateral cleft palate.


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FEEDING AN INFANT

WITH A CLEFT

Feeding an infant is important not only inproviding nourishment, it also provides anintimacy and closeness for both the parentand the child. Infants with a cleft of the lip or

of the soft palate seldom have problems withfeeding either by bottle or breast.

In babies with clefts of the hard palate, theopening in the roof of the mouth often causesdifficulty in creating adequate pressure onthe nipple, thus creating an inability to suckwell enough to get adequate nourishment.Feeding the infant takes patience and prac-tice. At our center we recommend the use of asoft squeezable plastic bottle like Mead

Johnson with an orthodontic nipple such asNuk. You can increase the flow by gentlysqueezing or putting pressure on the bottle. Itis important to feed the infant before he/shebecomes too hungry. Position the infant in anupright position with the head tilted backslightly. This position allows the milk to flowdown into the throat and less into the nose.Infants with clefts do swallow more air andneed to be burped more frequently. At first, itmay take extra time, but this will steadilydecrease . Feeding time of the newborn variesfrom 20-30 minutes. When feeding takeslonger than 45 minutes, the infant may beburning up calories necessary to gain weightIf this occurs the feeding consultant should becontacted to help with the feeding technique.

Breast feeding the newborn with a cleft ofthe hard palate is often unsuccessful.Generally the infant cannot produce enough

negative pressure to obtain ample breast milkto provide adequate nourishment. Using abreast pump to extract the milk and feedingthe infant breast milk from a squeezablebottle is recommended.

Clefts of the palate can vary in severity.Some may involve just the uvula and the softpalate. These are incomplete clefts of thepalate. Others extend the length of the palateand are complete clefts. They may involveone side of the palate (unilateral) or bothsides (bilateral).

Etiology: The exact cause of lip andpalatal clefting is not known, but mostexperts feel that it is due to both genetic andenvironmental factors. Clefts are associatedwith abnormalities in the genes which maybe a result of inheritance or from a sponta-neous mutation during fetal development.We recommend genetics counseling to dis-cuss causes of the cleft and the recurrencerisk factors.

Team Assessment: Children born withclefts should be carefully assessed by thecraniofacial team in order to detect potential-ly serious abnormalities that can be associat-ed with clefting. There are over 150syndromes that include cleft lip or palate intheir differential diagnosis. Generally, cleft-ing is the only congenital abnormality thatthe child has, but nearly 15 percent of all cleftlip or palate patients present clinically with

multiple problems.

The team concept allows a systematic,comprehensive treatment plan to be devel-oped and allows the team members to worktogether to identify problems before theybecome significant. The most common spe-cialities involved in the care of a child with acleft are: plastic surgery, otolaryngology, den-tistry, audiology, speech pathology, geneticsand pediatrics. Once a complete assessment

of the child with a cleft has been performed,a plan for treatment can be outlined.


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SURGERY

Cleft Lip Repair - The objec-tive in repairing the lip is toclose the cleft to create a pleas-ing face that will develop nor-mally with minimal scarring.Closure of the lip is performed

by the plastic surgeon when thebaby is approximately 3 monthsof age and weighs at least 10pounds. When there is involve-ment of the alveolus and palate,an orthodontic appliance maybe placed in the maxillary seg-ments as the first procedure.This is performed by the teamdentist as an outpatient surgical

procedure. The appliance isused to align the alveolus so thatit can be repaired (gingivope-riosteoplasty) at the time of thelip repair or lip adhesion. Thisimproves nasal support on thecleft side and creates a tunnelthat should develop bone, clos-ing the cleft. If the alveolus is notclosed in infancy, then the alveo-lar ridges will be orthodontical-ly aligned and a bone graftperformed to stabilize the maxil-la (5-10 years of age). Correctionof the nasal deformity is usuallyperformed at the time of liprepair. Additional proceduresmay be necessary to enhance theappearance of either the lip ornose.

Unilateral Cleft Lip with Custom Latham

Photos to the right:

A unilateral cleft lip is shown. A customfitted Latham appliance has been fabricatedand placed to rotate alveolar segmentstogether. Postoperative result is shown afterrepair of alveolar cleft and lip.


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UNILATERAL

CLEFT LIP REPAIR

A unilateral cleft lip results fromfailure of the union of the maxillaryand median nasal processes, thuscreating a split or cleft in the lip on

either the left or right side. It may bejust a notching of the lip or extendcompletely through the lip into thenose and palate. A number of proce-dures have been described to repairthe unilateral cleft lip. The proce-dure used at our Center is theMillard rotation advancement tech-nique. The procedure is designed toreconstruct the lip, muscle, oralmucosa, and to reposition the nose.

It is performed under general anes-thesia with surgery lasting 2-3 hoursand a hospital stay of 2-4 days.Special considerations are necessaryfor feeding and positioning theinfant postoperatively. The babyselbows are restrained from bendingto prevent him/her from disruptingthe nose or lip. Positioning the childin an infant seat keeps him/her from

rolling over and injuring the lip ornose. Pacifiers and nipples are notallowed. The baby is fed with a spe-cial syringe feeder with a soft tube. Ittakes approximately 3 weeks for thewound to gain enough strength todiscontinue the above precautions.The lip scar is initially red andswollen, but it begins to mature andimprove in appearance in six-twelvemonths.

Unilateral Cleft Lip




Photos of infants who underwent repair ofunilateral cleft lip with rotation advance-ment technique.



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BILATERAL

CLEFT LIP

The bilateral cleft lip involvesseparation of the lip alongphiltral lines, isolating the centralsegment (prolabium). Fifteen per-

cent of children born with cleftlips have bilateral clefts. Theassociated nasal deformity is usu-ally more severe than the unilat-eral cleft due to a very shortcolumella and flaring of both nos-trils. Surgical correction of thebilateral cleft lip is usually per-formed in one procedure at threemonths of age; however, the pro-cedure may be staged, closing

one cleft at a time. Rotation of thenostrils to a more normal positionis performed in the first proce-dure. A second procedure is per-formed by 2-3 years of age tolengthen the columella. Patientswith complete bilateral cleft lipsfrequently require additional pro-cedures to enhance the appear-ance of the lip and nose.

Performed under general anes-thesia, the operation generallyrequires 2-3 hours. A hospitalstay of 2-4 days should be expect-ed. Feeding, positioning andelbow restraints are the same asthose for repair of the unilateralcleft lip.

Bilateral Cleft Lip





Photos of infants who underwent onestage repair of bilateral cleft lip.


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CLEFT PALATE REPAIR

The objective of cleft palatesurgery is to close the palate torestore normal function to eatingand drinking and to enhance thedevelopment of normal speech.

Clefts of the palate can occur asisolated deformities or in combina-tion with a cleft of the lip. Cleftpalates result from failure of fusionof the embryonic facial processesresulting in a fissure through thepalate. This may be complete(extending through the hard andsoft palates) or any degree ofincomplete (partial cleft). Thepalate forms the roof of the oralcavity and the floor of the nose;thus, a cleft causes a free communi-cation between these two cavities.As a result, treatment of palatalclefts is complex because of poten-tial problems with feeding, speech,middle ear infections, occlusionand jaw alignment.

Surgical treatment of the cleft

palate is best accomplished in onesurgical procedure before the childreaches 12-14 months of age. Thecleft palate is surgically closed byelevating two muscoperiostealflaps. The levator muscles are ele-vated, redirected and repaired; anda three layer closure of nasalmucosa, muscle and oral mucosaaccomplished. Surgery under gen-eral anesthesia usually lasts about

2 hours. Special precautions asthose after the repair of the cleft lipare necessary for 2-3 weeks. Weprefer that the child be weanedfrom the bottle and pacifier prior tothe palatal repair. No hard orcrunchy foods are allowed for 3weeks post operatively.

Cleft Palate Repair:

Closure of cleft palate with pushback palatoplasty. A). Twomucoperiosteal flaps are outlined. B). Flaps are elevated off thehard palate. C,D). The abnormal levator muscle insertion to thehard palate is identified and cut free. E). The nasal lining isclosed as a separate layer and the levator muscle reapproximated.F). The palatal mucoperiosteal flaps are closed in a V-Y fashion.


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PHARYNGEAL FLAP

Approximately 70-80 percent ofall cleft palate patients will developvelopharyngeal competence afterpalate closure and thus the potentialfor normal speech. The remaining20-30 percent will require speech

therapy and/or an additional surgi-cal procedure called a pharyngealflap. To correct persistent hypernasalspeech, this procedure involves rais-ing a flap of tissue from the posteriorpharynx and inserting it into the softpalate. This flap is indicated whenthe repaired palate is too short or themuscles do not function properly,causing a persistent hypernasal

speech. The procedure is performedusually after the age of 4-5 whenspeech and velopharyngeal compe-tence can be thoroughly assessedand before the child begins school.

LATE CLEFT

TREATMENT

The Craniofacial Center can also

help those individuals that havegrown up without access to a com-prehensive, coordinated teamapproach. For adults with speechproblems, the previously mentionedpharyngeal flap, combined with anintensive regimen of speech therapy,can produce significant improve-ments. Orthognatic surgery is avail-able to patients with deformities ofthe jaws to improve their appearanceas well as to correct dental occlusion.For soft tissue revision of a severelytightened or notched upper lip, anAbbe flap is the surgical option. Thisprocedure is usually indicated inbilateral cleft patients who have ashort or deficient columella and atightened upper lip. This operation

Pharyngeal Flap:

The pharyngeal flap procedure for hypernasal speech. A superior-ly based flap of tissue is raised from the posterior pharynx andsutured to the soft palate thereby decreasing the amount of airthrough the nose. Lateral ports or holes are left so that the nosewill not be obstructed.

Inadequate Closure

Adequate Closure

Corrected Closure


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patients will have velopharyngeal incompe-tence or hypernasal speech after surgery, andmay require a pharyngeal flap to correct itaround the age of 4-5 years.

DENTAL

Clefts of the palate generally have an effecton dental development. In the area of the cleft,teeth often erupt in a crooked position withextra teeth or missing teeth being common inthe cleft area. Radiographs are often taken todetermine the exact position of the teeth.Dental problems have an effect on speech,chewing, appearance and frequently requireorthodontic treatment. Early orthodontic inter-vention may require a palatal expansion devicewith further alignment of the dental arches.

Later treatment after the primary teeth haveerupted can begin at 10-12 years of age.Orthognathic surgery may be indicated if amalocclusion develops due to abnormalgrowth of the maxilla.

PIERRE ROBIN

SYNDROME

This syndrome was described in 1923 by

Pierre Robin in which he described airwayobstruction associated with glossoptosis andhypoplasia of the mandible. Today this syn-drome is characterized by retrognathia ormicrognathia, glossoptosis, and airway obstruc-tion. An incomplete cleft of the palate is associ-ated with the syndrome in approximately 50%of these patients.

In patients with micrognathia (small jaw) orretrognathia, the chin is posteriorly displaced

causing the tongue to fall backward toward theposterior pharyngeal wall. This results inobstruction of the airway on inspiration. Cryingor straining by these children can often keepthe airway open. However, when the childrelaxes or falls asleep, airway obstructionoccurs. Due to these respiratory problems, feed-ing may become very difficult. This can lead to


can add fullness to the upper lip as well aslengthen the columella. A number of addi-tional surgical therapies, similar to the onesdescribed, are available to patients whodesire further improvements.

HEARING

Children with cleft palate have a higherincidence of hearing problems. TheEustachian tube connects the middle earspace to the back of the throat. It normallyopens and shuts to relieve pressure thatbuilds up behind the ear drum. If theEustachian tube does not open, then the pres-sure increases until mucus or fluid accu-mulates behind the eardrum. The musclesresponsible for opening the Eustachian tube

do not function as well in children with cleftpalates resulting in more frequent problemswith fluid, otitis media and ear infectionswhich can be very painful. Because of thisproblem, it is important to have the infantshearing tested during the first few months. Ifhearing is impaired by fluid buildup orunequal pressure, it may be necessary for theotolaryngologist to place pressure equalizing(PE) tubes. Tubes are often placed at the time

of the lip or palate surgery. It is crucial thatchildren with cleft palates have regular hear-ing tests to monitor middle ear problems thatcould alter the development of normal hear-ing as well as speech. As the child grows, thefrequency of ear infections and fluid in theears seem to decrease.

SPEECH

Speech development in children with cleftlip only should be normal. The unrepairedcleft palate causes speech to sound hyper-nasal because air passes through the nosewhile talking. Most speech sounds requirethe nose to be closed off from the mouth. Cleftpalate surgery usually remedies the problem,but speech therapy is still recommended.Approximately 20-30 percent of cleft palate


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a sequence of events: glossoptosis,airway obstruction, crying or strain-ing with increased energy expendi-ture and decreased oral intake. Thisvicious cycle of events if untreatedcan led to exhaustion, cardiac fail-ure, and ultimately death.

Treatment of this syndrome canbe divided into conservative therapyversus surgical intervention. Themajority of these patients can bemanaged by placing the infant in theprone position until adequategrowth of the jaw occurs. This caus-es the jaw and the tongue to fall for-ward opening the airway. If this typeof treatment fails the infant should

then be considered for a tongue-lipadhesion (a procedure to pull thetongue forward) or a tracheostomy.

In children with severe underde-velopment of the lower jaw, a newtechnique called mandibular boneexpansion is now available. Thistechnique also called distractionosteogenesis involves placement ofan expansion device that is turned

daily to slowly lengthen the jaw. Anexternal incision is required to makea surgical cut through the jaw bonewith placement of pins that aresecured to the expansion device.Once the amount of expansion of the

bone has been obtained (4-5 weeks)the device is then kept in place untilthe bone gap heals with new boneformation (8 weeks). This techniquecan be performed at a very early agewhich is a significant advantageover the traditional technique oflower jaw lengthening.

Results after removal of lengthening devices. Patient now readyfor removal/closure of tracheostomy.

Bilateral mandibular lengthening devices surgically placed withlengthening in progress.

Two year old with Pierre Robin and severe airway obstruc-tion requiring tracheostomy.

Pierre Robin


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Clefts of the Lip and Palate


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Each year approximately 227,500 or 7percent of births in the United States areaffected by birth defects of the head andface. The most common of these areclefts of the lip and palate which occuronce in every 700 births. Clefts occur ininfants of all races with a 2:1 male tofemale ratio. The incidence of clefts ishighest in the Asian population andlowest in African Americans. Of all oro-facial clefts, 21 percent present as cleftlip only (unilateral and bilateral), 46 per-cent present as cleft lip and palate, whilethe remaining 33 percent have cleftpalate alone.

What is a cleft? A cleft is a divisionor separation of parts of the lip or roof ofthe mouth that is formed during theearly months of development of the

unborn child. All of the parts of the lipor roof of the mouth are present; theysimply failed to fuse in a normal way.Surgical intervention is necessary toalign the parts and join them. Often thebones of upper jaw (maxilla) and/or theupper gum are affected. Acleft lip can beincomplete with a variable degree ofnotching of the lip, or complete, extend-ing through the lip and into the nose.


CLEFTS OF THE

LIP AND PALATE

Variations in clefts of the palate.

A). Cleft of soft palate only. B). Incomplete cleftof soft and hard palate. C). Complete unilateralcleft palate. D). Complete bilateral cleft palate.


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FEEDING AN INFANT

WITH A CLEFT

Feeding an infant is important not only inproviding nourishment, it also provides anintimacy and closeness for both the parentand the child. Infants with a cleft of the lip or

of the soft palate seldom have problems withfeeding either by bottle or breast.

In babies with clefts of the hard palate, theopening in the roof of the mouth often causesdifficulty in creating adequate pressure onthe nipple, thus creating an inability to suckwell enough to get adequate nourishment.Feeding the infant takes patience and prac-tice. At our center we recommend the use of asoft squeezable plastic bottle like Mead

Johnson with an orthodontic nipple such asNuk. You can increase the flow by gentlysqueezing or putting pressure on the bottle. Itis important to feed the infant before he/shebecomes too hungry. Position the infant in anupright position with the head tilted backslightly. This position allows the milk to flowdown into the throat and less into the nose.Infants with clefts do swallow more air andneed to be burped more frequently. At first, itmay take extra time, but this will steadilydecrease . Feeding time of the newborn variesfrom 20-30 minutes. When feeding takeslonger than 45 minutes, the infant may beburning up calories necessary to gain weightIf this occurs the feeding consultant should becontacted to help with the feeding technique.

Breast feeding the newborn with a cleft ofthe hard palate is often unsuccessful.Generally the infant cannot produce enough

negative pressure to obtain ample breast milkto provide adequate nourishment. Using abreast pump to extract the milk and feedingthe infant breast milk from a squeezablebottle is recommended.

Clefts of the palate can vary in severity.Some may involve just the uvula and the softpalate. These are incomplete clefts of thepalate. Others extend the length of the palateand are complete clefts. They may involveone side of the palate (unilateral) or bothsides (bilateral).

Etiology: The exact cause of lip andpalatal clefting is not known, but mostexperts feel that it is due to both genetic andenvironmental factors. Clefts are associatedwith abnormalities in the genes which maybe a result of inheritance or from a sponta-neous mutation during fetal development.We recommend genetics counseling to dis-cuss causes of the cleft and the recurrencerisk factors.

Team Assessment: Children born withclefts should be carefully assessed by thecraniofacial team in order to detect potential-ly serious abnormalities that can be associat-ed with clefting. There are over 150syndromes that include cleft lip or palate intheir differential diagnosis. Generally, cleft-ing is the only congenital abnormality thatthe child has, but nearly 15 percent of all cleftlip or palate patients present clinically with

multiple problems.

The team concept allows a systematic,comprehensive treatment plan to be devel-oped and allows the team members to worktogether to identify problems before theybecome significant. The most common spe-cialities involved in the care of a child with acleft are: plastic surgery, otolaryngology, den-tistry, audiology, speech pathology, geneticsand pediatrics. Once a complete assessment

of the child with a cleft has been performed,a plan for treatment can be outlined.


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SURGERY

Cleft Lip Repair - The objec-tive in repairing the lip is toclose the cleft to create a pleas-ing face that will develop nor-mally with minimal scarring.Closure of the lip is performed

by the plastic surgeon when thebaby is approximately 3 monthsof age and weighs at least 10pounds. When there is involve-ment of the alveolus and palate,an orthodontic appliance maybe placed in the maxillary seg-ments as the first procedure.This is performed by the teamdentist as an outpatient surgical

procedure. The appliance isused to align the alveolus so thatit can be repaired (gingivope-riosteoplasty) at the time of thelip repair or lip adhesion. Thisimproves nasal support on thecleft side and creates a tunnelthat should develop bone, clos-ing the cleft. If the alveolus is notclosed in infancy, then the alveo-lar ridges will be orthodontical-ly aligned and a bone graftperformed to stabilize the maxil-la (5-10 years of age). Correctionof the nasal deformity is usuallyperformed at the time of liprepair. Additional proceduresmay be necessary to enhance theappearance of either the lip ornose.

Unilateral Cleft Lip with Custom Latham


A unilateral cleft lip is shown. A customfitted Latham appliance has been fabricatedand placed to rotate alveolar segmentstogether. Postoperative result is shown afterrepair of alveolar cleft and lip.


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UNILATERAL

CLEFT LIP REPAIR

A unilateral cleft lip results fromfailure of the union of the maxillaryand median nasal processes, thuscreating a split or cleft in the lip on

either the left or right side. It may bejust a notching of the lip or extendcompletely through the lip into thenose and palate. A number of proce-dures have been described to repairthe unilateral cleft lip. The proce-dure used at our Center is theMillard rotation advancement tech-nique. The procedure is designed toreconstruct the lip, muscle, oralmucosa, and to reposition the nose.

It is performed under general anes-thesia with surgery lasting 2-3 hoursand a hospital stay of 2-4 days.Special considerations are necessaryfor feeding and positioning theinfant postoperatively. The babyselbows are restrained from bendingto prevent him/her from disruptingthe nose or lip. Positioning the childin an infant seat keeps him/her from

rolling over and injuring the lip ornose. Pacifiers and nipples are notallowed. The baby is fed with a spe-cial syringe feeder with a soft tube. Ittakes approximately 3 weeks for thewound to gain enough strength todiscontinue the above precautions.The lip scar is initially red andswollen, but it begins to mature andimprove in appearance in six-twelvemonths.

Unilateral Cleft Lip




Photos of infants who underwent repair ofunilateral cleft lip with rotation advance-ment technique.



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BILATERAL

CLEFT LIP

The bilateral cleft lip involvesseparation of the lip alongphiltral lines, isolating the centralsegment (prolabium). Fifteen per-

cent of children born with cleftlips have bilateral clefts. Theassociated nasal deformity is usu-ally more severe than the unilat-eral cleft due to a very shortcolumella and flaring of both nos-trils. Surgical correction of thebilateral cleft lip is usually per-formed in one procedure at threemonths of age; however, the pro-cedure may be staged, closing

one cleft at a time. Rotation of thenostrils to a more normal positionis performed in the first proce-dure. A second procedure is per-formed by 2-3 years of age tolengthen the columella. Patientswith complete bilateral cleft lipsfrequently require additional pro-cedures to enhance the appear-ance of the lip and nose.

Performed under general anes-thesia, the operation generallyrequires 2-3 hours. A hospitalstay of 2-4 days should be expect-ed. Feeding, positioning andelbow restraints are the same asthose for repair of the unilateralcleft lip.

Bilateral Cleft Lip





Photos of infants who underwent onestage repair of bilateral cleft lip.


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CLEFT PALATE REPAIR

The objective of cleft palatesurgery is to close the palate torestore normal function to eatingand drinking and to enhance thedevelopment of normal speech.

Clefts of the palate can occur asisolated deformities or in combina-tion with a cleft of the lip. Cleftpalates result from failure of fusionof the embryonic facial processesresulting in a fissure through thepalate. This may be complete(extending through the hard andsoft palates) or any degree ofincomplete (partial cleft). Thepalate forms the roof of the oralcavity and the floor of the nose;thus, a cleft causes a free communi-cation between these two cavities.As a result, treatment of palatalclefts is complex because of poten-tial problems with feeding, speech,middle ear infections, occlusionand jaw alignment.

Surgical treatment of the cleft

palate is best accomplished in onesurgical procedure before the childreaches 12-14 months of age. Thecleft palate is surgically closed byelevating two muscoperiostealflaps. The levator muscles are ele-vated, redirected and repaired; anda three layer closure of nasalmucosa, muscle and oral mucosaaccomplished. Surgery under gen-eral anesthesia usually lasts about

2 hours. Special precautions asthose after the repair of the cleft lipare necessary for 2-3 weeks. Weprefer that the child be weanedfrom the bottle and pacifier prior tothe palatal repair. No hard orcrunchy foods are allowed for 3weeks post operatively.

Cleft Palate Repair:

Closure of cleft palate with pushback palatoplasty. A). Twomucoperiosteal flaps are outlined. B). Flaps are elevated off thehard palate. C,D). The abnormal levator muscle insertion to thehard palate is identified and cut free. E). The nasal lining isclosed as a separate layer and the levator muscle reapproximated.F). The palatal mucoperiosteal flaps are closed in a V-Y fashion.


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PHARYNGEAL FLAP

Approximately 70-80 percent ofall cleft palate patients will developvelopharyngeal competence afterpalate closure and thus the potentialfor normal speech. The remaining20-30 percent will require speech

therapy and/or an additional surgi-cal procedure called a pharyngealflap. To correct persistent hypernasalspeech, this procedure involves rais-ing a flap of tissue from the posteriorpharynx and inserting it into the softpalate. This flap is indicated whenthe repaired palate is too short or themuscles do not function properly,causing a persistent hypernasal

speech. The procedure is performedusually after the age of 4-5 whenspeech and velopharyngeal compe-tence can be thoroughly assessedand before the child begins school.

LATE CLEFT

TREATMENT

The Craniofacial Center can also

help those individuals that havegrown up without access to a com-prehensive, coordinated teamapproach. For adults with speechproblems, the previously mentionedpharyngeal flap, combined with anintensive regimen of speech therapy,can produce significant improve-ments. Orthognatic surgery is avail-able to patients with deformities ofthe jaws to improve their appearanceas well as to correct dental occlusion.For soft tissue revision of a severelytightened or notched upper lip, anAbbe flap is the surgical option. Thisprocedure is usually indicated inbilateral cleft patients who have ashort or deficient columella and atightened upper lip. This operation

Pharyngeal Flap:

The pharyngeal flap procedure for hypernasal speech. A superior-ly based flap of tissue is raised from the posterior pharynx andsutured to the soft palate thereby decreasing the amount of airthrough the nose. Lateral ports or holes are left so that the nosewill not be obstructed.

Inadequate Closure

Adequate Closure

Corrected Closure


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DENTAL



PIERRE ROBIN

SYNDROME







HEARING




SPEECH



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Pierre Robin


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DENTAL



PIERRE ROBIN

SYNDROME







HEARING




SPEECH



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Pierre Robin


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Ear Reconstruction


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Reconstruction of the ear is one ofthe most challenging problems fac-ing a reconstructive surgeon as itdemands precise technique com-bined with artistic creativity.Microtia is a congenital deformity ofthe external ear where the auricle(the external ear) is severelydeformed. There may be a spectrumof external ear deformities with var-ious degrees of involvement of themiddle and inner ear. This type of

ear deformity is commonly seen inpatients with hemifacial microsomiaand Treacher-Collins syndrome.

Psychological effects of an eardeformity play a significant role intiming of reconstruction. Most sur-geons prefer to initiate treatmentwhen the patient is between 5 and 7years of age since this early inter-vention will reduce anxiety as a

result of peer pressure. This alsoallows for sufficient rib growth toprovide the quantity of cartilageneeded by the surgeon for adequateframework fabrication. Surgery atthis time can give a more consistentresult than earlier intervention dueto the fact that the child has had achance to grow, thus making it easi-er for the surgeon to balance the size

and shape of the reconstructed earto the childs normal ear.

MICROTIA

The treatment of microtiainvolves surgical reconstruction ofthe external ear framework. Ear

EAR RECONSTRUCTION


Costal cartilage harvested

from left chest wall.

Cartilage carved into ear

framework.

Microtia

reconstruction requires a carefully planned, stagedreconstruction that involves 3-4 operative procedures.The first procedure involves the construction of the car-tilage framework for the ear. Under general anesthesia,donor cartilage for the frame is obtained en bloc from the

Tennessee Craniofacial Center 1(800) 418-32231997 Erlanger Health System


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rib area contralateral to the ear being recon-structed to take full advantage of the carti-lages natural curvature. Working frompre-surgical templates that have been drawnas well as from photographs, the surgeon thencarves the cartilage into its new shape andcarefully positions the graft into position. Theoverlying skin then redrapes to the newlycarved cartilage framework. Subsequent oper-ations are required to rotate the lobule and toelevate the framework into its final position.

Costal cartilage harvested fromchest wall.

Cartilage carved into earframework.

It is of significance to note that if there isnormal hearing in one ear, surgery to improvehearing in the abnormal ear is not recom-mended. Almost 90% of all patients withmicrotia have only unilateral involvement andquickly adjust to this condition followingbirth. Potential gains from working on the

middle ear are outweighed by the inherentrisks of the surgery itself. Therefore, middleear surgery should be performed only on thetrue bilateral microtia patient or the patientwith significant hearing loss in both ears.

Nine year old with left microtia.

Microtia

Postoperative result after staged ear reconstruction.



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Reconstruction of traumatic ear deformity

TRAUMATIC EAR

DEFORMITIES

The traumatic amputation ofan ear is another circumstance inwhich this type of staged carti-

lage reconstruction can be effec-tively used. The amount of earloss determines the types andstages of reconstruction needed.If only a small part of the ear islost from trauma or tumor resec-tion, then helical or rim advance-ment flaps may be used toreconstruct this portion. If largersections are lost then a stagedreconstruction is necessary.

Effective ear reconstruction isdependent upon meticulous sur-gical technique and careful pre-operative planning.

Microtia

Four year old born withright microtia.

Appearance of ear after first stagereconstruction.

Postoperative result after stagedreconstruction.


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Aquired Deformity (Human Bite to Ear)

Human bite to ear with loss ofmiddle third.

Helical rim flap reconstruction. Postoperative result.

Protruding Ears

Eight year old with bilateralprotruding ears.

Postoperative result afterotoplasty.

OTOPLASTY

Another congenital deformi-ty of the ears is called prominentor protruding ears. The earsprominence is due to lack ofdevelopment of the antihelical

fold. The primary reason to cor-rect this deformity is to elimi-nate the psychological traumathat this condition can cause.Peer ridicule can be severe withthis particular deformity. Theoperation designed to correctthis problem involves recreatingthe antihelical fold curling orsetting the ears back closer to the

head. With experience, consis-tently good results can beobtained.


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Craniosynostosis


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Craniosynostosis can be defined as thepremature closing of one or more of thenormally present bony gaps between thedifferent bones of the skull. These linearareas where the bones have not yet healedtogether are called cranial sutures. Theyintersect in certain areas of the skull form-ing soft spots or fontanelles. The cranialsuture lines or non-ossified zones accom-modate the brains growth and expansion

during the first years of life. The brainincreases in volume about 2-1/2 to 3 timesthe first two years of life with these non-ossified areas of bone remaining open for avariable length of time and then closing in apredictable manner. Premature synostosisor closure of the suture will stop the growthof the skull in the direction perpendicularto that suture; however, growth will contin-ue in the direction parallel to the suture.The result is that synostosis of a particularsuture will alter the shape of the skull in apredictable manner with recognizable pat-terns. With multiple suture involvement,increased intracranial pressure can occursince the skull cannot sufficiently expand toaccommodate normal brain growth. Inmoderate craniosynostosis with only onesuture involved increased intracranial pres-


3-D Scan of Sagittal Synostosis

CRANIOSYNOSTOSIS

sure has been found to be present in approxi-mately 13-23% of patients.

The surgical treatment of craniosynostosishas been transformed by the development andapplications of craniofacial techniques toreshape the skull and upper face in the infant.Functionally, the goal in treatment is to releasethe fused sutures to prevent any problems asso-ciated with increased intracranial pressure aswell as creating the potential for normal growth.Cosmetically, the goal is to obtain normal shapeof the skull and face, thus minimizing psychoso-cial problems.


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together to insure that the patients are evalu-ated and treated in a coordinated manner andthat all their needs, both physical and psy-chosocial, are met. The team combines theexpertise of each specialist to provide a levelof comprehensive care that cannot be provid-ed by a single physician. The primary physi-cian or pediatrician plays a key role in earlydiagnosis and referral so that a successfultreatment is accomplished.

Surgical Treatment: Tremendousadvancements have been made in the treat-ment of infants with isolated craniosynostosisdue to the application of craniofacial surgeryprinciples. Past treatments such as stripcraniectomies which remove only the fusedsuture have resulted in unpredictable results

These procedures have often resulted in skulland facial deformities that are difficult to cor-rect in the older child. Current surgical treat-ment involves a team approach with acombined expertise of a neurosurgeon andcraniofacial surgeon. The goal of surgery is toestablish normal contour of the brow, fore-head and skull; and to allow the brain togrow and expand in a normal manner withrelease of synostosis. The timing of surgicalcorrection depends on the age at diagnosis

and referral. In our experience we feel thatthe best results are obtained when infantsundergo surgical repair between 4 and 8months of age. This period has severaladvantages:

(1) remodeling is easier because the bone isvery malleable;

(2) rapid brain growth benefits boneremodeling;

(3)bone defects heal rapidly.Early correction also prevents further

deformity caused by unreleased, fusedsutures. Therefore, early diagnosis and refer-ral to a craniofacial center is crucial. We advo-cate extensive release of the fused suture,total reshaping of the brow, superior orbits,and skull as necessary between 4 and 8months of age. Older patients will require

Approximately one infant in every onethousand births will have a premature clo-sure of a cranial suture associated with askull deformity. Infants born with abnormalskull shapes should be suspected of havingcraniosynostosis. However, abnormal skullshapes at birth may be related to fetal headposition or birth trauma; this type of defor-mity will usually correct itself in severalmonths. True craniosynostosis will notimprove with time and usually worsens withgrowth. On examination palpation of theabnormal portion of the head often reveals aridge in the area of the fused suture. Oncecraniosynostosis is suspected, the diagnosiscan be verified with plain skull radiographsand CT scans. We routinely obtain CT scanswith three-dimensional reconstructions to

confirm the diagnosis, rule out other intracra-nial abnormalities, and to fully assess thedeformity. The 3-D scans can very clearlyshow the skull shape and help in pre-opera-tive planning. Isolated craniosynostosis mustbe distinguished from a syndrome that has asone of its components craniosynostosis. Thespecific diagnosis is an important startingpoint in treatment planning.

Etiology: While the pattern of embryonic

craniofacial development has been welldefined through extensive research, very lit-tle is known about the etiology of many cran-iofacial anomalies. There is strong evidencefor a genetic role in the occurrence of somecraniosynostosis. An inherited tendency orfamily history has been reported in manycases in the literature, however, most cases ofisolated craniosynostosis are usually spo-radic in occurrence with no known etiology.

Team Evaluation: Patients with cran-iosynostosis need to be evaluated and treatedat a recognized craniofacial center with ateam approach. We have a multidisciplinaryteam at our center to provide the comprehen-sive care necessary to adequately evaluateand treat craniofacial patients and their fami-lies. Members of the craniofacial team work


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modification of thisplan due to the pres-ence of more rigid boneand slower cranialgrowth. In addition,older patients mayrequire reconstructionof secondary facialdeformities.

In the event thatintraoperative or post-operative blood trans-fusions are needed, theparents are asked toprovide designateddonor blood. In addi-tion, aspirin and other

medications that mightprolong the bloodclotting time are to beavoided.

FUSED SUTURE NAME DESCRIPTION

Sagittal Scaphocephaly Boat Skull

Metopic Trigonocephaly Triangular Skull

Unilateral Coronal Plagiocephaly Asymmetric Skull

Bicoronal Brachycephaly Short Skull

Lambdoid Plagiocephaly Asymmetric Skull

TERMINOLOGY OF CRANIOSYNOSTOSIS

Multiple suture lines shown in infant skull.

Lambdoid Suture

Sagittal Suture

Coronal SutureMetopic Suture


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SAGITTAL SYNOSTOSIS

Premature closure of the sagittalsuture, the longitudinal suture on thetop of the head, stops growth laterallyproducing a narrow head. There is acompensatory growth in the antero-posterior direction with elongation of

the skull and a bulging of the frontand back of the head. This particulardeformity is called scaphocephalydue to the skulls boat shaped appear-ance. Synostosis of the sagittal sutureis the most frequent type of singlesuture craniosynostosis. It makes up50-60% of all patients within NorthAmerica with craniosynostosis. Theparticular infants with sagittal synos-

tosis characteristically have normalintelligence.

In sagittal synostosis or scapho-cephaly we most frequently performtotal skull reshaping. In this type ofsynostosis the forehead and back ofthe skull are usually bulging, over-projected, and the width of the skulltoo narrow. The front, back and later-al bone plates are removed and then

reshaped to a more normal contour.The forehead is tilted back (as well asthe occiput) and the entire anteropos-terior dimension of the skull is short-ened. The reshaped bones are securedin position with wires and/ormicroplates. Certain gaps between thebones are left unsecured to avoidrestriction of growth. The dura, orouter covering of the brain, may be

plicated in the frontal region todecrease the prominence. Barrel stavecuts are made in the lateral bone at thebase of the skull to allow more projec-tion of the skull laterally, increasingthe transverse width. This type of pro-cedure gives a more definitive andimmediate improvement in skullshape.

Scaphocephaly




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METOPIC

SYNOSTOSIS

The metopic suture runs down themidline of the forehead. Premature fusionof this suture results in a triangular shapedforehead called trigonocephaly. A bony

ridge is usually palpable that extends fromthe bridge of the nose to the upper part ofthe forehead. This premature fusion pro-duces a prominent midline keel with later-al recession of the brows. From the frontalview these patients typically have theappearance of hypotelorism or decreaseddistance between the eyes.

The incidence of metopic synostosis inmost cases of craniosynostosis is between

5-10%. This deformity is usually obvious atbirth, but the severity of the anomaly mayvary. We prefer to correct these deformitiesbetween 4 and 8 months of age. Our cur-rent surgical treatment consists of frontalbone remodeling and supralateral orbitaladvancement. The triangular or keel-shaped forehead is removed and recon-toured to the appropriate shape or bonemay be taken from another area of theskull to replace this bone. The entire bonysupraorbital bar is removed and reshapedwith supralateral orbital advancement torestore the normal brow contour.Microplates are used to secure the frontalbar to the facial bones and maintain thenormal contour.


Intraoperative photograph of triangular shaped forehead(left) and brow (right).

Trigonocephaly


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UNILATERAL

CORONAL SYNOSTOSIS

Premature fusion of one of thecoronal sutures results in an asym-metric forehead and brow. On theaffected side the forehead is flattened

and recessed with the brow andsupraorbital rim both elevated andrecessed. The contralateral foreheadmay exhibit compensatory bulgingor bossing. This ultimately results ina very asymmetric malformationcalled plagiocephaly. Technically,plagiocephaly refers to any typeof asymmetric skull deformity.However, many authors have usedthis synonymously with unilateral

coronal synostosis.

The incidence of unilateral coro-nal synostosis in most series of cran-iosynostosis is between 10-20%. Ifuntreated, this type of synostosismay result in a severe deformity ofthe forehead, orbit, and nose thatpersists or worsens with growth. Wefeel the best treatment for plagio-cephaly should involve a bilateralapproach with remodeling of theentire forehead and brow betweenthe ages of 4 and 8 months. Our cur-rent surgical treatment consists ofextended bicoronal craniectomieswith bilateral frontal bone recon-struction. The supraorbital bar iscompletely removed, reshaped, andstraightened. The supraorbital bar isadvanced and lowered as necessary

on the affected side and rigidly fixedin position to the face. The recon-structed forehead is then secured tothe supraorbital bar. We feel that thebilateral approach obtains a moresymmetric forehead and brow thanthe unilateral approach.



Plagiocephaly


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BICORONAL

SYNOSTOSIS

Bicoronal suture fusionresults in a flat retrudedforehead with increasedheight to the skull. This

condition is also calledbrachycephaly due to theshort anteroposteriordiameter. As a result of thisshortening there is a com-pensatory bulging of thetransverse diameter orwidth of the skull. Thebrow is usually recessedand elevated with thedeformity being symmet-

ric. The incidence ofbicoronal synostosis orbrachycephaly is between10-20% in most series ofcraniosynostosis. This mayoccur as an isolated occur-rence or part of a morecomplex syndrome withfacial retrusion such asAperts or Crouzons.

Surgical treatment consistsof extended bicoronalcraniectomies with recon-struction of the forehead.The supraorbital bar orbrow is reshaped andadvanced forward with theforehead. The reconstruct-ed forehead and brow arerigidly fixed to the noseand lateral orbits with

microplates. If there isexcessive height to theskull then total calvarialremodeling is also per-formed, decreasing theheight of the skull.


Intraoperative lateral view of skullbefore remodeling.

Intraoperative lateral view of skull afterremodeling.

Brachycephaly


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POSTERIOR

PLAGIOCEPHALY

There has been a significant increasein the past several years of infantsreferred for evaluation of occipitaldeformities. This increase in occurrence

of posterior skull deformities appears tobe related to the 1992 recommendationfor supine sleep positioning by TheAmerican Academy of Pediatrics toavoid Sudden Infant Death Syndrome.Occiput deformities can be divided intolambdoid synostosis and deformationalor positional plagiocephaly.

The lambdoid suture is a pairedstructure that transverses in a symmetric

Posterior Plagiocephaly

Postoperative result after posteriorskull remodeling.

PostoperativePreoperative Postoperative

Preoperative photo showing leftposterior skull flattening.

Postoperative result after posteriorskull remodeling.


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fashion across the posterior aspect of theskull. Premature fusion or synostosis of thissuture is rare but may be unilateral or bilat-eral.

Characteristically in unilateral synosto-sis the posterior skull is flattened on theinvolved side. Displacement of the ear with

compensatory skull changes may be presentin severe cases. Bilateral synostosis is char-acterized by extensive flattening across theposterior skull and increased verticalgrowth to accommodate brain enlargement.In mild cases the deformity can be coveredby hair and surgical repair may not be indi-cated. When there is significant flatteningor asymmetry, we recommend release of thefused sutures and posterior skull remodel-

ing. Surgical treatment consists of extensiveposterior skull craniectomies with remodel-ing of both sides of the occiput and posteri-or advancement of the affected side. Rigidfixation with microplates is used to hold thereconstructed bones in position and main-tain the contour until healing takes place.

Occipital plagiocephaly without synos-tosis must be distinguished from lambdoidsynostosis. Most patients referred to our

center for posterior skull deformities aredeformational or positional plagiocephaly.In these infants, the skull becomes mis-shaped from repeated pressure on the sameposition, without the premature fusion ofthe lambdoid suture. Positional plagio-cephaly usually improves without surgery.Once the childs sleep patterns change andhe spends more time awake, the brains nor-mal growth forces help to reshape the poste-rior skull. Some centers recommend acustom fitted helmet to mold the head backinto position over a period of severalmonths. However, in cases where the poste-rior skull deformity progresses or is severe,we recommend surgical remodeling asdescribed, even though a true synostosismay not be present.

CRANIOFACIAL DYSOTOSIS

Craniofacial dysotosis (Crouzons and Apertsdiseases) are characterized by craniostenosiswith cranial dysmorphia and facial deformities(hence, the term craniofacial dysotosis).

APERT SYNDROMEApert syndrome or acrocephalosyndactyly

syndromes are rare conditions. In 1906, Apertdescribed the skull, facial, and hand deformitiesof several patients characteristic of this syn-drome that now bears his name. The incidence ofinfants born with Apert syndrome is one forevery 100,000 to 160,000 live births. Many of theinfants born with this syndrome show a sporadictransmission, which means that a family may

have a child with Aperts when no other mem-bers of the family are affected. The recurrent riskof having another child with Aperts for twounaffected parents is negligible. However, if theparent is affected there is a 50% chance of eachoffspring having Apert syndrome with bothmales and females affected equally.

Clinical Features: Patients with Apert syn-drome have very distinct facial and extremityfeatures. Abnormal skull shape is due to cran-

iosynostosis or premature fusion of the suturesor soft spots. The skull usually demonstrates ashort anteroposterior diameter (brachycephaly)and may be excessively tall (turricephalic) orabnormally wide (euryprosopia). The foreheadis generally always retruded, but this may not beobvious due to the hypoplasia of the midface.The orbits or bony sockets which contain theeyes are very shallow causing a bulging or prop-tosis. The orbits are usually rotated downward

and lateral causing a downward shape to the lat-eral corners of the eyes. There may be a moder-ate increased distance between the eyes(hypertelorism) with muscle imbalance.

The middle of the face in Aperts is bothretruded and very hypoplastic. This causes thecentral midface to have a characteristic sunken-


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in appearance with the nose beingthick and beaked. The upper jaw ormaxilla characteristically shows anarrow arch with an open bite anddental crowding. The maxilla issignificantly retruded compared tothe mandible with the teeth of thelower jaw projecting in front of theupper teeth. Other possible clinicalfeatures include moderate hearingloss, speech impairment, acne, anddecreased mental capability insome individuals. Intellectualpotential may be difficult to evalu-ate due to communication prob-lems. Some patients with Apertsyndrome may have normal intelli-gence. All patients with Apert syn-

drome demonstrate a unique handmalformation. This is characterizedby a complex syndactyly or fusionof the skin, soft tissue, and bones ofthe fingers. Both hands are affectedequally, as are the feet. This unusu-al variation of syndactyly can beused to identify Aperts from othersimilar syndromes.

Treatment: The treatment of

patients with Apert syndrome isnot uniform due to significant vari-ations in the facial anomalies, ageof patients when first seen, andprevious operations. Our primaryconcern of the infant born with thissyndrome is: compression of thebrain, breathing problems, pro-truding eyes with corneal expo-sure, and lack of facial growth. The

surgical plan must be flexibleand individualized to the patient.Multiple stages or operationsat different ages are usuallynecessary.

When we see these patients asinfants, the first stage is treatmentof the craniosynostosis with total

Apert Syndrome

Preoperative photo of infantwith Apert syndrome.

Postoperative result after fore-head and brow advancement.


Characteristic appearance of complex syndactyly of hand.


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calvarial reshaping. We prefer to do thisprocedure at four to six months of age. Afrontal-orbital advancement is per-formed which increases the intracranialspace and size of both orbits. Total skullreshaping helps correct the tower skullproblem which is not addressed byfrontal-orbital advancement alone. Aventriculo-peritoneal shunt may beneeded for treatment of a hydro-cephalus. This is performed prior toskull remodeling. Occasionally a repeatcraniotomy is needed to further reshapethe calvarial vault and advance theorbits. The next stage of the reconstruc-tion is midfacial advancement. We usu-ally perform this procedure between theages of four to six years old prior to

starting school. If necessary, anintra/extracranial advancement of theentire face and forehead (monobloc) canbe performed with correction of mild

hypertelorism or lateral rotation of the orbits.This procedure, as described by Tessier, is calleda facial bipartition and corrects several deformi-ties at once. It effectively widens the maxilla andderotates the orbits, and narrows the upper face.In milder cases an extracranial LeFort IIIadvancement may be used.

The final steps in the reconstruction are max-illary/mandibular osteotomies to complete thecorrection of any further dental discrepancies.These procedures are usually performed aftereruption of permanent dentition and completionof growth (teen years). Additional proceduressuch as rhinoplasty, genioplasty and eyelidsurgery may be beneficial. Surgical separation ofthe fingers is usually started in the first year oflife and completed by three to four years of age.

The patient with Apert syndrome representsa complex combination of multiple deformities.Our goal is to treat not only the function andphysical problems but the psychosocial issues as

Postoperative result after facialadvancement.

Apert Syndrome

This little girl has the character-istic facial appearance of ApertSyndrome which is also associat-ed with complex syndactyly.

Preoperative


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CROUZON

SYNDROME

This syndrome was originallydescribed in 1912 by a French neuro-surgeon. He described four essentialcharacteristics: exorbitism, retromaxil-

lism, inframaxillism and parodoxicretrogenia. The incidence of this syn-drome appears to be approximatelyone in 25,000 in the general popula-tion. It is inherited as an autosomaldominant pattern with variableexpression. However, approximately25% of reported cases have no familyhistory and represent a new mutation

Clinical Features: Patients with

Crouzon syndrome have very distinctfacial features with craniofacial bonemorphology similar to Apert syn-drome. Premature fusion of thebicoronal suture is the most commontype of craniosynostosis with abrachycephalic or oxycephalicappearance to the skull. The extent ofthe craniosynostosis may be variableas well as the age at onset. There isretrusion of both the forehead andbrow, with midface hypoplasia andshallow orbits with bulging eyes(proptosis). Orbital hypertelorism isless commonly seen in Crouzons thanAperts syndrome. The lateral canthimay be slanted downward with somedegree of upper eyelid ptosis. Theproptosis is usually more prominentin Crouzons than Aperts; however,in Apert syndrome, the other craniofa-

cial deformities are more complex andsevere. In addition, hydrocephalusand developmental delay are less fre-quently seen in Crouzons thanAperts. The nasomaxillary retrusionmay cause some degree of nasal air-way obstruction with mouth breath-

Apert Syndrome

Four year old girl with Apertsyndrome.

Postoperative result aftermonobloc facial advancement

with facial bipartition (medialrotation of orbits).


well. We feel these individuals are best evaluated andtreated by a craniofacial center that utilizes the multi-disciplinary team approach providing a coordinated,comprehensive, long-term treatment plan with carefulmonitoring of growth and development.


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osteotomy is used to correct the dental discrepancies incombination with orthodontic intervention. This is usual-ly performed after facial growth is completed and may becombined with a genioplasty (chin reduction/advance-ment). Additional procedures such as rhinoplasty may beneeded.

ing. The chin is usually large withincreased vertical height but oftenretruded resulting in a paradoxicalretrogenia. The maxillary teeth areretruded compared to the mandiblewith a class III malocclusion. Otherpossible abnormalities include con-

ductive hearing loss, inverted V-shape to palate and exposurekeratitis. Variations of this type ofcraniofacial dysostosis are seen in thesyndromes of Carpenter, Pfeiffer andSaethre-Chotzens.

Treatment: Our approach to thetreatment of Crouzon syndrome issimilar to

craniofacial book

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