Tiirkis/i Neiirosiirgeni 6: 28 - 32, 1996

Frontoethmoidal

Report of

Sinus MucocelesTwo Cases

Dösog/ii: Ml/coeei,'

MURAT DÖSOGLU, FUAT M. MUTLU, SERDAR ARMAGAN, FÜGEN AKER,

LEVENT ÇELIK, MAHIR TEVRÜZ

Haydarpasa Numune Hospital, Department of Neurosurgery (MD, FMM, SA, MT),Pathology (FA) and Radiology (LÇ), Istanbul, Türkiye.

Abstract: Mucoceles are chronic and benign lesions caused byaccumulation and retention of mucus secretion in a sinus due to

partial or complete occlusion of the ostium which causesprogressive distention of the bony waii because of increasedpressure. They occur most commonly in the frantal and ethmoidalsinuses.We present two patients with anterior frantoethmoidal mucoceleswho were treated surgicaiiy in our clinic. The clinical presentationwas pain and deformation of the frantal region and medialcanthus in both cases. Chronic sinusitis was detected as a

predisposing factor in one case. A transfrontat approach and

INTRODUCTION

Ocelusion of the sinus ostium due to various

pathologies can cause slow and progressiveaccumulation of secretions. The obstmetion may becaused primarily by eystic dilatation of the gobletceii gland besides secondary reasons (2, 7).Congenital pathologies such as agenesis of the ostium(8), tumoral pathologies such as osteoma, fibrousdysplasia, bone !ipoma, osteoclastoma,haemangioma, epidermoid, metastatic tumours,craniopharyngioma, cyst formation by embryonicpituitary rests and nasal polyps (3,4, 15) are primaryreasons, while secondary reasons are inflammatorydiseases such as asthma, all ergi es and chronicsinusitis, traumatic causes such as displaced fraeturefragments and iatrogenic reasons such as surgery (6,12). For it is possible mucocele to develop with orwithout occlusion (8). it has been reported thatcalcifying fibroma, meningioma and acousticneurinoma may accompany mucoceles (4, 9).Osteoma may be seen in 12.5 - 50 % of cases duringsurgery (15). A previous history of sinusitis andrhinitis occurs in 35 - 50 % of cases, trauma in 10 - 28

28

obliteration of the ostium foiiowed by aspiration of the sinuscontent was the treatment of clioice in both cases. No recurrence

\Vas observed in a one-year foiiow-up.Although mucoceles are often see n in ENT praetice, intracranialand/or orbital extension may be see n in neurosurgery. Butrecurrence may occur despite surgical intervention. Surgery offersthe only effective treatment; the type of procedure selecteddepends on the location and extent of the mucocele and the natureof any existing complication.Key word s: Ethmoid sinus, frontal sinus, mucocele, paranasalsinus

% and allergies in 11 % (4,5). Although retention cystsresulting from obstruction and dilatation oftubuloacinar glands are used synonymously withmucocele, theyare different in origin and clinicalprogress. Usually asymptomatic, they do not requiretreatment (16).

CASE REPORTS

Case 1A 55-year-old woman was admitted with

swelling of the medial side of the left orbit and frontalregion, epiphora, frontal and periorbital headache.She had been treated for sinusitis for three years priorto admission. Deformation of the orbital region haddeveloped and progressed in the last eight months.On physical examination, inferomedial displacementof left eye and hyposmia in the right side wereobserved. The nasolacrimal canal was open. Eyemovements, visual acuity and fundus were normaland there was no loss of sensation on the face. Skull

x-rays demonstrated selerotic bone lesions in thefrontoethmoidal region, irregularity in the superiorand medial orbital wall, lateral displacement of the

Turkisli Neiirosiirgery 6: 28 - 32, 1996

lamina papyraeea and opacifieation in the leftmaxillary sinus (Figure 1). Computed tomography(CT) showed an isodense lesion (55 HU) in the leftfrontal and ethmoidal sinuses SxSx4 cm in sizeeausing orbital displaeement by destruetion of themedial orbital wall. The lesion had extended to the

superior nasal eavity and anterior eranial fossa withthe erosion of the anterior and posterior walls of thefrontal sinus and the superior wall of the ethmoidalsinus via eribriform, perpendieular andethmoidomaxillary plate destruetion and invaded theorbit with the erosion of the lamina papyraeea andsupramedial wall. The lesion had not infiltrated theanatomical struetures. There was a thin fat planebetween the medial reetus musele and the lesion

(Figure 2). Bioehemieal examinations were normaL.

1-"lg 1: Skitil x-my film sliawiiig a /esiaii iii Ilie /eft froliloellllllVidal

regioii oCCllpyilig and remodelliiig Ilie siiius aiid

opacificalioii iii Ilie lefl maxillary siiiiis.

The patient was operated on via a bifrontalincision. Twenty-five ml of green, viseous mueousfluid was aspirated from the eavity. The mueosa wasexcised and the ostium of the sinus was obliterated

by wax. The dura was intaet.In the early postoperative eourse, the patient's

pain disappeared. Pathological examination revealedhyalinized fibrotie wall eovered with eiliatedpseudostratified euboidal eells. There werehaemorrhages, histioeytes, lymphoplasmoeytie eellinfiltration, multinuelear giant eells and eholesteroleleft in all layers of the wall. Examination of themueous material revealed dense nuelear debris and

mixed inflammatory eells espeeially neutrophilleueoeytes.

DösoglH: Miicocele

Fig. 2: CT senii sliowiiig aii isodeiise II/IlS, aiid laleml displaceiiiciif

of ilie lamiiia papyrncea aiid Ilie orbil, erosioii of Ilie

el/llllOidomaxillar plale willi exleiision lo Ilie superior

Ilasal envily iii Ilie lefl froiilal and ethmoidal sinus.

Case 2

A 60-year-old man was hospitalized withswelling and pa in of the left frontal region that hadstarted five years ago and progressed in the last threemonths. On physieal examination, there were noabnormalities exeept a fluetuating lesion witheosmetie deformation in the left orbital region. X-raysof the skull showed a marginal selerosis eonsistentwith an expanding mass of the left frontal sinus. Thesuperior and medial orbital walls were displaeedinferiorly and laterally (Figure 3). CT revealed ahomogeneous mass lesion (47 HU) 3x4xS cm in sizewith s1ight enhaneement in the frontal and anteriorethmoidal sinuses and extending to the orbitofrontalregions (Figure 4). Bioehemieal tests were normaL.

Frontal sinus exploration was performed usinga left frontal incision. Brownish, odoriferous, mueousfluid was aspirated that had eroded the anterior,posterior and inferior walls of the sinus. The lesionwas removed, and the ostium was obliterated. Thedura was intaet and pulsatile. Histologiealexamination of the mueous membrane showed

pseudostratified eiliary eolumnar epitheliumeontaining the goblet eells among the condensedmu co us fragments. Lymphoplasmoeytie eellinfiltration, histioeytes, giant eells, hyalinized stromaeontaining eholesterol cleft were also observed(Figure 5). On mierobiologieal examination,polymorphonuelear leueoeytes were seen. Cultures

29

Tiirkish Neiirosiirgery 6: 28 - 32, 1996 Dösog[ii: Miicoce[e

Fig. 3: Aiiteroposterior fi[iii of the skiill showiiig iiifenordisp[ncemeiit of the siiperior niid medini ianlls of theorbit niid elenr-ciit boiie deslmctioii (iiinrgiiin[ selerosis)iii the [efi fralllnl regioii.

Fig. 4: CT scan reveals a homogeneolis mass with destriictionof the anterior and posterior waiis of the siniis.

of the mucous material were sterile. The case was

considered to be a mucocele in the light of thesefindings. Regarding the slight contrast enhancement,microbiological examination and character of theaspirated fluid, the cas e was considered to be

30

Fig. 5: Pliotomicrogrnpliic nppenmiice of Ihe lesioii s/iowiiig the

nrens of /IlOiioiiiielenr cell iiifillmlioii niid Iliiicoid [nkeiii the //Iiderlyiiig fibroiis tissiie. (/inemntoxyliii niideosiii; origiiinl mngiiificntioii, X 100)

mucopyocele. Following ten days antibiotic therapy,the patient's complaints disappeared and he wasdischarged.

DISCUSSION

Mucoceles are paranasal sinus lesions usuallyseen in adults with equal incidence in both sexes (6).Infrequently, they may be seen in children (5, 8). Theyare common in frontal and ethmoidal sinuses, andrare in sphenoid and maxillary sinuses (5, 11, 18).Atypical localizations like frontal lobe (7), intrasellarand retroorbital regions O) as well as anterior dinoidprocesses (4) have also been described. In these cases,the sinus connection has not been shown because of

early dosure of the path, agenesis of the sinus ostiumand ectopic or aberrant sinus without an ostium (5,

8). Three types of ethmoidal mucocele are describedas anterior, middle and posterior. Posteriorethmoidal mucoceles are called sphenoethmoidalmucoceles because they originate from thesphenoidal sinus 02, 18). Mucoceles may showextensions to various regions according to theirlocalization. Sphenoid mucoceles may extend to thecavernous sinus, sella turcica, divus, superior orbitalfissure, orbit and ethmoidal sinus. MaxiJlarymucoceles may narrow the pterygopalatine and nasalfossae, and extend into the ethmoidal sinus and orbitwith erosion of the ethmoidomaxillary plate (6, 12)..Ethmoidal mucoceles may show extensions into themaxillary sinus and the anterior cranial fossa bydestmetion of the perpendicular and cribriform plate,into the orbit and optic canal by erosion of the lamin<ipapyracea (9, 11, 18). Frontal mucoceles may extentinto the ethmoidal sinus, orbit or anterior cranial

Tiirkish Neiirosiirgery 6: 28 - 32, 1996

fossa. The medial rectus muscle and globe aredisplaced anterolaterally or usually inferiorly infrontoethmoidal mucoceles because of erosion of the

supramedial and medial wall of the orbit. Thenasolacrimal canal and the nasal cavity may beinvolved. Mucoceles may be attached to the dura ifthe posterior wall of the frontal sinus is destroyed(9). Delfini eloquently classified paranasal sinusmucoceles according to their localization andintracranial extensions to establish the surgicalapproach (4). Type 1: anterior without extension,Type 2: anterior with extension, Type 3: posteriorwithout extension, Type 4: posterior with extension.The presented cas es were evaluated as type 2mucoceles because of the anterior frontoethmoidallocalization and intracranial and orbital extension.

Symptoms and signs may show differences accordingto localization. Mucous and mucopurulant nasaldischarge, frontal and periorbital pain radiating tothe vertex, temporal and occipital regions, facialdeformation, diplopia and loss of smeii may be seen.Cliniçal findings are ophthalmoplegia,exophthalmos, epiphora, chemosis, gradual loss ofvisual acuity, optic atrophy, nasal polyp,spontaneous pneumocephaly, sweating dysfunctionwith sympatic system involvement. Chemicalmeningitis may be see n (3, 6, 8, 11, 16). Deformation,pain, globe dislocation and hyposmia were the mainsymptoms because of the anterior localization of themucoceles in our cases. Orbital apex syndrome, opticcanal syndrome and endocrinopathy may be seen inposterior localization of mucoceles (2,4, 18). X-raysmay provide information about destruction of theiimer table of the frontal bone and roof of the orbit,

cloudy appearance of the sinus, disappearance andremodeiiing of the normal sinus walls and marginalsclerosis (8, 16). There may be erosion ordisplacement in the frontozygomatic arcus, sellar andorbital waiis (2,5,6). Conventional tomograms andCT have been very helpful in evaIuating the characterand extent of mucoceles (9). CT has distinct

advantages over MRI for assessment ofbony erasionsand extensions of the lesions as seen in our cases (2,

7). Perugini et aL.praposed some criteria for accuratediagnosis of mucoceles (13). a. homogeneousisodense mass occupying the sinus with exophyticalterations, b. clear margins without signs ofinfiltration of adjacent anatomical structures, c.patchy osteolysis d. no enhancemenL Spontaneoushypodense and hyperdense lesions have also beendescribed (4). Differences in the densities of these

lesions may be related to the age and consistency ofthe entrapped secretions (6). Enhancement may beseen if the mucocele is infected (mucopyocele) as in

Döso,~lii: Miicocele

one of our cases (9, 15). There may be ossificationand calcification in the osteolytic region. On MRimages, the mucocele's contents will have varyingsignal intensities on Ti and Ti weighted imagesaccording to the protein concentration and may bemisleading (4, 15). The mucosa will enhance at theperiphery of the nonenhancing secretions af tercontrast administration. Angiogram and orbitalvenogram reveal an avascular mass, displacementof the vascular structures but are not used today (5,7,8).

Destruction of the sinus wall forms a

communication between the epidural space and theatmosphere which causes intracranial andextracranial complications that include extraduralaerocele, CSF fistula, pneumocephalus, orbitalceiiulitis, meningitis, brain abscess and even sepsis(l5, 16, 18).

Pathological examination shows PAS (+),pseudostratified ciliary columnar epitheliumcontaining the goblet cells or smaIl cubic epithehumcovering the mucoperiostal wall. it mayaiso showchronic nonspecific inflammatory tissue revealingsquamous metaplasia or fibrous tissue consisting ofmononuclear ceii infiltration (4, 10, 12, 17). Similar

findings were observed in our cases. Culture resultsmay be positive, when the mucocele is infected. Noorganisms were isolated from the mucous content ofour second case with mucopyocele.

Management of a mucocele that has extendedinto the anterior cranial fossa and / or orbit is surgery.The main purpose of surgical treatment is to evacuatethe lesion, radically remove the sinus mucosa toprevent relapse and re-estabhshment of apiane ofseparation between the extracranial and intracranialspace as well as reconstructive surgery to achievesatisfactory cosmetic results. Frontotemporal, frontal,bifrontal craniotomies; transnasa!, transethmoidal,

transpalata!, transorbital or combined approachesmay be used according to localization of the lesion.

Sphenoidotomy, transfrontal sinusotomy andethmoidectomy mayaiso be used for simple drainage(4, 7, 11, 18). Basically, transcranial or maxillofacialapproaches should be used for mucoceles with orwithout intracranial extensions respectively.Maxillofacial approaches were usually performed inanterior mucoceles (Type 1) and posterior midlinemucoceles without intracranial extension (Type 3) inENT practice (4, 10, 14). Some maxillofacial surgeonsprefer two - stage operations, draining the sinuscontent first followed by removal of the sinus mucosa(5, 12, 15). Removal of the anterior waIl of the frontalsinus is generally performed only for osteomyehtis.Cranialization of frontal sinus involves removal of

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T'I/'kl'I' .\t'lIrtlsiirgery 6: 28 - 32, 1996

the posterior table and all sinus eontents. Theposterior wall of the sinus should be restored with apateh of galea graft beeause of the risk of infeetionirhinorrhea and pneumoeephaly. The sinus ostiumshould be oecluded by melted bone wax and musclegrafts to prevent reeurrenee (4, 8, 15). Haemorrhagienasal diseharge and 4-10 % reeurrenee may be seenfollowing the operation (2 ,4, 14). In our casesibifrontal and frontal approaehes were used due tofrontoethmoidal loealization with intraeranialextension (Type 2). After exeision of the mueoeeleithe mueosa was eompletely removed and the ostiumwas obliterated to prevent reeurrenee of themueoeele. The anterior and posterior walls of thesinus were left intaet beeause of the absenee of

osteomyelitis. There was no reeurrenee in one yearfollow-up.

Correspondence: Murat Dösoglu,Haydarpasa Numune Hastanesi,Nörosirürji Klinigi,Üsküdar, IstanbulTel: 0216.3454680,3454083,4144502 - 1764Fax: 0216. 3360565

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