Pediatric Radiology

Paranasal Sinus Mucoceles in Children 1 Marilyn J. Siegel, M.D., Gary D. Shackelford, M.D., and William H. McAlister, M.D.

The clinical and radiographic features of paranasal sinus mucoceles in 6 pediatric patients are discussed. These lesions may be associated with clinical signs of a periorbital or intracranial mass. Characteristic radiographic findings can suggest the preoperative diagnosis, although a sphenoid sinus mucocele may simulate more serious conditions. INDEX TERM:

Sinuses, paranasal (Paranasal sinus, mucocele , 2[3J.255)

Radiology 133:623-626, December 1979

CASE REPORTS

UCOCELE of the paranasal sinus is a rare lesion in

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children and is seen as a dilated, fluid-filled mass caused by the accumulation of mucus and secretions within an occluded sinus cavity. It may result from infection, trauma, surgery, congenital deformity of the ostium, or cystic degeneration of the mucosa (2,4, 7, 11). A paranasal sinus mucocele is interesting not only because of its rarity but also because the clinical features suggest a periorbital or intracranial mass. Radiographicallythe lesion may produce rather characteristic features, but occa sionally it simulates a more serious lesion. We wish to report 6 cases of mucoceles in children, with attention directed to the clinical and radiographic features. Included are 2 patients with sphenoid mucoceles who presented interesting diagnostic problems: one was initially thought to have a chordoma or histiocytosis and the other was diagnosed as having a meningoencephalocele. Three other patients had an ethmoid mucocele, and one had multiple sinus involvement. The radiographic features of CASE III have been mentioned elsewhere (12).

CASE I: A 12-year-old boy was admitted for evaluation of a right medial canthal mass and " watering" of the right eye of 2 months duration. On physical examination, a firm, nontender 1-cm mass was palpable in the region of the inner canthus. Radiographs of the paranasal sinuses showed a dense right frontal sinus, with slight depression of the superomedial margin of the right orbital roof (Fig. 1, a). The right ethmoid sinus was opacified and expanded, and a small mucus retention cyst was present in the left maxillary sinus. Tomograms confirmed a soft-tissue mass in the right ethmoid sinus, thinningand bulging the walls in all directions but without bone destruction (Fig. 1, b). The radiographic diagnosis was a frontoethmoid mucocele. Usinga right transethmoidapproach, an ethmoid mucocele was found and removed. Although it had thinned the anterior two thirds of the lamina papyracea, there was no extension into the frontal sinus. The patient recovered satisfactorily, and sinus radiographs one month after surgeryshoweddiminishingopacification of the right ethmoid and frontal sinuses.

COMMENT: Pressure on the superomedial aspect of the orbit initially suggested a coexistent frontal mucocele;

1a,b

Fig. 1. CASE I. a. Frontal radiograph shows an expanded right ethmoid sinus. There is deformity of the superomedial orbital wall as a result of pressure by expanded anterior ethmoid air cells . b. Tomogram demonstrates expansion of an opaque right ethmoid sinus. An ethmoid mucocele was proved surgically . 1 From theMallinckrodtlnstituteofRadiology.WashingtonUniversitySchoolofMedicine .St.Loui~.Mo. Received March 6, 1979; accepted and revision requested June 26; revision received Aug. 2. sjh

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2a,b

Fig. 2. CASE III. a. Lateral radiograph shows destruction of the walls of the sella and sphenoid sinus. Note the extension to the clivus. The fine line crossing the sella on the right is the thinned sellar floor. b. Lateral radiograph 5 years after surgery shows a normal sella.

3a,b

Fig. 3. CASE IV. a. Submentovertex projection shows an opaque right sphenoid sinus and anterior bowing of the right maxillary sinus (arrows) . b. Tomogram demonstrates an expanded, opacified right sphenoid sinus.

however, the orbital deformity was actually due to expanded anterior ethmoid air cells. CASE II: A 12Yz-year-old boy with a 3-month history of left periorbital swelling and erythema showed roentgen features suggestive of a left ettvnoid mucocele, which was confirmed surgically. Initial improvement occurred but was followed by recurrence of swelling, and repeat radiographic findings in various modalities again suggested left ethmoid involvement. Further antibiotic therapy ameliorated the symptoms and the patient stabilized.

CASE III: An 8-year-old boy was admitted for evaluation of a 3-week history of severe throbbing left retro-orbital pain associated with acute esotropia, nausea, and vomiting. On physical examination there was limitation of motion of the left eye, a left temporal field cut superiorly, palsy of the left sixth nerve, and mild papilledema, greater on the left.

Skull radiographs and tomograms revealed erosion of the sella from the tuberculum to the dorsum sellae, with the sellar floor remaining thinned on the right. The walls of the right and left sphenoidsinuses were destroyed except for the right lateral wall, which was thinned (Fig. 2,

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Fig. 5. CASE VI. CT scan through the orbits, showlnq an expanded, opacified right ethmoid sinus. The left ethmoid sinus is also dense.

Fig. 4. CASE VI. Frontal radiograph demonstrates expanded right frontal and ethmoid sinuses. The medial right orbital wall is deformed and thinned. The lesion was surgically proved to be a frontoethmoid mucocele.

a). The destruction extended to the left pterygoid process and the medial wall of the foramen rotundum; posteriorly it reached the clivus and spheno-occipital synchondrosis. There was no tumor calcification. The radiological diagnosis was histiocytosis or chordoma . Left carotid and vertebral arteriograms demonstrated a space-occupying mass in the pituitary fossa and adjacent portion of the sphenoid sinus. There was slight lateral and superior elevation of the cavernous segment of the internal carotid artery, with minimal extrinsic narrowing of the carotid siphon. No definite suprasellar extension was noted. Using a left transethmoid intranasal approach , a cystic lesion containing a thickened membrane and brown gelatinous fluid was found in the sphenoid sinus and drained. The diagnosis was a sphenoid mucocele. Biopsies of mucosa from the left ethmoid and sphenoid sinuses revealed chronic inflammation and edema. The patient made a satisfactory recovery, and the palsy gradually subsidedover a 6-week period. Repeat skull radiographs 5 years later showed a normally mineralized sella and a surgical defect in the lateral wall of the left ethmoid (Fig. 2, b). There was no evidence of a recurrent mucocele. CASE IV: An 11-year-old girl was referred for evaluation of progressive loss of vision in the right eye. At 5 years of age a pin had perforated the globe . Physical examination revealed proptosis, decreased visual acuity , and a nasopharyngeal mass. Skull radiographs showed a soft-tissue mass in the pterygomaxillary fossa, displacing the posterior wall of the right maxillary sinus anteriorly and the right pterygoid process posteriorly (Fig. 3, a). There were also erosion of the inferolateral margin of the right supra-orbital fissure and elevation of the lesser wing of the sphenoid. Tomograms of the sinuses demonstrated an airless and expanded right sphenoid sinus (Fig. 3, b). In view of the prior trauma, the radiographic diagnosis was a meningoencephalocele or leptomeningeal cyst. A right frontal craniotomy was performed and a sphenoid mucocele extending into the greater wing of the right sphenoid was found and drained. Biopsy of mucosa from the sphenoid sinus revealed chronic inflammatory changes. Following an unremarkable recovery, the patient was discharged with residual visual loss in the right eye. CASE V: A 14-year-old girl was admitted because of recurrent nasal polyps and sinusitis. She had had a right nasal polypectomy five months earlier, and physical examination on admission revealed polyps in the right middle turbinate.

Plain radiographs and tomograms of the paranasal sinuses showed opacification of the right maxillary antrum , with expansion and opacification of the right ethmoid sinus. The walls of the sinus were intact but attentuated and flattened. The radiographic diagnosis was a right ethmoid mucocele. Right transantral ethmoidectomy revealed a mucocele which had destroyed many of the bony septa. Sections of the mucocele revealed acute and chronic inflammation. Recovery was unremarkable, and follow-up radiographs one year later showed minimal residual density in the left ethmoid sinus. CASE VI: A 9-year-old girl presented with a firm mass in the medial aspect of the right orbit of 5 months duration. Mild epiphora had been present for about 1 month and right nasal drainage for 3 weeks. Radiographs and tomograms of the paranasal sinus revealed an expansile soft-tissue mass in the anterior and middle portions of the right ethmoid and in the right frontal sinus, with expansion laterally and anteriorly into the superomedial aspect of the orbit (Fig. 4). The walls of the right frontal and ethmoid sinuses were thinned but intact. The remainder of the paranasal sinuses were airless, consistent with sinusitis. The CT scan confirmed an expanding right ethmoid and frontal mass, compatible with a mucocele (Fig. 5). At operation, a large pyocele was found extending downward from the right frontal sinus into the ethmoid and maxillary sinuses on the right, with a second, smaller pyocele in the sphenoid sinus. Sections of the resected pyoceles showed acute and chronic inflammation and fibrosis. The patient made a satisfactory recovery, and repeat sinus radiographs one month later revealed residual opacification of all sinuses.

COMMENT: In contrast to CASE I, the deformity of the superomedial orbital wall was caused by a combined frontoethmoid mucocele. CLINICAL AND RADIOLOGICAL MANIFESTIONS

Most mucoceles are reported in adults, with ages ranging from 13 to 80 years (6, 11). Sites of involvement, in order of decreasing frequency , are the frontal, ethmoid, sphenoid, and rarely the maxillary sinus (1, 5, 8, 14, 15). Slightly more than half of our patients had ethmoid involvement, followed in frequency by sphenoid mucoceles. The clinical signs and symptoms of mucoceles vary depending on the size and location of the lesion and the direction of expansion. Ethmoid and frontal mucoceles in

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children and adults are usually not discovered until they grow large enough to produce a palpable mass in the superomedial aspect of the orbit, mild to moderate proptosis, some limitation in eye movement, diplopia, epiphora, or headaches. Generally there are no acute signs unless the mucocele has become infected. Although chronic sinus disease has frequently been described in adults, this was present in only one of our patients. Infrequently the mucocele is an incidental finding as in CASE V. Diplopia, proptosis, pulsating exophthalmos, opthalmoplegia, and optic atrophy are common with sphenoid mucoceles, both in our series and in the adult literature. This complex of symptoms has been termed the orbital inlet syndrome and results from pressure on the adjacent optic foramina and nerves (1, 7, 13). Encroachment into the nasopharynx is unusual (7) but was present in one patient. None of our patients had meningitis or brain abscess (7), endocrine dysfunction (10), or loss of olfaction (1), although these complications have been reported in adults. Radiographs of the paranasal sinuses may demonstrate unilateral or bilateral involvement. The patients analyzed had features characteristic of mucoceles, especially in the ethmoid or frontal sinuses: (a) Enlargement of the sinus with destruction of septa and thinning of the bony walls, so that the normal scalloped margins disappear. On occasion the sinus margins may be so thinned that they are visible on only one of many tomographic sections. (b) Opacification of the sinus, althoughthe involved sinus may be radiolucent if the bone destruction cancels out the increased density due to fluid accumulation in the sinus. (c) Pressure deformity or destruction of adjacent bone, with scattered areas of osteolysis or hyperostosis. Similar features can occur with sphenoid mucoceles, although they were not present in either of our patients. An interesting finding was present in CASE I; expansion of anterior ethmoid air cells extrinsically deformed the superomedial orbital wall, suggesting a frontal as well as ethmoid mucocele. Pluridirectional tomography (3,9) or CT is often helpful in demonstrating the characteristic radiographic features which otherwise may be missed or underestimated. Sphenoid sinus mucoceles are often misdiagnosed on radiographic examinations because of their association with more extensive bony alterations. Destruction of the floor of the sella turcica, erosion of the optic canals, widening of the superior orbital fissures, elevation of the anterior clinolds, and bulging into the nasopharynx, clivus, posterior ethmoids, and pterygomaxillary fossa are not unusual (1). Carotid angiography may show elevation or lateral displacement of the carotid siphon. Radiographic evidence of suprasellar extension of the mass is rare (10). DISCUSSION

Sinus opacification in older children is frequently due to sinusitis, either allergic or infectious. Other common causes of opacification include retention or mucoserous

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cysts and benign solid tumors, including papillomas, osteomas, and mucosal polyps. A useful distinguishing feature of mucoceles is expansion, distortion, and erosion of the bony walls by an indistinct sott-tissue mass: in contrast, solid tumors and most cystic masses generally have more distinct margins and less tendency toward destruction (6). The radiographic appearance in one of our patients with a sphenoid mucocele was indistinguishable from a meningocele or encephalocele. Typically the latter lesions exhibit a midline skull defect, a soft-tissue mass, an increased interethmoid distance, and a bifid nasal septum (3), but on occasion a definite diagnosis is made only at surgery as in CASE IV. Various types of malignant neoplasms may have radiographic features similar to those of a mucocele, including carcinoma, sarcoma, lymphoma, and nasopharyngeal angiofibroma. Sphenoid mucoceles must also be differentiated from intrasphenoid craniopharyngiomas and pituitary tumors (1, 10, 12). Although these lesions are prone to be more destructive and invasive than mucoceles, differentiation may be difficult (as in CASE III) until the patient is surgically explored. Mallinckrodt Institute of Radiology Washington University School of Medicine 510 S. Kingshighway St. Louis, Mo. 63110 REFERENCES 1. Bloom DL: Mucoceles of the maxillary and sphenoid sinuses. Radiology 85:1103-1109, Dec 1965 2. Bordley JE, Bosley WR: Mucoceles of the frontal sinus: causes and treatment. Ann Otol Rhinol Laryngol 82:696-702, Sep-Oct 1973 3. Dubois PJ, Schultz JC, Perrin RL, et al: Tomography in expansile lesions of the nasal and paranasal sinuses. Radiology 125: 149-158. Oct 1977 4. Hartung A, Wachowski T: Mucocele of the frontal sinus. With special reference to the roentgen aspects and report of four cases. Am J RoentgenoI34:30-36, Jul 1935 5. Hayes GJ, Creston JE: Mucocele of the sphenoid sinus. Arch Otolaryngol 79:653-656, Jun 1964 6. Lloyd GAS, Bartram CI, Stanley P: Ethmoid mucocoeles. Br J Radiol 47:646-651, Oct 1974 7. Lundgren A, Olin T: Muco-pyocele of sphenoidal sinus or posterior ethmoidal cells with special reference to the apex orbitae syndrome. Acta Otolaryngol 53:61-79, Feb 1961 8. McHenry LC Jr, Sullivan JF, Ryan MJ, et al: Mucocele of the sphenoid sinus. A benign lesion simulating a malignant process. N Engl J Moo 262:549-551, 17 Mar 1960 9. Minagi H, Margolis MT, Newton TH: Tomography in the diagnosis of sphenoid sinus mucocele (SSM). Am J Roentgenol 115: 587-591,JuI1972 10. Nugent GR, Sprinkle P, Bloor BM: Sphenoid sinus mucoceles. J Neurosurg 32:443-451, Apr 1970 11. Palubinskas AJ, Davies H: Roentgen features of nasal accessory sinus mucoceles. Radiology 72:576-584, Apr 1959 12. Roberson GH, Patterson AK, EI Deeb M, et al: Sphenoethmoidal mucocele: radiographic diagnosis. Am J Roentgenol 127: 595-599, Oct 1976 13. Simon HM Jr, Tingwald FR: Syndrome associated with mucocele of the sphenoid sinus. Report of two cases and their radiographic findings. Radiology 64:538-545, Apr 1955 14. Wigh R: Mucoceles of the fronto-ethmoidal sinuses. Analysis of roentgen criteria. Relation of frontal bone mucoceles to ethmoidal sinuses. Radiology 54:579-590, Apr 1950 15. Zizmor J, Noyek AM: Cysts and benign tumors of the paranasal sinuses. Semin Roentgenol 3:172-201, Apr 1968

Paranasal sinus mucoceles in children.

Pediatric Radiology Paranasal Sinus Mucoceles in Children 1 Marilyn J. Siegel, M.D., Gary D. Shackelford, M.D., and William H. McAlister, M.D. The c...
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