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Show Journal of Neuro- Ophthalmology 15( 2): 105- 108, 1995. © 1995 Raven Press, Ltd., New York Isolated Third- Nerve Palsy Associated with Frontal Sinus Mucocele Susan J. Ehrenpreis, M. D., and John F. Biedlingmaier, M. D., F. A. C. S. Isolated third- nerve palsies are seen most commonly with aneurysms, vascular disease, trauma, or neoplasms ( 1- 5). Sinus mucoceles have been known to cause orbital symptoms including proptosis and cranial nerve palsies. Rarely, an oculomotor nerve abnormality may be seen in the setting of mucoceles of the paranasal sinuses. We describe an unusual case of an isolated pupil- sparing third- nerve palsy as the presenting sign of a frontal sinus mucocele. Emphasis is placed on the discussion of sinus mucoceles and their relation to orbital symptoms. Key Words: Frontal- Sinus- Mucocele- Third nerve palsy. Manuscript received August 23, 1994. From the Friedenwald Eye Institute of Maryland General Hospital ( S. J. E.) and Division of Otolaryngology, Head & Neck Surgery, University of Maryland Hospital, Baltimore, Maryland, U. S. A. Address correspondence and reprint requests to Dr. S. J. Ehrenpreis, Friedenwald Eye Institute of Maryland General Hospital, 827 Linden Avenue, Baltimore, MD 21201, U. S. A. CASE REPORT A 79- year- old African- American man with a history of chronic open angle glaucoma, borderline hypertension, branch retinal vein occlusion OD, and hemiretinal vein occlusion OS had a 1- week history of ptosis of the left upper lid. The patient had no ocular pain, headaches, or diplopia. Pupils were equal and normally reactive, and visual acuity was 20/ 50 OD and 20/ 200 OS. There was 5 mm of left ptosis ( Fig. 1), and motility in the left eye was limited in elevation ( Fig. 2). Fourth- and sixth-nerve function was normal. Movement of the right eye was full. There was resistance to retrodisplace-ment of the left globe. Corneal sensation was normal in both eyes. Anterior segment examination revealed bilateral nuclear sclerotic cataracts. There were normal intraocular pressures by applanation tonometry. Goldmann perimetry revealed nasal steps and paracentral scotomas in both eyes ( stable from previous visual fields). Ophthalmoscopy revealed inferior intraretinal hemorrhages respecting the horizontal meridian OS, consistent with hemiretinal vein occlusion. There was a moderate amount of macular edema. The arteries were narrowed OU, and a pigmentary macular disturbance was present OD. Cup- to- disc ratios were 0.6 OD and 0.75 OS. There were healthy rims and sharp disc margins. A contrast- enhanced computed tomography ( CT) scan of the brain and orbits revealed a soft-tissue density in the left orbital area and maxillary sinus. An eye, ear, nose, and throat ( ENT) consult was requested to evaluate the maxillary sinusitis noted on orbital CT. Full- sinus CT scan revealed opacification of the left frontal sinus with extension into the orbit ( Fig. 3). Magnetic resonance imaging ( MRI) of brain/ orbits/ facial bones revealed an opacification of the left maxillary sinus and a cystic mass extending from the floor of the frontal sinus into the orbit with displacement of the superior 105 106 S. J. EHRENPRE1S AND ]. F. BIEDLINGMAIER muscles ( Fig. 4). Surgical exploration was performed using a bicoronal scalp flap. After removal of the anterior frontal sinus table, a mucocele of the frontal sinus was found. Histopathological examination of the biopsy material revealed moderate acute and chronic inflammation and granulation tissue consistent with a mucocele. A gram-negative rod { Achromobacter xylosoxidans biotypes I and II) grew from frontal sinus cultures. The cyst had eroded a 2- cm defect through the orbital roof, but the orbital periosteum was intact ( Fig. 5). The sinus was stripped of the mucosal lining and obliterated with calvarial bone dust. The free anterior FIG. 2. Patient preoperatively with limited upgaze of left eye. FIG. 3. Axial view computed tomography scan showing mucocele in left frontal sinus ( arrow). table flap was replaced using micro- mini plates. Postoperatively, the patient did well, and both the ptosis and third- nerve palsy recovered after 1 month of follow- up ( Figs. 6 and 7). DISCUSSION This patient was first seen with an isolated pupil- sparing superior division oculomotor nerve palsy. The main differential diagnosis for pupil-sparing third- nerve palsies includes vasculopathic FIG. 4. Coronal view magnetic resonance imaging scan showing mucocele extension into left orbit and opacified left maxillary sinus. / Neuro- Ophthalmol, Vol. 15, No. 2, 1995 THIRD- NERVE PALSY WITH TRONTAL SINUS MUCOCELE 107 FIG. 5. Operative view showing large orbital roof defect after mucocele removal ( arrow). paresis, myasthenia gravis, thyroid eye disease, chronic progressive external ophthalmoplegia, orbital inflammatory pseudotumor, and midbrain lesions ( 23). Although this patient has vascular disease that would put him at risk for cranial nerve palsies, his case emphasizes the importance of considering sinus pathology when evaluating a third- nerve palsy. The fact that patients may have FIG. 6. Resolution of ptosis 1 month postoperatively. FIG. 7. Return of upgaze 1 month postoperatively. predisposing factors for cranial nerve palsies makes sinus disease a diagnosis of exclusion. The distribution and etiology of third-, fourth-, and sixth- nerve palsies have been reported ( 1,4,5). In a series of 1,000 patients of all ages, sixth- nerve palsy was noted in 45% of the cases, third- nerve palsy in 30%, fourth- nerve palsy in 11%, and multiple nerve involvement in 14%. The etiology was undetermined in 26% of cases, head trauma in 20%, vascular in 17%, neoplastic in 14%, due to other in 15%, and aneurysms in 7% ( 3). As mentioned previously, mucoceles may also cause cranial nerve palsies. A mucocele is a chronic cystic lesion of the paranasal sinuses that is slowly expanding and may require 10 or more years to become symptomatic ( 6). Cyst- like lesions of the paranasal sinuses have been described almost 100 years ago, with Rollett describing the first mucocele in 1896 ( 7). With increasing size, bony erosions can occur, and the mucocele extends outside the sinus. Once a mucocele expands beyond its natural bony cavity and damages important adjacent structures, dramatic symptoms are likely to occur. A mucocele can exert pressure on the optic nerve and chiasm, causing progressive loss of vision or even acute blindness. It may displace the cavernous portion of the carotid artery laterally and create transient or permanent palsies of the oculomotor nerves ( 8). Mucoceles are most frequently found in the frontal sinus ( 9). Frontal headaches and proptosis are the most common complaints, with displacement of the globe in a downward and outward direction ( 10). Diplopia can occur secondary to displacement of the globe. There have been no reported cases on the association between oculomotor nerve dysfunction and frontal sinus mucocele in almost 20 years. In 1975, Guerry and Smith reported three cases of frontal sinus mucoceles presenting as otherwise asymptomatic squamous cell carcinoma. The possibility of third- nerve involvement existed in one of these cases in which there / Neuw- Ophthalmol, Vol. 15, No. 2, 1995 108 S. J. EHRENPRE1S AND J. F. BIEDLINGMAIER was blepharoptosis and restriction of movement in the affected eye up and in ( 11). Mucoceles may erode through the anterior wall of the frontal sinus as a tender, fluctuant mass beneath the periosteum of the frontal bone ( Pott's puffy tumor). If the posterior sinus wall is destroyed, epidural abscess, subdural empyema, and meningitis or a brain abscess may develop ( 12). Ethmoid sinus mucoceles commonly destroy the lamina papyracea and displace the orbital contents laterally or downward, causing exophthalmos and diplopia. If the superior orbital fissure becomes compressed, ocular palsies and decreased sensation in the forehead could result ( 13). A cause of recurrent unilateral, painful oculomotor nerve palsy has been reported with an ipsilateral posterior ethmoidal mucocele ( 14). Maxillary sinus mucoceles rarely lead to neurologic complications. Several documented cases have been reported with proptosis due to upward displacement of orbital contents, exophthalmos caused by destruction of the orbital floor, diplopia due to displacement of orbital contents, and paresis of oculomotor and optic nerve, as in the orbital apex syndrome ( 15- 18). Only 1% of all mucoceles are seen in the sphenoid sinus ( 19). Sphenoid sinus mucoceles must be suspected when there is an expanding mass in the sphenoid sinus and sella turcica. Recurrent headaches, diplopia, and visual- field defects are characteristic ( 20). Several cases have been recently reported of a sphenoid sinus mucocele mimicking diabetic ophthalmoplegia and retrobulbar neuritis and a calcified sphenoid mucocele with a third-nerve palsy ( 19,21). Two cases of sphenoid sinus mucoceles, the first with a chiasmatic syndrome and the second with oculomotor paralysis, have also been reported ( 22). It is rare that an oculomotor nerve abnormality is seen in association with a mucocele. Although older patients with vascular disease and an oculomotor nerve palsy can often be managed conservatively, this case illustrates the importance of a more aggressive diagnostic approach. Sinus pathology must be considered in the work- up of a patient with a cranial nerve palsy. More emphasis should be placed on the CT scan evaluation to include the sinuses. Plain radiographs may be sufficient to make the diagnosis, but CT scan is the procedure of choice for defining the extent of the lesion and assuring appropriate surgical approach. If the screening CT scan is suggestive of sinus pathology, a following coronal CT scan through the sinus is better than an MRI because water density will increase false positives. The case presented here describes a rare finding of a frontal sinus mucocele seen as a partial third- nerve palsy. Surgical exploration and repair resulted in a rapid return of ocular function. REFERENCES 1. Rucker CW. 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