Skull Base Surgery Ocular Manifestations of Sphenoid Mucoceles: CLINICAL FEATURES AND NEUROSURGICAL MANAGEMENT OF THREE CASES AND REVIEW OF THE LITERATURE Nedal Hejazi,* Alfred Witzmann* and Werner Hassler† *Department of Neurosurgery, LKH Feldkirch, Austria; †Department of Neurosurgery, Klinikum Kalkweg, Duisburg, Germany Hejazi N, Witzmann A, Hassler W. Ocular manifestations of sphe- noid mucoceles: clinical features and neurosurgical management of three cases and review of literature. Surg Neurol 2001;56:338 – 43. BACKGROUND Sphenoid mucoceles (SMCs) are unusual lesions, with about 130 cases reported in the literature. Although be- nign, they may involve the orbit and cause acute restric- tive ophthalmoplegia, proptosis, and reduced visual acuity. METHODS AND RESULTS We present three cases (1 male, 2 females, aged 35, 36, and 46 years) of orbital involvement with acute de- creased visual acuity by SMC. The lesions were promptly excised via a transnasal approach to decompress the optic nerve. After the decompression, the patients recov- ered completely. CONCLUSION We believe that immediate surgical drainage of the SMC and prolonged antibiotic therapy are necessary and re- sulted in recovery of visual acuity. Prompt microsurgical transsphenoidal decompression is simple, effective, safe, and is necessary for avoiding persistent visual loss. An- tibiotic therapy alone usually results in a poor outcome. We discuss the etiology, clinical manifestations, and man- agement of this rare condition in the light of 130 other cases in the literature. © 2001 by Elsevier Science Inc. KEY WORDS Mucocele, sphenoid sinus, optic nerve decompression. S phenoid mucoceles (SMCs) are unusual le- sions, with about 130 cases reported in the world literature since Berg’s first description in 1889 [5]. Although rare, SMC should be considered in the differential diagnosis of cystic lesions of the anterior visual pathways. Ophthalmic manifesta- tions may include restrictive ophthalmoplegia, proptosis, and reduced visual acuity because of optic nerve compression. The etiology of the SMC is still uncertain, but it is almost unanimously thought to be caused by an accumulation of mucus due to phlogistic stenosis of the sinus ostium or of one of its recesses [16,18]. The optic nerve may be in- volved either in a phlogosis because of contiguity or in a direct compression with development of a retro-bulbar neuropathy [1,17]. We present three personal cases of SMC with optic nerve compres- sion and review briefly 130 other cases of SMC in the literature. Summary of Cases In the period from 1990 through 2000, we treated 3 patients with large SMCs accompanied by visual disturbances. CASE 1 A 36-year-old woman was referred for an opinion from a neurological department after she devel- oped left-sided headache associated with lacrima- tion and nausea, which had intensified over a 4-day period. Visual acuity remained unaffected until the third day in hospital, when it was noted to decrease to 0.2 in the left eye with left-sided color-blindness. The left side of her face was slightly swollen. There was no pain on palpation of her frontal or maxillary sinuses. Palpation of the orbit rim and regional lymph nodes showed no abnormality and percus- sion of the sinuses was not painful; extraocular movements were full and did not evoke diplopia. Ophthalmoscopic examination of the left eye Address reprint requests to: Dr. Nedal Hejazi, Department of Neurosur- gery, LKH Feldkirch, Carinagasse 9, A-6800 Feldkirch, Austria. Received November 22, 2000; accepted June 12, 2001. 0090-3019/01/$–see front matter © 2001 by Elsevier Science Inc. PII S0090-3019(01)00616-4 655 Avenue of the Americas, New York, NY 10010