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Mucoceles arising from the anterior clinoid is rare. Although intrinsically benign, mucoceles in the sphenoid sinus (anterior clinoid variant) can lead to complications due to their proximity to other structures. We present a case of a young man whose visual complaints were typical for retrobulbar optic neuritis. Results of radiologic tests for diagnosis led us to the early detection of a surgically treatable condition, a sphenoid sinus mucocele (anterior clinoid variant). Early surgery was helpful in ameliorating the symptoms, leading to full recovery of visual function. The clinician should be aware of this rare entity with its presentation in a wide variety of signs and symptoms.

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PURPOSE: To determine the most effective, safe, and cost-efficient treatment for ocular adnexal Kaposi's sarcoma in the acquired immune deficiency syndrome. METHODS: Eighty-two patients with ocular adnexal Kaposi's sarcoma related to the acquired immune deficiency syndrome were examined, and 25 were selected to participate in a 3-year study that treated the ocular lesions based on stage of development and location. RESULTS: Of 14 patients with bulbar conjunctival Kaposi's sarcoma treated with surgical excision, 2 stage III lesions recurred during a follow-up that ranged from 8 to 31 months; no stage I or stage II lesion recurred. Of 7 patients with eyelid Kaposi's sarcoma treated with cryotherapy, 2 stage III lesions recurred during a follow-up that ranged from 9 to 24 months; no stage I or stage II lesion recurred. Four patients with stage III Kaposi's sarcoma of the bulbar conjunctiva were treated with fluorescein angiography-based surgical excision. None of these lesions recurred during a follow-up that ranged from 4 to 8 months. CONCLUSION: A treatment regimen for ocular adnexal Kaposi's sarcoma related to the acquired immune deficiency syndrome based on tumor stage and location, using cryotherapy and surgical excision with or without fluorescein angiography, is effective, safe, and cost-efficient.

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Computer-assisted photokeratoscopy was used to evaluate the topographic characteristics of corneas preoperatively and postoperatively in seven patients who underwent surgery for correction of postkeratoplasty astigmatism. The steep hemimeridians were typically separated by an angle other than 180 degrees (mean, 162.5 degrees) and the flat hemimeridians were often not orthogonal to the steep hemimeridians. Asymmetry of power (1.5 or more diopters) between these two major hemimeridians was also observed in three patients. Relaxing incisions were placed in the two steep hemimeridians and compression sutures were placed in the flat hemimeridians. The mean percent of reduction of astigmatism (vector-corrected) was 81.1%. The amount of keratometric astigmatism, and the degree of asymmetry of the hemimeridians were not correlated with the percent of reduction of astigmatism after placement of the relaxing and compression sutures. Computer-assisted topographic analysis may prove useful in planning transverse keratotomies centered on the steep hemimeridians and in placement of compression sutures in flat hemimeridians.

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We examined histopathologically 18 ocular adnexal Kaposi's sarcoma lesions related to acquired immunodeficiency syndrome. These lesions were classified into three types. Type I consisted of thin, dilated vascular channels lined by flat endothelial cells with lumen-containing erythrocytes. Type II featured plump, fusiform, endothelial cells, often with a hyperchromatic nucleus and foci of immature spindle cells and occasional slit vessels. Type III was characterized by large aggregates of densely packed spindle cells with hyperchromatic nuclei, occasional mitotic figures, and abundant slit spaces often containing erythrocytes in between. Clinically, type I and type II tumors were patchy and flat (less than 3 mm in height) and of less than four months' duration. Type III tumors were nodular and elevated (greater than 3 mm in height). We describe the clinical and histopathologic types of Kaposi's sarcoma that may help in diagnosis.

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Two patients, both seropositive for the human immunodeficiency virus, developed rapidly progressive retinal necrosis associated with a systemic herpes zoster infection. The retinitis in these patients was characterized by primary involvement of the outer retina, with sparing of the inner retina and retinal vasculature until late in the disease process; a rapidly progressive course; poor response to intravenous acyclovir; and development of rhegmatogenous retinal detachment. In one of the patients, the retinitis was initially multifocal. Electron microscopy of a retinal biopsy specimen from one of the patients demonstrated virus particles consistent with a herpesvirus, and polymerase chain reaction disclosed herpesvirus in a retinal biopsy specimen of the other patient. This entity may represent a distinct form of acute retinal necrosis that is seen in immunocompromised individuals.

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Pneumocystis carinii pneumonia is a major cause of morbidity and mortality in patients with the acquired immunodeficiency syndrome. When P. carinii is disseminated, the choroid may be involved and the infection is often fatal. We examined, treated, and followed up two patients who developed choroidal lesions typical of P. carinii while taking aerosolized pentamidine for prophylaxis. The choroidal lesions gradually resolved after three weeks of therapy with intravenous trimethoprim and sulfamethoxazole in one patient, and after three weeks of therapy with parenteral pentamidine in the other patient. The patients did not have clinical or laboratory evidence of P. carinii infection other than in the eye. It thus appears that early ophthalmologic examination may detect disease before it is threatening to sight and allow systemic therapy to be instituted before widely disseminated infection results in a fatal outcome.

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The time course of the appearance of fibronectin (Fn) on wounded corneal surfaces was studied in vitamin A-deficient (A-) and pair-fed control rats. At various times following a central epithelial abrasion, the tissue was harvested, and Fn was localized on frozen corneal sections by an indirect immunofluorescence technique. There was no detectable Fn in intact, nonwounded control or intact A-corneas, except for Descemet's membrane. Within a half hour after abrasion, a band of Fn appeared on the denuded corneal surface of pair-fed control rats and became a continuous, prominent layer at four hours. This layer remained until 16 hours but disappeared at 24 hours when the epithelium had resurfaced over the defect. In severely A- rats, reepithelialization following central epithelial abrasion was delayed, and no Fn band was discernible at any time from one to 32 hours after injury. Light microscopy revealed a progressive increase in polymorphonuclear neutrophil infiltration with time in the underlying stroma in severely A- rats. This study indicated that in severe vitamin A deficiency, delayed epithelial migration is associated with an inflammatory cell layer and occurred in the absence of Fn.

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Six epikeratoplasty (EKP) specimens were obtained 1 to 10 months after refractive keratoplasty for aphakia (2 cases), keratoconus (1 case), and myopia (3 cases). The EKP grafts were removed because of postoperative complications such as delayed reepithelialization, lenticule edema, stromal ulceration, graft opacification, and inability to improve vision in a clear graft. The extent of epithelial abnormalities was associated directly with the severity of structural changes in the underlying basal lamina, Bowman's layer, and stroma as evidenced by electron microscopic examination. Epithelial defects were associated with varying degrees of sterile stromal ulceration of the underlying lenticule. In one case, an extensive full-thickness ulceration of the EKP button occurred in the area of persistent epithelial defects and was associated with a major ulceration of the underlying recipient's corneal stroma. Removing the failing button halted the ulcerative process with prompt reepithelialization. In the absence of inflammation and infection, the persistent epithelial abnormalities and defects leading to proteolytic degradation of the EKP buttons constitute an important mechanism of graft failure with major visual consequences and possibly irreversible ocular damage.

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The cytomorphologic findings of a vitrectomy specimen from the right eye of an 80-year-old woman with an unsuspected primary choroidal malignant melanoma are described. The patient had undergone a closed vitrectomy because of chronic vitreous hemorrhage. Histocytology of the vitreous fluid specimens revealed melanoma cells of variable shape and size (from 30-150 microns) with eccentric nuclei. Many of these cells were binucleated or multinucleated with small, uniform, evenly dispersed intracytoplasmic melanin granules. The histocytologic findings together with the postoperative tumor characteristics by ultrasonography and fluorescein angiography suggested the diagnosis of choroidal malignant melanoma.

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A two-month-old girl with aniridia was found to have an optic pit in one eye and the morning glory disc anomaly in the other eye. The patient's mother had corectopia and atypical iris defects. The findings are described and the embryology of the various abnormalities briefly reviewed. This is the first report of such an association of findings in a family with aniridia. Failure of normal neuroectodermal development may play a role in the development of optic pits and the morning glory disc anomaly.

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The data on 58 patients with retinoblastoma managed at the American University of Beirut, Medical Center over the last 35 years is reviewed. The epidemiology, genetics and clinical features of this tumor appear to be the same in the Middle-East as well as the rest of the world. The notable difference between the data in this study and that from other studies is the marked delay in diagnosis of the tumor in this part of the world leading to a higher incidence of extraocular extension, a more advanced stage of the disease and poorer survival rates. This seems to be a feature of medically underdeveloped societies. Increased public awareness of the early signs and symptoms of the disease, as well as routine fundoscopy on all infants are advocated to improve early detection, leading to improved survival rates and prevention of unnecessary enucleations.

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The case report of a 16-month-old girl with lamellar ichthyosis and unilateral megalocornea is presented. This is a previously unrecorded association and the first report of a unilateral megalocornea in a female.

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A clinical and histopathologic review of 23 cases of melanocytoma of the ciliary body discloses that the tumor is unilateral, extends into contiguous structures, slowly enlarges, and commonly undergoes necrosis. We report the clinical and histopathologic features of four cases of melanocytoma of the ciliary body, review the previously reported and presented cases, and discuss the differential diagnosis of melanocytoma of the ciliary body.

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We describe the occurrence of bilateral occipital cortical infarction following respiratory failure in two patients of differing ages. The precipitating factors, clinical manifestations, and ophthalmologic findings also differed, but the hypoxic effects on the cortex were the same as evidenced by the CT scanning studies.

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Morphea, or localized scleroderma, of the eyelids is an uncommon disease. Morphea usually involves the thorax, trunk, lower and upper extremities, face, and genitalia. In the present report a patient with a biopsy-proven morphea of both upper eyelids is described. The salient histopathologic features included thinning of the epidermis with thickening and sclerosis of the collagen fibers in both the papillary and reticular dermis. There was a marked decrease in the fibrocytes. The eccrine sweat glands were entrapped by sclerotic collagen fibers. The pilosebaceous units were markedly decreased in number. There was a moderate lymphocytic infiltration in the dermis and a prominent lymphocytic perivasculitis. The clinical and histopathologic features of morphea are compared with those of lichen sclerosus et atrophicus.

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Light and transmission electron microscopic examinations of two postmortem eyes from an 80-year-old woman with clinically well-documented Best's macular dystrophy disclosed striking abnormalities in the retinal pigment epithelium (RPE), photoreceptors, and sub-RPE area. The RPE cells were flattened, with displacement of the nuclei toward the apex and diffuse deposition of abnormal lipofuscin and pleomorphic melanolipofuscin granules. A PAS-positive, acid-mucopolysaccharide-negative, electron-dense, finely granular material was deposited in the inner segments of the degenerating photoreceptors and the Mueller cells. An abnormal fibrillar material was present underneath and in close association with the RPE cells, just beneath the area of photoreceptor cell loss. The choriocapillaris was normal. Breaks in Bruch's membrane and neovascularization were noted in the foveolar region. There is evidence that the sensory-retinal changes are primary and that the RPE changes are secondary.

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Occlusions of nine branch retinal veins in eight eyes of seven patients were studied histopathologically by serial sections through the affected areas. Intravitreal neovascularization from the disc, retina, or both was noted in four eyes. Two additional eyes had intraretinal neovascularization (intraretinal microvascular abnormalities). Cystoid macular edema was present in five eyes. A fresh or recanalized thrombus was noted at the site of vein occlusion in all eyes. Inner ischemic atrophy of the retina was found distal to the area of occlusion in six of the nine affected quadrants of the eight eyes. Although the corresponding branch retinal arteries showed varying degrees of sclerosis (severe, three eyes; moderate, five eyes; and minimal, one eye), no definite thrombus was observed in any of them.

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Forty-four eyes from 39 patients with either lamellar macular holes (17 eyes), full-thickness macular holes (18 eyes), and/or macular cysts (9 eyes) were studied histopathologically. Lamellar and full-thickness holes were frequently found in eyes with a history of trauma or ocular surgery. Diabetes mellitus was the most common condition associated with macular cysts. Four lamellar holes and four full-thickness holes occurred in eyes with senile macular degeneration. Six lamellar holes, eight full-thickness holes, and one macular cyst developed on an idiopathic basis. Residual cystoid macular edema was the most prevalent accompanying pathologic feature. Cystoid macular edema was also noted in the opposite eye in seven cases of lamellar and full-thickness holes. A preretinal glial membrane, thought to be a secondary change, was found at or near the edges of the lamellar or full-thickness holes or over the macular cyst in the majority of cases. Wrinkling of the internal limiting membrane (ILM) was present in five cases. Vitreous traction, with or without an operculum, was infrequently associated with these entities.

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