Richard B. O'Grady

Conjunctival inflammation and amyloidosis in allergic granulomatosis and angiitis (Churg-Strauss syndrome).

A 37-year-old woman developed nodular, waxy conjunctival lesions associated with the exacerbation of Churg-Strauss syndrome. Conjunctival and skin biopsy specimens demonstrated a diffuse inflammatory process composed of eosinophils, plasma cells, lymphocytes, and focal collections of histiocytes. There were amyloid deposits in the conjunctiva and skin. To our knowledge, this is the first report of conjunctival changes and amyloidosis associated with the Churg-Strauss syndrome.

Endogenous intraocular Nocardia asteroides in Hodgkin's disease.

A 60-year-old man receiving antituberculous and corticosteroid therapy for a granulomatous disease of uncertain etiology was found to have a chorioretinal mass in his right eye. Fluorescein angiography showed blockage of fluorescence by the mass and late leakage. Autopsy findings were compatible with Hodgkins disease with disseminated nocardiosis caused by Nocardia asteroides. Organisms typical of Nocardia were found in the choroid and subretinal space. The patients history, ophthalmic examination, and fluorescein angiographic findings suggested a type of chorioretinal involvement.

Cyst of the Intraorbital Optic Nerve Sheaths

A 43-year-old woman had a history of gradual unilateral visual acuity loss and proptosis. A 2.5-cm cystic dilatation of the intraorbital dura mater was removed. Microscopic examination revealed a vascular proliferation within the leptomeninges. We inadvertently injected the lesion during orbitography, providing us with an unusual radiographic appearance.

Scleritis and Wegener's Granulomatosis in Children

We treated two children with scleritis (one unilateral, one bilateral), in whom Wegeners granulomatosis was diagnosed on the basis of pathologic changes in respiratory tract mucosa. Both patients were girls, 13 and 14 years of age, respectively. One patient had otitis media and a nodular scleritis. Laboratory test results demonstrated an increased erythrocyte sedimentation rate and microscopic hematuria. A biopsy of the sinus confirmed the diagnosis of Wegeners granulomatosis. The second patient had fever, arthralgias, a nonproductive cough, and bilateral scleritis. Laboratory test results demonstrated an increased erythrocyte sedimentation rate, positive test results for rheumatoid factor, and bilateral pulmonary nodules on chest x-ray. Open-lung biopsy confirmed the diagnosis of Wegeners granulomatosis. Both patients responded well to treatment with a combination of prednisone and cyclophosphamide.

Pilomatrixoma (Benign Calcifying Epithelioma of Malherbe)

A 24-year-old white man had a large subcutaneous discolored tumefaction of the right lower lid, successfully treated by two separate incision and drainage procedures. Microscopic appearance initially suggested basal cell epithelioma, but further studies provided a correct diagnosis of pilomatrixoma (calcifying epithelioma of Malherbe). This lesion probably represents aberrant primitive germ cells differentiating towards a hair matrix cell. Basaloid and squamoid (shadow) cells with or without focal calcifications in shadow cell areas are typical, and the clinical presentation is also highly suggestive of pilomatrixoma.

Cat model for intraocular pressure reduction after transscleral Nd:YAG cyclophotocoagulation

Transscleral neodymium:yttrium-aluminum-garnet (Nd:YAG) laser cyclophotocoagulation (TSNYC) is used to lower intraocular pressure (IOP) in glaucoma patients refractory to conventional medical and surgical therapy. Our study investigates the ability of TSNYC to lower IOP in normal cats. One eye of 13 cats was treated with non-contact TSNYC. Mean pretreatment IOP was 25 mm Hg (vs. 25.3 mm Hg in contralateral control eyes). Eyes received 80 laser applications over 360 degrees delivered at least 3 mm posterior to the limbus with maximum power (8 to 9 joules) and maximum retrofocus (3.6 mm). Eyes were retreated if IOP was not reduced below baseline after 2 weeks. By 4 weeks, IOP was decreased in all treated eyes by a mean of 29.2% and was maintained as long as 20 weeks (mean decrease 14.8%). IOP in 10 eyes was lowered after a single treatment session. Of these, 2 eyes had IOP spikes > 10 mm Hg prior to IOP reduction. Three cats required retreatment to maintain IOP reduction. All eyes developed transient (< 4 weeks) postoperative uveitis and 3 eyes developed rubeosis iridis which resolved with topical corticosteroids. Histologic examination (6 weeks post-treatment) showed focal disruption of the pigment epithelium and to a lesser degree the nonpigmented epithelium at the base of the ciliary body and in the valleys of the pars ciliaris. The epithelium at the apices of the ciliary processes appeared intact. Vascular engorgement was variably present. This study demonstrates that TSNYC lowers IOP in cats. This animal model will be useful for investigating mechanism(s) responsible for TSNYC-induced IOP reduction.

Mucolipidosis IV in an African American patient with new findings on electron microscopy.

Purpose. We report an unusual case of mucolipidosis IV in a patient of African ancestry, with intracytoplasmic inclusions of the corneal endothelium found on electron microscopy. Method. Clinical description with light and electron microscopy. Results. We describe a case of mucolipidosis IV diagnosed in a patient of African ancestry after penetrating keratoplasty. Electron microscopic evaluation revealed intracytoplasmic inclusions in both the corneal epithelium and endothelium. Conclusion. The diagnosis of mucolipidosis in a patient of African ancestry is unusual, as this genetic disorder is found predominantly in individuals of Jewish descent. Corneal endothelial involvement in mucolipidosis IV has not previously been reported.