Faculty Bibliography

We report on retinal detachments in six eyes of four patients with incontinentia pigmenti. These nonrhegmatogenous traction retinal detachments are characterized by extensive preretinal and vitreous fibrous organization that pull the retina anteriorly behind the lens. The clinical course and fundus appearance of the retinal detachments, beginning with the avascular peripheral retina and leading to traction retinal detachment, are similar to those of cicatricial retinopathy of prematurity. Vitreous surgery was performed on three eyes (two patients), with partial reattachment of a total retinal detachment in one eye and complete reattachment of a partial retinal detachment in a second eye. These cases represent the first successful surgical interventions reported for retinal detachment in incontinentia pigmenti

We prospectively evaluated a modified version of a previously reported technique of retrobulbar anesthesia for vitreoretinal surgery involving the use of a blunt 19-gauge cannula to directly infuse anesthetic into the retrobulbar space. Using this method, 60 consecutive patients undergoing scleral buckling surgery had effective anesthesia and akinesia, with no complications

Posterior scleritis was diagnosed in four adolescent boys. These patients represented a distinct subgroup of patients with posterior scleritis that differed from the adult variant by gender, lack of systemic disease, and absence of the associated ocular findings often seen in the adult variant of the disorder. The four patients had diminished visual acuity, ocular pain, and exudative retinal detachments at initial examination. Fluorescein angiography demonstrated multiple pinpoint leaks at the level of the retinal pigment epithelium in three patients with late-phase patchy staining of a mass-like lesion in one patient. B-scan ultrasonography demonstrated choroidal and scleral thickening with increased acoustic density of the choroid in all patients. Systemic evaluation of these patients disclosed no underlying disease. Clinical signs and symptoms resolved in three of the patients after treatment with low-dose, orally administered corticosteroid or noncorticosteroid anti-inflammatory medication. High systemic doses of corticosteroid in combination with noncorticosteroid anti-inflammatory medication and local corticosteroid therapy was required to induce remission in one patient. All patients recovered good visual acuity

Two patients with renal disease and choroidal effusions are presented. The renal disease was mediated by humoral immunologic mechanisms as demonstrated by immunofluorescent studies of renal biopsies. Experimental and clinical studies provided evidence of simultaneous immune-complex deposition in both the renal glomerulus and the uvea of the eye. Immune-mediated vascular damage may result in glomerulonephritis and choroidal effusions