Delayed onset of bilateral acute retinal necrosis syndrome: a 34-year interval.

The case is described of a male patient who had a history of acute retinal necrosis (ARN) OS at age 13 years. The eye subsequently had an inoperable retinal detachment with residual light perception visual acuity. After a 34-year disease-free interval, ARN developed OD that responded to medical treatment. This case represents the longest reported interval of ARN quiescence with eventual bilateral involvement and illustrates the importance of long-term patient follow-up.

Dystrophic calcification of silicone scleral buckling implant materials.

In six patients, removal of solid silicone scleral buckling implant materials that had been in place between eight and 21 years disclosed gray-white deposits firmly adherent to the silicone. Four of the six patients had culture-proven infections, whereas two had no evidence of infection and had negative cultures. One of these two patients, however, had intermittent pain, which was the indication for removal of the implant. Analysis of the deposits disclosed that they were calcium phosphate. The exact mechanism responsible for the calcification on the silicone material is unknown. Dystrophic calcification can occur without infection in injured tissue wherein extracellular deposits of devitalized cells, blood cells, and lipids may act as a nidus for calcification. In the presence of infection, bacteria may serve as such a nidus. Additionally, it is possible that biofilm produced by the bacteria had a role in the deposition of calcium phosphate, as well as in its firm adhesion to the silicone materials. We considered the possibility of similar deposits developing on intraocular silicone lenses.

Choroidal effusions in two patients with glomerulonephritis.

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.

Abnormal scleral collagen in nanophthalmos. An ultrastructural study.

Ten patients with bilateral nanophthalmos underwent sclerectomies for uveal effusion. Ultrastructural examination of the sclera revealed abnormal collagen in seven patients. Four showed dramatic fraying of the collagen fibrils into fine filaments 2 to 3 nm in diameter. In three of these cases and three other cases without fraying, there were foci of 10- to 35-nm small collagen fibrils, some appearing to arise by splitting of otherwise normal collagen fibrils. In areas of fraying, elastic fibers were absent. All patients had a wider range of collagen diameters than did control subjects. The youngest patient with fraying also had Hallermann-Streiff syndrome. In three patients, no collagen abnormality was found. The clinical feature correlating best with the presence of abnormal collagen was an extremely small eye, since the three patients without collagen abnormality had the largest eyes (range of anteroposterior diameters, 19.2 to 20.3 mm). Nanophthalmos appears to result from several distinct defects.

Retinal manifestations of neurofibromatosis. Diagnosis and management.

Five patients presented with vision-threatening retinal tumors and systemic signs of neurofibromatosis, including neurofibromatosis type 1 (four patients) and familial cafe-au-lait spots (one patient). These tumors included large retinal astrocytic hamartomas, multiple retinal capillary hemangiomas, and combined hamartomas of the retina and retinal pigment epithelium, which resulted in rubeotic glaucoma, vitreous hemorrhage, and retinal detachment. Surgical therapy included retinal cryopexy, xenon and argon photocoagulation, scleral buckling, and pars plana vitrectomy with excisional retinal biopsy. Retinal tumors may result in marked visual loss in patients with neurofibromatosis, and vitreoretinal surgery may restore useful vision in some of these patients.

Argon laser photoablation of a choroidal osteoma.

Choroidal osteomas are rare, juxtapapillary choroidal tumors, which are usually unilateral but can be bilateral in as many as 30% of patients. Choroidal neovascularization may complicate this condition and be associated with severe visual loss. A patient treated with argon laser photocoagulation for SRNVM is described, and clinical and radiographic evidence of destruction of the osteoma during an 8-year follow-up is presented. The possible mechanisms of laser's ablative effect on bone, in addition to clinical application for choroidal osteoma treatment, are discussed.

Cataract surgery in nanophthalmic eyes.

Cataract surgery in nanophthalmic eyes may be complicated by postoperative uveal effusion. Evidence is presented that prophylactic lamellar scleral resection with decompression of the vortex veins, performed 2 months or more prior to cataract extraction, may reduce the incidence of this severe complication. A randomized study to determine the benefit of such prophylaxis is advocated.

Vascularized vitreous membranes in congenital retinoschisis.

Congenital retinoschisis is a bilateral x-linked disease characterized by a stellate maculopathy and retinoschisis. The pathogenesis is unknown. This article describes a patient with congenital retinoschisis and documents the unique development of vascularized vitreous membranes. A posterior vitreous separation with subsequent vascularization of the posterior hyaloid face occurs early in the course of the disease process and is coincident with the development of maculopathy and schisis cavities. This process may represent an important element in the pathogenesis of the maculopathy and schisis cavities in congenital retinoschisis. With atrophy of the neovascular tissue, a membrane remains and consists of a thickened area of the posterior hyaloid face.

Glial cell component in retinoblastoma.

The right eye of a 4-month-old girl with a large, unilateral, sporadic retinoblastoma was enucleated. The tumor was unusual because it contained Flexner-Wintersteiner rosettes with extremely large lumina. Smaller rosettes and undifferentiated tumor cells were observed within the lumina. Also of importance were cells resembling glial cells which were intermixed with more typical cuboidal retinoblastoma cells. These cells had electron microscopic features typical of glial cells and stained positively for glial fibrillary acidic protein in immunohistochemical studies. Rosettes and glial cells continued to be observed in the tumor carried in tissue culture through two passages over a 7-month period. This tumor is presented because of its unusual rosette structures and because it confirms recent reports describing a glial cell component in retinoblastoma.

Peripapillary subretinal neovascularization in peripheral uveitis.

A 29-year-old white female with peripheral uveitis developed bilateral disc edema and unilateral peripapillary subretinal neovascularization which was successfully treated with argon laser photocoagulation. To our knowledge, this is the first reported case of peripapillary subretinal neovascularization complicating the course of peripheral uveitis.

Peripapillary subretinal neovascularization and serous macular detachment. Association with congenital optic nerve pits.

Congenital anomalous disc changes were associated with acquired macular detachment and peripapillary choroidal neovascularization in two cases. The anomalous disc changes resembled optic nerve pits. In one case, the peripapillary choroidal neovascularization was treated with argon laser photocoagulation, with subsequent reattachment of the macula and considerable improvement in the visual acuity. Although the pathogenesis of macular detachment occurring with optic nerve pits is usually not disclosed by fluorescein angiography, leakage from choroidal neovascularization can occur with this congenital defect and may contribute to the formation of a neurosensory macular detachment. If found, choroidal neovascularization may represent a remedial cause for visual loss in a condition with an otherwise poor prognosis.

Retinal angiomatous mass. A complication of retinal detachment surgery.

Retinal angiomatous masses were observed as a very late complication in four patients following scleral buckling procedures with drainage of subretinal fluid. The lesions manifested 5-19 years after the original surgical procedure, and the presenting complaint was decreased vision secondary to vitreous clouding. In three patients a vascularized retinal mass greater than 3 disc diameters in size was observed at the site of choroidal perforation for drainage of subretinal fluid. A fourth patient demonstrated a similar lesion in the location of a suture used to anchor the encircling element, possibly the point of accidental choroidal perforation. Three of the four patients were treated by photocoagulation or cryocoagulation with cicatrization of the angiomatous mass and obliteration of the vascular net. This resulted in clearing of vitreous opacities and subretinal exudates, with restoration of vision to a level prior to the onset of symptoms.

Argon laser photocoagulation of subretinal neovascular membrane associated with osteoma of the choroid.

A 19-year-old white woman with osteoma of the choroid developed a subretinal neovascular membrane with hemorrhage. This was treated with Argon laser photocoagulation and she has shown no evidence of recurrence 13 months after treatment.

Nanophthalmic sclera. Ultrastructural, histochemical, and biochemical observations.

Scleral tissue from two cases of nanophthalmos was examined by amino acid analysis, light microscopy, histochemistry, and transmission electron microscopy. Perifibrillar aggregates, similar to proteoglycans, were prominent in the nanophthalmic sclera. The sclerae were thicker than normal and the bundles of collagen fibrils were less ordered. The clinical features of vortex vein compression seem causally related to the disordered and thickened sclera, which, in turn, may be caused by dysfunctional proteoglycans, or interaction with the scleral collagen, or both.

Nanophthalmos: a perspective on identification and therapy.

Nanophthalmos is a rare and blinding disease. Diagnostic features include a small eye, small cornea, shallow anterior chamber, narrow angle, high lens/eye volume ratio, and uveal effusion. Intraocular surgery has a high rate of disastrous complications and blindness. The 32 eyes (16 patients) presented are in three categories based on angle closure and intraocular pressure levels. Treatment methods included medication, laser iridotomy and gonioplasty, peripheral iridectomy, filtration surgery, and cataract extraction. Glaucoma medication was effective, although miotics sometimes increased pupillary block. Laser iridotomy was successful in 83% of six eyes; laser gonioplasty in 91.6% of 12 eyes. Peripheral iridectomy succeeded in two of seven eyes, and filtering operations provided tension control in two of five eyes. Thirteen of 15 eyes undergoing filtration surgery suffered severe postoperative visual loss. Cataract extraction improved vision in only three of six eyes. The authors' experience confirms that surgery in nanophthalmic eyes has an extremely high complication rate with disastrous results. Medication and laser therapy are the procedures of choice for angle-closure glaucoma in nanophthalmos.