Vortex vein decompression for nanophthalmic uveal effusion.

Uveal effusion in nanophthalmic eyes probably is the result of choroidal congestion secondary to imparied vortex venous drainage through the thick sclera that is characteristic of nanophthalmos. Ten eyes with nonrhegmatogenous retinal detachment, occurring in nanophthalmic eyes, were treated by vortex vein decompression and, in some cases, drainage of choroidal and/or subretinal fluid with air injection into the vitreous cavity. Eight of the ten eyes showed reattachment of the retina after this procedure. There results support the hypothesis that choroidal congestion is the basic mechanism of nanophthalmic uveal effusion.

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.

Nanophthalmos with uveal effusion. A new clinical entity.

Five patients exhibited the association of nanophthalmos and uveal effusion, apparently on a familial basis. Glaucoma, occurring in the fourth to sixth decades of life, required surgical intervention that was followed by the development of secondary retinal and choroidal detachment. Recognition of this syndrome is important because: (1) surgical procedures for glaucoma should be avoided, if possible, in order to prevent the development of uveal effusion; (2) retinal detachment surgical procedures are ineffective in uveal effusion and should be avoided, and (3) choroidal elevation occurring in the uveal effusion phase may be erroneously diagnosed as an intraocular tumor and unnecessary enucleation may follow.

Neovascular fundus abnormalities in peripheral uveitis.

Neovascular fundus abnormalities are an unusual complication of peripheral uveitis. Peripheral retinal neovascularization was seen in 11 patients, six of whom had retinal angioma-like lesions. Most of the patients had loss of vision caused by vitreous hemorrhage. In four additional patients with peripheral uveitis, neovascularization developed either at the disc or elsewhere in the posterior pole.

Hemorrhagic detachment of the peripheral retinal pigment epithelium.

Six patients had hemorrhagic detachment of the peripheral retinal pigment epithelium. Abnormal neovascular nets in older individuals, under the retinal pigment epithelium and originating from the choriocapillaris, may well be the cause of such hemorrhagic detachments. This lesion must be differentiated from choroidal tumors, choroidal detachment, and uveal effusion.

Perforating diathermy coagulation for retinal angiomas.

Perforating diathermy coagulation can be used efficiently to destroy large angiomas of the retina. Five patients who had developed large angiomas (greater than 5 disc diameters) in association with various retinal diseases were treated with this technique. The follow-up period ranged from 1 1/2 to 7 years, except for one case in which the patient died five days after the surgical procedure. Several techniques of perforating diathermy are described, and the indications, surgical precautions, and complications of each method are discussed.

Cellular blue nevus of the sclera.

An isolated cellular blue nevus of the sclera was discovered and removed during a retinal detachment operation. Such benign tumors may be misdiagnosed clinically as an extension of a melanoma of the choroid or histologically as melanoma.

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.

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.

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.

Proton irradiation of choroidal melanomas. Preliminary results.

Choroidal malignant melanomas in nine patients were treated with proton beam irradiation at the Harvard Cyclotron Laboratory, Cambridge, Mass. Each patient received five proton beam treatments in eight to ten days, totalling 4,730 to 8,570 rads at the tumor. No complications occurred during the treatment or follow-up period, which, at the time of this writing, ranges from one to 24 months, with an average of 12 months. No further growth of the tumor has been observed in any patient. Different signs of tumor regression have been noted. Resolution of the serous retinal detachments that accompanied some tumors is the earliest finding. Pigment changes over the surface of the tumor and adjacent pigment epithelium is a usual initial tumor response. Fluorescein angiography initially showed decreased leakage of dye; later, destruction of the tumor's vasculature and elimination of fluorescein leakage became evident. Only large choroidal vessels remained patient. Ultrasonography revealed decreased height of the tumors postirradiation, and the radioactive phosphorus (32P) uptake test, repeated in one patient, turned negative on postirradiation measurements.

Photocoagulation of active toxoplasmic retinochoroiditis.

Active, recurrent, protracted toxoplasmic retinochoroiditis that had been unresponsive to intensive medical therapy with pyrimethamine, sulfamethoxypyridazine, intramuscular folicic acid, or clindamycin and corticosteroids, was treated with photocoagulation in five eyes. Four eyes healed rapidly within a few weeks. In one patient's eye, lens and vitreous opacities prevented adequate treatment with the red III intensity level of the xenon are photocoagulator. Subsequent surgical diathermy and cryocoagulation resulted in prompt healing of the lesion. Noninvasive photocoagulation of active toxoplasmosis retinochoroiditis is recommended in protracted cases if the media are clear, the macula is threatened, or there are severe complications from systemic medications.

Angle-closure glaucoma in nanophthalmos.

Two patients had nanophthalmos with uveal effusion and angle-closure glaucoma. They were treated with a method based on the use of the laser, not only to perform iridotomy but to shrink the iris stroma, which appears to open the anterior chamber angle even without iridotomy. Additionally, we introduced three factors that may be diagnostic of nanophthalmos.

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.

Optic pits and posterior retinal detachment.

Six cases of congenital pit of the optic nervehead associated with posterior serous retinal detachment are presented. All were treated by photocoagulation along the disc margin in the area of retinal detachment. In five cases reattachment of the retina occurred, after the clinical development of a film chorioretinal adhesion at the disc margin, and appeared to be secondary to the treatment. The sixth case (Case 4), although treated, appeared to represent a spontaneous reattachment. This disorder, which frequently results in permanent decrease of central vision, affected the better, or only, eye in two of the six cases herein reported. Fluid, probably from the vitreous cavity, appears to gain access to the subretinal space via the pit. Reattachment in treated cases occurred only if an effective chorioretinal adhesion was created over the entire area of the fistulous detachment at the disc margin. Field defects after treatment appear to be secondary to either the optic pit itself or the longstanding retinal detachment, oftern accompanied by pigmentary degeneration and cystic macular degeneration, rather than juxtapapillary photocoagulation treatment.