[Solitary albinotic spot of the retinal pigment epithelium: a functional and imaging study]. / Solitärer, albinotischer "Fleck" des retinalen Pigmentepithels: klinische und funktionelle Befunde.

PURPOSE: The presence or absence of functional changes associated with solitary, congenital, hypopigmented lesions of the retinal pigment epithelium (RPE) have been a matter of controversy. This case report describes retinal and functional findings in a young patient with such a lesion. METHODS: A 10-year-old Hispanic female with a solitary congential hypopigmented spot of the RPE was examined using fundus photography, fluorescein angiography, autofluorescence imaging (AF) and optical coherence tomography (OCT). Functional analyses were performed using the Humphrey 24 - 2 visual field, Goldmann perimetry and the multifocal ERG (mfERG). RESULTS: A small visual field defect was demonstrated on both Goldmann perimetry (I/ 2e test object) and on Humphrey 24 - 2 visual field testing (significant at the 0.5 % level for pattern deviation). The multifocal ERG response amplitudes were decreased in the corresponding area and increased in implicit time. Autofluorescence imaging showed an absence of fluorescence corresponding to the area of the lesion. OCT findings were indicative of a small amount of subretinal fluid or schisis-like changes overlying the RPE anomaly. CONCLUSION: The results indicate that solitary, albinotic spots of the RPE can be associated with visual field defects and outer retinal deficits; these may be related to impaired RPE function and/or chronic exudative changes.

Giant cysts of the conjunctiva following scleral buckling.

Four patients developed giant conjunctival cysts following scleral buckling for retinal detachment. Histologically these cysts showed a core of fibrous tissue lined by stratified, non-keratinizing epithelium and with goblet cells. They probably arose from inadvertent implantation of epithelium during surgery. In three cases the cysts were surgically excised without recurrence. Alternate modes of therapy are discussed.

Retinal arteriolar occlusion in multiple sclerosis.

A 25-year-old white man presented with acute multiple sclerosis manifested by right blindness, difficulty urinating, and paresthesias and weakness of both legs. Retinal examination revealed a distal occlusion of the descending branch of the superior temporal arteriole leading to an area of retinal ischemia of the right eye. The optic disc was edematous, and there were focal areas of periphlebitis. All retinal signs resolved in three weeks, and the only abnormality that persisted was a pale right optic disc. The finding of small arteriolar disease is unusual and may represent another possible pathogenetic mechanism in multiple sclerosis.

Scleral ruptures during retinal detachment surgery.

Two cases of retinal detachment are presented in which significant scleral ruptures occurred at the time of scleral buckling surgery. Retinal reattachment was achieved and scleral ruptures successfully closed utilizing standard scleral buckling elements. Long-term follow-up of both cases failed to disclose any significant ocular complications occurring at the rupture site. Intraoperative management and techniques recommended for the avoidance of this complication are discussed.

A rare choristoma of the optic nerve and chiasm.

Two patients with a heretofore undescribed unilateral choristomatous malformation of the intracranial optic nerve and chiasm underwent craniotomy for partial resection of the optic nerve in the belief that the tumor was either a glioma or a meningioma. Visual acuity in the affected eye was reduced to 20/200 in one patient and to finger counting in the other. Chiasmal involvement in the first case was associated with a superotemporal field defect in the opposite eye. Ophthalmoscopy disclosed atrophic nerve heads in both affected eyes. Heteroplastic masses of smooth muscle and adipose tissue replaced most of the resected optic nerve in each case.

Silastic sponge implants in retinal detachment surgery.

Two hundred consecutive primary rhegmatogenous retinal detachments repaired with Silastic sponge implants were evaluated. A primary reattachment rate of 94% and an overall reattachment rate of 95% were achieved. Two of the successful repairs became detached after six months giving a redetachment rate of approximately 1%. Eight of the 12 failures were due to massive periretinal proliferation (MPP) (overall incidence of MPP = 4%). To date, only one Silastic sponge has had to be removed because of late infection. No cases of extrusion or erosion occurred. We recommend this procedure as safe and effective.

Tuberous sclerosis associated with a retinal angioma.

A 62-year-old woman known to have tuberous sclerosis had an ususual peripapillary retinal elevation in her left eye. The patient had no specific ocular symptoms and we considered the diagnosis of papilledema secondary to increased intracranial pressure. Retinal angiography established that she had a peripapillary exophytic retinal angioma associated with an epipapillary astrocytic hamartoma in this eye.