Vitreoretinal Degeneration Research Articles

533 WAGNER-STICKLER SYNDRCME: A GENETIC STUDY

Based on our study of 32 affected individuals in 10 families, we suggest that the Wagner and the Stickler syndromes could be the same disorder. Both the Wagner and the Stickler syndromes are autosomal dominant disorders with a similar pattern of eye abnormalities, including myopia, presenile cataracts, strabismus, vitreo-retinal degeneration, retinal detachment and retino-schisis. Only the Stickler syndrome has been associated with non-ocular problems, such as cleft palate, micrognathia, mid facial hypoplasia, hearing loss and degenerative arthritis. All of the index cases in our study were ascertained because of their retinal symptoms as having the Wagner syndrome. Three families were included in a report on Wagner syndrome in Arch Ophthal. 89:176, 1973 by Hirose et al.

Pigmentation and Retinal Breaks

Of 260 patients with primary, nontraumatic, rhegmatogenous retinal separation analyzed for the presence of retinal or subretinal pigmentation associated with the retinal breaks, some 48.5% of all patients had significant pigmentary changes, especially occurring with lattice degeneration and nonlattice horseshoe tears. Critical analysis of the age of the patient, type of retinal break, vitreoretinal degeneration, and type and extent of pigmentation is essential before ascribing a protective effect of the pigment in the prevention of subsequent retinal separation.

Natural history of retinal breaks without detachment.

One hundred seventy-six eyes with retinal breaks without detachment or with subclinical detachment were followed up without prophylactic treatment for six months to 16 years. In 31 (18%) progression occurred. The likelihood of progression was greater in the following circumstances: (1) fresh symptomatic horseshoe tears, (2) breaks with subclinical detachment, (3) aphakic eyes. Progression in eyes containing breaks with opercula or round holes without opercula or in phakic eyes containing asymptomatic horseshoe tears was unusual (5% to 10%). In the patient first seen with a fresh clinical retinal detachment in one eye due to a horseshoe tear or hole with operculum and an asymptomatic break of either of these types without detachment in the fellow eye, the eye with the asymptomatic break usually represents the chronologically more-advanced stage of vitreoretinal degeneration.

Wagner's hereditary vitreoretinal degeneration and retinal detachment.

Forty-one patients with Wagner's vitreoretinal degeneration were studied. The polymorphous ocular signs of this disease included vitreous changes, myopia, cataract, retinal pigmentation, retinal breaks, patchy areas of thinned pigment epithelium or of chorioretinal atrophy, narrowing or sheathing of the retinal vessels, extensive white with pressure, lattice degeneration, marked meridional folds, optic atrophy and subnormal or normal electroretinograms. The main cause of blindness was retinal detachment of relatively poor surgical prognosis due to a variety of causes. Various types of retinal breaks were found in close association with vitreous and retinal disease. Early diagnosis of this disease is important. Members of the patient's family should be checked for retinal breaks. Prophylactic treatment for retinal detachment should be considered before cataract develops.

Hereditary Vitreoretinal Degeneration and Night Blindness

Hereditary Vitreoretinal Degeneration and Night Blindness

New and Controversial Aspects of Retinal Detachment.

This book was published as the result of an assembly at The Texas Medical Center in Houston, June 3 through 5, 1965. An international symposium on the new and controversial aspects of retinal detachment was conducted with nearly 50 authorities from various parts of the world contributing to the understanding of this disease. Although the monograph is not exhaustive, it none the less is broad in scope, containing chapters on morphology, pathology, etiology, classification, examination, treatment complications, and results. The first section of the book presents the latest concepts of the ultrastructure of the retina and the vitreous, details recent studies of autopsy eyes containing pathology that is known to be associated with retinal detachment, and investigates the genetic determination of vitreoretinal degeneration. The management of retinal detachment is considered at length. New diagnostic instrumentation and a classification of retinal detachment, based on prognosis, are described, and the judiciousness of

Retinal detachment due to ocular contusion.

The causal relationship between trauma and retinal detachment is frequently uncertain because two separate etiologic factors are involved: the traumatic insult and the preexisting state of vitreoretinal degeneration. The relative influence of each factor depends upon its severity. The purpose of this paper is to describe the clinical characteristics of retinal detachments caused by severe contusion in eyes selected so as to minimize the influence of vitreoretinal degeneration. These characteristics are compared to those of nontraumatic cases as previously reported by Schepens and Marden.1,2 Materials and Methods One hundred and sixty patients with retinal detachment due to ocular contusion were selected from the records of the Retina Associates and the Retina Service of the Massachusetts Eye and Ear Infirmary according to the following criteria: Unilateral retinal detachment preceded by ocular contusion. Objective signs of contusion in the affected eye. Absence of visible vitreoretinal degeneration of the types known to