Congenital diffuse necrotizing herpetic retinitis.

Neonatal Herpes simplex infections are usually contracted from the birth canal, and the systemic lesions develop several days to weeks after delivery. We present the clinicopathologic findings in a newborn with a prenatal diagnosis of hydrocephalus who died at 1 day of age. Severe liquefaction necrosis and foci of calcification were present in the brain, adrenal glands, and retina. Cowdry type A intranuclear inclusions were present in the adrenal glands and retina. There was no clinical evidence of genital herpes in either parent. This is the first documented case of in utero transmission of Herpes simplex infection, confirmed by the polymerase chain reaction, and causing fulminant necrotizing retinitis and encephalitis.

Congenital herpes simplex retinitis.

An 1,810-g girl born at 37 weeks of gestation had true congenital herpes simplex virus type 2 infection. The neonate was born with hypo-pigmented skin lesions, brain lesions, and old heavily pigmented retinal scars in the posterior pole. There was no active ocular disease. Viral cultures, immunoperoxidase stains, and antibody determination by enzyme-linked immunosorbent assay were all positive for herpes simplex virus. Treatment with acyclovir healed the lesions within ten days. The clinical and laboratory findings indicated that the herpetic infection probably occurred in the second trimester.

Congenital retinitis in the rat following maternal exposure to lymphocytic choriomeningitis virus.

A combined clinical and histopathological study of the eyes of the offspring of females inoculated with LCMV shows that about two-thirds of the pups develop some degree of retinal inflammation. This may range from a mild, subclinical reaction to an overt retinitis characterized clinically by demonstrable inflammatory and degenerative changes. Histopathologically, the latter condition presents the picture of an inflammatory reaction with extensive loss of photoreceptors and retinal neurons in general, macrophagic invasion, mild microcystoid degeneration and total or subtotal retinal detachment. This vertically-transmitted disease does not show the relentless progression and hemorrhagic tendency characteristic of the retinitis which occurs after direct viral inoculation. However, in the most severe cases the final outcome is the same, namely severe retinal impairment subsequent to widespread loss of photoreceptors. In the presence of consistently negative virological data, the hypothesis is proposed that this retinitis could be the result of a vertically-transmitted autoimmune disease.

Choroidoretinal vascular anastomosis.

Vascular anastomoses between the retina and choroid may occur in different congenital and acquired conditions. It has been postulated that the direction of blood flow in these anastomoses follows a pressure gradient characterized as follows: choroidal artery greater than retinal artery greater than retinal vein greater than choroidal vein. Of the four possible directions of blood flow between the two circulations that this theory allows, only three have previously been reported. The fourth, described in this report, is a choroidoretinal vascular anastomosis with blood flow from a choroidal vessel into a retinal vein.

Congenital herpes simplex virus, type 2, bilateral endophthalmitis.

A set of dizygotic twins, both born prematurely, developed herpes simplex encephalitis shortly after birth. The second twin had unilateral keratoconjunctivitis and bilateral endophthalmitis and subsequently died from disseminated herpes infection. Herpes simplex, type 2, was isolated from conjunctiva of both eyes, cerebrospinal fluid, nasopharynx and trachea. Histologic examination of the eyes revealed bilateral endophthalmitis with a necrotizing retinitis. Intranuclear inclusion bodies were demonstrated by light microscopy in the retina, choroid and iris, and virus particles were demonstrated by electron microscopy in the retina. The other twin recovered from a much milder disseminated herpes simplex infection without apparent ocular involvement. During pregnancy, the mother had vaginitis which was suspected, but not documented, as being herpetic in origin. The father was diagnosed as having a penile infection caused by herpes simplex. Repeated attempts to locate the family of the surviving baby girl in order to obtain follow-up information have failed.

[Electrophysiological and fluorescence-angiographical examinations in a case of fundus albipunctatus cum hemeralopia congenita (author's transl)]. / Elektrophysiologische und fluoreszenzangiographische Untersuchungen bei einem Fall von Fundus albipunctatus cum hemeralopia congenita.

Electrophysiological and fluorescenceangiographical examinations were carried out in a case of fundus albipunctatus cum hemeralopia congenita. In the ERG a diminished scotopic b-wave was conspicuous, which reached the normal size after longer dark adaptation. It was possible to distinguish between albipunctatus-dots and druses by fluorescenceangiography. While the latter became clearly visible in the phase of choroidic-fluorescence the ophthalmoscopically perceptible albipunctatus-dots lost their visibility.

Audiometric and ophthalmological findings in rubella deafness.

In an attempt to study the relevance of ophthalmological changes to the diagnosis of cochlear hearing impairment due to fetal rubella infection, a survey of the literature combined with a clinical investigation was carried out. Based on the investigation of 57 patients, 31% had congenital hearing impairment due to fetal rubella infection. Of these patients, 61% had typical rubella retinal changes. In the remaining 69%, ophthalmoscopy revealed no abnormality, except in 1 patient. As the cause of the hearing impairment in this patient was unknown, it was concluded that the mother had suffered from subclinical rubella infection in the first trimester of her pregnancy. It is concluded that rubella retinitis is found with such a high incidence in rubella children that it can be used as a tool in the diagnosis of cochlear hearing impairment due to fetal rubella infection. Patients with congenital hearing impairment ought to undergo a routine ophthalmoscopy which will detect eventual pigmentary changes.

Genetic approach to the nosology of retinal disorders.

During the last 20 years we have carried out, with the collaborators of our Institute, a series of genetic and epidemiologic investigations on tapetoretinal degenerations and allied diseases in Switzerland. Basing conclusions both on the results of this inquiry and on the findings in the literature, we give in this study a general survey of the chorioretinal abiotrophies and describe in particular their clinical and genetic aspects as well as their association with other congenital and hereditary anomalies. A nosologic classification is proposed which takes into account the clinical and genetic features of the systemic heredodegenerative conditions of the retina.