Retinal Vascular Changes of Incontinentia Pigmenti

Ocular manifestations of incontinentia pigmenti

“…Treatment of the avascular, ischaemic areas has been the most common, although the success rate varies (Best & Rentsch 1974;Nishimura et al 1980;Tornabe & Schochet 1981;Catalano et al 1990;Rahi & Hungerford 1990;Wald et al 1993). A few authors have treated the abnormal vascular tissue directly (Watzke et al 1976;Nix & Apple 1981). This has also been recommended by Catalano et al (1990), who base their view on the assumption that the neovascular response is induced by the abnormal retinal tissue, rather than the avascular retina.…”

“…Several reports on selected groups of cases with ocular manifestations in incontinentia pigmenti have been published (Watzke et al 1976;Raab 1983;Eisenhaure et al 1985;Spallone 1987;Goldberg & Custis 1993) and also a few metaanalyses of the literature (Scott et al 1955;Carney 1976). The aims of the present study were to make an inventory of the disease in Sweden, to acquire better knowledge of it from the genetic, dental, neurological and ophthalmological points of view, to give information to the patients and their families, to promote creation of an association for patients with incontinentia pigmenti, and to suggest a programme for follow-up of these patients.…”

So-Called ‘Central Retinal Vein Occlusion’

“…2). These latter angiographic findings are not unique to patients with hemorrhagic retinopathy (i.e., central retinal vein occlu sion with retinal ischemia); exactly identical changes are also seen in many other conditions including diabetic retinopathy, pulseless disease [14], postradiation retinopathy [5], Eales' disease [7,16], sickle cell retino- pathy [2], incontinentia pigmenti [15] and retrolental fibroplasia [13]; in the last four conditions the changes usually start at the periphery of the retina. The common factor in all these conditions, and also hemorrhagic retinopathy, is ocular ischemia.…”

Retinal Telangiectasis and Proliferation of the Retinal Vessels in a Family

“…Vascular changes in the retinal periphery similar to those found in the retinopathy of premature babies have been observed in wom en with Bloch-Sulzberger syndrome [64]. In this condition, small haemorrhages and micro aneurysms can also appear near the macula.…”

“…There were also no signs of Takayasu-Ohnishi arteritis. 64 Schmidt/Soriano Vessel Anomalies in a Family I □ Fig. 7.…”