Retinitis pigmentosa, unilateral Coats's disease, and thalassemia minor--a case report.
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Adult↗
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Results for “Retinal Telangiectasis”
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[Association of cavernous hemangioma of the retina and Leber-Coats angiopathy (apropos of 2 cases)].
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Adult↗
Flat preparations of the retinal vessels in Coats' disease.
Flat preparations of the retina were prepared by trypsin digestion in ten cases of Coats' disease. All of the patients were young males with advanced disease leading to enucleation. All eyes showed marked abnormalities in the arterioles, venules, and capillaries. The findings most characteristic of Coats' disease were large aneurysms and thick PAS positive deposits in vessel walls. The aneurysms ranged from 50 to 350 microns and frequently formed large sausage-like or beaded out-pouchings and were sometimes situated on shunt vessels. Other findings frequently seen, but not specific for Coats' disease, were capillary dilatation, small aneurysms, hypocellular vessel walls, and fibrous strands.
Aneurysm↗
Peripapillary fluorescein leakage in 11778 Leber's optic neuropathy.
A 17-year-old young man presented with a 2-month sequential visual loss in both eyes. Vision was 20/200 in the right eye and 20/400 in the left eye. Both discs were hyperemic with circumpapillary telangiectasia and nerve fiber swelling. Fluorescein angiography revealed a fuzzy hyperfluorescence due to leakage from dilated capillaries on both discs in the late transit. Molecular mitochondrial DNA study confirmed a homoplasmic 11778 Leber's optic neuropathy.
Adolescent↗
Origin of ghost cell in Coats' disease.
A morphological study of Coats' disease was performed with light and electron microscopy. The choroid was intact throughout. The pigment epithelial cells showed proliferative changes, being thickened in two or three rows. The transitional form of ghost cell was observed in contact with the pigment epithelial layer, then migrated toward the subretinal space, and finally invaded the detached retina. It was concluded that the origin of ghost cell in Coats' disease was the pigment epithelium.
Child, Preschool↗
Parafoveal telangiectasia in association with CREST syndrome.
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CREST Syndrome↗
The use of perfluorocarbon liquid in the surgical repair of a bullous retinal detachment secondary to Coats' disease.
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Aneurysm↗
Myelinated nerve fibers and retinal vascular abnormalities.
BACKGROUND/PURPOSE: Abnormal retinal vessels may develop in a region of myelinated nerve fibers, and these vessels may cause vitreous hemorrhages. METHODS: The clinical histories of seven patients with retinovascular abnormalities in a patch of myelinated nerve fibers are presented. None of the reported patients had other evidence of systemic disease. The cases were traced by a multicentric retrospective study. RESULTS: Retinal vascular abnormalities ranged from mild telangiectasis to frank neovascularization, with or without obstruction of the capillary network and signs of branch artery and vein occlusion. Age at diagnosis ranged from 15 to 68 years. Vitreous hemorrhages occurred in the four youngest patients and occurred at 15, 27, 27, and 43 years of age. Laser photocoagulation was applied in three patients and vitrectomy was performed in one. CONCLUSION: The authors' findings suggest that the abnormal structure of the myelinated nerve fibers and the thickened nerve fiber layer of the affected portions of retina may play a role in the onset of retinal vascular abnormalities and eventually cause telangiectasis, branch artery and vein occlusion, neovascularization, and vitreous hemorrhages. This suggestion is based on the absence of other causes of neovascularization or vitreous hemorrhage in all seven patients, and on the relatively young age of four of the patients with this association.
Adolescent↗
Increased aqueous lactate dehydrogenase in Coats' disease.
A 3-year-old boy had glaucoma and a total left retinal detachment that displayed peripheral intraretinal telangiectasis. The most probable clinical diagnosis was Coats' disease. A suspicion of retinoblastoma also existed, and an aqueous aspirate was assayed for lactate dehydrogenase (LDH) and found to contain three times the level present in a matching sample of serum. We enucleated the eye and verified the diagnosis of Coats' disease pathologically. The results of aqueous LDH determinations in eyes with glaucoma, phthisis, or large numbers of histiocytes, erythrocytes, and polymorphonuclear leukocytes should be interpreted with caution.
Aqueous Humor↗
Atypical parafoveal telangiectasis with subsequent anterior and posterior segment neovascularization.
Bilateral parafoveal telangiectasis typically includes the presence of fluorescein staining of outer retinal capillaries, no macular ischemia, right-angled venules, and migration of pigment along these venules. In the bilateral form, atypical parafoveal telangiectasis with macular ischemia but without right-angled venules or migration of pigment along these venules has been reported in only five patients previously. An atypical case is presented of bilateral parafoveal telangiectasis complicated, not only by areas of macular nonperfusion, but also by peripheral ischemia with subsequent anterior and posterior segment neovascularization in the absence of any systemic abnormalities. As in previously reported cases, this patient had no evidence of right-angled venules or pigment migration. Pan-retinal photocoagulation resolved the rubeosis and caused regression of the retinal neovascularization.
Fluorescein Angiography↗
Visual field changes in cone-rod degenerations.
The visual field test results from 20 patients with con-rod degeneration (CRD) from all inheritance patterns were reviewed. Typical fundus findings of CRD included optic disc pseudoedema, temporal disc atrophy, parapapillary and disc telangiectasis, and few to no retinal pigmentary deposits. Visual field changes were seen to be distinctive and, like electrophysiologic tests, were helpful in pointing to a retinal degenerative process rather than an optic neuropathy.
Adolescent↗
[Ocular manifestation of progressive essential telangiectases].
Generalized essential telangiectasia is a rare skin disorder characterized by the development of dilated venules beginning at the lower extremities and progressively spreading out to the rest of the body. It is not related to any known affection and is therefore considered to be essential. The condition tends to affect mostly women in their midthirties. We report the case of a 49-year-old healthy man worried by the progressive extension of dilated vessels on the conjunctivae of both eyes and the progressive worsening at the condition over the last five years. On clinical examination he presented also telangiectasies on both arms and legs which had also grown worse over the last five years. All further investigations and complementary examinations showed no disease. A cutaneous biopsy showed dilated venules in the superior dermis supporting the diagnosis of telangiectases. Several differential diagnoses are discussed. With confirmation of the clinical diagnosis we started treatment with oral tetracycline. Because of the poor response we stopped treatment after a period of three months.
Biopsy↗
Visceral larva migrans of the eye. Subretinal tube formation.
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Child, Preschool↗
Ocular fundus in acute Leber optic neuropathy.
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Acute Disease↗
Autosomal recessive retinitis pigmentosa and Coats disease: a presumed familial incidence.
Three of four siblings of normal parents had a combination of Coats disease and retinitis pigmentosa bilaterally. Our report of their respective cases is, to our knowledge, the first report of a familial incidence of the combination of the two diseases in an individual and supports the implication that Coats disease may be a genetic abnormality. The pathological process was essentially the same in all three patients, although it occurred differently in one of the three siblings and gave rise to an altered functional and clinical picture. The similarities may indicate that the convenient categories of "tapeto-retinal degenerations" are clinical variations of the same genetic abnormality.
Adolescent↗
Coats' disease and persistent hyperplastic primary vitreous. Role of MR imaging and CT.
Coats' disease is an idiopathic disorder in which telangiectatic and aneurysmal retinal vessels leak a lipoproteinaceous exudate, with consequent bullous retinal detachment. It is a diagnostic challenge, and CT and MR imaging provide valuable information to differentiate it from other pathologies, particularly from retinoblastoma. Typical, advanced Coats' disease shows on CT a denser substance posterior to the vitreous, which on MR is hyperintense on all pulse sequences. Contrast administration on both CT and MR might give slight linear enhancement at the boundary between vitreous and exudation. Persistent hyperplastic primary vitreous (PHPV) is a unilateral disorder in a microphthalmic eye, seen in full-term infants. PHPV rarely is bilateral in patients with Norrie's disease, Warburg syndrome, or patients with retinal dysplasia. Persistent fetal vasculature leads to fibrosis, resulting in elongation of the ciliary processes, retinal detachment, and spontaneous cataracts. The CT appearance in the disorder is quite variable; however, MR imaging may be superior in demonstrating the enhancing retrolental mass and the elongated ciliary processes.
Aneurysm↗