[Vitreoretinochoroidal heredo-dystrophy, microcornea, glaucoma and cataract].
Vitreoretinochoroidopathy with microcornea, glaucoma and cataract must be considered to be a distinctively autosomal dominant affection. The authors present evidence in the form of 18 carriers of the same anomaly detected with a pedigree extending up to six generations. Microcornea and vitreoretinochoroidal dystrophy are the prime characteristics; hypertonia and cataract are induced complications. The syndrome may be attributed to a hereditary dysgenesis affecting the anterior part of the globe with trabecular and preequatorial corneal alterations. The dystrophy has a slow development as shown by the clinical and electroretinographic course. Present treatment only consists of controlling ocular hypertonia and cataract.
François
P
P
Clinique Ophtalmologique de Lille, CHRU.
Puech
B
B
Hache
J C
JC
Laffineur
Q
Q
D000293:Adolescent; D000328:Adult; D000368:Aged; D002386:Cataract / Q000235:genetics*; D002648:Child; D002675:Child, Preschool; D015862:Choroid Diseases / Q000235:genetics; D003317:Corneal Dystrophies, Hereditary / Q000235:genetics*; D015785:Eye Diseases, Hereditary / Q000235:genetics*; D005260:Female; D005500:Follow-Up Studies; D005901:Glaucoma / Q000235:genetics*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D012164:Retinal Diseases / Q000235:genetics; D013577:Syndrome; D014822:Vitreous Body / Q000002:abnormalities
Hérédo-dystrophie choriorétinovitréenne, microcornée, glaucome et cataracte.