释义 |
DictionarySeeangiomatosiscerebroretinal angiomatosis
von Hip·pel-Lin·dau syn·drome (făn hip'el lan'dow), [MIM*193300] a type of phacomatosis, consisting of retinal vascular malformations, which may be multiple and bilateral, associated with hemangioblastomas primarily of the cerebellum and walls of the fourth ventricle, occasionally involving the spinal cord; sometimes associated with renal cell carcinomas or cysts or hamartomas of kidney, adrenal, or other organs; autosomal dominant inheritance due to mutation in the von Hippel-Lindau gene (VHL) on 3p. Synonym(s): cerebroretinal angiomatosis, Hippel disease, Lindau disease, von Hippel disease von Hip·pel-Lin·dau syn·drome (făn hip'el lan'dow), [MIM*193300] a type of phacomatosis, consisting of retinal vascular malformations, which may be multiple and bilateral, associated with hemangioblastomas primarily of the cerebellum and walls of the fourth ventricle, occasionally involving the spinal cord; sometimes associated with renal cell carcinomas or cysts or hamartomas of kidney, adrenal, or other organs; autosomal dominant inheritance due to mutation in the von Hippel-Lindau gene (VHL) on 3p. Synonym(s): cerebroretinal angiomatosis, Hippel disease, Lindau disease, von Hippel diseasevon Hippel, Eugen, German ophthalmologist, 1867-1939. Hippel keratoplastyHippel trephinevon Hippel disease - retinal hemangiomatosis.von Hippel-Lindau syndrome - a type of phacomatosis, consisting of hemangiomas of the retina associated with hemangiomas or hemangioblastomas primarily of the cerebellum and walls of the fourth ventricle, occasionally involving the spinal cord. Synonym(s): cerebroretinal angiomatosis; Lindau disease |