Turkish Abstract Article PDF Similar Articles

¹İzmir Atatürk Eğitim Hastanesi Göz Kliniği
²İzmir Atatürk Eğitim Hastanesi 2. Ortopedi Kliniği
³İzmir Atatürk Eğitim Hastanesi 2. KBB. Kliniği Stickler syndrome, also called as "Hereditary progressive arthroophthalmopathy " is a generalized connective tissue dysplasia, with an autosomal dominant hereditary trait, skeletal and joint anomalies, typical facial appearence, hearing loss and retinal detachment. 6 cases of familial Stickler syndrome were evaluated ophthalmologically, orthopedically, audiologically and cardiologically.
Severe myopia, cataract, glaucoma and empty vitreous view because of syneresis, membranes in vitreous, retinal pigmentary lesions, retinal detachments were observed. Orthopedical examination revealed vertebral pathologies, archnodactili, early arthropathy, a broad valgus femoral neck, protrusio acetabuli and pes planus. All cases had moderate sensory-neural hearing loss bilaterally, at pure tone audiometry.
As a conclusion; blindness, as a result of retinal detachments at 3 eyes of 6 cases at young ages, revealed the requirement of prophylactic treatment for retinal detachments in these cases. Keywords : Stickler syndrome, Hereditary progressive arthro-ophthalmopathy, sensory-neural hearing loss, premature vitreous syneresis, retinal detachments.