Rhegmatogenous retinal detachment in Kniest Syndrome : Clinical model of abnormal vitreoretinal traction in children .

Abstract

Introducing the management of non traumatic rhegmatogenous retinal detachment in pediatric patient in the context of a genetic syndrome associated with failure of type 2 collagen synthesis which manifests itself in ocular , hearing , facial , bone and joint abnormalities. We describe the clinical presentation of the picture and surgical repaire technique consisted of lensectomy , vitrectomy , peeling of vitreoretinal proliferation membranes , giant retinal tear management and prolonged tamponade by silicone oil. Long term clinical follow-up was carried out. We highlight this case report to be a therapeutic challenge in a deaf and high myopic child , with motor disabilities and atypical vitreoretinal disorders have generated difficulties in surgical resolution , there is also little scientific reference in world literature and represent a clinical model of abnormal vitreoretinal interaction

Keywords

• Kniest syndrome
• retinal detachment
• children
• giant retinal tear
• myopia.