Abstract
Purpose:
The Wagner-Stickler syndrome is a hereditary progressive arthro-ophthalmopathy with an autosomal dominant pattern of inheritance. Both conditions involve varying degrees of degeneration of eye structures including myopia, presenile cataracts, strabismus, vitreo-retinal degeneration and rhegmatogenous retinal detachments. Only the Stickler syndrome has been associated with other variable non ocular symptoms such as deafness and facial, oral and skeletal abnormalities. <br /> OBJECTIVE: To study the outcome of the treatment of retinal detachment in Wagner-Stickler patients.<br />
Methods:
Retrospective analysis of 14 patients (19 eyes) with retinal detachments. Mean visual acuity was 0,90 logMar. Mean age was 7,29. Mean refractive errors was -12 sph. Mean follow up is 5,46 years. All the eyes were managed with 20G vitrectomy.
Results:
Five eyes devoloped proliferative vitreoretinopathy and was re-operated and the retina remained attached. Three eyes developed an ocular hypertension well managed with topical beta blocker therapy. <br /> One eyes had a central corneal opacity.<br /> Mean visual acuity after surgery was 0,50 logMar.<br />
Conclusions:
Wagner-Stickler Syndrome retinal detachment is often complex in nature. Vitrectomy is effective in the repair of retinal detachment and can improve visual outcomes.