Clinical observation of natural course of congenital retinoschisis

BackgroundCongenital retinoschsis is rare X-linked inherited disorder,mainly affects bilateral retina and is seldom in clinic.Some severe complications,such as retinal detachment and vitreous bleeding,remain difficult to treat.ObjectiveThe aim of this study was to observe the natural course of clinical development in patients with congenital retinoschsis.MethodsThis was a retrospective case-observation study.The clinical data and fundus characteristics of 32 eyes from 16 cases with congenital retinoschsis were analyzed.These patients were diagnosed in West China Hospital through fluorescence fundus fluorescein angiography(FFA)and optical coherence tomography(OCT).ResultsAll of the 16 cases of congenital retinoschesis were bilateral with the foveoschisis.Peripheral retinoschisis was found in 12 eyes of 6 cases.The patients aged from 6 to 29 years old with the average age 14.2 years.During the follow-up period,3 eyes developed macular hole,and 3 eyes progressed retinal detachment,and 1 eye developed vitreous hemorrhage.The macula was involved in 1 eye with peripheral retinoschisis.Twenty-four eyes presented the stable course.ConclusionThe state of illness in majority of patients with congenital retinoschisis can be stabilized for a long duration.But some complications and illness progress are found in partial patients.Regular follow-up should be performed for the patients with congenital retinoschsis.OCT is a useful tool for the monitoring the manifestation of fundus in congenital retinoschsis.