Clinical Characteristics And Treatments In Pediatric X-linked Retinoschisis

Objectives: To analyze the clinical characteristics,the risk factors of longterm complications and treatments in a pediatric cohort of X-linked retinoschisis and define the classification system of the disease.Subjects and Methods: 97 children(194 eyes)referred to our department with a diagnosis of XLRS from December 2007 to March 2017 are included in this study.Clinical data of ocular manifestations,auxiliary examinations,ocular treatments and outcomes,follow-ups,and family history were collected in order to summarize the clinical features of a pediatric cohort,and to define the clinical staging and classification of Xlinked retinoschisis.Risk factors for the occurrence of complications and the clinical effect of preventive laser photocoagulation were analyzed.Surgical interventions were performed on children with severe complications.The diagnosis and treatments in this study were performed by the same experienced ophthalmologist.Results: 97 children(194 eyes)were all male(100%)in this study.The average age of these children was 54.55 ± 37.21 months,range from 3 to 195 months.A total of 80 children(82.47%)were younger than 7 years old at the first visit.35 children(36.08%)were younger than 3 years old.Misdiagnosis rate was 26.80%(26 cases).8 eyes(4.12%)developed secondary cataract at the first visit.143 eyes(73.71%)presented typical macular schisis with a spoke-wheel pattern.156 eyes(80.41%)presented peripheral schisist.Exudative lesions were observed in 19 eyes(9.79%).49 eyes of 27 patients(27.84%)underwent fundus fluorescein angiography(FFA).FFA findings included vascular abnormalities(14 eyes,28.57%),peripheral vascular tortuous expansion with fluorescence leakage(6 eyes,12.24%)and macular fluorescence accumulation(14 eyes,28.57%).51 eyes(26.29%)developed severe complications,including retinal detachment(12.37%),severe vitreous hemorrhage(8.25%),lamellar macular hole(2.58%),secondary cataract(8.76%)and full-thickness macular hole(0.52%).There was no statistically significant difference in the incidence of complications between the prophylactic laser group and the observation group.32 eyes of 23 patients(16.49%)underwent surgical interventions.23 patients(96.88%)achieved anatomical reduction after surgeries.Postoperative complications were observed in 13 eyes(40.63%),of which 9(28.13%)received secondary surgery.Conclusions: X-linked retinoschisis is a congenital macular degeneration and vitreoretinopathy.There is a wide range of long-term visionthreatening complications.There is a high variety in the phenotype of XLRS with asymmetry manifestation and early-onset of severe complications.The presence of exudation and concurrent vascular leakage and vascular anomalies on FFA are common in XLRS.Preventive LIO may be helpless in preventing complications and surgical intervention is the only effective treatment.“Early discovery,early diagnosis,close clinical follow-up and timely treatment” is crucial in improving the prognosis of XLRS.