Congenital retinal splitting, also known as x-linked retinoschisis (XLRS ), is an inherited fundus disease caused by mutations in the XLRSl gene and is the main cause of macular degeneration in male adolescents with an incidence of approximately 1:30,000-1:5,000 [1, 2 ]. The clinical manifestation is a radially whorled arrangement of retinal folds and microcystic changes in the macula, with peripheral retinal cleavage in about 50% of patients [3]. The disease is often complicated by retinal detachment of retinal origin and vitreous hemorrhage in the later stages of the disease, leading to a dramatic loss of vision or even blindness. There is no definite and effective treatment for this disease, and the corresponding treatments are mainly chosen for its complications, such as vitreous surgery, scleral buckling, laser photocoagulation, and so on. Among them, vitreous surgery has been more frequently reported [4-9] at home and abroad to treat its complications, but most studies have small sample sizes and few reports on the long-term outcomes after surgery. We conducted a long-term follow-up of 57 patients with congenital retinal splitting who underwent vitreous surgery in our hospital between February 2000 and August 2010, and achieved good clinical results. Yu Honghua, Ophthalmology Department, Guangzhou General Hospital, Guangzhou Military Region XLRS is a rare hereditary degenerative blinding fundus disease, the cause of which has been confirmed to be a mutation in the RS1 gene located in the Xp22 fragment. It can be diagnosed in infancy with vitreous hemorrhage as the first symptom [11], and most patients present with poor visual acuity in childhood, but it is often detected at the time of consultation after school when they feel they have difficulty learning. It has been shown that patients with XLRS may experience a significant period of vision loss in the early stages, which may be followed by a long period of stabilization [12, 13]. However, about 5-20% [14, 15] and 33% [2, 16] of patients develop serious complications such as secondary retinal detachment and vitreous hemorrhage, respectively, leading to a dramatic decrease in visual function or even blindness. Therefore, it is clinically important to explore effective surgical methods to save the visual function of XLRS patients with concomitant retinal detachment and vitreous hemorrhage. In this study, we treated XLRS patients with complications of vitreous hemorrhage and/or retinal detachment with vitreous surgery to observe the long-term outcome. All patients underwent vitreous surgery, including vitrectomy, inner boundary membrane peeling, laser photocoagulation, and C3F8/silicone oil filling. Studies [17] have shown that vitrectomy of the vitreous cortex and peeling of the inner boundary membrane play a crucial role in the treatment of macular clefts caused by high myopia, and Ikeda.F et al [7] also reported that vitreous traction plays an important role in the progression of macular clefts in congenital retinal clefts. We observed that intraoperative care should be taken to remove the vitreous as cleanly as possible and peel the inner macular boundary membrane intact, which can effectively release the vitreous traction on the retina, especially the macula, and promote the repositioning of the cleft cavity. If the retinal flap has been rolled over, it is often necessary to release the vitreous traction and use heavy water to flatten the overturned retina, and the edge of the fissure should be laser photocoagulated. In the selection of intraocular filler, C3F8 can be used for single condition; silicone oil can be used for more complex and severe condition with severe PVR or recurrent retinal detachment. In addition, patients with high-risk PVR can be combined with extra-scleral ring ligation to fully release retinal traction and prevent retinal re-detachment. The 7 eyes that failed to improve their visual acuity at the final follow-up were those with severe preoperative retinal detachment or vitreous hemorrhage, 2 of which had undergone vitreous surgery because of complications after the first vitrectomy, 2 of which had preoperative vitreous hemorrhage and retinal detachment, and 3 of which had extensive preoperative retinal detachment with prolonged macular involvement. The lack of visual acuity improvement in these 7 eyes may be related to apoptosis of photoreceptor cells due to repeated surgical injury and prolonged retinal detachment [18]. In summary, vitreous surgery for XLRS, including vitrectomy, inner boundary membrane peeling, laser photocoagulation, and C3F8/silicone oil filling, can effectively remove the cloudy refractive interstitium, release vitreous traction, and reset the detached retina, as well as reset the retinal splitting or prevent the splitting cavity from continuing to expand and endanger the macula, thereby improving visual function and restoring retinal tissue anatomy in patients with XLRS. It has a good long-term efficacy. Therefore, we believe that patients with XLRS should be closely followed up, and regular fundus photography and OCT should be performed, and vitreous surgery should be performed in a timely manner once complications such as vitreous hemorrhage and retinal detachment occur. For those who have not yet developed complications such as vitreous hemorrhage and retinal detachment, but the extent of cleavage is expanding during the follow-up process, whether vitreous surgery should be performed or not, will be further studied.