Marfan综合征伴孔源性视网膜脱离

目的：分析Marfan综合征伴孔源性视网膜脱离眼发病特征与治疗原则。方法：回顾并随访了1998年至2001年间上海市第一人民医院进行视网膜脱离手术治疗的10例12眼。结果：平均随访5．2个月，总的视网膜解剖复位率83．3％(10／12)，其中只作巩膜外手术7眼，复位率100％，玻璃体手术5眼，复位率60％。视力提高7眼(58．3％)。结论：仔细采用多种方法检查可以提高术前裂孔检出率。经巩膜环扎加压手术可以有效处理简单视网膜脱离眼，而毋实施玻璃体手术联合晶体摘除术。治疗中需加倍警惕对侧眼病变。     

Retinal detachment in Marfan syndrome: clinical characteristics and surgical outcome

PURPOSE: To describe the clinical characteristics of retinal detachments in patients with Marfan syndrome and report the surgical outcome of vitreoretinal surgery. METHODS: Records relating to 53 eyes of 45 patients with Marfan syndrome who underwent surgery for rhegmatogenous retinal detachment were reviewed. Of the 53 eyes, 24 (45.3%) underwent scleral buckling as the first procedure and 29 (54.7%) underwent vitrectomy surgery with scleral buckle as the first procedure. Main outcome measures included clinical characteristics of retinal detachment, reattachment rates, and functional improvement in vision. RESULTS: Characteristic findings included total retinal detachment in 40 (75.5%) eyes, atrophic holes in 24 (45.3%) eyes, more than four retinal breaks in 21 eyes (39.6%), preequatorial and postequatorial breaks in 20 (37.7%) eyes, giant retinal tears in six (11.3%) eyes, and proliferative vitreoretinopathy (posterior, anterior, or both) in nine (17%) eyes. In 30 (56.6%) eyes, retinal breaks were located only in the temporal half of the retina. Of the 24 eyes with myopia, 13 (54.2%) had a myopic correction greater than 7 diopters. At the median follow-up of 10.7 months, complete retinal reattachment was obtained in 87.6% and 86.2% of patients undergoing scleral buckling (including additional procedures such as vitrectomy) and vitrectomy surgery, respectively. In eyes with reattached retinas, a final visual acuity of 20/200 or better was obtained in 81% of the patients after scleral buckling and in 56% of the patients after vitrectomy surgery (P = 0.07). CONCLUSIONS: Retinal detachment in Marfan syndrome is complete in 75% of the eyes. More than half (56%) the eyes had a retinal break only in the temporal half of the retina, and 83% had at least a break in the temporal half of the retina. Currently available vitreoretinal surgical techniques result in successful reattachment of the retina in approximately 86% of the eyes.     

Retinal detachment in Axenfeld-Rieger syndrome.

Three cases of the association between the Axenfeld-Rieger syndrome and retinal detachment in one family are described. Serious damage to the posterior segment of the eye in the Axenfeld-Rieger syndrome has not previously been reported.     

Retinal detachment in median cleft-face syndrome.

A 7-year-old boy had an unusual association of median cleft-face syndrome, a characteristic physiognomy, unilateral retinal dysplasia, and bilateral retinal detachment. Despite surgical treatment to both eyes he became blind. The hereditary pattern operating in this complex disorder was found to be consistent with an autosomal dominant trait.     

Results of retinal detachment surgery in Marfan syndrome in asians

PURPOSE: To report the anatomic and visual results of retinal detachment in patients with Marfan syndrome in an Asian population. METHOD: A retrospective review of all patients with Marfan syndrome and retinal detachment operated on by a single surgeon was conducted. RESULTS: Thirteen eyes of 12 patients were reviewed. Eleven patients were males, with an average age of 24.6 years. Three eyes had a history of trauma. Ten eyes were successfully treated with scleral buckle and encircling band. Three eyes were also treated with vitrectomy, scleral buckle, and encircling band. One hundred percent success was achieved with only one surgery. Best-corrected visual acuity of at least 20/40 was achieved in 7 of 12 eyes (58.3%). Eight of 12 eyes (66.7%) had at least two lines of improvement on the Snellen chart after 6 months. CONCLUSIONS: With appropriate surgical intervention, an excellent anatomic reattachment rate and good visual outcome can be achieved in Marfan syndrome patients with retinal detachment regardless of their lens status.     

Retinal detachment associated with morning glory syndrome

CASE REPORT: A twenty three year old woman was diagnosed of a morning glory papillary anomaly, then with normal visual acuity (VA). Nine years later, the VA decreased to 0.4, secondary to a serous macular detachment, confirmed by optical coherence tomography (OCT). After treatment with C2F6 gas injection, positioning, and peripapillary laser, the VA improved to 0.7 and the foveolar area reattached. DISCUSSION: The morning glory Syndrome usually has an early diagnosis due to poor visual acuity. Thirty eight percent of the cases have retinal detachment. We show an unusual case of morning glory syndrome with a serous detachment, successfully treated with gas and laser.     

AIDS／HIV感染合并视网膜脱离

目的探讨获得性免疫缺陷综合征（AIDS）／艾滋病病毒（HIV）感染合并视网膜脱离（RD）的临床特点、药物及手术治疗。方法回顾11例（16眼）AIDS／HIV感染患者RD的实验室检查和临床诊治资料,分析其病因、与细胞免疫的关系、RD发生的高危因素、治疗方法及治疗效果的影响因素。6例（6眼）做了玻璃体视网膜手术。结果13眼孔源性RD由病毒性视网膜炎及可疑病毒性视网膜炎引起,2眼RD由梅毒性葡萄膜炎引起,1眼为原发性孔源性视网膜脱离;发病时,6例CDfT淋巴细胞计数低于100个／μL,3例CD4T淋巴细胞计数高于200个／μL;未手术跟视力无改变,手术眼术后视力有不同程度提高,病程短及单纯性孔源性网脱眼术后矫正视力好于病程长及感染性视网膜炎导致的网脱眼。结论病毒性视网膜炎是CD4^＋T淋巴细胞计数较低的AIDS患者发生孔源性RD的主要病因,原发性孔源性RD及非病毒感染葡萄膜视网膜炎是CD4^＋T淋巴细胞计数较高的AIDS／HIV感染患者RD的主要病因。CD4^＋T淋巴细胞计数较低是RD的高危因素;玻璃体视网膜手术是视网膜解剖复位、改善视力的首选治疗方法。RD的病因、病程及病情严重程度是影响手术后视力的因素。     

Retinal detachment in pseudophakia

In a group of 2,281 eyes after extraction of a cataract and implantation of an iris clip lens (1,362 eyes after intracapsular extraction and 927 eyes after extracapsular extraction) motion of the retina occurred in 36 eyes (1.67%), incl. 27 after intracapsular extractions (1.98%) and 9 after extracapsular extractions (0.97%). In patients with pseudophakic retinal detachment after implantation of an iris clip lens the frequency of factors which might interfere with the development of this complication was investigated. The latter proved to be the following: prolapse of the vitreous body, luxation of the implanted lens, posterior uveitis and myopia. Detachment of the retina was preceded by: peroperative prolapse of the vitreous body in 34.21%, luxation of the implanted lens in 28.95%, posterior uveitis in 36.85% and myopia (up to -4.0 Ds) in 21.05%. Anatomical and functional results after operation of pseudophakic motion were evaluated in 38 eyes (36 eyes operated on account of cataract in the authors' department, two in other departments). The minimal follow up period after operation of retinal detachment was six months. Anatomical restoration of the retina was achieved in 23 eyes (60.53%), in 9 eyes a residual motion remained (23.68%) and in four eyes total motion (10.53%). In two eyes keratopathy developed (5.26%). Central vision 6/6-6/12 was achieved in 18.42%, 6/15-6/36 in 21.05%, 6/60-3/60 in 28.95% and less than 3/60 in 31.58%. In the poorer anatomical and functional results after operation of pseudophakic motions participate in addition to the above mentioned ones difficult visualization of the retinal periphery and the extent of surgical trauma during surgery of detached retinas.     

Retinal detachment in aphakia

In a prospective study five hundred consecutive cases of cataract operations were followed during ten years. Nine cases of retinal detachment were observed. In a retrospective study 77 cases with aphakic retinal detachment were studied according to type of cataract operation, interval between this operation and the onset of retinal detachment, refraction, area of detachment and results of treatment. About fifty percent of the cases of retinal detachment are observed during the first year after the cataract operation. At the first examination the detached area is usually larger in aphakic eyes than in phakic eyes. The anatomical and functional cure after detachment operation is probably less in aphakic than phakic eyes.