儿童眼皮肤白化病合并婴儿型眼球震颤综合征的手术治疗

摘要

Objective To evaluate the effects of surgery for children with octlocutaneous albinism (OCA) and infantile nystagmus syndrome (INS).Methods Children diagnosed oculocutaneous albinism (OCA) and infantile nystagmus syndrome (INS) was collected from Shandong Provincial Hospital affiliated to Shandong University between April 1,2012 and May 30,2015.The subjects underwent evaluation of general eye examination and ocular motility recordings.The surgical procedure and surgical amount were designed according to type of anomalous head posture (AHP),strabismus,with or without convergence damping.The post-op follow-up was at least 3 months.Postoperative observation included the change of strabismus deviation,AHP and the intensity of nystagmus.Pre-and post-operative best corrected visual acuity (BCVA) were tested for the older albinotic children.Results Eighty-seven cases with OCA and INS (49 males and 38 females;mean age of 2.41 ± 1.78 years) were enrolled in the study.The mean follow-up was 13.6±12.7 months (3-41 months).Of all the participants,77(89％) subjects had AHP,of which Chin-down position (n=63) was the most common.Heterotropia was observed in 38 participants (44％),26 subjects were concomitant esotropia,12 subjects were concomitant exotropia.BCVA of older albinos increased 0.1 logMAR after surgery.The AHP and intensity of nystagmus improved significantly.Eye positions of all strabismus patients were orthotopic.No visual acuity decreased in any patients.Conclusions Surgery is a safe and efficient method for children with OCA and INS,which can increase visual function,improve AHP and decrease the intensity of nystagmus.%目的 探讨儿童眼皮肤白化病(OCA)合并婴儿型眼球震颤综合征(INS)的手术治疗效果.方法 收集2012年4月至2015年5月在山东省立医院眼科门诊就诊的OCA伴INS儿童.患儿均行详细眼科一般检查、眼球运动描记等检查.手术方式及手术量根据患儿伴或不伴代偿头位、斜视、集合阻滞等设计.术后随访至少3个月.术后观察最佳矫正视力(BCVA)、斜视度、代偿头位、眼球震颤强度的变化.结果 收集符合条件的患者87例,其中男性49例,女性38例,平均年龄(2.41±1.78)岁.平均随访(13.6±12.7)月(3～41月).伴代偿头位者77例,其中下颌内收型最多见(63例).合并斜视者38例,其中共同性内斜视26例,共同性外斜视12例.存在集合阻滞现象者2例.术后观察,无一例出现视力下降.较大年龄患儿BCVA较术前平均增加0.1 logMAR.代偿头位均明显改善,眼球震颤强度均明显减低.斜视患者眼位均正位.结论 对OCA合并INS的儿童进行眼球震颤手术能提高视功能,改善代偿头位,减轻眼球震颤,是一种安全、有效的方法.