Ghosh Debangshu, Saha Somnath, Basu Sumit Kumar
Department of ENT, R. G. Kar Medical College, Kolkata, West Bengal, India.
Indian J Ophthalmol. 2015 Oct;63(10):800-3. doi: 10.4103/0301-4738.171524.
Ectrodactyly-ectodermal dysplasia and clefting syndrome or "Lobster claw" deformity is a rare congenital anomaly that affects tissues of ectodermal and mesodermal origin. Nasolacrimal duct (NLD) obstruction with or without atresia of lacrimal passage is a common finding of such a syndrome. The authors report here even a rarer presentation of the syndrome which manifested as bilateral NLD obstruction and lacrimal fistula along with cleft lip and palate, syndactyly affecting all four limbs, mild mental retardation, otitis media, and sinusitis. Lacrimal duct obstruction and fistula were managed successfully with endoscopic dacryocystorhinostomy (DCR) which is a good alternative to lacrimal probing or open DCR in such a case.
缺指（趾）-外胚层发育不良及腭裂综合征或“龙虾爪”畸形是一种罕见的先天性异常，会影响外胚层和中胚层起源的组织。鼻泪管（NLD）阻塞伴或不伴有泪道闭锁是该综合征的常见表现。作者在此报告了该综合征更罕见的一种表现，即双侧鼻泪管阻塞和泪瘘，同时伴有唇腭裂、四肢并指（趾）畸形、轻度智力发育迟缓、中耳炎和鼻窦炎。泪道阻塞和泪瘘通过内镜下泪囊鼻腔造口术（DCR）成功得到治疗，在这种情况下，这是泪道探通术或开放性DCR的良好替代方法。
