Busse H, Austermann K H, Pawlowitzki I H
Klin Monbl Augenheilkd. 1977 Jun;170(6):933-8.
A case of malformation of the tear duct associated with clefts in the face is described. To our knowledge this combination has not been described before. The whole symptoms complex consisted of: bilateral cleft lip upper jaw and gum, choanalatresia, mucocele of the left nasal cavity from adhesions of the lower concha with the nasal septum, cystic dilatation of the tear sac at the connection to a bony naso-lacrimal duct. Right inguinal hernia, syndactyly of the first and second left toes, iris-chroid coloboma right with congenital cataract and unilateral right convergent squint. Because of the advanced dacryocystitis on the basis of the congenital dacryostenosis, and external dacryostorhinostomy (modified after Kaleff 1937) was performed after pre-treatment by instillation of antibiotic solutions in the tear ducts. At the same time the nasal cavity mucocele was opened by partial excision of the lower concha and opening the choanal atresia. After a post-operative course free of complications and with clear patency of the tear ducts the oral surgical operation was done--closure of the face clefts. The chromosome analysis was normal the only abnormal result here was an unexplained slow rate of multiplication of lymphocytes in four cultures inoculated at various points of time. Because we could not find any hereditary factors, this symptom complex is probably due to exogenous embryo damage during the early development phase.
本文描述了一例泪道畸形合并面部裂隙的病例。据我们所知，这种组合此前尚未有过描述。整个症状复合体包括：双侧唇裂、上颌和牙龈裂、后鼻孔闭锁、因下鼻甲与鼻中隔粘连导致的左侧鼻腔黏液囊肿、泪囊与骨性鼻泪管连接处的囊性扩张。右侧腹股沟疝、左侧第一和第二趾并指、右侧虹膜脉络膜缺损伴先天性白内障和右侧单侧共同性内斜视。由于先天性泪道狭窄导致泪囊炎进展，在泪道内滴注抗生素溶液预处理后，进行了外部泪囊鼻腔吻合术（根据1937年卡莱夫改良）。同时，通过部分切除下鼻甲并打开后鼻孔闭锁来处理鼻腔黏液囊肿。在术后无并发症且泪道通畅的情况下，进行了口腔外科手术——修复面部裂隙。染色体分析正常，唯一异常结果是在不同时间接种的四种培养物中淋巴细胞增殖速率不明原因地缓慢。由于我们未发现任何遗传因素，这种症状复合体可能是由于早期发育阶段的外源性胚胎损伤所致。
