Raghunath B V, Shankar Gowri, Babu M Narendra, Kini Usha, Ramesh S, Jadhav Vinay, Aravind K L
Dept. of Pediatric Surgery, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India,
Pediatr Surg Int. 2014 Jan;30(1):119-22. doi: 10.1007/s00383-013-3367-8. Epub 2013 Aug 15.
Skip segment Hirschsprung's disease is a condition where an area of normally ganglionated intestine is interspersed proximally and distally by aganglionic segments. The occurrence of skip segment has no clear embryological explanation, contrary to the present concept of failure of cranio-caudal migration of neural crest-derived cells in Hirschsprung's disease. Only 25 case reports have been published in literature so far and this is possibly the first to be reported from Asia. We report one such case with novel surgical management.
跳跃段型先天性巨结肠是一种正常有神经节的肠段在近端和远端被无神经节段穿插的疾病。与目前关于先天性巨结肠中神经嵴衍生细胞头尾迁移失败的概念相反,跳跃段的出现尚无明确的胚胎学解释。迄今为止,文献中仅发表了25例病例报告,这可能是亚洲报道的首例。我们报告了这样一例采用新型手术治疗的病例。