Siu K L, Kwok W K, Lee W Y, Lee W H
Department of Paediatrics, Queen Elizabeth Hospital, Hong Kong 30 Gascoigne Road, Kowloon Hong Kong.
Pediatr Surg Int. 1999;15(2):141-2. doi: 10.1007/s003830050538.
A case of colonic atresia (CA) and aganglionosis is presented, which is probably the tenth in the English literature. The boy presented on day 3 of life with delayed passage of meconium, milk intolerance, and progressive abdominal distension. A barium enema study was complicated by barium peritonitis. Emergency peritoneal lavage was done and the intestinal obstruction was relieved by a proximal defunctioning ileostomy; type III CA (Grosfeld classification) was identified. The proximal atretic end was the blind-ending caecum and the distal atretic end commenced in the splenic area as a microcolon extending to the pelvis and replacing the normal colon. Total colonic aganglionosis (TCA) was confirmed by biopsies. This is the first case in the literature of TCA with CA documented before any attempts at colonic resection and anastomosis were made. This association highlights the recommendation for biopsy of the micro-colon and rectum when Type III CA (Grosfeld classification) is encountered in a newborn. The infant underwent a cholecystectomy and Duhamel-Martin operation at 14 months that was complicated by abdominal wound dehiscence; the ileostomy was closed at 22 months of age.
本文报告一例结肠闭锁（CA）合并无神经节症病例，这可能是英文文献中报道的第十例。该男婴出生第3天出现胎粪排出延迟、牛奶不耐受及进行性腹胀。钡剂灌肠检查并发钡剂性腹膜炎。行急诊腹膜灌洗，并通过近端失功能回肠造口术缓解肠梗阻；确诊为III型CA（格罗斯菲尔德分类法）。近端闭锁端为盲端盲肠，远端闭锁端起自脾区，为延伸至盆腔并取代正常结肠的小结肠。活检证实为全结肠无神经节症（TCA）。这是文献中首例在进行任何结肠切除和吻合术尝试之前记录到的TCA合并CA病例。这种关联凸显了新生儿遇到III型CA（格罗斯菲尔德分类法）时对小结肠和直肠进行活检的建议。该婴儿14个月时接受了胆囊切除术和杜哈梅尔 - 马丁手术，术后出现腹部伤口裂开；22个月时关闭回肠造口。
