Sweeney B, Surana R, Puri P
Children's Research Centre, Our Lady's Hospital for Sick Children, Dublin, Ireland.
J Pediatr Surg. 2001 May;36(5):774-6. doi: 10.1053/jpsu.2001.22958.
Duodenal atresia is associated with a higher incidence of associated congenital malformations than jejunoileal atresia, supporting the hypothesis that the duodenal obstruction occurs early in fetal life. In this study, the authors analyzed the incidence of major associated malformations in jejunal atresia (JA) and ileal atresia (IA) to determine if there is a positive correlation between the proximity of the intestinal atresia and the association of other major anomalies.
Records of all patients with jejunoileal atresias treated at the authors' institution between 1980 and 1997 were examined.
There were 83 patients with jejunoileal atresias, 38 with JA, and 45 with IA. Sixteen (42%) of the JA patients had an associated major congenital malformation, whereas only 1 (2%) of the IA patients had an associated malformation. A single atresia was found in 18 (47%) of JA patients and 41 (91%) of IA patients. Twenty (53%) of the JA patients had either multiple or apple-peel atresia. Thirteen patients (16%) died, 11 with JA, and 2 with IA. Of the 11 patients with JA who died, 6 had multiple atresias, 4 had cystic fibrosis, and 1 had small bowel volvulus.
The higher incidence of associated major congenital extraintestinal malformations in JA compared with IA patients suggests that some cases of JA may arise from a malformative process.
十二指肠闭锁比空肠回肠闭锁合并先天性畸形的发生率更高，这支持了十二指肠梗阻发生于胎儿生命早期的假说。在本研究中，作者分析了空肠闭锁（JA）和回肠闭锁（IA）合并主要畸形的发生率，以确定肠道闭锁的位置与其他主要异常的合并之间是否存在正相关。
查阅了1980年至1997年间在作者所在机构接受治疗的所有空肠回肠闭锁患者的记录。
共有83例空肠回肠闭锁患者，其中38例为JA，45例为IA。16例（42%）JA患者合并有主要先天性畸形，而IA患者中只有1例（2%）合并有畸形。18例（47%）JA患者和41例（91%）IA患者为单发闭锁。20例（53%）JA患者为多发或苹果皮样闭锁。13例患者（16%）死亡，11例为JA，2例为IA。在11例死亡的JA患者中，6例为多发闭锁，4例患有囊性纤维化，1例患有小肠扭转。
与IA患者相比，JA患者合并主要先天性肠外畸形的发生率更高，这表明某些JA病例可能源于一种致畸过程。
