Dalla Vecchia L K, Grosfeld J L, West K W, Rescorla F J, Scherer L R, Engum S A
Department of Pediatric Surgery, James Whitcomb Riley Hospital for Children, Indiana University Medical Center, Indianapolis 46202, USA.
Arch Surg. 1998 May;133(5):490-6; discussion 496-7. doi: 10.1001/archsurg.133.5.490.
To evaluate the causes, clinical presentation, diagnosis, operative management, postoperative care, and outcome in infants with intestinal atresia.
Retrospective case series.
Pediatric tertiary care teaching hospital.
A population-based sample of 277 neonates with intestinal atresia and stenosis treated from July 1, 1972, through April 30, 1997. The level of obstruction was duodenal in 138 infants, jejunoileal in 128, and colonic in 21. Of the 277 neonates, 10 had obstruction in more than 1 site. Duodenal atresia was associated with prematurity (46%), maternal polyhydramnios (33%), Down syndrome (24%), annular pancreas (33%), and malrotation (28%). Jejunoileal atresia was associated with intrauterine volvulus, (27%), gastroschisis (16%), and meconium ileus (11.7%).
Patients with duodenal obstruction were treated by duodenoduodenostomy in 119 (86%), of 138 patients duodenotomy with web excision in 9 (7%), and duodenojejunostomy in 7 (5%) A duodenostomy tube was placed in 3 critically ill neonates. Patients with jejunoileal atresia were treated with resection in 97 (76%) of 128 patients (anastomosis, 45 [46%]; tapering enteroplasty, 23 [24%]; or temporary ostomy, 29 [30%]), ostomy alone in 25 (20%), web excision in 5 (4%), and the Bianchi procedure in 1 (0.8%). Patients with colon atresia were managed with initial ostomy and delayed anastomosis in 18 (86%) of 21 patients and resection with primary anastomosis in 3 (14%). Short-bowel syndrome was noted in 32 neonates.
Morbidity and early and late mortality.
Operative mortality for neonates with duodenal atresia was 4%, with jejunoileal atresia, 0.8%, and with colonic atresia, 0%. The long-term survival rate for children with duodenal atresia was 86%; with jejunoileal atresia, 84%; and with colon atresia, 100%. The Bianchi procedure (1 patient, 0.8%) and growth hormone, glutamine, and modified diet (4 patients, 1%) reduced total parenteral nutrition dependence.
Cardiac anomalies (with duodenal atresia) and ultrashort-bowel syndrome (<40 cm) requiring long-term total parenteral nutrition, which can be complicated by liver disease (with jejunoileal atresia), are the major causes of morbidity and mortality in these patients. Use of growth factors to enhance adaptation and advances in small bowel transplantation may improve long-term outcomes.
评估肠闭锁婴儿的病因、临床表现、诊断、手术治疗、术后护理及预后。
回顾性病例系列研究。
儿科三级护理教学医院。
以人群为基础的样本，包括1972年7月1日至1997年4月30日期间接受治疗的277例患有肠闭锁和狭窄的新生儿。梗阻部位为十二指肠的有138例婴儿，空回肠的有128例，结肠的有21例。在这277例新生儿中，10例存在一个以上部位的梗阻。十二指肠闭锁与早产（46%）、母亲羊水过多（33%）、唐氏综合征（24%）、环状胰腺（33%）和肠旋转不良（28%）相关。空回肠闭锁与宫内肠扭转（27%）、腹裂（16%）和胎粪性肠梗阻（11.7%）相关。
138例十二指肠梗阻患者中，119例（86%）接受了十二指肠十二指肠吻合术，9例（7%）接受了十二指肠切开并切除隔膜，7例（5%）接受了十二指肠空肠吻合术。3例危重新生儿放置了十二指肠造瘘管。128例空回肠闭锁患者中，97例（76%）接受了切除术（吻合术45例[46%]；缩窄肠成形术23例[24%]；或临时造口术29例[30%]），25例（20%）仅接受了造口术，5例（4%）接受了隔膜切除，1例（0.8%）接受了比安基手术。21例结肠闭锁患者中，18例（86%）首先进行了造口术并延迟吻合，3例（14%）进行了切除并一期吻合。32例新生儿出现短肠综合征。
发病率以及早期和晚期死亡率。
十二指肠闭锁新生儿的手术死亡率为4%，空回肠闭锁为0.8%，结肠闭锁为0%。十二指肠闭锁患儿的长期生存率为86%；空回肠闭锁为84%；结肠闭锁为100%。比安基手术（1例患者，0.8%）以及生长激素、谷氨酰胺和改良饮食（4例患者，1%）降低了对全胃肠外营养的依赖。
心脏异常（与十二指肠闭锁相关）以及需要长期全胃肠外营养的超短肠综合征（<40 cm）（可并发肝病[与空回肠闭锁相关]）是这些患者发病和死亡的主要原因。使用生长因子促进适应以及小肠移植技术的进步可能会改善长期预后。
