肠系膜憩室索带所致小儿急性肠梗阻的临床探讨

A clinical study of acute intestinal obstruction due to mesodiverticular band in children

目的总结肠系膜憩室索带导致的小儿急性肠梗阻的临床特点,分析其病因,以提高对该病的认识和诊治水平。方法回顾性分析1998年3月至2018年3月间收治的17例肠系膜憩室索带所致急性肠梗阻患儿的临床资料。其中,男13例,女4例。其中1例(男)为妊娠7个月死产儿,于一项尸体解剖研究中发现。其余16例,年龄7 d至14岁,平均4.20岁;均以不明原因的"呕吐、腹胀和哭闹或腹痛"就诊。结果手术探查发现存在麦克尔憩室并索带残留致肠梗阻6例(35.3%),不伴麦克尔憩室的肠系膜憩室索带11例(64.7%)。其中9例索带送检病理诊断,其余患儿根据索带起止走行及表现等临床诊断。除1例患儿解除梗阻后因合并全结肠无神经节细胞症最后死亡外,余15例患儿均通过手术治疗得到治愈。其中切除索带及绞窄坏死肠管5例,切除索带及受累穿孔、撕裂肠管各1例,其余行索带松解切除解除肠梗阻(合并麦克尔憩室者相应切除)。除最近1例外,所有存活病例术后均获随访1年以上,未见明显并发症和后遗症。结论肠系膜憩室索带源于胚胎期卵黄血管发育异常,发病机制及术中表现复杂,缺乏特异症状体征和检查手段,漏诊误诊率高。对不明原因急性肠梗阻怀疑肠绞窄者,应考虑积极手术探查,发现索带组织应仔细探查并送病理检查。

Objective To explore the clinical characteristics, pathogenesis, diagnosis and treatment of acute intestinal obstruction due to mesodiverticular band in children. Methods The clinical data of 17 patients with acute intestinal obstruction due to mesodiverticular band between 1998 and 2018 were retrospectively analyzed along with a review of literatures. Results The male-to-female ratio was 13∶4. One case was 7-month-old stillbirth from an autopsy study. The median age at diagnosis was 4.2 years. The common symptoms were vomiting, abdominal pain and/or distension. None of them had any operative history. And 35.3%(6/17) of them had both mesodiverticular band (MB) and Meckel's diverticulum (MD) and 64.7%(11/17) had MB only. Nine cases received a pathologic diagnosis while the others were clinical diagnosed by intraoperative findings. Except 1 case didn’t rescue because of total colonic aganglionosis after removal of the obstruction, all children through surgery treatment were cured. MB led to strangulation of necrotic bowel (n=5), intestinal perforation (n=1) and intestinal rupture (n=1). Surgical resection of MB and bowel were performed in 7 cases, just MB resection (partially with MD resection) in the remainder. Except for one recent case this year, the remainder were operated without any complication during 1-year follow-up. Conclusions Resulting from the remnant of vitelline vessel, MB frequently leads to acute intestinal obstruction. Lacking specific clinical symptoms and physical examinations, it has a high rate of underdiagnosis and misdiagnosis. Unexplained abdominal pain and distension without an operative history should raise a high alert particularly for strangulated intestinal obstruction. Timely operative intervention is beneficial for preventing intestinal necrosis or even sudden death. The bands causing acute intestinal obstruction should be closely examined and a routine pathological examination is necessary.