Wright J R, Barth R F, Neff J C, Poe E T, Sucheston M E, Stempel L E
Pediatr Pathol. 1986;5(3-4):421-48. doi: 10.3109/15513818609068868.
Prune belly syndrome (PBS), a triad consisting of abdominal musculature hypoplasia, urinary tract malformations, and cryptorchidism, is frequently associated with other congenital malformations. Although it is acknowledged that gastrointestinal (GI) malrotation and mesenteric anomalies are frequent in PBS, other GI anomalies are generally considered to be exceedingly rare. Here we describe 3 autopsy cases with severe malformations of both midgut and hindgut derivatives and review the world literature to evaluate the spectrum of GI malformations associated with this syndrome. The relatively high frequency of distal stenoses and atresias suggests that the anomalous mesenteric attachments may predispose to prenatal volvulus and subsequent anatomic bowel obstruction. Postnatal volvulus is also occasionally observed. Infants with PBS also appear to be at a higher risk for persistence of the common fetal cloaca.
梅干腹综合征（PBS），一种由腹部肌肉发育不全、泌尿系统畸形和隐睾症组成的三联征，常与其他先天性畸形相关。尽管人们公认胃肠（GI）旋转不良和肠系膜异常在PBS中很常见，但其他GI异常通常被认为极其罕见。在此，我们描述3例中肠和后肠衍生物均有严重畸形的尸检病例，并回顾世界文献以评估与该综合征相关的GI畸形谱。远端狭窄和闭锁的相对高发生率表明，异常的肠系膜附着可能易导致产前肠扭转及随后的解剖性肠梗阻。产后肠扭转也偶尔可见。患有PBS的婴儿似乎也有更高的持续性胎儿泄殖腔的风险。
