Phillips J Duncan, Raval Mehul V, Redden Courtney, Weiner Timothy M
Division of Pediatric Surgery, Department of Surgery, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
J Pediatr Surg. 2008 Dec;43(12):2208-12. doi: 10.1016/j.jpedsurg.2008.08.065.
Intestinal atresia (IA) occurs in 10% to 20% of infants born with gastroschisis (GS). We describe a distinct subset of these children with severe dysmotility, without mechanical intestinal obstruction, and with adequate intestinal length for enteral nutrition and propose possible treatment strategies.
A total of 177 patients with GS managed at a single institution between 1993 and 2007 were retrospectively reviewed.
Twenty-one (12%) patients had IA. Six (29%) did well, with gradual progression to full diet; 1 died at birth because of complete midgut infarction; 1 died of necrotizing enterocolitis; 4 with short bowel syndrome (SBS) (small bowel length, 30-41 cm) died of liver failure because of total parenteral nutrition (3) or sepsis (1). Nine (43%) were felt to have gastroschisis/atresia/dysmotility, defined as adequate small bowel length for survival as measured at time of laparotomy (mean, 146 cm; range, 66-233 cm), massive intestinal dilatation, and stasis. Of 9 patients with gastroschisis/atresia/dysmotility, 5 (56%) survived. All had surgery to "rescue" their dysfunction intestine, at mean age 128 days (range, 52-271 days): 4 had tapering enteroplasties of mean 37 cm (range, 5-115 cm)-all 4 survived; 3 had diverting stomas created for intestinal decompression, followed by stoma closure-1 survived; 1 nonsurvivor had a Kimura "patch"; 1 nonsurvivor underwent redo anastomosis. Survivors weaned off total parenteral nutrition at mean age 331 days after rescue surgery (range, 42-814 days).
More than one third of patients with GS/IA appear to have significant intestinal dysmotility without true SBS nor obstruction. Successful treatment of these infants may be achieved with the use of tapering enteroplasty and/or temporary diverting stomas.
先天性腹裂（GS）患儿中，10%至20%会发生肠闭锁（IA）。我们描述了这些患儿中的一个独特亚组，他们存在严重的动力障碍，无机械性肠梗阻，且肠道长度足以进行肠内营养，并提出了可能的治疗策略。
回顾性分析了1993年至2007年在单一机构接受治疗的177例GS患儿。
21例（12%）患儿患有IA。6例（29%）情况良好，逐渐过渡到正常饮食；1例出生时因完全性中肠梗死死亡；1例死于坏死性小肠结肠炎；4例患有短肠综合征（SBS）（小肠长度30 - 41厘米），因全肠外营养（3例）或败血症（1例）死于肝功能衰竭。9例（43%）被认为患有腹裂/闭锁/动力障碍，定义为剖腹手术时测量的小肠长度足以维持生存（平均146厘米；范围66 - 233厘米）、肠道大量扩张和淤滞。9例腹裂/闭锁/动力障碍患儿中，5例（56%）存活。所有患儿均接受手术以“挽救”其功能障碍的肠道，平均年龄128天（范围52 - 271天）：4例行平均长度为37厘米（范围5 - 115厘米）的肠管缩窄成形术，4例均存活；3例行造口术以进行肠道减压，随后关闭造口——1例存活；1例非存活者接受了木村“补片”手术；1例非存活者接受了再次吻合术。存活者在挽救手术后平均331天（范围42 - 814天）停用全肠外营养。
超过三分之一的GS/IA患儿似乎存在明显的肠道动力障碍，既无真正的SBS也无梗阻。使用肠管缩窄成形术和/或临时造口术可能成功治疗这些婴儿。
