Velasco A L, Holcomb G W, Templeton J M, Ziegler M M
Department of Surgery, Children's Hospital of Philadelphia, PA 19104.
J Pediatr Surg. 1990 Feb;25(2):192-7. doi: 10.1016/0022-3468(90)90401-t.
Congenital microgastria is a rare anomaly. Only 22 cases have been reported in the literature. Its clinical manifestations depend on the stage at which the embryologic development of the stomach is arrested. The outcome of most patients with severe microgastria previously reported has either been death or extreme malnutrition. Since 1978, four patients with congenital microgastria have been managed at The Children's Hospital of Philadelphia. All patients had severe feeding intolerance and associated congenital malformations. Three patients were treated with a double lumen Roux-en-Y jejunal reservoir (Hunt-Lawrence pouch). This treatment improved the nutritional management of these patients. On follow-up two patients are at the 50th percentile for height and weight, and one is at the 25th percentile. One patient with associated severe congenital heart disease was treated nonoperatively. Her nutritional management was difficult, and she ultimately died of congestive heart failure at 3 years of age. In patients with the most severe forms of microgastria, early operative treatment improves nutritional management and facilitates the achievement of normal growth and development.
先天性小胃是一种罕见的异常情况。文献中仅报道了22例。其临床表现取决于胃胚胎发育停滞的阶段。先前报道的大多数严重小胃患者的结局要么是死亡，要么是极度营养不良。自1978年以来，费城儿童医院收治了4例先天性小胃患者。所有患者均有严重的喂养不耐受及相关先天性畸形。3例患者接受了双腔Roux-en-Y空肠储袋（亨特-劳伦斯袋）治疗。这种治疗改善了这些患者的营养管理。随访时，2例患者的身高和体重处于第50百分位，1例处于第25百分位。1例伴有严重先天性心脏病的患者未接受手术治疗。她的营养管理困难，最终在3岁时死于充血性心力衰竭。对于最严重形式的小胃患者，早期手术治疗可改善营养管理，并有助于实现正常的生长发育。
