Management of congenital microgastria.

ABSTRACT

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.