Congenital intestinal atresia requires emergency surgery soon after birth. Lymphangioma, a benign tumor, is caused by an anomalous lymphatic system. We report a case of congenital intestinal atresia associated with a mesenteric cystic lymphangioma in a low birth weight neonate.
At 21 weeks of pregnancy, ultrasonography revealed a cystic lesion in the fetal abdominal cavity. At 31 weeks, magnetic resonance imaging showed dilatation of the small intestine. This low birth weight (1752 g) male infant was born by vaginal delivery at 32 weeks 3 days’ gestation. Laparotomy on day 2 of life revealed jejunal atresia and a mesenteric cyst. The cyst was removed and intestinal anastomosis was performed. Histologically, the cyst proved to be a mesenteric lymphangioma.
The most popular theories regarding the mechanism of congenital intestinal atresia include reperfusion injury and intestinal tract blood flow disturbance. In this fetus, intestinal torsion had occurred around the mesenteric cystic lymphangioma, which apparently disturbed the mesenteric blood flow and caused intestinal atresia.
There have been few reports of the combination of a mesenteric cystic lymphangioma and congenital intestinal atresia. This case supports the theory that small bowel atresia and stenosis are caused by accidental blood flow disturbance.

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