Giant mesenteric lymphatic malformation presenting as small bowel volvulus
A 4-year-old previously healthy male presented to an outside hospital with 2 days of nausea and bilious emesis. An abdominal plain film (Fig. a) revealed small bowel obstruction and an abdominal ultrasound was nondiagnostic. Upon transfer to our institution, he had persistent bilious emesis with periumbilical abdominal pain. His medical and surgical history was unremarkable, with no history of antecedent trauma. His mother noted an increasing abdominal girth over the prior several months, but did not seek medical attention. On physical examination, the child's abdomen was soft, mildly distended and with periumbilical tenderness. He had no rebound tenderness or guarding, and no hernias were present. Review of the ultrasound revealed extensive intra-abdominal fluid. An abdominal computed tomography (CT) scan was obtained and revealed a large intraperitoneal cystic mass measuring 13 × 21 × 9 cm in the left abdomen (Fig. b). A small bowel obstruction was also present with concern for volvulus due to mesenteric swirl seen on axial imaging. There was no evidence of compromised intestine on CT (Fig. c). The patient was taken to the operating room for laparoscopy. The cyst, which arose from the mesentery of the small intestine, resulted in a segmental volvulus with congestion of the involved segment (Fig. a–c). A short vertical midline incision was made to allow the giant cyst to be delivered and the volvulus reduced. After reduction, the congested small bowel returned to a normal appearance. The cyst was excised by removing the 8 cm segment of small bowel from whose mesentery it arose (Fig. c). The abdomen was closed and the patient was discharged in excellent condition.\nPathologic inspection of the mass revealed a thin-walled cyst containing milky fluid. Immunohistochemistry showed that the cyst lining expressed D2–40 (a marker of lymphatic endothelium), but was negative for keratin, WT-1 and calretinin, confirming the diagnosis of lymphatic malformation (Fig. ).\nThe patient continues to do well with no postoperative complications at 7-month follow-up.