A complicated case of mesenteric cystic lymphangioma (MCL) with resulting short bowel syndrome

Abstract

Background: Lymphangiomas are benign conditions characterized by proliferation of lymphatic spaces. The most frequent localizations are craniofacial region, neck, chest, and abdomen (5% of cases). Lymphangioma of the small-bowel mesentery is rare, having been reported for less than 1% of all lymphangiomas. MCL is often diagnosed before the fifth year of age (50% of cases are symptomatic by 1 year). Fetal ultrasonography and MRI are important tools in prenatal diagnosis. We report the case of a newborn with a complicated MCL (without prenatal diagnosis) who developed short bowel syndrome due to massive intestinal volvulus. Case report: A male baby (birth weight 3500 g, + 1.95 SDS), born at 36 weeks and 1 day of gestation by cesarean section due to non-reassuring fetal cardiotocography, was admitted to our Neonatal Intensive Care Unit (NICU) at 12 h of life for bilious vomiting and abdominal distension. Pregnancy was unremarkable except for maternal pre-gravidic diabetes treated with insulin; fetal ultrasonographic monitoring was normal. At admission, abdominal palpation revealed a large flank swelling occupying the left side of the abdomen. Ultrasonographic examinations detected an anechoic mass measuring 6 cm. MRI confirmed a large multilocular cystic mass with septations, suspected for MCL, dilated jejunal loops and volvulus of the small bowel. Laboratory results were normal. Emergency surgical intervention was performed on day 2 because of deterioration of clinical conditions: the cystic mass originating from the mesentery was completely excised, and intestinal derotation and enterostomy were performed. Pathologic examination showed a thin-walled multicystic lymphangioma with chylous fluid inside (6 cm in size). A second look procedure was performed on day 45: necrotic bowel was removed and 60 cm of small bowel were left in place. Bowel continuity was not restored and a double enterostomy was created. The patient developed short bowel syndrome which required prolonged parenteral nutrition. Severe cholestasis occurred and was treated with intravenous omega-3 fatty acids emulsion. Discussion: Intestinal lymphangiomas are considered benign tumors, and usually a conservative approach is pursued. Emergency surgery is required in case of complications (intestinal obstruction, volvulus, torsion or rupture of the cyst and obstruction of the urinary tract). Although rare, intestinal lymphangiomas should be considered in differential diagnosis in all newborns with bilious emesis, in order to avoid massive intestinal resection and consequent short bowel syndrome in otherwise healthy babies.