Abdominal cystic lymphangioma in children: benign lesions that can have a proliferative course

Twenty-one pediatric cases of abdominal cystic lymphangioma (CL) treated in the past 20 years are reviewed. To date, this is the largest reported series. CL is a rare congenital malformation that presents either with chronic abdominal distension (and is detected by palpation of a cystic mass) or acutely with bowel obstruction or signs of peritonitis. It is more common among boys and most often occurs in children under 5 years of age. Abdominal ultrasonography is the procedure of choice for establishing the diagnosis. Acute cases with intracystic hemorrhage are more difficult to diagnose. Computed tomography and celioscopy may be useful. With these techniques, a correct diagnosis should be achieved in nearly every case. Enucleation (when feasible) or segmental intestinal resection (when the cyst is intimate to the bowel) is effective treatment. In a few cases the malformation is diffuse, and extensive bowel resection is necessary, with the risk of short bowel syndrome.