Intra-abdominal cystic lymphangiomas in infancy and childhood

Background: Cystic lymphangiomas (CL) rarely present as intra-abdominal masses. Abdominal CL is often discussed in conjunction with mesenteric cysts; however, their histology, location and age of presentation differ significantly. In an attempt to establish a best diagnostic and treatment modality, we report our experience dealing with intra-abdominal CL during a 5-year period. Methods: Between January 1998 and December 2003, 12 patients, 7 boys and 5 girls, with a diagnosis of CL were reviewed. Modes of clinical presentation, location of CL, methods of diagnosis, surgical intervention and histological examination were all analyzed. Results: The ages of the 12 patients ranged from 8 days to 6 years. Eleven of the 12 patients were symptomatic with abdominal pain, abdominal distention or palpable mass, dysuria and severe acute abdominal pain mimicking appendicitis. Abdominal ultrasound was done preoperatively in all patients. At laparotomy, 5 CL were located in the omentum, 5 in the mesentery, and another 2 in the retroperitoneum. All omental CL were completely excised without difficulty. CL removal required resection of both the cyst and intestine in 2 patients. One of 2 retropritoneal CL was removed with small areas of the posterior wall of the cyst remaining on the inferior vena cava (IVC). There were no major postoperative complications, deaths, or recurrences in this series. Conclusions: Intra-abdominal CL are usually involved in young children and are usually symptomatic. A preoperative diagnosis is possible with ultrasound study. Complete excision of the cysts with or without intestinal resection is mandatory to prevent recurrence. The long-term prognosis is excellent.