Intraperitoneal cystic lymphangioma and Crohn's disease: an exceptional association

Cystic lymphangioma is a rare benign malformative tumor of the lymphatic vessels which may occur in various locations. Intra-abdominal cystic lymphangioma is less frequent than cervicoaxillary cystic lymphangioma. Clinical presentation is polymorphic. Diagnosis is based on imaging data but it requires histological confirmation. Surgery is the gold standard treatment. We here report a rare case of acquired intraperitoneal cystic lymphangioma secondary to subtotal colectomy in a female patient with severe evolutive Crohn's disease treated with anti-TNF alpha. The patient presented with irreducible right painful paramedian mass with no impulse on coughing, suggesting the diagnosis of strangulated eventration within a surgical scar from midline laparotomy. She underwent emergency surgery. Surgical exploration showed multi-cystic intraperitoneal mass protrunding through the right paramedian eventration. Anatomo-pathological examination helped to confirm the diagnosis of cystic lymphangioma. Postoperatively, the mass was punctured twice to evacuate the fluid, because of incomplete surgical resection. This is the first reported case of cystic lymphangioma in a patient under anti-TNF alpha. It could be caused by disruption of the immune system and more specifically of the lymphocyte population. This association has not hitherto been established and experimental studies are necessary to accept or refuse this hypothesis.