Congenital lymphangiomatosis: Multidisciplinary approach

Abstract Background Diffuse lymphangiomatosis is a rare disorder of the lymphatic vessels. Its prognosis is poor and its treatment not codified. Methods We describe the case of a 14 years old boy known for a lymphatic malformation of the left chest wall with pleuropulmonary, bone and visceral involvement. He presented with respiratory difficulties and a left pleural effusion. The treatment associated thoracic drainage, surgical pleurectomy and iterative sclerotherapy. Results The patient is currently asymptomatic. On imaging, there is a decrease of the left lateral thoracic component and a resolution of the intra-pleural portion. Conclusions The treatment of diffuse lymphangiomatosis is based on a multimodal management.