Congenital lymphangiomatosis: Multidisciplinary approach

Diffuse lymphangiomatosis is a rare disorder of the lymphatic vessels. Its prognosis is poor and its treatment not codified. 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 res...

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Bibliographic Details
Published in:Journal of pediatric surgery case reports 2020-10, Vol.61, p.101621, Article 101621
Main Authors: El Ezzi, Oumama, Saliou, Guillaume, Gengler, Carole, de Buys Roessingh, Anthony
Format: Article
Language:English
Subjects:
Congenital
Lymphangiomatosis
Pediatrics
Sclerotherapy
Vascular malformations
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Summary:Diffuse lymphangiomatosis is a rare disorder of the lymphatic vessels. Its prognosis is poor and its treatment not codified. 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. 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. The treatment of diffuse lymphangiomatosis is based on a multimodal management.
ISSN:2213-5766
2213-5766
DOI:10.1016/j.epsc.2020.101621