Open AccessLinfangiomatosis pulmonar difusa con compromiso pleural y pericárdico. Reporte de un caso pediátrico
Author(s) -
Rodolfo P Moreno,
Yanina Hernández,
Patrícia Garrido,
Bethy Camargo Vargas,
Alberto D. Hernandez,
Javier Faín,
Cecilia Seligra,
Patricia Topp,
Alberto Maffey,
Gustavo Cardigni
Publication year - 2021
Publication title -
archivos argentinos de pediatría
Language(s) - Spanish
Resource type - Journals
SCImago Journal Rank - 0.236
H-Index - 19
eISSN - 1668-3501
pISSN - 0325-0075
DOI - 10.5546/aap.2021.e264
Subject(s) - medicine , lymphangiomatosis , pleural effusion , chyle , chylothorax , surgery , pericardiocentesis , mediastinum , pleural cavity , pericardial effusion , lymphatic system , pathology , complication
Diffuse pulmonary lymphangiomatosis is a rare disease characterized by marked proliferation and dilation of lymphatic vessels in the lungs, pleura, and mediastinum. The prevalence is unknown and the etiology is not fully understood. A 22-month-old girl was admitted for polyserositis, with pericardial and pleural effusion. She required pericardiocentesis and pleural drainage, presenting chyle drainage (1.5-4 liters/ day) without response to medical treatment (fasting, parenteral nutrition and octreotide). A lung biopsy was performed. The pathological anatomy showed findings compatible with diffuse pulmonary lymphangiomatosis. Treatment with sirolimus and propanolol began, decreasing losses due to pleural drainage one week after treatment. She progressed well, discontinued oxygen supply and pleural drainage was removed, leaving the patient after the fourth month of hospitalization. Early diagnosis of diffuse pulmonary lymphangiomatosis is difficult to achieve, but it allows the application of therapies that prevent disease progression, reducing morbidity and mortality.