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Case Reports
. 2021 Jun;119(3):e264-e268.
doi: 10.5546/aap.2021.e264.

[Diffuse pulmonary lymphangiomatosis with pleural and pericardial involvement. Pediatric case report]

[Article in Spanish]
Rodolfo P Moreno  1 , Yanina Hernández  2 , Patricia Garrido  3 , Bethy Camargo Vargas  2 , Alberto Hernández  2 , Javier Faín  2 , Cecilia Seligra  2 , Patricia Topp  2 , Alberto Maffey  4 , Gustavo Cardigni  2
Affiliations
Free article
Case Reports

[Diffuse pulmonary lymphangiomatosis with pleural and pericardial involvement. Pediatric case report]

[Article in Spanish]
Rodolfo P Moreno et al. Arch Argent Pediatr. 2021 Jun.
Free article

Abstract
in English, Spanish

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.

La linfangiomatosis pulmonar difusa es una enfermedad rara caracterizada por una marcada proliferación y dilatación de los vasos linfáticos en los pulmones, la pleura y el mediastino. Se desconoce la prevalencia, y la etiología no se comprende completamente. Una niña de 22 meses ingresó por poliserositis, con derrame pericárdico y pleural. Requirió pericardiocentesis y avenamiento pleural, y presentó drenaje de quilo (1,5- 4 litros/día) sin respuesta al tratamiento médico (ayuno, nutrición parenteral y octreotide). Se realizó biopsia pulmonar. La anatomía patológica mostró hallazgos compatibles con linfangiomatosis difusa pulmonar. Comenzó tratamiento con sirolimus y propanolol, que disminuyeron las pérdidas por el drenaje pleural a la semana. Presentó buena evolución; suspendió aporte de oxígeno y se retiró el drenaje pleural. Se externó al cuarto mes de internación. El diagnóstico temprano de la linfangiomatosis pulmonar difusa es difícil de lograr, pero permite aplicar terapéuticas que evitan la progresión de enfermedad y disminuir la morbimortalidad.

Keywords: chylothorax; diffuse pulmonary lymphangiomatosis; pediatrics.

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Conflict of interest statement

None

References

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