Rare. Unknown etiology. Proliferation and expansion of the intrathoracic lymphatic vessels due to a regional anomaly of their embryogenesis. The first symptoms appear between birth and adolescence in 70 % of cases but sometimes the problem is diagnosed in adulthood. Although it is a benign condition, prognosis is poor because there is no known effective treatment.

Nonspecific symptoms: shortness of breath, cough, wheezing, hemoptysis, chest pain, chylothorax or chylopericardium...

RX, CT and MRI: pleural and/or pericardial effusions,  mediastinal or pulmonary masses; diffuse thickening of the interlobar septae with sometimes a nodular appearance, mediastinal infiltration, abundant pericardial effusion without signs of tamponade.

DIC cases have been resported, the mechanism of which is poorly understood.

Differential diagnosis: chest lymphoma

Treatments: surgical resection if the problem is localized otherwise; corticosteroid therapy; vincristine; somatostatin; interferon; colchicine, lung transplantation

Anesthetic implications:

RX thorax and echocardiography to quantify the pulmonary and pericardial lesions.

References : 

-          Espinosa V, Martin-Achard A, Guinand O. 
Lymphangiomatose pulmonaire diffuse : description de cas et revue de la littérature.
Rev Med Suisse 2012 ; 8 : 1125-9.

-         Nèji H, Hantous-Zannad S, Zidi A, Hamzaoui A, Mezni F et al. 
Lymphangiomatose pulmonaire diffuse. 
Arch Pediatr 2013 ; 20 : 1244-6.

Updated: October 2021