MULTIMODAL IMAGING IN THE CONGENITAL PULMONARY LYMPHANGIECTASIA-CONGENITAL CHYLOTHORAX-HYDROPS FETALIS CONTINUUM

C Bellini, M Mazzella, C Campisi, G Taddei, F Mosca, P Toma, G Villa, F Boccardo, AR Sementa, RC Hennekam, G Serra

Abstract


We report on three infants with congenitalchylothorax (CC) and congenital pulmonarylymphangiectasia (CPL). CPL appears to be acharacteristic pathological finding in Cc.Through the use of lymphoscintigraphy andcomputed tomography, this study suggests thatCC and CPL are strongly correlated entitiesand that the dysplasia of the lymphatic systemresults in a pulmonary lymphatic obstructionsequence. The initial microscopic dilatation ofthe lymph channels may lead to progressiveweeping of lymphatics and, consequently, topleural effusion. Non-Immune HydropsFetalis (NIHF) may be the final consequenceof impaired systemic venous return and mayhelp to explain pleural-pulmonary involvementin this generalized lymph-vessel malformationsyndrome.

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