Multimodal imaging in the congenital pulmonary lymphangiectasia-congenital chylothorax-hydrops fetalis continuum

Carlo Bellini, M. Mazzella, C. Campisi, G. Taddei, F. Mosca, P. Tomà, G. Villa, F. Boccardo, A. R. Sementa, R. C. Hennekam, G. Serra

Research output: Contribution to journalArticle

Abstract

We report on three infants with congenital chylothorax (CC) and congenital pulmonary lymphangiectasia (CPL). CPL appears to be a characteristic pathological finding in CC. Through the use of lymphoscintigraphy and computed tomography, this study suggests that CC and CPL are strongly correlated entities and that the dysplasia of the lymphatic system results in a pulmonary lymphatic obstruction sequence. The initial microscopic dilatation of the lymph channels may lead to progressive weeping of lymphatics and, consequently, to pleural effusion. Non-Immune Hydrops Fetalis (NIHF) may be the final consequence of impaired systemic venous return and may help to explain pleural-pulmonary involvement in this generalized lymph-vessel malformation syndrome.

Original languageEnglish
Pages (from-to)22-30
Number of pages9
JournalLymphology
Volume37
Issue number1
Publication statusPublished - Mar 2004

ASJC Scopus subject areas

  • Immunology

Fingerprint Dive into the research topics of 'Multimodal imaging in the congenital pulmonary lymphangiectasia-congenital chylothorax-hydrops fetalis continuum'. Together they form a unique fingerprint.

  • Cite this

    Bellini, C., Mazzella, M., Campisi, C., Taddei, G., Mosca, F., Tomà, P., Villa, G., Boccardo, F., Sementa, A. R., Hennekam, R. C., & Serra, G. (2004). Multimodal imaging in the congenital pulmonary lymphangiectasia-congenital chylothorax-hydrops fetalis continuum. Lymphology, 37(1), 22-30.