Document Detail

Multimodal imaging in the congenital pulmonary lymphangiectasia-congenital chylothorax-hydrops fetalis continuum.
MedLine Citation:
PMID:  15109074     Owner:  NLM     Status:  MEDLINE    
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.
C Bellini; M Mazzella; C Campisi; G Taddei; F Mosca; P Tomà; G Villa; F Boccardo; A R Sementa; R C Hennekam; G Serra
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Lymphology     Volume:  37     ISSN:  0024-7766     ISO Abbreviation:  Lymphology     Publication Date:  2004 Mar 
Date Detail:
Created Date:  2004-04-27     Completed Date:  2004-07-08     Revised Date:  2006-11-03    
Medline Journal Info:
Nlm Unique ID:  0155112     Medline TA:  Lymphology     Country:  United States    
Other Details:
Languages:  eng     Pagination:  22-30     Citation Subset:  IM    
Servizio di Patologia Neonatale, Dipartimento di Pediatria, Università di Genova, Istituto G. Gaslini, Genova, Italia.
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MeSH Terms
Abnormalities, Multiple / radiography*,  radionuclide imaging*
Chylothorax / congenital*,  radiography,  radionuclide imaging
Hydrops Fetalis / radiography*,  radionuclide imaging*
Infant, Newborn
Lung Diseases / congenital*,  radiography,  radionuclide imaging
Lymphangiectasis / congenital*,  radiography,  radionuclide imaging
Lymphatic System / radionuclide imaging
Tomography, X-Ray Computed

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