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Hepatic Pulmonary Fusion: Two Cases with Diaphragmatic Hernia and One Case with Pentalogy of Cantrell.

Authors :
Lin, Jenny
Durham, Megan M
Ricketts, Richard
Abramowsky, Carlos R.
Steelman, Charlotte Katherine
Shehata, Bahig M.
Source :
Fetal & Pediatric Pathology; Dec2012, Vol. 31 Issue 6, p401-409, 9p, 2 Color Photographs
Publication Year :
2012

Abstract

Hepatic pulmonary fusion (HPF) is characterized by a fibrous connection between the liver and lung tissue. We present two cases of hepatic pulmonary fusion diagnosed with right diaphragmatic hernia and a third case with Pentalogy of Cantrell exhibiting complete agenesis of the diaphragm and finger-like projections of liver adhered to the right lung. It has been proposed that this anomaly is secondary to developmental failure of the mesoderm between days 14-18 after conception and is attributed to diaphragmatic maldevelopment. Understanding the molecular-genetic basis of diaphragmatic hernias may shed light on this unusual presentation and explain why other cases show no fusion. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
15513815
Volume :
31
Issue :
6
Database :
Complementary Index
Journal :
Fetal & Pediatric Pathology
Publication Type :
Academic Journal
Accession number :
82560465
Full Text :
https://doi.org/10.3109/15513815.2012.659406