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

• Published in: Fetal and pediatric pathology Vol. 31; no. 6; pp. 401 - 409
• Main Authors: Lin, Jenny, Durham, Megan M, Ricketts, Richard, Abramowsky, Carlos R., Steelman, Charlotte Katherine, Shehata, Bahig M.
• Format: Journal Article
• Language: English
• Published: England Informa Healthcare 01-12-2012; Taylor & Francis
• Subjects: congenital diaphragmatic hernia; Fatal Outcome; Gestational Age; hepatic pulmonary fusion (HPF); Hernia, Diaphragmatic - pathology; Hernia, Diaphragmatic - surgery; Hernias, Diaphragmatic, Congenital; Humans; Liver - abnormalities; Liver - surgery; Lung - abnormalities; Lung - surgery; Pentalogy of Cantrell; Pentalogy of Cantrell - pathology; Premature Birth; Thoracotomy; Treatment Outcome
• ISSN: 1551-3815; 1551-3823
• DOI: 10.3109/15513815.2012.659406

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.