title: Hepatic pulmonary fusion: two cases with diaphragmatic hernia and one case with Pentalogy of Cantrell.
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.
authors.affiliation: Department of Pathology, Children's Healthcare of Atlanta, Atlanta, Georgia 30322, USA.
authors.forename: Jenny
authors.initials: J
authors.lastname: Lin
authors.forename: Megan M
authors.initials: MM
authors.lastname: Durham
authors.forename: Richard
authors.initials: R
authors.lastname: Ricketts
authors.forename: Carlos R
authors.initials: CR
authors.lastname: Abramowsky
authors.forename: Charlotte Katherine
authors.initials: CK
authors.lastname: Steelman
authors.forename: Bahig M
authors.initials: BM
authors.lastname: Shehata
mesh_terms: D017809:Fatal Outcome; D005865:Gestational Age; D006548:Hernia, Diaphragmatic / Q000473:pathology* / Q000601:surgery; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D008099:Liver / Q000002:abnormalities* / Q000601:surgery; D008168:Lung / Q000002:abnormalities* / Q000601:surgery; D058502:Pentalogy of Cantrell / Q000473:pathology*; D047928:Premature Birth; D013908:Thoracotomy; D016896:Treatment Outcome