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Eur J Pediatr Surg 2022; 32(06): 477-496
DOI: 10.1055/s-0042-1749213
Review Article

Congenital Hepatopulmonary Fusion

Gustavo M. D. Rocha 
1   Department of Neonatology, Centro Hospitalar Universitário de São João, Porto, Portugal
› Institutsangaben Funding None.
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Abstract

Congenital hepatopulmonary fusion (HPF) is a rare anomaly characterized by a fusion between the liver and lung parenchyma. HPF cases have been scarcely reported in the literature. An extensive search of publications was performed in the PubMed and Google Scholar databases without a time limit. In total, 34 clinical case reports were found in the literature, and a study by the Congenital Diaphragmatic Hernia (CDH) Study Group reported data on 10 patients with HPF. Of these 44 infants, 20 were male, 20 were female, and four were reported without gender specification. Nineteen (43.2%) patients required intubation on the first day of life. Six (13.6%) patients had their clinical presentation during the first year of life, and four (9%) clinically presented with HPF between 2.5 and 11 years of age. In these patients, cough, asthma-like symptoms, dyspnea, hemoptysis, right-side chest pain, respiratory infections, and pneumonia were the relevant clinical signs. Right-lung vascular anomalies were present in 18 (40.9%) patients. A complete liver and lung separation was successful in 17 (38.6%) patients. The overall survival was 56.8%. Congenital HPF has no gender predominance. In most cases, it behaves similar to a right CDH in need of resuscitation and intubation after birth. The majority of the cases are discovered during the surgical procedure for CDH. The best surgical approach has not been established and depends on the degree of fusion and vascular anomalies. An advanced imaging assessment is necessary before a surgical approach is attempted. The prognosis is ominous.

Keywords

case series - congenital diaphragmatic hernia - hepatopulmonary fusion - liver - lungs - diaphragm - neonate

Note

This review gathers information on the clinical presentation, association with other congenital anomalies, diagnosis, clinical evolution, treatment, and prognosis of congenital hepatopulmonary fusion. It is of added value for clinicians, since it allows them to provide correct information to parents and to make decisions.


Abbreviations

AD – autosomal dominant


AR – autosomal recessive


CDH – congenital diaphragmatic hernia


CNS – central nervous system


CT – computed tomography


D – day of life


ECMO – extracorporeal membrane oxygenation


GA – gestational age


HPF – hepatopulmonary fusion


IVC – inferior vena cava


MR – magnetic resonance


NICU – neonatal intensive care unit


VACTERL – vertebral, anal, cardiac, oesophageal, renal/kidney, and limb defects


VSD – ventricular septal defect




Publikationsverlauf

Eingereicht: 30. November 2021

Angenommen: 11. Februar 2022

Artikel online veröffentlicht:
26. August 2022

© 2022. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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