Front Pediatr
. 2025 Mar 25:13:1497203.
doi: 10.3389/fped.2025.1497203. eCollection 2025. https://pubmed.ncbi.nlm.nih.gov/40201661/
Case Report: Hepatopulmonary fusion: to separate or not to separate? From a clinical case to A literature review
Marta Tedesco # 1 2, Simonetta Costa # 1 2, Pierpaolo Agresti 1 2, Francesca Priolo 1 2, Alessandro Perri 1 2, Annamaria Sbordone 1 2, Stefano Nobile 1 2, Filomena Valentina Paradiso 2 3, Maria Vittoria Stern 2 3, Riccardo Rizzo 2 3, Maria Cristina Giustiniani 2 4, Lorenzo Nanni 2 3, Giovanni Vento 1 2
Affiliations Expand
- PMID: 40201661
- PMCID: PMC11975665
- DOI: 10.3389/fped.2025.1497203
Abstract
Objective: Hepatopulmonary fusion (HPF) is a rare congenital malformation, frequently associated to right-sided congenital diaphragmatic hernia (CDHR). The presence of HPF often leads to a fatal outcome. The most effective approach to managing this condition remains uncertain due to the limited number of documented cases in the literature.
Study design: This case presents a 11-day old full-term female neonate with HPF associated to CDHR. The definitive diagnosis of HPF was made during surgery for CDHR. Our team opted for a simple repair of the diaphragmatic defect and no attempts were made to separate the liver from the right lung.
Results: Our approach was successful, as our patient not only survived the procedure but also showed favorable cardiorespiratory adaptation, consistent growth, and regular neurodevelopment, according to follow-up data, available at six months of life.
Conclusion: The adopted surgical management strongly suggests that when the diagnosis is made intraoperatively and detailed knowledge of the vascularization is lacking, partial separation of the viscera, preserving the medial hepatopulmonary fusion and suturing the diaphragm, is the successful approach.
Keywords: congenital diaphragmatic hernia; hepatopulmonary fusion; management; neonatal intensive care unit; right-sided congenital diaphragmatic hernia.
© 2025 Tedesco, Costa, Agresti, Priolo, Perri, Sbordone, Nobile, Paradiso, Stern, Rizzo, Giustiniani, Nanni and Vento.
