Hepatopulmonary Fusion: A Rare Variant of Congenital Diaphragmatic Hernia
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In brief
In brief
This registry study of 10 infants with hepatopulmonary fusion—a rare complication of right-sided congenital diaphragmatic hernia—demonstrates that partial surgical separation of fused liver and lung tissue yields better outcomes than complete separation. Complete separation resulted in catastrophic complications due to underlying vascular anomalies.
Written by the GCMD Library team from the article.
Background
Hepatopulmonary fusion (HPF), a rare anomaly associated with right congenital diaphragmatic hernia (CDH), is characterized by a fibrovascular fusion between herniated liver and lung parenchyma. We aimed to clarify patient characteristics, management strategies, and outcomes in HPF.
Methods
Data on infants with HPF were obtained from the Congenital Diaphragmatic Hernia Registry (CDHR). Patient characteristics, management, and outcomes were compared with the results of a literature review.
Results
Ten cases of HPF were identified in the CDHR. Five patients survived. The median estimated gestational age was 38 weeks (range 36–40). Median birth weight was 2.7 kg (range 2.0–3.8 kg), but non-survivors had a lower median birth weight (2.3 kg vs. 3.5 kg). All patients had at least 1 congenital anomaly in addition to CDH. Operative approach varied, but most surgeons performed only partial separation of the liver and lung (n = 6). The 2 patients who underwent complete separation both ultimately died, 1 due to significant postoperative complications and 1 due to severe pulmonary hypertension with multiple vascular anomalies.
Conclusion
Partial separation of liver and lung appears to be the wisest surgical approach in HPF, as complete separation has resulted in catastrophic complications due to frequent underlying vascular anomalies.
Level of evidence
IV.