Hepatopulmonary fusion: A rare variant of congenital diaphragmatic hernia

Abstract

BackgroundHepatopulmonary 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. MethodsData 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. ResultsTen 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. ConclusionPartial 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 evidenceIV.