Congenital hepatopulmonary fusion in right congenital diaphragmatic hernia: Surgical strategy and outcomes in a multicenter cohort

Aim: Congenital hepatopulmonary fusion (CHPF) is a rare anomaly associated with right congenital diaphragmatic hernia (RCDH), for which optimal diagnosis and surgical management remain undefined. This study aimed to explore the impact of surgical separation on outcomes. Methods: A retrospective multicenter study reviewed neonates with intraoperatively or radiologically confirmed CHPF associated with RCDH. Demographic data, imaging, surgical strategy, and outcomes were analyzed. Patients were stratified according to whether surgical separation of the fusion was performed (CHPF-S) or not (CHPF-NS). Results: Forty-three patients were included (26 CHPF-S and 17 CHPF-NS). Prenatal diagnosis of RCDH occurred in 20.9% of cases, while most diagnoses were made postnatally. Imaging findings suggestive of CHPF were identified preoperatively in a minority of patients, with computed tomography angiography providing additional information regarding vascular anatomy in selected cases. Baseline demographic characteristics, gestational age, birth weight, and CDH defect distribution were comparable between groups. The age at surgical repair differed, however not significantly, with earlier intervention in the CHPF-S group (9.0 +/- 15.8 vs 17.9 +/- 24.2 days, p = 0.099). Complete parenchymal fusion was more frequently observed in the CHPF-NS group, whereas partial fusion was more common in the CHPF-S group. Pulmonary hypertension occurred in 55.8% of patients and extracorporeal life support was required in 7.0%. Overall mortality was 27.9%, including 7 deaths in the CHPF-NS group and 5 in the CHPF -S group (41.2% vs 19.2%, p = 0.117), predominantly related to severe pulmonary hypertension. Conclusions: Surgical separation of the fusion may be selectively advisable in hemodynamically stable patients with a thin membrane or partial fusion, although non-separation often remains prudent. In critically ill neonates, patch repair or closure around the fused organs without separation represents a safer alternative. Early suspicion of CHPF in RCDH and thorough preoperative planning are essential to optimize outcomes.