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Hepatopulmonary Fusion in Right Congenital Diaphragmatic Hernia: Can Conservative Approach Win?
Silviana Ribeiro 1, Joana Brandão 1, Inês Teixeira 2, José Miguel Campos 1, Leonor Carmo 1
1 Department of Pediatric Surgery, Centro Hospitalar Universitário de São João, Porto, Portugal
2 Unidade Local de Saúde de Gaia e Espinho
Introduction:
Hepatopulmonary fusion (HPF) is a rare malformation associated with right-sided congenital diaphragmatic hernia (CDH). Preoperative diagnosis is uncommon and often made intraoperatively. The optimal management strategy remains controversial.
Case Presentation:
We present the case of a term newborn from a twin pregnancy, with a prenatal diagnosis of right-sided CDH, poor prognostic indicators, and congenital heart disease. The patient was intubated at 3 minutes of life and placed on conventional mechanical ventilation with good initial response. The prenatal findings were confirmed postnatally, and further imaging studies were conducted.
On day of life (DOL) 2, clinical deterioration necessitated escalation of ventilatory support, with consideration for ECMO, which was ultimately not feasible. At DOL 9, exploratory laparotomy revealed a large diaphragmatic defect with extensive HPF and no identifiable surgical dissection plane. Surgical repair was aborted, and further anatomical characterization was pursued. Angio-CT demonstrated features consistent with HPF and multiple vascular shunts.
Given the high mortality associated with extensive HPF and vascular anomalies, a multidisciplinary team opted for conservative, non-surgical management. The patient showed progressive clinical improvement, was extubated on DOL 50, and discharged on DOL 110 on 2 L/min nasal oxygen and nighttime BIPAP. At three years of age, the child remains off ventilatory support and shows gradual psychomotor development.
Conclusion:
HPF associated with right-sided CDH carries a high perioperative mortality (approximately 40%). The extent of fused parenchyma and associated vascular anomalies are emerging as critical prognostic markers. Hemodynamic compromise due to altered vascular dynamics may contribute to poor post-operative outcomes, highlighting the potential role for conservative management in selected cases. This case supports the feasibility of non-surgical treatment with favourable long-term outcomes. Long-term follow-up is essential to monitor vascular dynamics and consider interventions such as embolization if necessary.