Non-Isolated Congenital Diaphragmatic Hernia. Can We Think of a Beacon of Hope?

Abstract

Background: The TOTAL Trial demonstrated the efficacy and safety of prenatal treatment of isolated severe left Congenital Diaphragmatic Hernia (CDH). Since this trial was completed, the application of the fetal approach in selected non-isolated CDH cases has become a daily clinical and ethical reality, raising the question of extending the indication to fetoscopic endotracheal occlusion (FETO) to selected non-isolated cases where no guidance is available.

Method: This study examines the ethical and clinical implications of offering FETO for non-isolated Congenital Diaphragmatic Hernia (CDH). It analyzes current ethical frameworks in fetal surgery and draws comparisons with similar debates concerning patients with genetic conditions and/or neurological impairment that necessitate major procedures, such as organ transplantation. We revised the literature, including data from extensive CDH registries, to assess survival rates and the variability of associated anomalies. A multidisciplinary, patient-centered decision-making framework was developed to guide clinical considerations.

Results: Recent data suggest that survival outcomes in some syndromic CDH cases may be comparable to those of isolated CDH, challenging the rationale for a priori exclusion from fetal therapy. Ethical analysis highlights the need for individualized assessments rather than categorical restrictions, emphasizing the principles of beneficence, non-maleficence, autonomy, and justice. Decision-making should extend beyond survival rates to consider long-term quality of life and parental values.

Conclusions: The absence of definitive evidence should not lead to the denial of potential benefits when a reasonable chance of improved outcomes exists. Instead, a multidisciplinary, patient- and family-centered approach should guide fetal therapy decisions, ensuring ethical integrity while adapting to the evolving landscape of prenatal medicine.