Perioperative management of infected skin defect with poor wound healing in a neonate with Pentalogy of Cantrell associated with single ventricle and ectopia cordis: a case report and literature review.

BACKGROUND

Complete Pentalogy of Cantrell (POC) with ectopia cordis, full-thickness skin defects, and functionally univentricular physiology is an extremely rare congenital malformation with limited reported management experience.

CASE DESCRIPTION: We report a male infant with complete POC, including ectopia cordis, a 4 × 4 cm skin defect, and complex congenital heart disease (single ventricle, tricuspid atresia, double-outlet right ventricle, and pulmonary artery hypoplasia) associated with lower sternal defect and omphalocele. Diagnostic workup confirmed the diagnosis according to the Toyama classification; genetic analysis identified no pathogenic variants related to the phenotype. A multidisciplinary staged strategy was adopted. Following targeted antimicrobial therapy for an early local infection, postoperative incision dehiscence resulted in recurrent cardiac exposure. Rather than immediate reoperation, the team elected a conservative approach involving condition-adapted local wound management guided by wound bed preparation principles, alongside optimization of cardiorespiratory function and nutrition.

RESULTS

The exposed cardiac surface achieved complete epithelialization, obviating the need for short-term secondary skin grafting and stabilizing the patient for future staged interventions.

CONCLUSION

This case illustrates that an individualized, multidisciplinary staged approach-including meticulous local wound care-may facilitate favorable outcomes in selected patients with this life-threatening condition.