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

Abstract

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.