Video 1_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.mp4

Background<p>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.</p>Case description<p>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.