Single-stage combined cardiac and abdominal wall reconstruction in Pentalogy of Cantrell using a cemented sandwich mesh technique: a case report.
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BACKGROUND: Pentalogy of Cantrell (POC) is a rare congenital malformation defined by five midline defects involving the sternum, anterior diaphragm, diaphragmatic pericardium, abdominal wall, and heart. Surgical strategies vary between staged and single-stage approaches, and optimal timing remains debated. We report a case of complete POC managed with a single-stage combined cardiac and abdominal wall reconstruction at six months of age, using a cemented sandwich mesh, previously used by our team after sternal tumor resection, for the lower sternal defect. CASE PRESENTATION: A six-month-old Saudi male infant, born at 38 weeks by emergency cesarean section (birth weight 1.99 kg, Apgar 8/9), was diagnosed antenatally with ectopia cordis, ventricular septal defect (VSD), and epigastric omphalocele. Amniocentesis had excluded trisomy. Postnatal echocardiography confirmed a large perimembranous VSD with inlet extension, a mid-muscular VSD, a small atrial septal defect (ASD), and an ejection fraction of 57%. A multidisciplinary team elected to proceed with a single-stage combined operation once his weight exceeded 5 kg (6.2 kg at surgery). Cardiac surgery was performed first through sternotomy (bypass time 150 min; cross-clamp time 124 min), with patch repair of the VSD and ASD and excision of a left ventricular diverticulum. The pediatric surgery team then addressed the omphalocele via midline laparotomy extension, closed the anterior diaphragmatic defect primarily, and reconstructed the lower sternal defect using gentamicin-impregnated bone cement within doubled Gore-Tex mesh (a sandwich mesh configuration). Fascial closure reached the umbilicus without clinical signs of raised intra-abdominal pressure. The patient was extubated on postoperative day three and achieved full oral feeds by day five. Echocardiography before discharge showed no residual shunt and an ejection fraction of 54%. A small incisional hernia was repaired electively about one year later, and the child remained asymptomatic at two years. CONCLUSIONS: This case demonstrates that single-stage combined cardiac and abdominal wall repair in complete POC is feasible and can reduce the burden of repeated surgical interventions. The cemented sandwich mesh achieved stable reconstruction of the lower sternal defect in this patient, offering a potential alternative for centers managing similar complex congenital anomalies.