Complex management of ectopia cordis complicated by pentalogy of cantrell: Report of two cases and review of current evidence

Int J Surg Case Rep. 2025 Jun;131:111353. doi: 10.1016/j.ijscr.2025.111353. Epub 2025 Apr 23.

ABSTRACT

INTRODUCTION AND IMPORTANCE: Ectopia cordis is a rare congenital anomaly characterized by the abnormal positioning of the heart outside the thoracic cavity, frequently associated with additional congenital defects such as those seen in Pentalogy of Cantrell. Due to the rarity and severity of these anomalies, timely diagnosis and appropriate management remain clinically challenging but critical for patient outcomes.

CASE PRESENTATION: We report two distinct cases of ectopia cordis managed at our institution. The first case presented with complete ectopia cordis and absence of the sternum, requiring initial management with a Bogota bag followed by placement of bovine pericardium; unfortunately, the patient succumbed to complications of sepsis and heart failure at 12 days of life. The second case involved partial ectopia cordis accompanied by features of Pentalogy of Cantrell, managed conservatively with a Bogota bag and spontaneous epithelialization, resulting in successful discharge with stable clinical status.

CLINICAL DISCUSSION: Management of ectopia cordis, especially when complicated by associated anomalies, presents significant surgical and clinical challenges. Survival rates remain low due to complications such as sepsis, cardiac instability, and limited thoracic cavity space. Utilization of biomaterials like bovine pericardium has shown promise in providing necessary cardiac protection when primary closure is not feasible. Early multidisciplinary intervention, involving neonatologists, pediatric cardiologists, and surgeons, is crucial for optimizing outcomes.

CONCLUSION: While prognosis for ectopia cordis remains poor, careful and individualized management strategies, including timely intervention and infection prevention, can lead to favorable outcomes as demonstrated in our second case. This underscores the importance of a multidisciplinary approach and early prenatal diagnosis in enhancing survival rates for this rare condition.

PMID:40286694 | PMC:PMC12059706 | DOI:10.1016/j.ijscr.2025.111353