Fontan operation for the Cantrell syndrome using a clamshell incision

Area of Science:

• Pediatric Cardiac Surgery
• Congenital Heart Disease
• Cardiovascular Surgery

Background:

• Ectopia cordis presents significant surgical challenges, especially when combined with complex congenital cardiac anomalies.
• Standard median sternotomy can be difficult and carry increased risks in these pediatric patients.

Observation:

• A patient with ectopia cordis, a functionally single ventricle, and bilateral superior vena cava was identified.
• The complexity of the cardiac anatomy precluded a standard sternotomy approach.

Findings:

• A staged Fontan procedure was successfully performed using a combined clamshell incision and sternothoracotomy approach.
• This alternative surgical strategy facilitated access and management of the complex anatomy.

Implications:

• The clamshell incision and sternothoracotomy approach provides a feasible alternative for complex pediatric cardiac cases.
• This technique may improve outcomes for children with ectopia cordis and associated anomalies undergoing staged palliation.