A Case Study Examining the Current Evidence and Controversies in the Management of High-Risk Neonates With Malrotation

Introduction

Heterotaxy syndrome (HS) is a loss of the normal position of abdominal and thoracic organs and can be associated with severe cardiac disease and high mortality rates. Frequently associated intestinal malrotation further increases complications. Because of the high morbidity and mortality in this population, ongoing controversies exist regarding appropriate screening and management of these neonates.

Case

A neonate born with HS and cardiac disease underwent complex cardiac surgery on first day of life. He is later found to have malrotation with midgut volvulus and undergoes Ladd procedure with intestinal detorsion. He subsequently presents with small bowel obstruction necessitating a second abdominal surgical intervention.

Conclusion

Although surgery is necessary here, this case demonstrates why current controversy exists regarding initial screening and subsequent offering of prophylactic Ladd procedure in asymptomatic neonates with HS because of the risk of challenging complications.