Unusual case of unrepaired omphalocoele and uncorrected tetralogy of Fallot in a young adult

Alsaif HS, Venkatesh KS, Chai P, Cheah WK, Teo LSL
Correspondence: Dr Lynette Li San Teo, lynette_ls_teo@nuhs.edu.sg

ABSTRACT
An omphalocoele is a congenital defect that affects the development of the abdominal wall in the umbilical region, resulting in a hernial-type sac of variable size. The condition is usually diagnosed prenatally and corrected in early infancy to prevent rupture of the covering membranes, which carries a high mortality and morbidity rate. Tetralogy of Fallot is the most common cyanotic congenital heart defect during infancy that is associated with this condition. Most patients experience cyanosis at birth and die in childhood if there is no surgical intervention. Overall, it is uncommon for untreated patients with both omphalocoele and tetralogy of Fallot to survive into adulthood. We report the rare case of a 17-year-old young adult with untreated omphalocoele and uncorrected tetralogy of Fallot.

Keywords: omphalocoele, tetralogy of Fallot, young adult
Singapore Med J 2011; 52(10): e213-e216