RESULTS : The infant with DGS was successfully anesthetised with no perioperative complications

Abstract:
Introduction: Di George syndrome (DGS) is a genetic disorder with multisystemic involvement characterised by craniofacial, cardiac anamolies, parathyroid and immune system dysfunction. The estimated prevalence is 1 in 4000 live births yet there are few case reports available describing the perioperative management in children presenting for various surgeries.

Case Report: In this case report we discuss the perioperative management of a five month old child diagnosed as Di George syndrome (DGS) with concurrent problems such as anticipated difficult airway, congenital heart disease, aspiration pneumonia secondary to uvulopalatal insufficiency, gastroesophageal reflux and laryngomalacia undergoing gastric fundoplication and gastrostomy.

Conclusion: DGS is associated with highly variable clinical manifestations. Anaesthesiologists should focus on possibilities of difficult intubation due to facial anomalies. Optimum pre-operative cardiac evaluation in view of complex congenital anomalies is very essential. There is a need to exercise extreme caution in handling aspiration pneumonia occurring from uvulopalateal insufficiency and gastro-oesophageal reflux disease. Key words: Di George syndrome, aspiration pneumonia, craniofacial anomalies and laryngomalacia.