Anaesthetic challenges in peri-operative management of williams syndrome

Abstract

Introduction: Williams syndrome is a rare microdeletion disorder associated with significant craniofacial and cardiac anomalies. The unique presentation of this syndrome and increased risk for sudden cardiac death poses a significant challenge to the anaesthesiologist to cater to varied requirements in airway and intraoperative management. The cardiac anomalies associated with this syndrome are complex and require prompt surgical correction. Case report: A 2 and half year old male child, a known case of william’s syndrome underwent surgery for correction of supravalvular aortic stenosis. He succumbed to sudden cardiac arrest in the immediate postop period. Discussion: Williams syndrome are associated with multifaceted cardiac defects with poor prognosis due to increased risk of sudden cardiac death and cardiac arrest resistant to normal resuscitation. This emphasises the need for careful and thorough perioperative care taking it to consideration the unique problems associated with this syndrome. Conclusion: A case of willams syndrome who underwent cardiac surgery is presented here with special emphasis on the challenges and distinct issues faced in the perioperative period