Apert syndrome: Anaesthetic concerns and challenges

Abstract

Apert syndrome is a congenital autosomal dominant disease characterized by brachycephaly, craniosynotosis, midface hypoplasia, hypertelorism, choanal stenosis, multidigit hand and foot syndactyly. Anaesthetic management of a child with Apert syndrome poses a great challenge to anaesthesiologist. The child should be evaluated thoroughly by history, examination and investigations for associated anamolies and managed accordingly. There is no definite recommendation for general or regional anaesthesia and also there are no known contraindications to specific anaesthetic agent and drug. However, whenever possible, regional anaesthesia should be preferred as the incidence of obstructive sleep apnoea is high in these patients. If general anaesthesia is planned, difficult airway cart should be kept ready. Temperature monitoring should be done intraoperatively and the patient should be monitored postoperatively for signs of airway obstruction.

Keywords

• Apert syndrome
• Anaesthesia
• Airway problem