Successful epidural anesthesia in a patient of Hurler syndrome for hernia repair

Abstract

Mucopolysaccharidosis is a rare inherited lysosomal storage disease characterized by increasing deposition of unmetabolized glucosaminoglycans. The surgical procedures under general anesthesia in these patients is associated with high mortality due to difficulty in airway management .The regional anesthesia is useful alternative; however, previous case reports describing epidural/spinal anesthesia have reported failure of this technique. We describe a case of mucopolysaccharidosis type I (Hurler syndrome) for repair of umbilical and inguinal hernia repair conducted under epidural anesthesia with sedation, obviating the need of intubation.

Key words: Mucopolysaccharidosis; Hurler syndrome; Epidural anesthesia; Hernia repair

Citation: Kerai S, Sehrawat L. Successful epidural anesthesia in a patient of Hurler syndrome for hernia repair. Anaesth Pain & Intensive Care 2016;20(3):350-352

Received: 17 April 2016; Reviewed: 20 June 2016; Accepted: 20 June 2016