Anaesthetic Management of Fracture Neck of Femur in a Case of Hurler’s Syndrome – A Case Report

Abstract

Hurler’s syndrome is a type of mucopolysaccharidoses, a rare lysosomal storage disorder with a prevalence of 1 in 100,000 individuals, results from deficient α-L-iduronidase enzyme activity, leading to the accumulation of glycosaminoglycans within lysosomes. This condition presents unique challenges to the attending anaesthesiologist as it manifests with profound anomalies, including severe airway and cervical spine manifestation. Difficult intubation is reported in 54% of cases, with a 23% incidence of failed intubation, highlighting the critical need for specialized preoperative care.
We describe a case of a 20-year-old male diagnosed with Hurler’s syndrome since birth, presenting with a fracture of the neck of femur. Given the risks associated with manipulation of the airway, the patient underwent CC-screw fixation under regional anaesthesia. The anaesthetic plan focused on avoiding airway manipulation and optimizing perioperative conditions under the guidance of a multidisciplinary team, including experienced anaesthesiologists.