Anaesthetic Approach to Hurler Syndrome

Aim: We aimed to evaluate the anesthetic approach to a 6 years old female child, clinically diagnosed as Hurler syndrome, undergoing umbilical hernia repair and adenoidectomy operation, under the light of literatüre data. Case Report: Premedication was omitted. The case monitorised with EKG, non invasive arterial pressure, peripheric oxygen saturation (SpO2) and taken under operation. It is assumed to be a difficult airway case bacause of anatomical features of Hurler Syndrome, tracheostomy and difficult airway algorithm prepared. Preoxygenation via face mask with 3L/min 100% O2 inhalation applied to the case before induction. Anesthesia induced with increasing sevoflurane concentrations in 100% oxygen and fentanyl 1µg/kg. Neuromuscular blockage was maintained with 1.5 mg/kg iv succinylcholine. 18 mm diameter endotracheal tube was placed into trachea by using Macintosh blade (No:2) at third attempt. Anesthesia was maintained with 2% sevofluran in 50% O2-50% N2O. The patient was extubated without any complication at the end of the surgery. Conclusion: In Hurler’s sydrome; patients should be evaluated carrefully prior to operation. It is vital to monitor the patient in peroperative and postoperative period.