Anaesthetic Management for Patient of Mucopolysaccharidosis Undergoing Bilateral Functional Endoscopic Sinus Surgery: A Case Report

Mucopolysaccharidosis (MPS) represent rare, group of inherited lysosomal storage disease characterised by deficiencies in specific lysosomal enzymes involved in the metabolism of glycosaminoglycans. These conditions present significant anesthetic challenges due to progressive airway obstruction, cardiovascular complications, and skeletal abnormalities. Hereby, the authors present the case of a 12-year-old female patient with MPS who underwent bilateral Functional Endoscopic Sinus Surgery (FESS) and was successfully managed under general anaesthesia. The patient presented with characteristic features including short stature, short hands, receding mandible, short neck, corneal clouding, and mouth breathing. Her surgical history included two previous FESS procedures, the first 10 years ago and the second five years ago, both performed under general anaesthesia. She also had a history of recurrent convulsions, managed with antiepileptic therapy. Preoperative assessment revealed stable vital signs with a blood pressure of 95/60 mmHg. Anaesthetic management included premedication followed by induction, complicated by a difficult airway. Despite challenging mask ventilation, endotracheal intubation was successfully achieved. Maintenance of anaesthesia with sevoflurane provided stable intraoperative conditions. Surgery proceeded without complications, and the patient had a favourable postoperative recovery. The present case highlights the importance of thorough preoperative assessment, careful anaesthetic planning, and preparedness for airway management challenges in MPS patients undergoing Ear, Nose and Throat (ENT) procedures.