Abstract
INTRODUCTION: Mucopolysaccharidoses (MPSs) are a group of heredity storage diseases, transmitted in an autosomal recessive manner, associated with the accumulation of glycosaminoglycans (GAGs) in various tissues and organs. The concerned patients have multiple concomitant hereditary anomalies. Considering the craniofacial abnormality in these patients, airway management may be difficult for anesthesiologists. In these patients, preanesthetic assessment is necessary and performed with the accurate assessment of airways, consisting of the physical exam and radiography, MRI or CT of head and neck. An anesthesiologist should set up a "difficult intubation set" with a flexible fiber-optic bronchoscope and also, it may be necessary to discuss with an ear-nose and throat (ENT) specialist if required, for unpredicted emergency situations. CASE PRESENTATION: In this case-report we presented a 2-year-old boy with known MPSs with psychomotor retardation, bilateral corneal opacities, impaired hearing and vision, inguinal hernia, severe mitral regurgitation, micrognathia, coarse facial feature, stiff and short neck and restricted mouth opening. He scheduled for left inguinal hernia repair surgery. DISCUSSION: The patient's difficult airway was managed successfully and the anesthesia of his surgical procedure had an uneventful course.