Airway management of a child with mucopolysaccharidosis undergoing cervical spine surgery: A case report.

Abstract

"Mucopolysaccharidosis" (MPS) is a rare, autosomal recessive lysosomal storage disease characterized by deficiencies in 11 different lysosomal enzymes involved in the metabolism of glycosaminoglycans (GAGs) leading to its accumulation, the condition which results in anatomic abnormalities and multi-organ dysfunction that increases the risk of anesthesia complications. The patterns of accumulation form the basis of MPS classification into seven types of progressive diseases. Most of the MPS types have facial and oral characteristics that increase the risk of airway management. We are reporting a case of MPS with a challenging airway, an 11-years-old boy diagnosed with MPS type VI with a prominent facial character planned for cervical spine fixation versus decompression, successfully managed with fiberoptic bronchoscopy (FOB) guided by video laryngoscopy (VL).