Anaesthetic challenges faced in a child with Morquio syndrome- An orphan disease

Abstract

: Morquio syndrome is a progressive lysosomal storage disorder leading to accumulation of glycosaminoglycans in the soft tissue, cartilage, and bone causing skeletal dysplasia. : We present a four-year-old child diagnosed with right side inguinal hernia posted for herniotomy. He had a large head with short neck and kyphoscoliosis. Pre-operative MRI whole spine shows a hypoplastic dens causing canal stenosis, indenting the cord at C1 level. General Anesthesia was planned. With a video laryngoscope on standy by, patient was induced after stabilizing the neck with a custom neck collar and the airway was secured with a Proseal Laryngeal Mask Airway.: The airway management can be challenging because of accumulation of mucopolysaccharides in the soft tissue of upper airway and limited mouth opening due to involvement of temporo mandibular joints. A thorough pre-operative evaluation of airway must be done and a well planned anaesthetic technique must be executed.