Anesthetic considerations in Morquio syndrome: a case report

ABSTRACT

Anesthetic challenges in Morquio syndrome include the respiratory problems due to restrictive defect in the thoracic cage, upper airway obstruction during head flexion, atlantoaxial instability and compression of the cervical spinal cord due to hypoplasia of the dens, complicating intubation. After pre-anesthetic check up and informed written consent, the patient was premedicated with glycopyrrolate 0.08 mg and fentanyl 10 mcg. Induction was done with inhalational anesthetic agent sevoflurane along with Oxygen [O[2]] and Nitrous oxide [N[2]O] maintaining the spontaneous respiration. Intubation was done with Flexo metallic tube [FMT] no. 24 through right nostril avoiding forceful movements at atlantoaxial joint and cervical spine. Maintenance and recovery was uneventful. Paramount in the anaesthetic care of such patients is a thorough preoperative evaluation of airway in addition to cardiac, respiratory, neurological function. Inhalational induction technique may be useful in difficult intubation