Anaesthetic considerations of adults with Morquio's syndrome - a case report

Abstract

Background: The anaesthetic management of patients with Morquio syndrome is complicated by a number of factors including odontoid hypoplasia, atlantoaxial instability, thoracic kyphosis, and deposition of mucopolysaccharides in the soft tissue of the oropharnyx.

Case presentation: Herein we describe the anaesthetic considerations and management of a 26 year old adult with Morquio syndrome, who presented for an elective hip replacement.

Conclusion: This report details an awake fiberoptic intubation in an adult with Morquio syndrome. We recommend that this approach be considered in patients with Morquio syndrome undergoing general anaesthesia.

Figure 1

Lateral cervical spine x-ray demonstrating loss of normal cervical lordosis, anterior beaking of the cervical bodies and hypoplasia of odontoid peg. The atlanto axial space on flexion is 3 mm, the upper limit of normal.

Figure 2

MRI cervical spine saggital section, with saturation band, anterior beaking of the cervical vertebrae and syrinx between C3 and C7 is noted. Arrows indicate odontoid peg and syrinx.