Anesthetic Challenges in a Patient of Morquio Syndrome Associated with Acromegaly.

Abstract

Morquio syndrome is one of the rare storage disorders associated with excessive deposition of keratin sulfate and chondroitin-6-sulfate in bones, cartilages, heart valves, and cornea. Although most individuals with this syndrome appear normal at birth; skeletal abnormalities often develop within the first year of life. Restricted breathing, joint stiffness, and cardiac abnormalities are also common. The multisystem involvement in these patients poses unique anesthestic challenges and there is a paucity of literature regarding the anesthetic management of patients with this condition. We report a successful management of a rare case of 34 years old male with Morquio syndrome who presented an association of acromegaly and was planned for surgical resection of the tumor under general anesthesia. Such rare disorders primarily require thorough knowledge about the disease, its presentation and management strategies for a better outcome. Keeping in mind the multisystem involvement a proper teamwork and coordination of various specialities becomes an utmost importance.