Anesthetic management of a case of alkaptonuria
Abstract
Alkaptonuria is a rare inherited autosomal recessive disorder of metabolism due to deficiency of homogentisic
acid oxidase. This results in deposition of homogentisic acid in almost all the collagenous structures of the
body leading to progressive multisystem involvement (alkaptonuric ochronosis) and poses a big challenge
in anesthetizing such patients. We present one such case posted for total hip replacement and its successful
management under general anesthesia.