Anesthetic management of a case of alkaptonuria

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