Anesthetic management of a case of alkaptonuria

  • Pratheeba. N Department of anesthesiology, Pondicherry Institute Of Medical Sciences , Pondicherry
  • Neha Singh Department of anesthesiology, Pondicherry Institute Of Medical Sciences , Pondicherry
  • Ranjan R. V Department of anesthesiology, Pondicherry Institute Of Medical Sciences , Pondicherry
  • Ramachandran . Department of anesthesiology, Pondicherry Institute Of Medical Sciences , Pondicherry
  • Sageev Koshy George Department of anesthesiology, Pondicherry Institute Of Medical Sciences , Pondicherry
  • Ramya GS Department of anesthesiology, Pondicherry Institute Of Medical Sciences , Pondicherry
Keywords: Alkaptonuria, Ochronosis, Homogentisic acid oxidase

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.

Published
01-29-2019
Issue
Vol.17 No.2(2013): May-August
Section
Case Reports