Abstract: Case: Alkaptonuria is a disorder of tyrosine metabolism having excretion of homogentisic acid in urine which gets dark when exposed to air. The disease is expressed as ochronosis of connective tissue with the significant involvement of large joints and spine. We present a case of a 56-year-old male having arthritis secondary to ochronosis of all large joints and bluish black discoloration of the bilateral sclera, skin of palm & earlobes. During surgery, black discoloration of cartilage, menisci, joint capsule, and tendons was revealed in bilateral hip and knee replacement.
Conclusion: Due to stiffness of tissue encountered during surgical and anaesthetic procedure, this case serves as a learning high point for procedural modifications and surgical pearls which ensure a smooth management of the patient.
