Taylor’s Approach to Rescue: A Case of Undiagnosed Alkaptonuria Undergoing Bilateral Total Knee Replacement

Bhimala Naga Ramya,Sujil Sudhersan,Sivakumar Segaran

doi:10.4038/slja.v29i2.8743

Abstract

Alkaptonuria is a rare inherited autosomal recessive metabolic disorder due to the deficiency of homogentisic acid oxidase resulting in accumulation of homogentisic acid in collagen and cartilaginous tissue resulting in stiffening of joints, spine and involving other systems which pose challenges to anaesthesiologist in providing anaesthesia. Here we present a patient with undiagnosed alkaptonuria who was posted for bilateral total knee replacement. Since these patients usually have reduced intervertebral spaces, neuraxial anaesthesia was difficult with classic approach. Taylor’s approach was used for neuraxial anaesthesia and, post-operative analgesia was achieved with continuous femoral nerve block with catheter placement.

Highlights

Alkaptonuria is a rare autosomal recessive inherited metabolic disorder where the enzyme homogentisic acid oxidase is deficient which is needed for tyrosine metabolism
It is characterized by the triad of homogentisic aciduria, ochronosis, and arthritis.[1,2]
We present a case of alkaptonuria posted for total knee replacement for whom classical neuraxial technique failed, so Taylor’s approach was used as a rescue technique and post-operative analgesia was achieved by continuous femoral nerve block

Summary

Introduction

Alkaptonuria is a rare autosomal recessive inherited metabolic disorder where the enzyme homogentisic acid oxidase is deficient which is needed for tyrosine metabolism It is characterized by the triad of homogentisic aciduria, ochronosis, and arthritis.[1,2] Its reported incidence is about 1 in 2,50,000 to 1 in 10,00,000.3 The clinical manifestations are due to the accumulation of an ochre coloured homogentisic acid in the cartilages and connective tissues.[2] We present a case of alkaptonuria posted for total knee replacement for whom classical neuraxial technique failed, so Taylor’s approach was used as a rescue technique and post-operative analgesia was achieved by continuous femoral nerve block. After 4 days, patient was again posted for right knee total replacement which was successfully managed with planned Taylor’s approach and continuous femoral nerve block.

Airway Respiratory system Cardiovascular system

Preoperative pulmonary function testing

Conclusion