Bilateral Hip Arthroplasty for Ochronotic Arthropathy

Abstract

Ochronosis is a musculoskeletal manifestation of alkaptonuria, a rare hereditary metabolic disorder characterised by the absence of the enzyme homogentisic acid oxidase and associated with various systemic abnormalities related to the deposition of homogentisic acid pigment (ochronotic pigment). In this report, we describe a 53-year-old, HLA-B27(+) woman with ochronotic arthropathy. In addition to the typical clinical features of the disorder, she had bilateral hip involvement, which was improved by cementless total hip prosthesis.

Arthroplasty in Ochronosis “Tips and Pearls in Surgery”: Case Series

“…Ochronopathic arthropathy is a rare, developed in alkaptonuria patients as a result of deposition of polymerized homogentisic acid pigment in the cartilages and synovial tissue [7][8][9]. Life-table analyses show that, patients start to have joint replacement operations at a mean age of 55 years, which is comparable with our patients as they were 56 and 52 years old respectively [3].…”

“…This is also proven with one of our cases; normal bone healing was observed following internal fixation of proximal femoral fracture with sliding-screw plate system. And also bone-in-growth is not affected and cement less prosthesis can be used in eligible cases [8,11,14]. We used cement less femoral stems and acetabular components in one of our cases and we didn't encounter any stabilization and early loosening problems.…”

Co‐existence of Pott’s disease and alkaptonuria in a 21‐month‐old child

“…The only clinical sign in this age is the darkening of urine. Polymerization and auto‐oxidation of HGA into pigment is irreversible and this pigment results in degeneration of cartilage, particularly that of the spine and large joints (hips and knees) (2). As the disease progresses, postural deformities occur.…”

A case of ochronotic arthropathy treated with total hip arthroplasty

“…The total hip arthroplasties reported elsewhere were also successful. [11][12][13][14][15][16][17][18][19][20][21][22][23] For operations such as total hip arthroplasty, two complications of alcaptonuria are important. One is a tendency to form urinary tract stones from HGA product, which predisposes the patient to urinary tract infection.…”