Arasu J Arul Durai, Prasanna A Arul
Department of Orthopaedics, JJ Arul Hospital, Dindigul, Tamil Nadu, India.
J Orthop Case Rep. 2022 Nov;12(11):23-27. doi: 10.13107/jocr.2022.v12.i11.3400.
Ochronosis is a rare metabolic disorder, characterized by accumulation of homogentisic acid in the connective tissues due to the lack of the enzyme homogentisic acid oxidase. The musculoskeletal manifestation of alkaptonuria is black pigmentation of cartilage of the knee and the hip leading to arthropathy.
In this article, we report three patients with involvement of hip, knee, and spine, but with more severe involvement of the hips. Bilateral hip arthroplasty was done in one out of the three patients.
Being a rare disorder and often missed, the functional outcome of hip arthroplasty in these patients is similar to primary osteoarthritis. The importance lies in correct diagnosis and anticipation of intraoperative difficulties.
褐黄病是一种罕见的代谢紊乱疾病,其特征是由于缺乏尿黑酸氧化酶,导致尿黑酸在结缔组织中蓄积。尿黑酸尿症的肌肉骨骼表现为膝关节和髋关节软骨的黑色色素沉着,进而导致关节病。
在本文中,我们报告了3例累及髋关节、膝关节和脊柱的患者,但髋关节受累更为严重。3例患者中有1例行双侧髋关节置换术。
作为一种罕见且常被漏诊的疾病,这些患者髋关节置换术的功能结果与原发性骨关节炎相似。重要的是要做出正确诊断并预见术中困难。