Arthroscopic ankle arthrodesis in two alkaptonuria patients.
BMJ Case Rep
; 16(10)2023 Oct 25.
Article
em En
| MEDLINE
| ID: mdl-37880174
ABSTRACT
Alkaptonuria is a very rare disorder in which homogentisic acid accumulates due to a deficiency in the activity of homogentisic acid 1,2 dioxygenase. This deficiency results in deposition of a yellowish-brown pigment in connective tissue. Such deposition is termed 'ochronosis' and leads to deterioration in the formation and structure of proteoglycans in hyaline cartilage. These actions lead to fragmentation and rapid destructive arthritis. Often, ochronotic arthritis appears at 40-60 years of age, and many patients are treated symptomatically. Here, we report two patients (three ankles) with ochronotic arthritis who were treated with ankle arthrodesis. In all cases, the postoperative clinical score improved, but the time needed for fusion was prolonged and symptomatic subtalar arthropathy developed in the early postoperative period.
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Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Osteoartrite
/
Doenças das Cartilagens
/
Alcaptonúria
Limite:
Humans
Idioma:
En
Ano de publicação:
2023
Tipo de documento:
Article