Alkaptonuria with extensive ochronotic degeneration of the Achilles tendon and its surgical treatment: a case report and literature review.

Achilles tendon alkaptonuria homogentisic acid ochronosis rupture

Journal

Asian biomedicine : research, reviews and news
ISSN: 1875-855X
Titre abrégé: Asian Biomed (Res Rev News)
Pays: Thailand
ID NLM: 101465147

Informations de publication

Date de publication:
Jun 2021
Historique:
medline: 30 6 2021
pubmed: 30 6 2021
entrez: 8 8 2023
Statut: epublish

Résumé

Alkaptonuria is a rare genetic metabolic disorder due to deficiency of homogentisate 1,2-dioxygenase (HGD), an enzyme catalyzing the conversion of homogentisate to 4-maleylacetoacetate in the pathway for the catabolism of phenylalanine and tyrosine. HGD deficiency results in accumulation of homogentisic acid and its pigmented polymer. Ochronosis is a bluish-black discoloration due to the deposition of the polymer in collagenous tissues. Extensive ochronotic involvement of the Achilles tendon in alkaptonuria and its surgical treatment is rarely reported. A 43-year-old man presented to our clinic in March 2019 with sudden onset of left Achilles tendon pain with no history of prior trauma. Surgical exploration revealed a complete disruption of the tendon at its attachment to the calcaneus. Black pigmentation was extensive and reached the calcaneal tuberosity, extending about 7 cm from the insertion. Achilles reconstruction was performed using flexor hallucis longus tendon transfer. The patient experienced uncomplicated healing with satisfactory functional results. Orthopedic surgeons should be aware of the progressive nature of alkaptonuria. Extensive degenerative changes of the ruptured tendon should be suspected so that physicians can plan tendon repair and facilitate prompt surgical intervention.

Sections du résumé

Background UNASSIGNED
Alkaptonuria is a rare genetic metabolic disorder due to deficiency of homogentisate 1,2-dioxygenase (HGD), an enzyme catalyzing the conversion of homogentisate to 4-maleylacetoacetate in the pathway for the catabolism of phenylalanine and tyrosine. HGD deficiency results in accumulation of homogentisic acid and its pigmented polymer. Ochronosis is a bluish-black discoloration due to the deposition of the polymer in collagenous tissues. Extensive ochronotic involvement of the Achilles tendon in alkaptonuria and its surgical treatment is rarely reported.
Case report UNASSIGNED
A 43-year-old man presented to our clinic in March 2019 with sudden onset of left Achilles tendon pain with no history of prior trauma. Surgical exploration revealed a complete disruption of the tendon at its attachment to the calcaneus. Black pigmentation was extensive and reached the calcaneal tuberosity, extending about 7 cm from the insertion.
Discussion UNASSIGNED
Achilles reconstruction was performed using flexor hallucis longus tendon transfer. The patient experienced uncomplicated healing with satisfactory functional results.
Conclusion UNASSIGNED
Orthopedic surgeons should be aware of the progressive nature of alkaptonuria. Extensive degenerative changes of the ruptured tendon should be suspected so that physicians can plan tendon repair and facilitate prompt surgical intervention.

Identifiants

pubmed: 37551372
doi: 10.2478/abm-2021-0016
pii: abm-2021-0016
pmc: PMC10388780
doi:

Types de publication

Journal Article

Langues

eng

Pagination

129-136

Informations de copyright

© 2021 Nesrin Mwafi et al., published by Sciendo.

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Auteurs

Nesrin Mwafi (N)

Department of Biochemistry and Molecular Biology, Faculty of Medicine, Mutah University, Alkarak 61710, Jordan.

Ali Alasmar (A)

Department of Urology, Prince Hussein Urology Center, Jordanian Royal Medical Services, Amman 11855, Jordan.

Monther Al-Momani (M)

Department of Radiology, Jordanian Royal Medical Services, Amman 11855, Jordan.

Sattam Alazaydeh (S)

Department of Orthopedics and Trauma, Jordanian Royal Medical Services, Amman 11855, Jordan.

Omar Alajoulin (O)

Department of Orthopedics and Trauma, Jordanian Royal Medical Services, Amman 11855, Jordan.

Mohammad Alsalem (M)

Department of Anatomy and Histology, School of Medicine, The University of Jordan, Amman 11942, Jordan.

Heba Kalbouneh (H)

Department of Anatomy and Histology, School of Medicine, The University of Jordan, Amman 11942, Jordan.

Classifications MeSH