A Case of Xanthoma of Bilateral Tendoachilles Reconstructed with Fascia Lata Graft X

Aims and background: Cerebrotendinous xanthoma is an autosomal recessive disorder characterized by a lack of production of primary bile acids and accumulation of cholestanol in various tissues like the brain, spinal cord, peripheral nerves, eyes, and tendon in the form of xanthomas. This is a case r...

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Main Authors: S Jaikish, Balaji Sambandam
Format: Article
Language:English
Published: Jaypee Brothers Medical Publisher 2024-01-01
Series:Journal of Orthopedics and Joint Surgery
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Online Access:https://www.jojs.in/doi/JOJS/pdf/10.5005/jojs-10079-1124
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author S Jaikish
Balaji Sambandam
author_facet S Jaikish
Balaji Sambandam
author_sort S Jaikish
collection DOAJ
description Aims and background: Cerebrotendinous xanthoma is an autosomal recessive disorder characterized by a lack of production of primary bile acids and accumulation of cholestanol in various tissues like the brain, spinal cord, peripheral nerves, eyes, and tendon in the form of xanthomas. This is a case report of one such patient who had xanthoma of both tendoachilles. Case description: A 30-year-old female patient had swelling in both legs. Gradually, it became painful while walking. On examination, she had a swelling of size 10 by 4 cm attached to tendoachilles. Magnetic resonance imaging (MRI) showed features of xanthoma. Staged surgery was done. The mass was fleshy and pale yellow and replaced the entire tendon. The entire mass was removed, creating a defect of about 20 cm. Fascia lata of size 20 by 6 cm was harvested and made into a fourstrand graft. Proximally, it was sutured with fiber wires to the distal part of the muscle. Distally, it was fixed to the calcaneum using a 5 mm suture anchor. Flexor hallucis longus (FHL) tendon was used to augment the reconstruction. At 1 year, she was asymptomatic and gained good strength and ROM in the ankle. Conclusion and clinical significance: Cerebrotendinous xanthoma causes impairment in joint mobility and ambulation. Deposition of cholestanol in the neurons causes atrophy and secondary demyelination. Simple excision of tendoachilles xanthoma will do more harm to the patient who is more prone to neurological and mobility-related issues. Fascia lata graft has the advantage of limited donor site morbidity and adequate length.
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spelling doaj-art-331f6be21258498eb5fa8466ee6ccab62025-02-07T11:17:47ZengJaypee Brothers Medical PublisherJournal of Orthopedics and Joint Surgery2582-78632024-01-0161798110.5005/jojs-10079-112415A Case of Xanthoma of Bilateral Tendoachilles Reconstructed with Fascia Lata Graft XS Jaikish0Balaji Sambandam1Department of Orthopaedics, Atlas Hospitals, Trichy, Tamil Nadu, IndiaBalaji Sambandam, Department of Orthopaedics, Atlas Hospitals, Trichy, Tamil Nadu, India, Phone: +91 8754853535Aims and background: Cerebrotendinous xanthoma is an autosomal recessive disorder characterized by a lack of production of primary bile acids and accumulation of cholestanol in various tissues like the brain, spinal cord, peripheral nerves, eyes, and tendon in the form of xanthomas. This is a case report of one such patient who had xanthoma of both tendoachilles. Case description: A 30-year-old female patient had swelling in both legs. Gradually, it became painful while walking. On examination, she had a swelling of size 10 by 4 cm attached to tendoachilles. Magnetic resonance imaging (MRI) showed features of xanthoma. Staged surgery was done. The mass was fleshy and pale yellow and replaced the entire tendon. The entire mass was removed, creating a defect of about 20 cm. Fascia lata of size 20 by 6 cm was harvested and made into a fourstrand graft. Proximally, it was sutured with fiber wires to the distal part of the muscle. Distally, it was fixed to the calcaneum using a 5 mm suture anchor. Flexor hallucis longus (FHL) tendon was used to augment the reconstruction. At 1 year, she was asymptomatic and gained good strength and ROM in the ankle. Conclusion and clinical significance: Cerebrotendinous xanthoma causes impairment in joint mobility and ambulation. Deposition of cholestanol in the neurons causes atrophy and secondary demyelination. Simple excision of tendoachilles xanthoma will do more harm to the patient who is more prone to neurological and mobility-related issues. Fascia lata graft has the advantage of limited donor site morbidity and adequate length.https://www.jojs.in/doi/JOJS/pdf/10.5005/jojs-10079-1124case reportcerebrotendinous xanthomatosisfascia latatendoachillesxanthoma
spellingShingle S Jaikish
Balaji Sambandam
A Case of Xanthoma of Bilateral Tendoachilles Reconstructed with Fascia Lata Graft X
Journal of Orthopedics and Joint Surgery
case report
cerebrotendinous xanthomatosis
fascia lata
tendoachilles
xanthoma
title A Case of Xanthoma of Bilateral Tendoachilles Reconstructed with Fascia Lata Graft X
title_full A Case of Xanthoma of Bilateral Tendoachilles Reconstructed with Fascia Lata Graft X
title_fullStr A Case of Xanthoma of Bilateral Tendoachilles Reconstructed with Fascia Lata Graft X
title_full_unstemmed A Case of Xanthoma of Bilateral Tendoachilles Reconstructed with Fascia Lata Graft X
title_short A Case of Xanthoma of Bilateral Tendoachilles Reconstructed with Fascia Lata Graft X
title_sort case of xanthoma of bilateral tendoachilles reconstructed with fascia lata graft x
topic case report
cerebrotendinous xanthomatosis
fascia lata
tendoachilles
xanthoma
url https://www.jojs.in/doi/JOJS/pdf/10.5005/jojs-10079-1124
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