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Hand surgeons and amyloidosis specialists warning: transthyretin-associated amyloidosis with bifid median nerve as a cause of bilateral carpal tunnel syndrome. A case report and literature review

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Abstract

Introduction

Approximately 75% of patients with carpal tunnel syndrome (CTS) are diagnosed as idiopathic. Despite this, the presence of an underlying cause such as an anatomical variant or a systemic disease must always be suspected, especially in cases of bilateral presentation without an obvious cause, recurrence or complications. The anatomical variant known as the bifid median nerve (BMN) is a very rare abnormality that can occasionally lead to CTS. On the other hands, transthyretin-associated amyloidosis (ATTR) is one of the possible causes of bilateral CTS. We report a case where these two very rare pathologies converge as the cause of bilateral CTS and a review of the literature.

Case report

We report a 71-year-old male with prior history of lumbar canal stenosis, bilateral trigger finger, rupture of the supraspinatus muscle tendon and of the long portion of the right biceps brachial. He also had 8-year-old bilateral CTS that recurred after CTS surgery. He was surgically re-intervened and was diagnosed incidentally with BMN and an ultrasound of the other hands also showed BMN. Because of all the prior musculoskeletal history, a biopsy of the transverse carpal ligament was taken showing ATTR deposits that led to the diagnosis of cardiac ATTR wild type.

Conclusions

This case highlights the natural history of the multiple musculoskeletal manifestations related to ATTR and the importance of performing intraoperative biopsies in patients with CTS surgery as this can lead to early diagnosis of cardiac ATTR.

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Authors and Affiliations

  1. Department of Orthopaedic Surgery and Traumatology, Bellvitge University Hospital, L’Hospitalet de Llobregat, Barcelona, Spain

    Andreu Triguero, Silvia López-Marne & Alfred Llop

  2. Bellvitge Biomedical Research Institute (IDIBELL), L’Hospitalet de Llobregat, Barcelona, Spain

    Andreu Triguero, José González-Costello, Silvia López-Marne, Alfred Llop, Maria Pané & Sergi Yun

  3. Advanced Heart Failure and Heart Transplant Unit, Department of Cardiology, Bellvitge University Hospital, L’Hospitalet de Llobregat, Barcelona, Spain

    José González-Costello

  4. Department of Pathology, Bellvitge University Hospital, L’Hospitalet de Llobregat, Barcelona, Spain

    Maria Pané

  5. Community Heart Failure Program, Departments of Cardiology and Internal Medicine, Bellvitge University Hospital, L’Hospitalet de Llobregat, Barcelona, Spain

    Sergi Yun

  6. Department of Internal Medicine, Bellvitge University Hospital, L’Hospitalet de Llobregat, Barcelona, Spain

    Sergi Yun

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  1. Andreu Triguero
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  2. José González-Costello
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  3. Silvia López-Marne
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Correspondence to Sergi Yun.

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Triguero, A., González-Costello, J., López-Marne, S. et al. Hand surgeons and amyloidosis specialists warning: transthyretin-associated amyloidosis with bifid median nerve as a cause of bilateral carpal tunnel syndrome. A case report and literature review. Eur J Orthop Surg Traumatol 32, 575–581 (2022). https://doi.org/10.1007/s00590-021-03004-1

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