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A group of patients with Marfan’s syndrome, who have finger and toe contractures, displays tendons’ alterations upon an ultrasound examination: are these features common among classical Marfan patients?

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Abstract

The involvement of the musculoskeletal system with other mild pleiotropic manifestations represents a clinical criterion, called “systemic features,” to d iagnose Marfan’s syndrome. We aimed to investigate the features of the hands and feet redressable contractures present in a group of Marfan patients. In 13 patients with previously diagnosed Marfan’s syndrome, an accurate clinical examination was performed. In particular the characterization of the musculoskeletal system by visual analogic scale to measure muscle pain (VAS) and muscle strength (MRC system) was carried out; the Beighton scale score was used to evaluate the articular hypermobility. Ultrasound examination (US) was performed to detect deep-superficial flexor tendons and extensor tendons of both hands, and the short and long flexor and extensor tendons of the fingers and toes in static and dynamic positions. The ImageJ program was adopted to measure a profile of tendon echo-intensity. A reduction of the thickness of all tendons was detected by US in our patients; the VAS and Beighton scale scores were in normal ranges. The profile of tendon echo-intensity showed different textural details in all Marfan patients. This study provides evidence for other contractures’ localization, and for altered findings of the tendons in patients with Marfan syndrome and finger/toe contractures. These changes may be associated with structural modifications in connective tissue.

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Acknowledgments

We would like to thank Dr. Maurizio Abbate for his cooperation regarding the program ImageJ management.

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Authors and Affiliations

  1. Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, VialePieraccini, 18, 50139, Florence, Italy

    Daniela Melchiorre & Marco Matucci-Cerinic

  2. Agenzia Recupero e Riabilitazione, Careggi Hospital, University of Florence, Florence, Italy

    Elisa Pratelli

  3. Department of Heart and Vessels, Regional Marfan Syndrome and Related Disorders Center, Careggi Hospital, Florence, Italy

    Elisa Pratelli, Elena Torricelli, Francesco Sofi, Rosanna Abbate, GianFranco Gensini & Guglielmina Pepe

  4. Section of Critical Medical Care and Medical Specialities, Department of Experimental and Clinical Medicine, DENOTHE Center, University of Florence, Florence, Italy

    Francesco Sofi, Rosanna Abbate, GianFranco Gensini & Guglielmina Pepe

  5. Maria Agli Ulivi Center, Fondazione Don Carlo Gnocchi, Onlus, IRCCS, Florence, Italy

    GianFranco Gensini

Authors
  1. Daniela Melchiorre
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  2. Elisa Pratelli
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  3. Elena Torricelli
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  4. Francesco Sofi
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  5. Rosanna Abbate
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  6. Marco Matucci-Cerinic
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  7. GianFranco Gensini
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  8. Guglielmina Pepe
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Correspondence to Daniela Melchiorre.

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The authors declare there are no conflicts of interest. No financial support or other benefits from commercial sources were received for the present work. None of the authors has financial interests which could create a potential conflict of interest or the appearance of a conflict of interest with regard to the work.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1984 Helsinki Declaration and its later amendments or comparable ethical standard.

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Melchiorre, D., Pratelli, E., Torricelli, E. et al. A group of patients with Marfan’s syndrome, who have finger and toe contractures, displays tendons’ alterations upon an ultrasound examination: are these features common among classical Marfan patients?. Intern Emerg Med 11, 703–711 (2016). https://doi.org/10.1007/s11739-016-1399-5

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  • DOI: https://doi.org/10.1007/s11739-016-1399-5

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