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Oral Surgery Procedures in a Patient with Hajdu-Cheney Syndrome Treated with Denosumab—A Rare Case Report

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Listed:
  • Magdalena Kaczoruk-Wieremczuk

    (Department of Oral Surgery, Medical University of Gdańsk, 7 Dębinki Street, 80-211 Gdańsk, Poland
    Co-first author, these authors contributed equally to this work.)

  • Paulina Adamska

    (Department of Oral Surgery, Medical University of Gdańsk, 7 Dębinki Street, 80-211 Gdańsk, Poland
    Co-first author, these authors contributed equally to this work.)

  • Łukasz Jan Adamski

    (Department of Oral Surgery, Medical University of Gdańsk, 7 Dębinki Street, 80-211 Gdańsk, Poland)

  • Piotr Wychowański

    (Department of Oral Surgery, Medical University of Warsaw, 6 St. Binieckiego Street, 02-097 Warsaw, Poland)

  • Barbara Alicja Jereczek-Fossa

    (Department of Oncology and Hemato-Oncology, University of Milan, 7 Festa del Perdono Street, 20-112 Milan, Italy
    Division of Radiotherapy, IEO European Institute of Oncology, IRCCS, 435 Ripamonti Street, 20-141 Milan, Italy)

  • Anna Starzyńska

    (Department of Oral Surgery, Medical University of Gdańsk, 7 Dębinki Street, 80-211 Gdańsk, Poland)

Abstract

Background: Hajdu-Cheney syndrome (HCS) is a very rare autosomal-dominant congenital disease associated with mutations in the NOTCH2 gene. This disorder affects the connective tissue and is characterized by severe bone resorption. Hajdu-Cheney syndrome most frequently affects the head and feet bones (acroosteolysis). Case report: We present an extremely rare case of a 34-year-old male with Hajdu-Cheney syndrome. The patient was admitted to the Department of Oral Surgery, Medical University of Gdańsk, in order to perform the extraction of three teeth. These teeth were not eligible for conservative treatment and prosthetic reconstruction. The patient was treated with denosumab (angiogenesis and receptor activator of nuclear factor-κB RANK ligand inhibitor, RANKL). Discussion: Denosumab is a monoclonal antibody against RANKL. This drug works through a suppression of osteoclast activity. In cases of patients in which the pathway of the RANK/RANKL/osteoprotegerin is dysregulated, denosumab has been approved for the treatment off-label. In patients receiving denosumab, a delayed wound healing in the oral cavity and osteonecrosis may occur. Dental procedures involving the alveolar bone process (tooth extractions and bone alveoloplasty) may be a risk factor for medication-related osteonecrosis of the jaw (MRONJ). Spontaneous osteonecrosis is rarely observed. MRONJ consists of the destruction of exposed bone, with the exposure persisting for a minimum of 6–8 weeks. This is the first article about an HCS patient treated with denosumab who underwent invasive oral surgery procedures. This case report highlights the difficulties for professionals occurring during the oral surgery procedures in such patients.

Suggested Citation

  • Magdalena Kaczoruk-Wieremczuk & Paulina Adamska & Łukasz Jan Adamski & Piotr Wychowański & Barbara Alicja Jereczek-Fossa & Anna Starzyńska, 2021. "Oral Surgery Procedures in a Patient with Hajdu-Cheney Syndrome Treated with Denosumab—A Rare Case Report," IJERPH, MDPI, vol. 18(17), pages 1-10, August.
    • Handle: RePEc:gam:jijerp:v:18:y:2021:i:17:p:9099-:d:624323
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    References listed on IDEAS

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    1. Giuseppina Campisi & Rodolfo Mauceri & Francesco Bertoldo & Giordana Bettini & Matteo Biasotto & Giuseppe Colella & Ugo Consolo & Olga Di Fede & Gianfranco Favia & Vittorio Fusco & Mario Gabriele & An, 2020. "Medication-Related Osteonecrosis of Jaws (MRONJ) Prevention and Diagnosis: Italian Consensus Update 2020," IJERPH, MDPI, vol. 17(16), pages 1-15, August.
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