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Numb chin syndrome in sickle cell disease: a case series of Jamaican patients

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Abstract

Numb chin syndrome is an uncommon presentation that has been reported as secondary to metastatic disease, trauma, and infections of the maxilla, mandible, or oral cavity. The hypoesthesia, paraesthesia, or pain are a result of injury to the inferior alveolar nerve, which is particularly vulnerable as it exits the mandible through the mandibular foramen as the mental nerve. In persons with sickle cell disease, it has been reported as a manifestation of mandibular vaso-occlusive crisis. This case series presents 13 patients with sickle cell disease who presented with numb chin syndrome, the largest number of cases that has been described in the literature to date. The report illustrates the wide variety of presentations and therefore possible differential diagnoses to consider. In this case series, the symptoms were associated with vaso-occlusive crises, allergic reactions, dental infections, malignancy, rheumatoid arthritis, and pregnancy. Most appeared to be self-limiting; however, one patient was having his second episode, and the numbness has persisted in three patients. The series illustrates that it is important not only to ensure that the source of the local vaso-occlusive crisis is treated, but also to not miss important differentials such as metastatic disease, where this can be the first presentation of malignancy and would represent a very poor prognosis. There is no reported successful treatment for the hypoesthesia in this case series, and this presents an area for further research.

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Author information

Authors and Affiliations

  1. Caribbean Institute for Health Research, Sickle Cell Unit, The University of the West Indies, Mona Campus, Kingston 7, Jamaica

    Zachary Ramsay, Christine Hammond Gabbadon & Monika Asnani

Authors
  1. Zachary Ramsay
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  2. Christine Hammond Gabbadon
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  3. Monika Asnani
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Contributions

ZR was involved in conceptualization, conducted telephone interviews, and wrote the manuscript and ethics proposal. CHG was involved in conceptualization, conducted the medical record search, and wrote the manuscript and ethics proposal. MA was involved in conceptualization, wrote the manuscript and ethics proposal.

Corresponding author

Correspondence to Zachary Ramsay.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Consent to participate

The study received ethical approval from the University of the West Indies Ethics Committee for both medical records review without direct patient consent and subsequent telephone interview with patient consent. All patients are de-identified. Fully described cases, which were all available by telephone, were consented.

Consent to publication

The study received ethical approval from the University of the West Indies Ethics Committee for both medical records review without direct patient consent and subsequent telephone interview with patient consent. All patients are de-identified. Fully described cases, which were all available by telephone, were consented.

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Not applicable.

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Appendices

Appendix 1

Telephone questionnaire: Numb chin syndrome in sickle cell disease: a case series of Jamaican Patients

  1. 1.

    Do you recall a time when you suffered from numbness of the chin, cheek or mouth?

  2. 2.

    If yes continue to question 2. If no, you can end the questionnaire - thank you for your participation.

  3. 3.

    Do you recall what other symptoms were present at the time that the numbness started? Tick all that apply ___ Toothache (specify cause such as abscess or cavity) ___ Cancer (specify type) ___ Trauma ___ Sickle cell pain ____ Stroke ___ Other (specify)

  4. 4.

    Has the numbness resolved? And if so do you recall the duration of the numbness?

  5. 5.

    Do you recall anything making the numbness better or worse?

  6. 6.

    Have you developed any other illnesses not related to your sickle cell, since that incident? Tick all that apply ___ Diabetes ____ Cancer (specify type) ____ Stroke ____ Other (specify)

  7. 7.

    If the numbness resolved at that time, did it ever return since then?

Appendix 2

Table 2 Diagnostic pathway of numb chin syndrome based on clinical findings
Full size table

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Ramsay, Z., Gabbadon, C.H. & Asnani, M. Numb chin syndrome in sickle cell disease: a case series of Jamaican patients. Ann Hematol 100, 913–919 (2021). https://doi.org/10.1007/s00277-021-04423-0

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  • DOI: https://doi.org/10.1007/s00277-021-04423-0

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