Numb Chin Syndrome in Sickle Cell Disease: A Systematic Review and Recommendations for Investigation and Management
Abstract
:1. Introduction
2. Materials and Methods
3. Results
4. Discussion
4.1. Pathophysiology
4.2. Differential Diagnosis
4.3. Imaging
4.4. Treatment
4.5. Recommendations
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
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Patient Characteristics (n = 33) | ||
---|---|---|
Age, years (mean) (SD) | 28.3 (11.7) | |
Sex, n (%) | Male | 17 (51.5) |
Female | 16 (48.5) | |
Genotype, n (%) | SS | 13 (39.4) * |
SC | 5 (15.2) * | |
Sβ-thalassemia | 3 (9.1) * | |
Data unavailable | 12 (36.4) * | |
Comorbidities +, n (%) | None | 27 (82) |
Diabetes | 1 (3) | |
Rheumatoid arthritis | 1 (3) | |
Asthma | 1 (3) | |
Membranoproliferative glomerulonephritis | 1 (3) | |
Splenectomy | 1 (3) | |
Retinal detachment | 1 (3) | |
Malignancy | 1 (3) | |
Numb chin presentation and management | ||
Location, n (%) | Unilateral | 18 (54.5) |
Bilateral | 10 (30.3) | |
Unclear | 5 (15.2) | |
Resolution of symptoms, n (%) | Yes | 11 (33.3) |
No | 10 (30.3) | |
Data unavailable | 12 (36.4) | |
Acute medical condition +, n (%) | None | 1 (3) |
Veno-occlusive crisis/Acute chest syndrome | 32 (97) | |
Osteomyelitis | 1 (3) | |
Infection | 5 (15) | |
Multiorgan failure | 1 (3) | |
Osteonecrosis of the mandible | 1 (3) | |
Treatment +, n (%) | Standard treatment of VOC | 15 (45) |
Treatment for infection | 2 (6) | |
Transfusion | 2 (6) | |
No treatment | 1 (3) | |
Data unavailable | 14 (42) | |
Geographical distribution, n (%) | Jamaica | 13 (39.4) |
United States of America | 7 (21.2) | |
Ghana | 5 (15.2) | |
France | 3 (9.1) | |
India | 2 (6.1) | |
England | 2 (6.1) | |
Turkey | 1 (3) | |
Duration | Min: 0.03 months Max: no resolution after 14 years |
Minimal Work-Up | |
---|---|
Clinical | Detailed history (trauma, infection, malignancy, autoimmune disease, VOC) General physical exam Detailed neurological exam Oral cavity examination by dentist or ENT |
Biological | Complete blood count Creatinine Pregnancy test |
Imaging | Xray of mandible or CT scan |
Further diagnostic tests to consider | |
Biological | Diabetes screening (Blood glucose or fructosamine/protein ratio) * Plasma protein electrophoresis with immunofixation, free light chains, cryoglobulin Serological tests for auto-immune diseases (ANA, ENA, Ro, La) Syphilis, HIV, HSV, Lyme testing Lumbar puncture (protein, cytology, flow cytometry) |
Imaging | CT scan MRI PET scan |
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Bedrouni, M.; Touma, L.; Sauvé, C.; Botez, S.; Soulières, D.; Forté, S. Numb Chin Syndrome in Sickle Cell Disease: A Systematic Review and Recommendations for Investigation and Management. Diagnostics 2022, 12, 2933. https://doi.org/10.3390/diagnostics12122933
Bedrouni M, Touma L, Sauvé C, Botez S, Soulières D, Forté S. Numb Chin Syndrome in Sickle Cell Disease: A Systematic Review and Recommendations for Investigation and Management. Diagnostics. 2022; 12(12):2933. https://doi.org/10.3390/diagnostics12122933
Chicago/Turabian StyleBedrouni, Mahdi, Lahoud Touma, Caroline Sauvé, Stephan Botez, Denis Soulières, and Stéphanie Forté. 2022. "Numb Chin Syndrome in Sickle Cell Disease: A Systematic Review and Recommendations for Investigation and Management" Diagnostics 12, no. 12: 2933. https://doi.org/10.3390/diagnostics12122933