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Acute painful crisis in adults with sickle cell disease

BMJ 2024; 386 doi: (Published 01 July 2024) Cite this as: BMJ 2024;386:e075099

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“Orofacial Pain Crisis”- A Frequently Overlooked Aspect of Sickle Cell Anemia

Dear Editor

Charles et al. provide valuable insights on acute painful crisis in sickle cell disease (SCD). (1) Nevertheless, as dentists from India, an Asian country mapped as an SCD "hotspot", we emphasize the importance of recognizing orofacial pain as part of this acute painful crisis. According to literature, nearly 49% of SCD patients suffer with orofacial pain. (2) Nonspecific generalized pain affecting various areas of maxilla and mandible, including teeth (‘sickle cell toothache’) and gingiva, has been reported. (3) This pain is linked to multiple episodes of vaso-occlusive events affecting the Oro-maxillofacial structures. The risk of developing jaw pain is nine times higher in SCD than normal-individuals. (4) This characteristic painful crisis is part of a larger spectrum of orofacial conditions, including pulpal necrosis (68%) and headaches (77%). (5) Clinically healthy but necrotic teeth occur 8.25 times more frequently in SCD due to occlusions of pulpal microvasculature. (6) Further, pulpal calcifications and external root resorption have been commonly observed in SCD, with higher frequency of changes in size, shape, periapex and root in sickle cell trait. (6,7) Other complications include mandibular neuropathy, temporomandibular joint arthritis, fibrous ankylosis, etc.

Several oral manifestations of SCD, although not pathognomonic of the disease, pose a diagnostic challenge that is crucial for successful management. This includes mucosal pallor, enamel/dentin mineralization disorders, changes in superficial cells of the tongue, multiple caries, and periodontal disease, which can lead to odontogenic abscesses. (3,5) A painful mental neuropathy (‘numb chin syndrome’) caused by vaso-occlusive SCD has been reported, particularly in the mandible, due to its relatively lower blood flow compared to other bones, eventually leading to permanent neuropathies, if left untreated. (8,9) Osteomyelitis of the jaws, secondary to SCD, although rare, has been reported in the mandible. An incidence of 3–5% of osteonecrosis without any dental origin was observed in the mandible, particularly the posterior regions, due to the single homolateral vascularization through the inferior alveolar artery and the periosteal vascularization. (10) Decrease in jaw bone densities and a coarse trabecular pattern described as 'ladder shape’ have been reported, owing to erythroblastic hyperplasia due to vaso-occlusion and compensatory bone expansion. (11,12) Hence, it is highly recommended to include maxillofacial imaging, such as, orthopantomograms/lateral cephalograms, as part of routine examinations for patients with SCD which aids in timely management and helps prevent tooth loss.

Further, dental professionals should consider acute painful crisis in the differential diagnosis of orofacial pain, particularly in patients with a history of SCD. As individuals with hemoglobinopathies may neglect their oral health due to coping with associated serious consequences, treatment of these oral complications must be tailored to their systemic condition and specific needs to improve their quality of life. (10)

1. Charles, K.S., Friday, M., & Rochford, E. (2024). Acute painful crisis in adults with sickle cell disease. BMJ, 386, e075099.
2. Caracas, Mda S., Jales, S.P., Jales, N.L.H., da Silva, C.J.C., Suganuma, L.M., Fonseca, G.H., Gualandro, S.F., & de Siqueira, J.T. (2013) Temporomandibular joint arthritis in sickle cell disease: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol, 115(2), e31-5.
3. Ali. R., Oxlade, C., & Borkowska, E. (2008). Sickle cell toothache. Br Dent J, 205(10), 524.
4. Cox, G.M. (1984). A study of oral pain experience in sickle cell patients. Oral Surg Oral Med Oral Pathol, 58(1), 39-41.
5. O’Rourke, C.A., & Hawley, G.M. (1998). Sickle cell disorder and orofacial pain in Jamaican patients. Br Dent J, 185(2), 90-2.
6. Costa, C.P., Thomaz, E.B., & Souza, Sde F. (2013). Association between sickle cell anemia and pulp necrosis. J Endod, 39(2), 177–8.
7. Souza, S., de Carvalho, H., Costa, C., & Thomaz, E. (2018). Association of sickle cell haemoglobinopathies with dental and jaw bone abnormalities. Oral Dis, 24(3), 393-403.
8. Friedlander, A.H., Genser, L., & Swerdloff, M. (1980). Mental nerve neuropathy: a complication of sickle-cell crisis. Oral Surg, 49(1), 15–7.
9. Chekroun, M., Chérifi, H., Fournier, B., Gaultier, F., Sitbon, I.Y., Ferré, F.C., & Gogly, B. (2019). Oral manifestations of sickle cell disease. Br Dent J, 226(1), 27–31.
10. Mulimani, P., Ballas, S.K., Abas, A.B., & Karanth, L. (2019). Treatment of dental complications in sickle cell disease. Cochrane Database Syst Rev, 12(12), CD011633.
11. Neves, F.S., de Almeida, D.A., Oliveira-Santos, C., dos Santos, J.N., Toralles, M.B., da Silva, M.C., Campos, M.I., & Crusoé-Rebello, I. (2011). Radiographic changes of the jaws in HbSS and HbSC genotypes of sickle cell disease. Spec Care Dentist, 31(4), 129 –33.
12. Costa, C.P., de Carvalho, H.L., Thomaz, E.B., & Sousa, Sde F. (2012). Craniofacial bone abnormalities and malocclusion in individuals with sickle cell anemia: a critical review of the literature. Rev Bras Hematol Hemoter, 34(1), 60-63.

Competing interests: No competing interests

07 July 2024
Divya Vinayachandran
Associate Professor in Oral Medicine and Radiology
Vivek Narayanan (Dean ,Professor and Head in Oral and Maxillofacial Surgery, SRM Kattankulathur Dental College and Hospital), Saravana Karthikeyan Balasubramanian (Professor in Conservative Dentistry and Endodontics, SRM Dental College, Bharathi Salai, Ramapuram), Sekar Mahalaxmi (Professor and Head in Conservative Dentistry and Endodontics, SRM Dental College, Bharathi Salai, Ramapuram)
SRM Kattankulathur Dental College and Hospital
SRM Institute of Science and Technology, Chennai, India