(64) The Management and Considerations of a Patient With Sickle Cell Disease in the Head and Neck With Two Case Reports of an Unusual Presentations

Abstract:

Background: Oral health and infections in sickle cell patients are concerning. We present two atypical cases of a crisis mimicking odontogenic infections, diagnosed as vaso-occlusive crises of the mandible. Both were managed non-surgically with medications and conservative therapy. This paper outlines management principles for sickle cell patients with mandibular abscess-like symptoms.

Clinical Case

Case 1:
A 28-year-old female with sickle cell disease (SCD) presented to the emergency department with hip, back, and chest pain consistent with a vaso-occlusive crisis. Additionally, she reported bilateral cheek swelling, trismus, headache, and numbness of the right mandible, lower lip, gingiva, and chin. Upon examination, the patient had significant pain in the posterior mandible, trismus, and hypoesthesia in the right posterior mandible and bilateral mental nerve distributions. CT and MRI imaging revealed fluid collections on both sides of the mandible and bone marrow edema indicative of acute bone infarction due to the vaso-occlusive crisis.

The patient was treated with IV antibiotics (Ceftriaxone, Metronidazole), dexamethasone, and pain management. Over the next 72 hours, she showed significant clinical improvement, with reduced facial swelling and restoration of sensation in the lower lip and chin. After 24 hours, her mouth opening increased, and by 48 hours, full sensation and function were restored, including normal excursive jaw movements. At 72 hours, the patient achieved a maximal incisal opening of 35 mm, and nearly complete resolution of hypoesthesia was observed. Conservative management led to a favorable outcome, avoiding the need for surgical intervention.

Case 2:
A 17-year-old male with sickle cell disease (SCD) presented with right-sided facial swelling, pain, limited mouth opening, and numbness of the lower lip and chin. He had a history of vaso-occlusive crises but no prior maxillofacial symptoms. On examination, his mouth opening was limited to 10 mm, and he had right facial swelling with no erythema. Neurological testing showed hypoesthesia of the right lower lip and chin. Laboratory results indicated mild anemia and elevated white blood cell count.

CT imaging showed inflammatory changes in the masticator, submandibular, and parapharyngeal spaces, with possible fluid collections around the right mandible. The temporomandibular joints appeared normal, and no dental pathology was seen. The patient was diagnosed with a vaso-occlusive crisis involving the maxillofacial complex and was treated with IV antibiotics, fluids, and corticosteroids.

After 72 hours, the patient showed significant improvement, with increased mouth opening (29 mm) and near resolution of facial swelling and numbness. He was discharged with antibiotics and continued to improve at follow-up, confirming the diagnosis of a vaso-occlusive crisis.

Conclusion: In patients that have sickle cell disease and signs and symptoms of an odontogenic infection, vaso-occlusive crisis should be considered. Effective treatment involves a thorough differential diagnosis and careful decision-making between surgical and non-surgical options. In both cases, antibiotics (IV Ceftriaxone, PO Metronidazole, or ampicillin-sulbactam), fluid management, dexamethasone, and physical therapy facilitated recovery without surgery.

Reference:
Chekroun M, Chérifi H, Fournier B, Gaultier F, Sitbon IY, Ferré FC, Gogly B. Oral manifestations of sickle cell disease. Br Dent J. 2019 Jan 11;226(1):27-31. doi: 10.1038/sj.bdj.2019.4. PMID: 30631169.
Kakkar, Mayank, et al. “Orofacial manifestation and dental management of Sickle Cell Disease: A Scoping Review.” Anemia, vol. 2021, 22 Oct. 2021, pp. 1–8, https://doi.org/10.1155/2021/5556708.
Souza S, de Carvalho H, Costa C, Thomaz E. Association of sickle cell haemoglobinopathies with dental and jaw bone abnormalities. Oral Dis. 2018 Apr;24(3):393-403. doi: 10.1111/odi.12742. Epub 2017 Sep 28. PMID: 28833998.
These are 2 case reports (n=2) of a rare phenomenon occurring where the presenting signs and symptoms appear to be a mandibular odontogenic abscess. We are hoping to bring awareness and recommendations to these types of patients. We also have additional images to include however there is not enough room for upload on the image upload sectionOrthopantomogram on initial admissionT2-weighted MRI Images for Case 1: Abnormal ill-defined hyperintense signals in the mandibleCT Maxillofacial soft tissue views of patient in Case 2