Primary chronic osteomyelitis (PCO) is a rare condition that usually affects the mandible. Although most often occurring isolated, it can be part of various systemic and cutaneous syndromes. Takayasu arteritis (TAK) is an autoimmune disease that can co-exist with osteomyelitis, but has only been reported once in conjunction with PCO of the mandible. We present a case of a 27-year old female with PCO of the mandible which was found to have co-existing TAK. Doctors treating patients with PCO should be aware of the associated diseases and actively seek out their signs and symptoms.
Primary chronic osteomyelitis of the mandible is a rare condition which most often occurs in isolation.
It can be associated with various cutaneous and systemic diseases.
This case illustrates that primary chronic osteomyelitis of the mandible can co-exist with Takayasu arteritis.
Clinicians treating patients with primary chronic osteomyelitis should be aware of the associated disease entities.
Primary chronic osteomyelitis (PCO) of the mandible is a rare condition.
We present a 27-year old pregnant woman with PCO of the mandible found to have Takayasu arteritis (TAK). To this date, there are seven cases reported in the literature of patients suffering from osteomyelitis of non-craniofacial bones that also developed TAK, but only one patient with osteomyelitis of the mandible [ ]. Additionally, there is only one case report of a patient with osteomyelitis of the mandible during pregnancy [ ].
This case report will give the reader an overview of PCO of the mandible and the different syndromes and disease association.
Initial presentation and treatment at the maxillofacial surgery department
A 27-year old woman was referred to the Maxillofacial Surgery Department at Oslo University Hospital, Ullevål. The patient had no past medical history. She was 15 weeks pregnant, expecting her first child. The third molar in the left mandible had been surgically extracted four years prior because of pericoronitis after which there was a brief period of surgical wound dehiscence. The third molar in the left maxilla was extracted 11 months before presenting to the department. She reported having had diffuse pain in the left jaw area four months ahead of the removal of the tooth. The pain had gradually moved to the left hemi-mandible which had increased in size the last couple of months. Panoramic radiograph ( Fig. 1 ) and cone beam computer tomography done at her dentist showed cortical thickening and signs suggesting long-standing inflammation in the left hemi-mandible compatible with osteomyelitis.
On examination, the patient had a bony swelling at the body of the left hemi-mandible from the first pre-molar to the second molar. The swelling was tender to palpation, but there were no other obvious signs of infection such as redness, fistula or increased skin temperature. Thus, antibiotic treatment was not initiated. A left-sided submandibular lymph node was tender to palpation and slightly increased in size. She had hypoesthesia on the chin and lip on the left side as a sign of involvement of the inferior alveolar nerve. There were no findings on the intraoral examination.
The patient was closely followed up during her pregnancy, and as there was no clinical detriment, we decided to wait until after birth before considering surgical treatment. After uncomplicated birth, the patient underwent a CT scan which showed what appeared as a chronic diffuse sclerosing osteomyelitis from the first premolar to the ramus of the left hemi-mandible ( Fig. 2 ). It was decided to do a decortication because of the substantial swelling on the lower left side of the face.
The patient was admitted for operation 11 months after her first visit to the Department. On pre-operative examination, a bruit was noted on her left carotid artery. Laboratory samples showed anemia with hemoglobin 10.6 g/dL and increased inflammatory parameters with ESR of 73 mm/h [ ], C-reactive protein of 23 mg/L (<4), and leucocytes at 13 cells per mcL (3.5–10). An ultrasound showed slightly increased speed (100–110 cm/sec) in the common carotid arteries (CCA) and internal carotid arteries (ICA) on both sides. The left vertebral artery appeared smaller in diameter than the opposite side, but the speed was equal (65 cm/sec). CT angiography showed increased tissue density in the aortic arch wall, a narrow left CCA, and high-grade stenosis of the left subclavian artery ( Fig. 3 ). The stenosis of the subclavian artery continued to the left vertebral artery, that was narrowed in its whole length. A tentative radiological diagnosis was vasculitis of the aortic arch and major precerebral arteries most compatible with TAK.
The patient was operated the following day with buccal decortication from the first premolar to the ascending ramus of the left hemi-mandible. Intraoperatively, there were scarce amounts of trabecular bone and the bone marrow appeared less vascularized than normal. Histology showed chronic inflammation with massive apposition of cortical bone and scarce amounts of trabecular bone in which there was noted fibrosis and few plasma cells. There were no adverse complications.
The patient was transferred for follow-up of the vasculitis at the Department of Rheumatology.
Follow-up and treatment at the department of rheumatology
The patient had experienced unspecific symptoms of fatigue, general weakness, increased sweating, and increasing numbness in the left hand for three years. There were no definite symptoms of ischemia in the left arm. Her left-sided facial pain was regarded as atypical of carotidynia, but she had over time developed jaw claudication that improved during the pregnancy. Inflammatory markers were increased with ESR 107 mm/h [ ], and C-reactive protein at 57 mg/L (<4). She was anemic with hemoglobin 9.8 g/dL (11.7–15.3), interpreted as a combination of iron deficiency and anemia of chronic disease. On examination, there was a bruit over the left carotid arteries and a blood pressure difference of more than 20 mmHg as well as reduced pulse amplitude in the left radial artery. CT angiography was repeated three days after the initial one, which showed wall thickening of the aortic arch, the thoracic artery, and precerebral arteries. There was high-grade stenosis of the left subclavian and left vertebral artery and wall thickening of the left CCA. A final diagnosis of type IIb Takayasu arteritis was made, with the involvement of the descending thoracic aorta, the aortic arch and its branches [ ].
Treatment was initiated with intravenous methylprednisolone 750 mg once daily over three days followed by oral prednisolone 1 mg/kg daily and subcutaneous methotrexate 20 mg once weekly. The treatment resulted in rapid recovery of her symptoms and normalization of the inflammatory parameters. Three months later, prednisolone had been tapered down to 12.5 mg/daily. An 18F-labeled fluoro-2-deoxyglucose ( 18 F-FDG) positron emission tomography showed slight FDG in the vessel walls of the aortic arch, the brachiocephalic trunk, the left proximal common carotid, and the left subclavian artery ( Fig. 4 ). There were no signs of inflammation of the mandible. The radiological results corresponded with the finding of a consistent difference in the blood pressure of about 20 mmHg between the left and right upper arm.