Takayasu Arteritis Presenting with Kidney Infarction, Cranial Nerve Palsy, and Osteomyelitis

Background Takayasu arteritis (TA) is a large-vessel vasculitis that primarily affects the aorta and its branches, increasing the risk of vascular complications like aneurysms and ischemic events. In the settings of aortic root involvement, aortic valve replacement can be prompted. However, the presence of a prosthetic valve poses risks of thromboembolism, infective endocarditis, and hemolytic anemia. The diagnosis and management of these conditions in the settings of coexisting TA can be challenging and prompt a multidisciplinary approach.

Case Report We present a case report of an African American female in her mid-30s with Takayasu arteritis, a prior Bentall procedure with mechanical aortic valve replacement, and coronary artery aneurysm bypass surgery. She presented with acute right-sided flank pain, right submandibular pain, and dysphagia. A month prior, she saw her primary care physician for a sore throat and right submandibular swelling with difficulty swallowing. She received several courses of antibiotics, with some improvement on levofloxacin. An ENT specialist noted paralysis of the right soft palate and right-sided tongue deviation. A head and neck CT scan was ordered, however, the patient presented to the ED before it could be performed. In the ED, she was diagnosed with right kidney infarction and an aneurysm of the right external carotid artery (Figure 1), causing partial paralysis of cranial nerve XII. Additional findings included C6-C7 osteomyelitis/discitis and ischemic infarctions in the left temporal and occipital lobes. Blood cultures grew Enterococcus faecalis, raising suspicion for septic emboli, prompting further evaluation for infectious endocarditis and mycotic aneurysm. Transthoracic echocardiography and transesophageal echocardiography performed at the primary facility did not reveal signs of infective endocarditis. However, given the high suspicion for mechanical valve endocarditis, further evaluation was pursued. The patient was transferred to a tertiary center, where reoperation of the prior aortic valve replacement revealed a perivalvular abscess. Intraoperative cultures grew Enterococcus faecalis. The carotid artery aneurysm was successfully repaired, leading to improvement in her neurologic symptoms. Pathology showed vessel wall fibrosis and thrombus formation.

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Figure 1.

External carotid artery aneurysm (saccular). A - the anuerysm is indicated with red arrows. B - connection of the saccular aneurysm to the external carotid artery is indicated with blue arrow.

Conclusion This case highlights the challenges of managing TA complicated by prosthetic valve infectious endocarditis with embolic phenomena. The unusual presentation required a multidisciplinary approach to balance medical and surgical interventions. Early recognition and individualized care are essential to optimize outcomes in complex cases.