Abstract
While superficial temporal artery (STA) vasculitis is typically a disease of the elderly, spontaneous STA aneurysm in children is anecdotic and usually caused by a subjacent vasculitis. Since 1948 around 40 cases have been listed in literature and just 6 of them under the age of 18. Three main forms have been classified: juvenile temporal arteritis, typical giant cells arteritis and temporal artery involvement secondary to systemic vasculitis. We report a rare case of STA aneurysm in an 8 year old patient already known for the surgical treatment of an aortic coarctation. The patient was asymptomatic, clinical exam was unremarkable and no traumatism, fever, arthralgias or recent infective episodes were reported. No residual coarctation at echocardiography. STA echo-colour Doppler suggested juvenile temporal arteritis, showing a dilation of 5.4 x 8.7 mm. Doppler scanning of the other districts was normal. At cerebral MRI a spindle-shaped dilation over the left STA was confirmed without other intracranial anomalies. Thoraco-abdominal angio-TC was normal to the entire aorta and at the visceral arteries level. Blood exams were unremarkable. A biopsy of the left STA was performed under general anesthesia: a true aneurysm of the artery (1.5×8 mm) was resected. At anatomopathologic examination neither epithelioid nor great cells were found but a lymphocytic infiltration was detected around the vasa vasorum. Isolated STA vasculitis in young individuals results in different findings, pathogenic triggers and clinical manifestations from affecting the elderly. Diagnosis is very important to direct the appropriate therapeutic strategy and must include histopathologic evaluation.
Highlights
Superficial temporal artery (STA) vasculitis is typically a disease of the elderly, being extremely rare in young patients and pediatric population
juvenile temporal arteritis (JTA) is the more frequent form and confers the best prognosis; all reported cases have been resolved with excision of the affected temporal artery without the need for systemic therapy
We present the case of an eight year old patient referred to our Unit for the incidental finding of a throbbing swelling on the left temple, characterized as a superficial temporal artery aneurysm
Summary
Superficial temporal artery (STA) vasculitis is typically a disease of the elderly, being extremely rare in young patients and pediatric population. Three main forms have been classified: juvenile temporal arteritis (JTA), typical giant cells arteritis (GCA) and temporal artery involvement secondary to systemic vasculitis [2]. The latter has been observed more frequently in polyarteritis nodosa (PAN) [3], Churg-Strauss syndrome (CSS) [4] and thromboangiitis obliterans (TAO) [5]. We present the case of an eight year old patient referred to our Unit for the incidental finding of a throbbing swelling on the left temple, characterized as a superficial temporal artery aneurysm. Immunological markers such as ANCA, anti-MPO and anti-PR3 antibodies were negative and so were
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