Transient perivascular inflammation of the carotid artery (TIPIC) syndrome, previously known as carotidynia or Fay syndrome, is a relatively newly recognized rare cause of acute unilateral cervical pain. It was described for the first time by Fay in 1927.1 The pain is typically localized over the carotid bifurcation, usually associated with tenderness on palpation, and may radiate to the adjacent regions. TIPIC syndrome is characterized by transient perivascular nonspecific inflammation of the carotid adventitia, but its pathogenesis remains unclear.2 The estimated prevalence of TIPIC among patients with acute neck pain is 2.8%,3 but this Figure may be underestimated due to generally mild symptoms and self-limiting resolution.
In this case, a 48-year-old man presented with a 2- to 3-day history of acute right-sided neck pain. The pain radiated to the right side of the head and was intensified by turning the head to the right. The patient denied a past history of cervical trauma, surgical intervention, or infection. Physical examination revealed tenderness when palpating the neck at level II (upper jugular lymph nodes area) on the right side, without any swelling. Ultrasound examination, performed on the second day after admission to the hospital (3–4 days from the onset of the symptoms), showed an eccentric normo- and hypoechoic thickening of the right common carotid artery (RCCA) wall at the level of bifurcation with propagation into the proximal right internal carotid artery (RICA) (Figure 1A). Doppler ultrasound examination demonstrated normal flow parameters in the affected carotid artery. Computed tomography, as per the emergency protocol performed on the day of the admission in the emergency room, and magnetic resonance imaging (MRI), performed 5 days after the admission revealed a perivascular infiltration of the distal RCCA in its bifurcation and around the proximal part of the RICA. MRI demonstrated hyperintense perivascular tissue edema on a T2-weighted image with enhancement after contrast administration on a T1-weighted, fat-saturated sequence (Figure 1B and 1C). TIPIC syndrome was diagnosed based on the results of the imaging examinations and after exclusion of other vascular disorders or causes of neck pain. Oral steroid therapy (methylprednisolone) was provided and resulted in a full clinical recovery. The follow-up ultrasound examination showed regression of the eccentric perivascular tissue. Within 1 year following the initial presentation, our patient presented 3 times with a relapse of the symptoms (after 1, 2, and 12 months since the first episode). Each time, he complained of acute right-sided neck pain radiating to the head. Ultrasound examination and MRI revealed a thickening of the RCCA wall at the level of the carotid bifurcation. Oral anti-inflammatory therapy was administered with clinical improvement.
TIPIC syndrome is a clinicoradiological entity usually manifested by severe, acute, unilateral neck pain, with the age of onset ranging between 30 and 50 years.3 It requires differentiation with autoimmune, laryngologic, ophthalmologic, and dental diseases. Imaging examinations, especially ultrasonography and MRI, may reveal abnormalities of the carotid bifurcation region, confirming an inflammatory process.4 Despite the self-limiting course of the disease, which usually resolves within 7 to 14 days, a good response to anti-inflammatory treatment has been proven. Recurrence occurs in 18.6% of patients.5
TIPIC syndrome as a group of diseases with abnormalities in the soft tissue surrounding the carotid artery was proposed by Lecler in 2017.3 Four diagnostic criteria have been set out: 1) presence of acute pain overlying the carotid artery, which may or may not radiate to the head, 2) eccentric perivascular infiltration on imaging, 3) exclusion of other vascular or nonvascular diagnoses with imaging, 4) improvement within 14 days either spontaneously or with anti-inflammatory treatment.
A self-limited intimal soft plaque was pointed out as an additional minor criterion.3 All 4 diagnostic criteria were met in the presented case.
TIPIC syndrome should be considered as a possible vascular cause of acute unilateral cervical pain in patients with characteristic radiologic imaging.
Justyna Chojdak-Łukasiewicz, MD, PhD, Department of Neurology, Wroclaw Medical University, ul. Borowska 213, 50-556 Wrocław, Poland, phone: +48 71 734 31 00, email: justyna.chojdak-lukasiewicz@umw.edu.pl
March 19, 2023.
June 26, 2023.
July 5, 2023.
None.
Publication of this report was supported by Wroclaw Medical University.
None declared.
Korbecka J, Chojdak-Łukasiewicz J, Sulima K, et al. Transient perivascular inflammation of the carotid artery syndrome as a rare cause of unilateral neck pain. Pol Arch Intern Med. 2023; 133: 16525. doi:10.20452/pamw.16525
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