Role of Ultrasound in the Diagnosis and Follow-Up of Transient Perivascular Inflammation of the Carotid Artery (TIPIC) Syndrome: a Case Report

  • Bhagyam R. MongaEmail author
  • Rahul J. Vakharia
  • Shirish M. Hastak
  • Gayatri M. Autkar
Part of the following topical collections:
  1. Topical Collection on Imaging


Idiopathic carotidynia (IC) is a rare, possibly under-diagnosed clinico-radiologic entity that consists of acute unilateral neck pain and tenderness over the carotid bifurcation. Lecler et al. have recently described this condition associated with characteristic imaging features of transient perivascular soft tissue inflammation at the level of the affected carotid bifurcation, by a new term TIPIC (transient perivascular inflammation of the carotid artery) syndrome. We report the case of an Indian man, with recurrent right-sided TIPIC syndrome and past history of transient left-sided symptoms. On initial presentation, the clinical history, ultrasound (US) features, magnetic resonance imaging (MRI) findings, and laboratory investigations of the patient were correlated to establish the diagnosis of TIPIC syndrome. Doppler US was performed for imaging follow-up of this patient, when he presented again with recurrence of similar symptoms of right neck pain. In this 45-year-old patient, US demonstrated a characteristic perivascular echogenic soft tissue around the symptomatic carotid artery. MRI confirmed these imaging findings and ruled out carotid dissection. Patient responded to anti-inflammatory medications. Recurrence and resolution of the soft tissue thickening surrounding the affected carotid arteries were documented on serial follow-up US examinations. This case report illustrates that US can be effectively used in the diagnosis as well as follow-up of TIPIC syndrome.


Neck pain Carotid artery diseases Diagnostic imaging Ultrasound Syndrome 


Carotidynia is a rare, idiopathic, unilateral neck pain syndrome, first reported by Fay in 1927 [1]. In 1988, the International Headache Society (IHS) Classification Committee classified it as idiopathic carotidynia (IC) based on the following criteria: (a) at least one of the following overlying the carotid artery: (1) tenderness, (2) swelling, and (3) increased pulsations; (b) appropriate investigations do not reveal structural abnormality; (c) pain over the affected side of the neck, may project to ipsilateral side of the head; (d) a self-limiting syndrome of less than 2 weeks duration [2]. However, some authors observed that several cases previously described as IC in the literature did not satisfy all the diagnostic criteria proposed by IHS (1988) and therefore considered it to be merely a symptom of various diseases causing neck pain [3, 4]. IC was subsequently removed from the IHS classification in 2004 [4].

Few publications in the following years reported consistent, characteristic clinical, and imaging features in patients presenting with neck pain at the level of carotid bifurcation. It was therefore deemed necessary to improve upon the description of this clinico-radiologic entity [5, 6]. On this background, in a multicenter study conducted in 2017, Lecler et al. have described this unclassified entity by the acronym: transient perivascular inflammation of the carotid artery (TIPIC) syndrome [7]. It responds to anti-inflammatory medications and is known to have a benign course [7]. Therefore, determination of an accurate diagnosis of this syndrome is imperative for its appropriate clinical management.

We present a case of recurrent right-sided TIPIC syndrome with the intent to highlight the role of ultrasound (US) in the preliminary diagnosis and follow-up of TIPIC syndrome.

Case Report

A 45-year-old Indian man presented to the department of neurology with pain on the right side of the neck. The pain was of 1 week duration and was not radiating to either the jaw or the face. He gave a recent history of similar pain in the left side of the neck that had disappeared spontaneously within 2 weeks. No significant medical history related to dyslipidemia, diabetes, autoimmune disease, vascular event, recent viral episode, or recent cervical trauma was reported. On physical examination, the patient had moderate to severe tenderness in the right neck at the level of the carotid bifurcation. There was no obvious cervical lymph node enlargement or thyroid swelling.

The patient’s laboratory investigations including peripheral blood and platelet counts, coagulation tests, liver and renal function tests, blood glucose, and lipid profile were all within normal ranges. In particular, the C-reactive protein (CRP) was 5.1 mg/L (reference range: 0–10 mg/L), erythrocyte sedimentation rate (ESR) was 10 mm/h (reference range: 0–22 mm/h for men), and antineutrophil cytoplasmic antibody (ANCA) test was negative.

He was referred for ultrasound (US) of the neck. Duplex US was performed on an EPIQ 7G Ultrasound Color Doppler system (Philips Healthcare, Amsterdam, The Netherlands) using linear transducers of 18–5 and 12–5 MHz frequency. On B-mode, the common carotid arteries (CCAs) showed a normal intima–medial thickness (IMT) of 0.6 mm, on either side. An echogenic soft tissue thickening was seen around the right CCA extending distally from its mid-segment, along the carotid bifurcation up to the proximal segments of right internal and external carotid arteries (Fig. 1a, b). This perivascular thickening extended over a length of about 6.6 cm and measured 4 mm in its maximum thickness. On Doppler studies, the affected carotid arteries on the right side demonstrated normal color flow and spectral trace with normal luminal diameters. The left carotid vasculature was unaffected. No obvious neck mass or cervical lymphadenopathy was noted. The thyroid gland was also normal. The patient underwent further evaluation with magnetic resonance imaging (MRI) of the neck. MRI performed on a 3T Ingenia MR system (Philips Healthcare, Amsterdam, The Netherlands) confirmed the US findings of a perivascular soft tissue thickening along the cervical portions of the carotid arteries on the right side. The soft tissue thickening was hypo-isointense on unenhanced T1-weighted black-blood images and showed intense enhancement on the fat-suppressed contrast-enhanced T1-weighted black-blood images (Fig. 2a–d). The following parameters were used for acquiring the unenhanced as well as contrast-enhanced, fat-suppressed, T1-weighted, black-blood images: repetition time/echo time: 1 beat/9 ms, matrix: 400, section thickness: 3 mm, field of view: 150 mm, and flip angle: 90°. No parietal carotid hematoma was evident on the MR images. These imaging features were consistent with a diagnosis of perivascular inflammation. Patient was treated with non-steroidal anti-inflammatory drugs (NSAIDSs) and steroids. His symptoms subsided within few weeks.
Fig. 1

Diagnostic ultrasound (US) on first presentation. a Axial US scan demonstrates an echogenic perivascular soft tissue thickening around the right CCA (arrow). b Longitudinal US image showing echogenic soft tissue thickening around the right distal CCA (arrow)

Fig. 2

MRI during the initial presentation. a Unenhanced fat-suppressed T1-weighted black-blood axial MR image shows hypo-isointense perivascular soft tissue thickening (arrowhead) along the distal segment of right common carotid artery(CCA). b The perivascular soft tissue demonstrates intense enhancement (arrowhead) on the axial post contrast fat-suppressed T1-weighted black-blood image. c Enhancing soft tissue seen extending along the right carotid artery bifurcation (arrowhead). d On the post contrast fat-suppressed T1-weighted black-blood coronal image, enhancing perivascular soft tissue seen extending along the distal CCA, carotid bifurcation, and the proximal internal carotid artery on the right side (arrows). No perivascular abnormality seen involving other parts of the common or internal carotid arteries

A year later, he presented again with similar symptoms of neck pain on the right side. There was no neck pain or tenderness on the left side. Duplex US of the neck showed an eccentric, echogenic soft tissue thickening along the anterior wall of the mid-distal right CCA extending to the right carotid bifurcation. The adjacent carotid space showed fat stranding (Fig. 3a, b). Mild luminal narrowing was seen involving the affected CCA segment. However, the color flow and Doppler velocities within the entire right CCA, its bifurcation, and the proximal internal and external carotid arteries were normal (Fig. 3c, d). The patient was treated with anti-inflammatory medications and had a full clinical recovery within 14 days.
Fig. 3

Duplex utrasound (US) performed for evaluation of recurrence of the right neck pain. a Axial US scan demonstrates an echogenic perivascular soft tissue thickening around the right CCA (arrow) with fat stranding in the surrounding carotid space. b Longitudinal scan shows the extent (4.36 mm) and maximum thickness (6.76 mm) of the perivascular soft tissue around the right mid-distal CCA (arrow). c Mild luminal narrowing of the affected portion of right CCA is seen with no hemodynamic abnormality on Doppler analysis. d Perivascular soft tissue thickening seen extending to the carotid bifurcation with normal spectral waveform in the right proximal ICA

Follow-up Duplex US performed nearly 3 months post-recovery, demonstrated resolution of the perivascular soft tissue thickening around the carotid arteries on the right side (Fig. 4a–d).
Fig. 4

Follow-up ultrasound (US) obtained 3 months after recovery of symptoms. a Longitudinal B-mode US image demonstrates resolution of the perivascular soft tissue thickening around the right mid-distal CCA. b Doppler US shows normal color flow within the right CCA. c Longitudinal scan shows resolution of the soft tissue around the right carotid bifurcation and the proximal right internal and external carotid arteries. d Normal color flow seen within these arteries on the duplex Doppler US


TIPIC is a newly recognized syndrome that describes a distinct clinico-radiologic entity, previously diagnosed as idiopathic carotidynia (IC) [2, 7, 8]. In a recent multicenter study, Lecler et al. proposed that TIPIC syndrome should be added to the International Classification of Headache Disorders-III [9] based on the following four major criteria: (1) presence of acute pain overlying the carotid artery, which may or may not radiate to the head, (2) eccentric perivascular infiltration (PVI) on imaging, (3) exclusion of another vascular or nonvascular diagnosis with imaging, and (4) improvement within 14 days either spontaneously or with anti-inflammatory treatment. Additionally, Lecler et al. stated that a minor criterion could be the presence of a self-limited intimal soft plaque [7].

TIPIC syndrome/IC is characterized by unilateral neck pain and tenderness associated with transient perivascular inflammation at the level of the carotid bifurcation [5, 6, 7]. It was previously considered to be a variant of migraine [10]. TIPIC syndrome rarely presents as a bilateral disease [11, 12]. The case we report presented with right neck pain and history of self-limiting left neck pain, suggesting symptoms of bilateral metachronous carotidynia similar to the case reported by Young et al. in 2015 [12]. The probability of recurrence of TIPIC syndrome is not well documented [7, 12]. Our patient presented with recurrence of symptoms of TIPIC syndrome, more than 1 year after full clinical recovery from the previous episode.

The pathogenesis of TIPIC syndrome is poorly understood. Upton et al. have described histology findings of chronic inflammation involving the adventitia of the affected carotid artery, in cases of carotidynia [13]. However, the cause of the inflammatory process is unknown. In line with some of the previously reported cases, the laboratory markers of inflammation were normal in our patient [14, 15].

In cases of IC, a characteristic abnormal soft tissue surrounding the symptomatic carotid bifurcation has been previously reported on various imaging techniques like ultrasound (US), magnetic resonance imaging (MRI), CT imaging, CT angiography, and [18F] fluoro-deoxy-glucose positron-emission tomography-CT [5, 7, 8, 13, 15, 16, 17, 18, 19, 20, 21]. Several studies have documented the role of ultrasound in the preliminary diagnosis of IC [14, 16, 17, 19]. In conjunction with these previous observations, the Duplex US performed in our patient showed the characteristic perivascular soft tissue thickening around the symptomatic carotid arteries. The lumen diameters of these arteries and their hemodynamic parameters were normal.

Cross-sectional imaging plays a major role in diagnosing other vascular conditions mimicking IC, such as giant cell arteritis, thrombosis, fibromuscular dysplasia, carotid dissection, aneurysm, lymphadenitis, submandibular gland disease, cervical arthrosis, and neck cancer [7, 15, 18, 19]. A contrast-enhanced MRI performed in our patient during his first episode of right-sided symptoms, delineated the characteristic perivascular inflammation around the affected carotid arteries, and excluded the other ominous causes of neck pain.

The diagnosis of TIPIC syndrome was made in our patient, after correlating his clinical presentation and the characteristic imaging findings that were in conformity with the four major criteria proposed by Lecler et al. [7]. Our case did not fulfill the minor criterion proposed by Lecler et al.

Similar to the earlier case reports, the patient responded to anti-inflammatory medications [7, 8, 12, 14, 15].

A good correlation was also demonstrated between the evolution as well as recurrence of our patient’s clinical symptoms and his follow-up Duplex US imaging findings [7, 14, 21].


Recurrent unilateral TIPIC syndrome with past history of transient symptoms on the contralateral side of the neck, as was seen in our patient, is a rather unusual clinical course of this disease entity. This case report illustrates that Duplex US being a non-invasive, widely available, cost-effective imaging technique; it can be used as a first line imaging investigation for diagnosis of TIPIC syndrome. The report also emphasizes the role of Duplex US in the follow-up of TIPIC syndrome.


Data Availability Statement

The data generated or analyzed during this paper will be available from the corresponding author by reasonable request.


The authors declare that no financial support was received for this case report.

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This article does not contain any studies with animals performed by any of the authors.

Informed Consent

Written informed consent was obtained from the patient included in this case report.


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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of UltrasoundWockhardt HospitalMumbaiIndia
  2. 2.Department of CT/MRIWockhardt HospitalMumbaiIndia
  3. 3.Department of Neurology and Stroke ServicesWockhardt HospitalMumbaiIndia

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