TIPIC Syndrome: Beyond the Myth of Carotidynia, a New Distinct Unclassified Entity

Abstract

Background and purpose: The differential diagnosis of acute cervical pain includes nonvascular and vascular causes such as carotid dissection, carotid occlusion, or vasculitis. However, some patients present with unclassified vascular and perivascular changes on imaging previously reported as carotidynia. The aim of our study was to improve the description of this as yet unclassified clinico-radiologic entity.

Materials and methods: From January 2009 through April 2016, 47 patients from 10 centers presenting with acute neck pain or tenderness and at least 1 cervical image showing unclassified carotid abnormalities were included. We conducted a systematic, retrospective study of their medical charts and diagnostic and follow-up imaging. Two neuroradiologists independently analyzed the blinded image datasets.

Results: The median patient age was 48 years. All patients presented with acute neck pain, and 8 presented with transient neurologic symptoms. Imaging showed an eccentric pericarotidian infiltration in all patients. An intimal soft plaque was noted in 16 patients, and a mild luminal narrowing was noted in 16 patients. Interreader reproducibility was excellent. All patients had complete pain resolution within a median of 13 days. At 3-month follow-up, imaging showed complete disappearance of vascular abnormalities in 8 patients, and a marked decrease in all others.

Conclusions: Our study improved the description of an unclassified, clinico-radiologic entity, which could be described by the proposed acronym: TransIent Perivascular Inflammation of the Carotid artery (TIPIC) syndrome.

Fig 1.

Diagnostic ultrasonography (A–D) shows an eccentric perivascular infiltration (arrowhead) at the level of bifurcation, with a soft intimal plaque (arrow) and a mild lumen narrowing without a hemodynamic change in Doppler mode. Follow-up ultrasonography (E) shows a marked decrease in the perivascular infiltration (arrowhead) and complete disappearance of the soft intimal plaque.

Fig 2.

CTA shows a left posterolateral eccentric perivascular infiltration (arrowhead) surrounding the carotid artery, with a distinct low-density soft intimal plaque (arrow).

Fig 3.

Initial diagnostic ultrasonography (A) and follow-up ultrasonography at 14 days (B) and 6 months (C) show a perivascular infiltration (arrowheads) at the level of the internal carotid artery just at the level of bifurcation, with a quick decrease at 14 days and the persistence of a thin abnormality at 6 months.

Fig 4.

Pre (A) and post (B) contrast fat-suppressed 3D T1 and 3D T2-weighted (C) MR imaging in an axial plane shows T1 hypointense and T2 hyperintense perivascular infiltration (arrowhead) at the level of the carotid artery bifurcation, enhanced after gadolinium injection. A distinct soft intimal plaque (arrow) is visible at the posterior part of the carotid artery. A sagittal curvilinear reconstruction of the right internal carotid artery on the postcontrast T1-weighted imaging (D) shows the PVI (arrowhead) centered at the level of the right carotid artery bifurcation and extended to both the distal common carotid artery and proximal internal carotid artery. Note that there is no vascular or perivascular abnormality involving other parts of the common or the internal carotid arteries.