Keyword
TIPIC syndrome - temporary perivascular infiltration - carotidynia - Fay syndrome
- neck pain
Introduction
Transient perivascular inflammation of the carotid artery (TIPIC) syndrome, historically
named idiopathic carotidynia, is a rare condition characterized by inflammation and
pain in the carotid artery. It was first described as carotidynia in 1927.[1] It was classified as an idiopathic neck pain syndrome by the International Classification
of Headache Disorders (ICHD) in 1988.[2] At that time, diagnosis of carotidynia or Fay syndrome could be evoked in the following
situations: pain over the affected side of the neck, associated with tenderness, swelling,
or increased pulsation on local pressure application and the concomitant exclusion
of structural causes of carotid pain or other local causes with a self-resolution
in less than 14 days.[2] However, due to the lack of symptom's specificity and after several discussions
and arguments,[3]
[4] ICHD decided to remove carotidynia as a distinct entity.[5]
Since 1927, few cases presenting unilateral or bilateral tenderness or pain located
in the carotid bifurcation area associated with atypical ultrasound imaging presentation
have been reported. In 2017, Lecler et al have enlightened the description on an unclassified,
clinical–radiological entity that has been proposed to be described under the acronym
TIPIC syndrome.[6] Our case is relevant as it is a poorly recognized cause of neck pain. Our goal is
to reinforce the necessity of better knowledge regarding the diagnosis and follow-up
of this rare clinical entity.
Case Presentation
We are reporting the case of a 37-year-old female who consulted her general practitioner
for increasing left neck pain, triggered by head movement or palpation, without a
history of trauma. In addition, she also reported fatigue without fever or any other
neurological symptoms. Her history was notable for class I obesity and migraines.
First examination showed a normal clinical status, especially no face or neck swelling
nor lymphadenopathy, whereas a tenderness at palpation and worsening by head movement
was noticed at the level of the left carotid bifurcation.
Ultrasound imaging described an increased mural thickening measured up to 1.8 mm associated
with hyperechogenicity, corresponding to eccentric perivascular infiltration (PVI)
at that level, without lumen narrowing or hemodynamic change in Doppler mode. No dissection
was diagnosed and the intima-media thickness was normal. Radiological status was completed
with a contrast-enhanced magnetic resonance imaging (MRI) using gadolinium, which
demonstrated a soft amorphous tissue replacing the fat surrounding the left carotid
bifurcation measuring 15 × 8 mm, with gadolinium enhancement, slight inflammatory
hypersignal T1, absolute hypersignal T2, without any diffusion restriction ([Fig. 1]). MRI showed no caliber differences nor atherosclerotic plaques regarding the carotid
arteries (common, intern, and extern) on both sides.
Fig. 1 Magnetic resonance imaging at diagnosis.
Blood work revealed no anemia, a normal white blood cell count at 9 g/L, as well as
a normal C-reactive protein level. Only a slight elevation of the erythrocyte sedimentation
rate at 23 mm/h was noted. Antinuclear antibody test came back negative.
Based on the clinical and radiological examination and according to previous descriptions,
the diagnosis of TIPIC syndrome was considered for our patient. A nonsteroidal anti-inflammatory
drug (NSAID) regimen was initiated with ibuprofen for a total duration of 14 days;
600 mg three times a day for 7 days, then 400 mg three times a day.
At the follow-up consultation 1 month after the treatment introduction, the patient
reported a significant improvement of her symptoms. Nevertheless, a nocturnal pain
was still present. We also performed a complete radiological follow-up, including
ultrasound and MRI, that showed a complete resolution of initial findings particularly
the absence of any PVI or amorphous tissue in the carotid bifurcation area ([Fig. 2]).
Fig. 2 Magnetic resonance imaging at follow-up.
Discussion
TIPIC syndrome typically presents with unilateral or bilateral neck pain in the region
of the carotid bifurcation that can be severe and persistent, often exacerbated by
movement or pressure over the affected area. Its prevalence is estimated at 2.8% among
subjects presenting with acute neck pain.[6] A slight female preponderance has been observed, with the highest incidence in the
fifth and sixth decades of life,[2] but definite epidemiological data have been hard to pull out considering the small
number of patient included in the available case series.
The exact cause of the syndrome remains unclear. Histological changes of TIPIC have
been described for the first time by Upton et al,[7] with a predominantly lymphocytic proliferation with scattered neutrophils in the
artery wall, with signs of fibrosis. Such findings suggested an inflammatory process,
and the condition is considered to be idiopathic.
Diagnosis of TIPIC syndrome is based on clinical evaluation (presence of acute pain
overlying the carotid artery, which may or may not radiate to the head) and exclusion
of other conditions that can cause similar symptoms, such as infection (thyroiditis,
sialadenitis), vascular disorders (dissection), other inflammatory conditions (vasculitis),
or cervical mass. A thorough personal history and general patient examination including
neurological and osteoarticular status should be routinely performed. Laboratory with
inflammatory parameters (C-reactive protein, erythrocyte sedimentation rate [ESR],
and white blood cells) is recommended. Inflammatory markers are usually not elevated
in TIPIC.[8]
[9] Nevertheless, a discrete increase in ESR have been reported by some authors,[7]
[10] which correlates to our case.
Imaging plays a pivotal role in the diagnosis of TIPIC syndrome. MRI and ultrasound
evaluation allows the exclusion of other pathological conditions of the artery and
confirms the absence of structural disorders. Ultrasound evaluation typically shows
an increased mural thickening associated with hyperechogenicity, corresponding to
eccentric PVI. In a systematic, retrospective study, conducted by Lecler et al in
2017, PVI was most often in a posterior and lateral location on imaging studies.[6] The median largest axial diameter of the PVI ranged from 4 to 5mm. An intimal soft
plaque was described in 58% ultrasound reviews and 27% of MRI reviews. No hemodynamic
change was observed in color duplex Doppler. However, it should be noted that in this
study, 4 of the 47 patients, didn't show PVI, which is a slightly atypical presentation.[6] MRI typically shows a thickened wall of the affected carotid artery, at the level
of the carotid artery bifurcation. It is associated with increased contrast uptake,
along with the presence of pericarotid infiltration and a T2 hyperintense captation.[11] The treatment of TIPIC syndrome is typically conservative and aimed at relieving
symptoms. NSAID may be used to reduce inflammation and alleviate pain. Rest, local
heat, and avoidance of pressure on the affected artery may also be recommended.
The prognosis of the syndrome is generally favorable, with most cases resolving spontaneously
within a few weeks. In a recent multinational retrospective cohort study, the resolution
of symptoms occurred after an average of 17 days, compared with 13 days as described
in the literature.[12] Recurrence occurred in 20% of patients in the cohort study, but the condition is
typically self-limiting and does not result in long-term complications or permanent
damage to the carotid artery.
Conclusions
TIPIC syndrome can be defined as follows: acute pain overlying the carotid artery,
which may or may not radiate to the head; presence of eccentric PVI on imaging studies
(ultrasound and/or MRI); exclusion of another vascular or nonvascular diagnosis; and
improvement within 13 to 17 days either spontaneously or with NSAID treatment. We
believe that multimodal imaging including Doppler ultrasound and MRI should be performed
in the diagnosis process for every patient presenting unilateral or bilateral pain
in the carotid bifurcation area. Those techniques would allow us to exclude any other
pathology and confirm the diagnosis of this clinical–radiological entity.
