Carotidynia, also known as Fay syndrome, is a rare syndrome characterized by neck pain in the region of the carotid bifurcation.
It was classified by the International Headache Society (IHS) in 1988 as an idiopathic neck pain syndrome associated with tenderness over the carotid bifurcation without structural abnormality.
Recently a discrete clinicoradiological entity has been described and named transient perivascular inflammation of the carotid artery syndrome (TIPIC syndrome) 14.
There remains confusion in the literature as to what the term carotidynia actually refers to, with some authors suggesting that the term should be reserved for a pain syndrome with no structural abnormality, whilst others contend that it is a defined inflammatory condition of the carotid (TIPIC syndrome). The latter seems to be more useful as it correlates to a specific syndrome with defined imaging features (thickening and inflammation around the carotid bifurcation) and prognosis (spontaneous resolution within 1-2 weeks).
Patients with carotidynia present with unilateral neck pain in the region of the carotid bifurcation 1. The pain typically tends to be dull and throbbing in nature, continuous, and usually localized over the carotid bifurcation, although it may radiate to the ipsilateral mandibular, buccal, ocular, or auricular regions.
The etiology of carotidynia remains unclear, however, it appears to be an inflammatory process involving the carotid wall, including the adventitia 1.
Carotidynia may be classified into three distinct entities 10:
- non-migrainous (or classic)
Many inflammatory markers tend to be elevated, including 4:
- C-reactive protein (CRP)
- erythrocyte sedimentation rate (ESR)
- serum amyloid A protein (SAP)
- fibrin degradation product / D-dimer
Contrary to the diagnostic criteria proposed by the Headache Classification Subcommittee of the IHS 3, which suggest that a carotid structural abnormality should be absent, relatively consistent imaging findings are encountered 1,2.
Regardless of modality (ultrasound, CT, MRI), typical features of carotidynia include 1,2:
- region: distal common carotid artery, bulb and proximal internal carotid artery
- lumen: mild narrowing
- eccentric thickening of the carotid wall/perivascular tissues
- enhancement (CT, and more so MRI)
- transient fatty plaque during healing 1
- surrounding soft tissues: fat stranding
"King Kong carotid" has been suggested by some neuroradiologists as an imaging sign of TIPIC syndrome. The eccentric thickening of the pericarotidian tissues with minimal narrowing of the vessel lumen is reminiscent of the hand of King Kong in the original motion picture incompletely encircling Ann Darrow without squeezing her too hard. Coincidentally, the actress who played the role of Ann Darrow was named Fay Wray, which has relevance to the eponymous name for this condition, Fay syndrome 15.
Treatment and prognosis
Carotidynia is usually self-limiting, spontaneously resolving within 2 weeks. Resolution can be expedited with non-steroidal anti-inflammatory drugs (NSAIDs) or steroids 1.
History and etymology
It was first described in 1927 by American neurologist Temple Fay (1895-1963) 7.
The differential includes other vascular causes of neck pain, including 1:
- arterial dissection
- large vessel vasculitides
- jugular vein thrombosis
Other wider clinical differential considerations include 2:
- cervical lymphadenitis
- submandibular gland diseases
- head and neck tumors
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- 14. Lecler A, Obadia M, Savatovsky J, Picard H, Charbonneau F, Menjot de Champfleur N, Naggara O, Carsin B, Amor-Sahli M, Cottier JP, Bensoussan J, Auffray-Calvier E, Varoquaux A, De Gaalon S, Calazel C, Nasr N, Volle G, Jianu DC, Gout O, Bonneville F, Sadik JC. TIPIC Syndrome: Beyond the Myth of Carotidynia, a New Distinct Unclassified Entity. (2017) AJNR. American journal of neuroradiology. 38 (7): 1391-1398. doi:10.3174/ajnr.A5214 - Pubmed
- 15. Peterson R [RyanBPetersonMD], Koontz N [nakoontz], Bull M [mattdbull]. (2018, Feb 2). Indeed. Only 'King Kong Carotid' has a better ring to it [Tweet]. Retrieved from https://twitter.com/mattdbull/status/966196758551425024