European Review for Medical and Pharmacological Sciences 2008; 12: 131-133 Atypical chronic head and neck : don’t forget Eagle’s syndrome

M. CASALE1, V. RINALDI1, C. QUATTROCCHI2, F. BRESSI1, B. VINCENZI3, D. SANTINI3, G. TONINI3, F. SALVINELLI1

Areas of 1Otolaryngology, 2Radiology and 3Medical , University Campus Bio-Medico, Rome (Italy)

Abstract. – We report a case of an adult drome” (ES) since 1937, when an otolaryngolo- woman with an Eagle’s Syndrome (ES) treated gist of the Duke University, Watt W. Eagle, de- with medical . ES is characterized by an scribed the first cases1-4. This syndrome was char- aspecific secondary to calcifica- acterized by symptoms typically occurring after tion of the stylohyoid ligament or elongated sty- loid process. In about 4% of general population pharyngeal trauma or tonsillectomy and presents an elongated styloid process occurs, while only as a nagging dull, long-term ache in the throat, about 4% of these patients are symptomatic. We sometimes radiated to the ipsilateral ear, sensation report a case of a 49-year-old lady with a 1-year of foreign body, occasionally odynophagia, dys- history of oro-pharyngeal foreign body sensa- phonia, increased salivation and headache. Not tion localized at the left , associat- rarely patients believe that they have not properly ed with a dull intermittent pain. A bony projec- tion was palpable with bimanual transoral explo- healed from their tonsillectomy. The second and ration. A lateral radiograph and a computed to- lesser-know presentation is constant throbbing mography scan of head and neck showed an pain through either the internal or external elongated styloid process of 57 mm on the left carotid artery distributions5. side and 48 mm on the right one. The patient re- Eagle considered tonsillectomy responsible for fused surgical treatment as first choice. She un- the formation of scar tissue around the styloid derwent a non-steroidal anti-inflammatory local apex, with consequent compression or stretching treatment, with progressive disappearance of symptoms. After 6 months she had no recur- of the vascular and nervous structures contained rence of symptoms. in the retrostyloid compartment (in particular In conclusion, a precise differential diagnosis glossopharyngeal nerve and perivascular carotid is crucial in order to choose the most adequate sympathetic fibres)1-4. treatment, which can be either surgical or non In the ensuing years, the term “Styloid Syn- surgical. Medical treatment represents the first drome” was created to describe a cervico-pha- choice, followed by surgical styloid process re- ryngeal pain related to the styloid process, when section, in the case of persistence or ingraves- 6 cence of the complaint. no previous history of trauma can be found . Pathophysiologically, the styloid syndrome is re- Key Words: lated to an irritation of the surrounding nerves, Elongated styloid process, Eagle’s syndrome, Head the carotid artery or the pharyngeal mucosa. and neck pain. The normal length of the styloid process varies greatly, but in the majority of patients it is 20 to 30 mm; it is considered elongated when it is longer than 25 mm7-9. In about 4% of general population an elongat- ed styloid process occurs, while only about 4% Introduction of these patients are symptomatic; thus the true incidence is 0.16% with a female predominance Aspecific orofacial pain secondary to calcifi- of 3:15,10. cation of the stylohyoid ligament or elongated We report a case of an adult woman with an styloid process has been known as “Eagle’s Syn- ES treated with medical therapy.

Corresponding Author: Vittorio Rinaldi, MD; e-mail: [email protected] 131 M. Casale, V. Rinaldi, C. Quattrocchi, F. Bressi, B. Vincenzi, D. Santini, G. Tonini, F. Salvinelli

Case Report A 49-year-old lady presented to the Otolaryn- gology Clinic of the University Campus Bio- Medico of Rome with a 1-year history of oro- pharyngeal foreign body sensation localized at the left tonsillar fossa, associated with a dull in- termittent pain on the left side of her throat. She didn’t reported odynophagia, , cervical pain, reflex ipsilateral otalgia. The patient was detailed interrogated and screened thoroughly, and pain due to other fac- tors, such as temporomandibular, dental, or- thopaedic, and pharyngoesophageal causes, was ruled out. Questioning disclosed that she under- went a tonsillectomy and a revision of tonsillec- tomy respectively 40 and 10 years before. There was no history of neck injury. Figure 2. CT scan showing a 48 mm right styloid process. The patient underwent detailed clinical ear, nose and throat (ENT) examination that included bimanual transoral palpation of left tonsillar fos- sa, on which a bony projection was felt; pain was of patient’s symptoms after infiltration of tonsil- elicited during palpation. The examination of the lar fossa with 1 ml of 2% lidocaine. controlateral tonsillar fossa was normal. The patient refused surgical treatment as first A lateral radiograph of the head was ordered: choice and she underwent an non-steroidal anti- the film showed remarkably elongated styloid inflammatory local treatment, with benefit. After processes ( > on left side), both measuring more 6 months she had no recurrence of symptoms. than one third of the length of the ramus of the . This anomaly was confirmed by head and neck computed tomography scan showing an elongated styloid process of 57 mm on the left Discussion side and 48 mm on the right one (Figures 1, 2, 3). No other densitometric alterations were found at Although the incidence of the styloid process the skull base. elongation or mineralization of the stylohyoid A positive lidocaine infiltration test was ob- complex is not uncommon, only a small percent- tained, this consisting in a temporarily subsiding age of these cases are symptomatic.

Figure 1. CT scan showing a 57 mm left styloid process. Figure 3. CT scan showing both styloid processes.

132 Atypical chronic head and neck pain: don’t forget Eagle’s syndrome

The vagueness of symptoms and the infrequent infection, while the disadvantages are the exter- clinical observation are often misleading. These nal scar, the longer surgical time and the risk of patients may be seen by a surgeon, a dentist, a injury to the facial nerve13. neurologist, and a psychiatrist, often receiving a In the case of clinical symptoms such as dys- variety of treatments that do not relieve the symp- phagia, foreign-body sensation and chronic neck toms and that cloud the clinical picture. Stylalgia or facial pain close to the ear, an ES should be is misdiagnosed or overlooked as a possible diag- considered in the differential diagnosis. nosis in cases of vague cervicofacial pain. All patients should be placed on a stepwise A variety of head and neck conditions should, therapy plan, which begins with a medical treat- however, be considered in the differential diagno- ment, followed by surgical treatment, in the case sis of ES and cervicopharyngeal pain. These in- of persistence or ingravescence of the complaint. clude temporomandibular disorders, glossopha- ryngeal , , mi- graine-type headaches, sphenopalatine neuralgia, References cervical arthritis, carotidynia, temporal arteritis, otitis, disease and possible tu- 1) EAGLE WW. Elongated styloid process: report of mours. Other should be eliminated by two cases. Arch Otolaryngol 1937; 25: 584-586. a careful medical history, clinical and radi- 2) EAGLE WW. Elongated styloid process: further ob- ographic examination5. servations and a new syndrome. Arch Otolaryn- gol 1948; 47: 630-640. The diagnosis of the condition requires aware- ness and vigilance. Diagnosis of ES is based on a 3) EAGLE WW. Symptomatic elongated styloid process: report of two cases of styloid good medical history and physical examination. process–carotid artery syndrome with operation. Tipically the patient refers earache, especially on Arch Otolaryngol 1949; 49: 490-503. swallowing with an history of tonsillectomy; 4) EAGLE WW. Elongated styloid process; symptoms more rarely, the patient can also have a foreign and treatment. AMA Arch Otolaryngol 1958; 67: body sensation in the pharynx with a persistent 172-176. dull aching sore throat. Stylalgia is confirmed 5) MENDELSOHN AH, BERKE GS, CHHETRI DK. Hetero- through palpation of the tonsillar fossa, transpha- geneity in the clinical presentation of Eagle’s syn- ryngeal injection of long-acting local anaesthetic drome. Otolaryngol Head Neck Surg 2006; 134: and /or steroids. With classic presentations of ES, 389-393. imaging is not necessary for diagnosis; however, 6) CAMARDA AJ, DESCHAMPS C, FOREST D. Stylohyoid especially in doubt cases, plain skull films is chain ossification: a discussion of etiology. Oral Surg Oral Med Oral Pathol 1989; 67: 515-520. enough. CT scan provides precious informations for the surgeon showing a more detailed view 7) MOFFAT DA, RAMSDEN RT, SHAW HJ. The styloid process syndrome: aetiological factors and surgical and measuring the precise styloid process length. management. J Laryngol Otol 1977; 91: 279-294. Once the diagnosis of ES or stylohyoid-related 8) KAUFMAN SM, ELZAY RP, Irish EF. Styloid process pain is made, surgical or non surgical treatment variation. Radiologic and clinical study. Arch Oto- should be considered. NSA-drugs, transpharyn- laryngol 1970; 91: 460-463. geal infiltration of steroidal drugs and lidocaine 9) STRAUSS M, ZOHAR Y, L AURIAN N. Elongated styloid in the tonsillar fossa have been suggested as non process syndrome: intraoral versus external ap- surgical treatments11. proach for styloid . Laryngoscope 1985; The surgical approach includes a transoral sty- 95: 976-979. loid fracture and/ or a surgical styloid shortening, 10) HARMA R, LINDEN WF. Indications for esophageal which can be carried out either transorally or reconstruction of corrosive structures. Acta Oto- transcervically12. laryngol 1966; 62: 27-32. The advantages of the trans-pharyngeal ap- 11) EVANS JT, CLAIRMONT AA. The nonsurgical treatment proach are safety, simpleness, shorter time, and of Eagle’s syndrome. Eye Ear Nose Throat Mon 1976; 55: 94-95. no external scar, even though such an approach has been criticised by some authors in view of 12) WEIDENBECHER M, SCHICK B, IRO H. Styloid syn- drome and its treatment. Laryngorhinootologie the possibility of infection of deep neck spaces, 2006; 85: 184-190. the risk of injury to major vessels and the poor 13) CHASE DC, ZARMEN A, BIGELOW WC, MCCOY JM. Ea- visualization of deep planes. The advantages of gle’s syndrome: a comparison of intraoral versus the external approach are the better visualization extraoral surgical approaches. Oral Surg Oral and the reduced possibility of deep neck space Med Oral Pathol 1986; 62: 625-629.

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