Jonathan Irish, MD, FRCSC, Section Editor
Styloid/C1 transverse process juxtaposition as a cause of Eagle’s syndrome Sandra Ho, MD,1 Adam Luginbuhl, MD,1 Steven Finden, MD,1 Joseph M. Curry, MD,1 David M. Cognetti, MD2* 1
Department of Otolaryngology Head and Neck Surgery, Thomas Jefferson University, Philadelphia, Pennsylvania, 2Department of Radiology, Thomas Jefferson University, Philadelphia, Pennsylvania.
Accepted 24 February 2015 Published online 6 July 2015 in Wiley Online Library (wileyonlinelibrary.com). DOI 10.1002/hed.24024
Background. The purpose of this case report was to characterize styloid/ C1 transverse process juxtaposition as a cause for Eagle’s syndrome. Methods and Results. A case series was conducted with a chart review of 5 patients with radiographic evidence of jugular vein compression who underwent styloid process excision between 2010 and 2013. There were 4 men and 1 woman, aged 35 to 62 years (mean, 46 years). Cervicalgia (4 of 5 patients) and otalgia (4 of 5 patients) were the most commonly reported symptoms. Styloid process length ranged from 2.4 to 8.5 cm. The distance between the styloid process and the transverse process of C1 ranged from 0.05 to 0.46 cm. All patients underwent a
transcervical approach for the excision of the styloid process with immediate postoperative resolution of symptoms and good cosmetic results. Conclusions. Styloid/C1 transverse process juxtaposition can produce symptoms of cervicalgia and otalgia even in the setting of a normal length styloid process. The transcervical approach is safe and effective for excision of the styloid process and has good functional and cosmetic C 2015 Wiley Periodicals, Inc. Head Neck 37: E153–E156, 2015 results. V
In 1937, Eagle presented the first case of clinical symptoms that occurred as a result of an elongated styloid process. He then went on to propose 2 syndromes associated with this phenomenon: (1) a classic syndrome and (2) a carotid artery syndrome. The former was thought to be due to scar tissue formation after a tonsillectomy that stretched nerve endings in the area, causing symptoms of dysphagia and otalgia. The latter was thought to be due to an elongated styloid process impinging upon the carotid artery and associated nerve endings, causing facial and cervical pain. In both syndromes, the length of the styloid process was thought to be the major causal factor. In this study, we propose that in addition to patients with elongated styloid processes, patients with styloid processes coursing adjacent to the transverse process of C1 may also have symptoms similar to those previously described by Eagle.1 Institutional review board approval was obtained for this retrospective analysis. Five patients who underwent styloid process excision for presumed Eagle’s syndrome from 2010 to 2013 were retrospectively analyzed. A detailed medical history and clinical examination were undertaken on all patients. Two-dimensional CT with contrast was acquired in all but 1 patient. Multiplanar reconstruction was carried out with Osirix software (Geneva, Switzerland) to allow for styloid process measurement.
*Corresponding author: D. M. Cognetti, Department of Otolaryngology Head and Neck Surgery, Thomas Jefferson University, 925 Chestnut Street, 6th Floor, Philadelphia, PA 19107. E-mail: [email protected]
HEAD & NECK—DOI 10.1002/HED
KEY WORDS: cervicalgia, Eagle’s syndrome, styloid process, transcervical, otalgia
Five patients who underwent styloid process excision for presumed Eagle’s syndrome were included in this study. There were 4 men and 1 woman with ages that ranged from 35 to 62 years (mean, 46 years). Symptom duration ranged from 9 months to 14 years (mean, 4.5 years). Two patients presented with symptoms primarily on the right side, and 2 patients had predominately leftsided symptoms. One patient presented with bilateral symptoms. Four of the 5 patients eventually progressed to bilateral symptoms and underwent staged excision of both sides (n 5 9). Patient #1 had a prior diagnosis of pseudotumor cerebri and had received multiple spinal taps without relief. Two of the patients reported a history of a motor vehicle accident. A summary of the patient demographics, styloid length, surgical approach, and outcomes are summarized in Table 1. Patients most commonly presented with cervicalgia (4 of 5 patients), otalgia (4 of 5 patients), and headaches (3 of 5 patients). These symptoms, along with others, are summarized in Table 2. On physical examination, 4 patients felt that their symptoms were made worse by head turning and 1 patient with chewing. With palpation of the tonsillar fossas, only 1 patient had a palpable styloid process on the right side (1 of 9 patients). Upon CT evaluation, 2 patients had dramatically elongated styloid processes. Patient 2 had a right styloid process measuring 6.8 cm and patient #5 had a left styloid process measuring 8.5 cm. Interestingly, for both patients, it was the side with the shorter styloid processes that were also the sides with the shortest distance between the styloid process and C1. It was also the side that these 2 patients had E153
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TABLE 1. Patient demographics, surgical approach, and outcomes. Patient no.
2 3 4 5
M M F M
51 44 35 57
Transcervical Transcervical Transcervical Transcervical
Recurrence at 1 y ! reexcision ! complete remission Complete remission Complete remission Complete remission Complete remission
Historically, Eagle1 proposed that elongated styloid processes could cause 2 distinct syndromes – one that occurred posttonsillectomy and another that occurred with carotid artery compression. He reported the incidence of elongated styloid processes to be around 4%, but of these, only 4% were symptomatic. Since Eagle’s initial report in 1937, numerous other cases of symptomatic elongated styloid processes have been documented.3–6 In our study, we propose that symptoms can result from the close proximity between the styloid process and the transverse process of C1. According to Eagle,1 the normal styloid process is about 2.5 to 3 cm long. In the literature, the normal length of the styloid process has been reported to range between 1.5 and 4 cm.7–9 Cadaveric studies have demonstrated the styloid process to range from 1.8 cm to 2.6 cm.9 In our series, the styloid processes ranged from 2.4 to 8.5 cm, with 7 of 9 styloid processes under 4 cm. Patients 2 and 5 had unequivocally long styloid processes on the right (6.8 cm) and left (8.5 cm), respectively. Interestingly, for both patients, it was the side with the shorter styloid process that also had smaller distances between the styloid process and C1, with the distances between the styloid process and the transverse process of C1 measuring 0.18 cm (patient 2) and 0.24 cm (patient 5). Both of these patients, along with patients 1 and 3 also presented initially with symptoms that corresponded to the side with the most significant compression. Although all 4 patients eventually had excision of both their styloid processes, the fact that all 4 had symptoms
their initial symptoms. The remaining patients had styloid processes within the normal range (