Eagle's and Ernest Syndrome

Number: 0400

Table Of Contents

Policy
Applicable CPT / HCPCS / ICD-10 Codes
Background
References

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Policy

Scope of Policy

This Clinical Policy Bulletin addresses Eagle’s and Ernest syndromes. Eagle’s syndrome, also termed stylohyoid or styloid syndrome, is a rare condition caused by elongation of the styloid process of the temporal bone. Ernest syndrome is also a rare condition caused by calcification of the stylomandibular ligament, especially at the mandibular insertion. Both are defined by orofacial pain.

1. Medically Necessary

   1. Eagle’s Syndrome treated by styloidectomy:

      Aetna considers styloidectomy through the transoral or transcervical approach medically necessary for Eagle’s syndrome when all of the following are met:

      1. Diagnosis of Eagle’s syndrome must be confirmed by all of the following:

         1. Documentation of at least 3 of the following symptoms

            1. pharyngeal pain with hemifacial radiation
            2. otalgia
            3. odynophagia
            4. sensation of foreign body in the pharynx
            5. discomfort with neck rotation
            6. increased salivation
            7. dizziness;
         2. In person exam documenting pain with palpation of the styloid process in the tonsillar fossa; and
         3. Formal imaging report showing an elongated styloid process of at least 30mm; and
         4. Other causes of pain ruled out including temporomandibular syndrome and dental problems; and

      2. Conservative treatment:

         1. At least 6 months of conservative management after confirmed diagnosis of Eagle’s syndrome, including at least one anticonvulsant or antidepressant taken for at least 3 months and at least one of the following:

            1. Trial of oral steroids or NSAIDs; or
            2. Local injections of steroid/anesthetic; or
            3. Physical therapy; and

      3. In-person follow up office visit documenting results of treatment and continued qualifying symptoms, and continued pain with palpation of the styloid process in the tonsillar fossa on repeat examination.

   2. Vascular Eagle’s syndrome:

      Stenosis and dissection of the carotid artery and stenosis of the jugular vein have been reported in association with elongated styloid processes. Aetna considers styloidectomy medically necessary for vascular Eagle’s syndrome when all of the following are met:

      1. Carotid artery dissection has been confirmed by CT angiography or conventional angiography; and
      2. Anticoagulation therapy has been started; and
      3. Formal imaging report shows an elongated styloid process of at least 30mm at the level of the dissection.

2. Experimental, Investigational, or Unproven

   The following are considered experimental, investigational, or unproven for treatment of Ernest or Eagle's syndrome:

   1. Radiofrequency (RF) thermoneurolysis (also known as RF ablation, RF neurolysis, RF thermoablation);
   2. Styloidectomy for stenosis of the jugular vein;
   3. Styloidectomy for stenosis of the carotid artery in the absence of carotid dissection;
   4. Styloidectomy for all other indications not noted as medically necessary above, including idiopathic intracranial hypertension, tinnitus, chronic fatigue, brain fog, headache and neck pain;
   5. Preventative styloidectomy.

3. Related Policies

   • CPB 0028 - Temporomandibular Disorders - noting that RF thermoneurolysis is considered experimental and investigational for treatment of TMD/TMJ syndrome

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Table:

CPT Codes / HCPCS Codes / ICD-10 Codes

Code	Code Description
CPT codes covered if selection criteria are met:
Styloidectomy (transoral or transcervical) - No specific code
CPT codes not covered for indications listed in the CPB:
Radiofrequency thermoneurolysis (also known as radiofrequency ablation, radiofrequency neurolysis, radiofrequency thermoablation - No specific code
Other CPT codes related to the CPB:
36221	Non-selective catheter placement, thoracic aorta, with angiography of the extracranial carotid, vertebral, and/or intracranial vessels, unilateral or bilateral, and all associated radiological supervision and interpretation, includes angiography of the cervicocerebral arch, when performed
36222	Selective catheter placement, common carotid or innominate artery, unilateral, any approach, with angiography of the ipsilateral extracranial carotid circulation and all associated radiological supervision and interpretation, includes angiography of the cervicocerebral arch, when performed
36223	Selective catheter placement, common carotid or innominate artery, unilateral, any approach, with angiography of the ipsilateral intracranial carotid circulation and all associated radiological supervision and interpretation, includes angiography of the extracranial carotid and cervicocerebral arch, when performed
36224	Selective catheter placement, internal carotid artery, unilateral, with angiography of the ipsilateral intracranial carotid circulation and all associated radiological supervision and interpretation, includes angiography of the extracranial carotid and cervicocerebral arch, when performed
+36227	Selective catheter placement, external carotid artery, unilateral, with angiography of the ipsilateral external carotid circulation and all associated radiological supervision and interpretation (List separately in addition to code for primary procedure)
+36228	Selective catheter placement, each intracranial branch of the internal carotid or vertebral arteries, unilateral, with angiography of the selected vessel circulation and all associated radiological supervision and interpretation (eg, middle cerebral artery, posterior inferior cerebellar artery) (List separately in addition to code for primary procedure)
64600	Destruction by neurolytic agent, trigeminal nerve; supraorbital, infraorbital, mental, or inferior alveolar branch
70250	Radiologic examination, skull; less than 4 views
70260	Radiologic examination, skull; complete, minimum of 4 views
70355	Orthopantogram (eg, panoramic x-ray)
70450	Computed tomography, head or brain; without contrast material
70460	Computed tomography, head or brain; with contrast material(s)
70470	Computed tomography, head or brain; without contrast material, followed by contrast material(s) and further sections
70486	Computed tomography, maxillofacial area; without contrast material
70487	Computed tomography, maxillofacial area; with contrast material(s)
70488	Computed tomography, maxillofacial area; without contrast material, followed by contrast material(s) and further sections
70498	Computed tomographic angiography, neck, with contrast material(s), including noncontrast images, if performed, and image postprocessing
97010 - 97028; 97032 – 97039	Physical medicine and rehabilitation modalities
97110 – 97140	Physical medicine and rehabilitation therapeutic procedures
Other HCPCS codes related to the CPB:
C9144	Injection, bupivacaine (posimir), 1 mg
J0665	Injection, bupivicaine, not otherwise specified, 0.5 mg
J0666	Injection, bupivacaine liposome, 1 mg
J0670	Injection, mepivacaine hydrochloride, per 10 ml
J0702	Injection, betamethasone acetate 3 mg and betamethasone sodium phosphate 3 mg
J1010	Injection, methylprednisolone acetate, 1 mg
J1094	Injection, dexamethasone acetate, 1 mg
J1100	Injection, dexamethasone sodium phosphate, 1 mg
J1130	Injection, diclofenac sodium, 0.5 mg
J1165	Injection, phenytoin sodium, per 50 mg
J1320	Injection, amitriptyline hcl, up to 20 mg
J1700	Injection, hydrocortisone acetate, up to 25 mg
J1710	Injection, hydrocortisone sodium phosphate, up to 50 mg
J1720	Injection, hydrocortisone sodium succinate, up to 100 mg
J1738	Injection, hydrocortisone sodium succinate, up to 100 mg
J1741	Injection, ibuprofen, 100 mg
J1953	Injection, levetiracetam, 10 mg
J2002	Injection, lidocaine hcl in 5% dextrose, 1 mg
J2003	Injection, lidocaine hydrochloride, 1 mg
J2004	Injection, lidocaine hcl with epinephrine, 1 mg
J2401	Injection, chloroprocaine hydrochloride, per 1 mg
J2402	Injection, chloroprocaine hydrochloride (clorotekal), per 1 mg
J2560	Injection, phenobarbital sodium, up to 120 mg
J2795	Injection, ropivacaine hydrochloride, 1 mg
J2919	Injection, methylprednisolone sodium succinate, 5 mg
J3299	Injection, triamcinolone acetonide (xipere), 1 mg
J3300	Injection, triamcinolone acetonide, preservative free, 1 mg
J3301	Injection, triamcinolone acetonide, not otherwise specified, 10
J3302	Injection, triamcinolone diacetate, per 5 mg
J3303	Injection, triamcinolone hexacetonide, per 5 mg
J3304	Injection, triamcinolone acetonide, preservative-free, extended-release, microsphere formulation, 1 mg
J7509	Methylprednisolone oral, per 4 mg
J7510	Prednisolone oral, per 5 mg
J7512	Prednisone, immediate release or delayed release, oral, 1 mg
J8540	Dexamethasone, oral, 0.25 mg
J8541	Dexamethasone (hemady), oral, 0.25 mg
Q2009	Injection, fosphenytoin, 50 mg phenytoin equivalent
S0078	Injection, fosphenytoin sodium, 750 mg
ICD-10 codes covered if selection criteria are met [Eagle's and Ernest Syndrome]:
H92.01 - H92.09	Otalgia
I65.21 – I65.29	Occlusion and stenosis of carotid artery [covered for vascular Eagle syndrome]
K11.7	Disturbances of salivary secretion
M54.2	Cervicalgia
M89.8X8	Other specified disorders of bone, other site
R07.0	Pain in throat
R09.A2	Foreign body sensation, throat
R13.19	Other dysphagia
R42	Dizziness and giddiness
Z79.01	Long term (current) use of anticoagulants
ICD-10 codes not covered for indications listed in the CPB:
G93.2	Benign intracranial hypertension
H93.A1 - H93.A9	Pulsatile tinnitus
H93.11 - H93.19	Tinnitus
K00.0 - K08.9	Dental problems
M26.601 - M26.69	Temporomandibular joint disorders
R41.89	Other symptoms and signs involving cognitive functions and awareness [Brain fog]
R51.9	Headache, unspecified
R53.82	Chronic fatigue, unspecified

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Background

Ernest or Eagle's syndrome, a problem similar to temporo-mandibular joint pain, involves the stylomandibular ligament, a structure that connects the styloid process at base of the skull with the hyoid bone.  Symptoms of Ernest syndrome, in decreasing order of occurrence, are: temporo-mandibular joint and temporal pain, ear and mandibular pain, posterior tooth sensitivity, eye pain, and throat pain (especially when swallowing). 

Ernest syndrome, or insertional tendinosis of the stylomandibular joint, is diagnosed by history of pain in the preauricular region or the mandible, with associated eye pain, jaw clicking or locking, and pain radiating to the neck, shoulder, or eye on the same side. Pain can be reproduced by palpation of the ligament. Conservative treatments include soft diet, medications, physiotherapy, and local injections. Treatment of Ernest syndrome is successful about 80 % of the time.

There are anecdotal reports that radiofrequency thermoneurolysis is effective in treating symptoms of Ernest syndrome.  However, these studies lack scientific rigor and the claims of effectiveness have not been validated in the peer-reviewed medical literature.

Surgical treatment of Eagle's syndrome includes styloidectomy (removal of the elongated portion of the styloid process). Mupparapu and Robinson (2005) stated that Eagle's syndrome refers to pain and discomfort in the cervico-facial region resulting specifically from the elongated styloid process.  Surgical shortening may be the only treatment that will alleviate the patient's symptoms.

Bargiel et al (2023) noted that stylohyoid syndrome, known as classical Eagle Syndrome (cES), is characterized by calcification of the stylohyoid chain with numerous non-specific symptoms, mainly pain.  These investigators introduced minimally invasive cervical styloidectomy (MICS).  MICS was carried out on 65 patients diagnosed with cES.  Patients underwent meticulous differential diagnosis.  Surgical plans were established based on the findings from neck computed tomography angiography (angioCT).  The healing process was uneventful, without significant complications.  The overall success rate was 97.0 %, with a follow-up of a minimum of 6 months.  In 1 case, the surgery did not yield the desired improvement.  In 1 case, a partial relapse of symptoms was observed.  The authors concluded that MICS is a straight-forward and efficient surgical technique for the treatment of cES.

Campisi set al (2024) carried out a systematic review of the existing literature on styloidectomy performed via transoral robotic surgery (TORS) in ES.  Two independent reviewers carried out a systematic review of PubMed and Embase databases, searching for studies on TORS carried out for ES treatment.  The search was performed in July 2023.  The review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.  The review included a total of 17 adult patients, comprising 5 men and 12 women, with an average age of 52.2 years, all diagnosed with ES.  For each patient, these investigators examined the overall length of the styloid process, the affected side, total intervention duration, hospital length of stay (LOS), pre- and post-operative visual analog scale (VAS) scores, and the presence of minor and major complications.  These researchers identified 4 studies describing 17 instances of TORS as a surgical treatment for ES in the literature, totaling 18 styloidectomies.  The average operation time, inclusive of the docking phase, was 68.8 mins.  A total of 16 patients (94.1 % of the total) experienced complete symptom disappearance or near-complete resolution following surgery; 1 patient (5.9 %) showed improvement categorized as “non-meaningful”.  Only 1 case of minor complication was reported among the 17 procedures (5.9 %).

Fitzpatrick, et al. (2020) explained that Eagle Syndrome (ES) is a rare disorder that can present with symptoms ranging from globus sensation to otalgia that is attributed to an elongated styloid process and/or calcified stylohyoid ligament. No standardized treatment algorithm exists, and although various surgical approaches have been described, data on the use of transoral robotic surgery (TORS) in this population is limited. To investigate the utility of TORS in the treatment of ES, a retrospective review in 19 ES patients was carried out at a single academic, tertiary medical center between 2000 and 2017. Nineteen patients underwent twenty-one styloid resections: 6 performed via TORS and 15 via transcervical approach. Across all patients, 90% reported some degree of lasting improvement in symptoms while 55% reported significant improvement. When TORS was compared to transcervical resection, there was no difference in the subjective rate of "meaningful" (83 vs. 57%) versus rate of "non-meaningful" symptom improvement (17 vs. 43%) (p = 0.35). There was a trend towards less estimated blood loss (EBL), operative time, and post-operative length of stay (LOS) with TORS versus transcervical cases (9.2 mL vs. 30.0 mL, 98 vs. 156 min, and 0.7 vs. 1.2 days); however, these did not reach statistical significance (p = .11, 0.13, and 0.42, respectively). Three patients experienced complications associated with an open approach, as compared to none with TORS. The authors concluded that, in select patients, TORS styloidectomy is a reasonable surgical alternative to traditional transoral and transcervical techniques as it provides similar symptom improvement, and reduced length of stay, blood loss, and operative time.

Egierska, et al. (2021) reported that Eagle's syndrome is a condition of orofacial pain related to the elongated styloid process or the calcification of the stylohyoid ligament. It affects around 4-10% of the population. The average length of the styloid process is about 15,2-47 mm, over 30 mm may be the cause of the Eagle's syndrome. The authors searched PubMed and Google Scholar using search terms Eagle's syndrome, styloid process, stylohyoid ligament, orofacial pain. They manually searched the references of selected articles for additional relevant articles. They selected articles relevant to a general medicine readership, prioritizing systematic reviews, cases and clinical practice guidelines. They stated that the literature contains the latest reports on the Eagle's syndrome. The researches, in which orofacial pain or other symptoms were connected to other disorders, have been rejected. Symptoms of Eagle's syndrome are: odynophagia, dysphagia, dysphonia, dizziness or difficulty in swallowing. Clinicians should pay attention to Eagle's syndrome in the differentiation. Treatment includes conservative approach such as NSAIDs, anticonvulsants, antidepressants, steroids, surgical approach as the main form of the treatment and manual therapy. Eagle's syndrome symptoms may be problematic to diagnose and what is the most important, they worsens the quality of patients' life. They noted that clinicians should pay attention to differentiation with other diseases.

Taneja, et al. (2023) wrote that stylalgia also referred to as Eagle's or stylohyoid syndrome (first described by Watt Eagle) is a constellation of signs secondary to an elongated styloid process or due to mineralization of the stylohyoid or stylomandibular ligament or the posterior belly of the digastric muscle (citing Malik et al., 2018). The Eagle's syndrome includes symptoms ranging from stylalgia (i.e., pain in the tonsillar fossa, pharyngeal or hyoid region) to foreign-body sensation in the throat, cervicofacial pain, otalgia or even increased salivation or giddiness. Diagnosis is primarily based on symptomatology, physical examination and radiographic investigations and should not be missed. However, palpation of tonsillar fossa with radiological demonstration of the elongated styloid process confirms the diagnosis. Surgical treatment by tonsillo-styloidectomy produces satisfactory results in stylalgia (citing Malik et al., 2018).

Pigache, et al. (2018) said that Eagle's syndrome is a rare disease responsible for polymorphic head and neck symptoms, often resulting in delayed diagnosis. The diagnosis of Eagle's syndrome is based on the presence of suggestive clinical signs associated with pain on palpation of the styloid process in the tonsillar fossa, a positive lidocaine test and elongation of the styloid process on 3D CT scan. The most commonly proposed curative treatment is styloidectomy, which allows complete resolution of symptoms in the great majority of cases. This procedure can be performed via a transoral approach or a transcervical approach. After briefly reviewing the embryology and anatomy of the styloid diaphragm region, the authors described the transcervical styloidectomy technique performed in their department. They stated that this simple technique is based on anatomical imperatives designed to limit the operating time and avoid damage to neurovascular structures.

Waters, et al. (2019) sought to define patient demographics and common symptoms in patients who undergo styloidectomy for stylohyoid pain syndrome (Eagle's syndrome), and to evaluate the effectiveness of styloidectomy in reducing symptoms of Eagle's syndrome. The authors retrospectively gathered demographic data on all patients at a single institution who underwent styloidectomy during a 5-year period. Using a patient symptom survey, they also gathered prospective data on a cohort of these patients presenting during the second half of the timeframe. Thirty-two patients underwent styloidectomy for Eagle's syndrome between November 2010 and June 2015. Of these patients, 22 (68.8%) were female, 29 (90.6%) were Caucasian, and 10 (31.3%) reported history of tonsillectomy. Mean age was 46.0 years, and mean BMI was 26.1 kg/m2. Nineteen patients completed the prospective survey. Average styloid length was 45.3 mm. Most severe preoperative symptoms were neck pain, otalgia, globus, facial pain, headache, and discomfort with neck turning. Thirteen of 17 individual symptoms demonstrated significant decrease in symptom scores after styloidectomy. Aggregate symptom scores also showed significant decrease postsurgically. Longer styloid length correlated with increased scores for dysphagia and odynophagia but not with conglomerate symptom scores. The authors summarized that, in this series, patients with Eagle's syndrome were mostly female, Caucasian, and had near-normal BMI. The observed that there is wide variability in presenting symptoms of Eagle's syndrome, but nearly all demonstrate improvement after styloidectomy. Thus, in appropriately selected patients, styloidectomy can effectively and reliably produce improvement in patient symptoms.

Wang, et al. (2022) stated that  Eagle's syndrome is not uncommon in clinical work. Because of its atypical symptoms, it is easy to be misdiagnosed as other diseases, further leading to misdiagnosis and mistreatment. At present, there is no expert consensus or treatment guidelines for the disease. The investigators evaluated the clinical characteristics and postoperative efficacy of 103 patients with Eagle's syndrome based on their clinical symptoms, radiological studies, and physical examination. Through the multicenter clinical study of Tongji Medical College and Dalian Medical University, they found some characteristics of Eagle's syndrome in operation and imaging. In total, 103 patients with Eagle's syndrome (treated from January 2010 to January 2020) were retrospectively enrolled. The postoperative curative effect was analyzed by three surgical methods: styloid process resection through the external cervical approach (styloid process could not be touched through the mouth or could be touched under the jaw or when the CT scan showed that the inclination angle was not large), tonsillectomy + styloidectomy, and preservation of the tonsil for styloidectomy (the styloid process bone could be touched directly during intraoral palpation or in whom the distal part of the styloid process could not be directly touched, but the CT scan showed that the bone inclined toward the oropharynx and its distal part was relatively close to the oropharynx cavity; whether tonsillectomy was performed depended on whether the patient's tonsil was too large to affect the surgical incision). According to the Quality of Well-Being Scale (QWB), they calculated the W value of the scale before operation and 30 days, 3 months, 6 months and 12 months after operation, and compared the W value of each group. The average length of the styloid process was 33 mm (range 25-61 mm). The patients were followed up for 12-36 months (average 15 months). Of the 103 patients, 21 underwent styloid process resection through the external cervical approach, 49 underwent tonsillectomy and styloidectomy, and 33 underwent styloidectomy with preservation of the tonsil. The treatment cured 48 (46%) cases, was effective in 35 (34%) and was ineffective in 20 (20%). The R language 3.6.3 software was used to perform the nonparametric rank sum test, differences in characteristics between groups were analyzed using the Kruskal-Wallis test with Dunn post hoc tests ( R package FSA) for categorical variables, and there was no significant difference between the three types of operations (H = 0.491, P = 0.782). QWB showed that the quality of life after operation was improved compared with that before operation. The investigators concluded that the operation is an effective method for treating Eagle's syndrome. There were no significant differences between the effects of the intraoral and external cervical approaches. Imaging examination-especially CT scanning and 3D reconstruction of the styloid process-is very helpful for diagnosis, but not an absolute criterion for the selection of surgery protocol.

Kumar, et al. (2022) said that Eagle's Syndrome is a much discussed yet controversial and debatable diagnosis of exclusion which is treated by many specialities with often unsatisfactory results. Due to entrapment/impingement on surrounding neurovascular structures by elongated styloid process patient may present with multitude of symptoms. Treatment is controversial and opinions are divided on choice of conservative and surgical management. Aim was to study outcomes of conservative and surgical modalities of treatment of Eagle's Syndrome and bring some clarity on management, what to offer, to whom and when. The authors reported on a prospective observational descriptive study which included 15 patients of Eagle's Syndrome, 7 of whom were treated with conservative method and 8 underwent resection of styloid process with intraoral approach. With objectives in mind to study efficacy of both management modalities, pain visual analogue scale (VAS) scores were recorded pre-intervention, post-intervention and during follow up on 1, 3 and 6 months and compared. Conservative management resulted in up to 70% reduction in pain VAS scores till 3 months of therapy (mean pre-intervention score being 3.71, 3 months-1, 6 months-1.29), while surgical modality resulted in nearly 99% reduction in mean pain VAS scores up to 3 months and even improved after 6 months (mean pre-intervention score being 6.75, 3 months-0.5, 6 months-0.13). The authors concluded that conservative management provides satisfactory short-term (up to 3 months) results but recurrences are known, while surgical resection of elongated styloid process gives better long-term results (6 months and beyond).

Bargiel, et al. (2024) stated that Stylohyoid syndrome, also known as classical Eagle syndrome (ES), is a rare condition characterized by elongation of the styloid process (SP) or calcification of the stylohyoid chain, presenting numerous non-specific symptoms. Most papers concerning this rare condition are case reports, case studies, or retrospective studies. The authors reported on a retrospective study that delves into the intricacies of Eagle syndrome and evaluates surgical outcomes in patients treated with minimally invasive cervical styloidectomy (MICS). The authors conducted a retrospective study of patients treated due to ES between September 2021 and August 2023. Ninety-seven patients were qualified for MICS. Evaluation before and after surgical treatment was conducted during follow-up visits and by a questionnaire that included various data, such as symptoms, pain intensity, evaluation of healing period, and overall patient satisfaction. The minimum follow-up period was six months. After applying inclusion and exclusion criteria, eighty-four patients were qualified for further assessment. The most common symptoms that subsided completely after treatment were pharyngeal foreign body sensation and pain with dysphagia or odynophagia. The authors stated that the MICS procedure proved to be highly effective, with 94.2% of patients experiencing a significant reduction in pain intensity and 97.1% reporting overall success. All patients were satisfied with the aesthetic outcome of the scar. The length of SP and time onset from the first symptoms to treatment positively correlated with the number, intensity, and time required for symptoms to subside. The authors concluded that  Eagle syndrome should be considered in diagnosing patients with orofacial and cervical pain symptoms, as the time from occurrence of the first symptoms to treatment is crucial. MICS is highly effective and has a low rate of complications in treating ES. Potential scar formation should not be a significant factor when deciding between the intraoral and external approaches.

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References