Ernest or Eagle's Syndrome (Stylomandibular Ligament Pain): Treatment with Radiofrequency Thermoneurolysis

Number: 0400

Table Of Contents

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

Policy

Scope of Policy

This Clinical Policy Bulletin addresses Ernest or Eagle's syndrome (stylomandibular ligament pain): treatment with radiofrequency thermoneurolysis.

  1. Experimental, Investigational, or Unproven

    Radiofrequency (RF) thermoneurolysis (also known as RF ablation, RF neurolysis, RF thermoablation) is considered experimental, investigational, or unproven for the treatment of Ernest or Eagle's syndrome because the effectiveness of this approach in treating this condition has not been established.

  2. Related Policies

Table:

CPT Codes / HCPCS Codes / ICD-10 Codes

Code Code Description

Information in the [brackets] below has been added for clarification purposes.   Codes requiring a 7th character are represented by "+":

Radiofrequency thermoneurolysis (also known as radiofrequency ablation, radiofrequency neurolysis, radiofrequency thermoablation):
No specific code

Other CPT codes related to the CPB:
64600 Destruction by neurolytic agent, trigeminal nerve; supraorbital, infraorbital, mental, or inferior alveolar branch

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).  Treatment of Ernest syndrome, which is successful about 80 % of the time, includes a soft diet, use of an intra-oral splint, physiotherapy, medication and injections of local anesthetics at the insertion of the ligament.

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 %).

References

The above policy is based on the following references:

  1. Baba A, Okuyama Y, Ojiri H, Nakajima T. Eagle syndrome. Clin Case Rep. 2017;5(2):201-202.
  2. Bargiel J, Gontarz M, Marecik T, et al. Minimally invasive cervical styloidectomy in stylohyoid syndrome (Eagle syndrome). J Clin Med. 2023;12(21):6763.
  3. Brown CR. Ernest syndrome: Insertion tendinosis of the stylomandibular ligament. Pract Periodontics Aesthet Dent. 1996;8(8):762.
  4. Campisi R, Caranti A, Meccariello G, et al. Transoral robotic styloidectomy for Eagle syndrome: A systematic review. Clin Otolaryngol. 2024 Jan 30 [Online ahead of print].
  5. Dominguez J, Bornhardt T, Wen S, Iturriaga V. Ernest syndrome: A systematic review of the literature. J Oral Facial Pain Headache. 2020;34(2):167-173.
  6. DuPont JS Jr. Panoramic imaging of the stylohyoid complex in patients with suspected Ernest or Eagle's syndrome. Cranio. 1998;16(1):60-63.
  7. Elimairi I, Baur DA, Altay MA, et al. Eagle's syndrome. Head Neck Pathol. 2015;9(4):492-495.
  8. Ferreira MS, Miranda G, Almeida FT, et al. Complications in intraoral versus external approach for surgical treatment of Eagle syndrome: A systematic review and meta-analysis. Cranio. 2022 Jan 10 [Online ahead of print].
  9. Han MK, Kim do W, Yang JY. Non surgical treatment of Eagle's syndrome - A case report. Korean J Pain. 2013;26(2):169-172.
  10. Jose A, Rawat A, Nagori SA. Insertion tendinosis of stylomandibular ligament: Ernest syndrome. J Craniofac Surg. 2021;32(3):e251-e253.
  11. Kumai Y, Hamasaki T, Yumoto E. Surgical management of Eagle's syndrome: An approach to shooting craniofacial pain. Eur Arch Otorhinolaryngol. 2016;273(10):3421-3427.
  12. Malik JN, Monga S, Sharma AP, et al. Stylalgia revisited: Clinical profile and management. Iran J Otorhinolaryngol. 2018;30(101):335-340.
  13. Mupparapu M, Robinson MD. The mineralized and elongated styloid process: A review of current diagnostic criteria and evaluation strategies. Gen Dent. 2005;53(1):54-59.
  14. Piagkou M, Anagnostopoulou S, Kouladouros K, Piagkos G. Eagle's syndrome: A review of the literature. Clin Anat. 2009;22(5):545-558.
  15. Rinaldi V. Eagle syndrome. eMedicine Surgery. New York, NY: Medscape; updated April 27, 2012.
  16. Shankland WE 2nd. Ernest syndrome as a consequence of stylomandibular ligament injury: A report of 68 patients. J Prosthet Dent. 1987;57(4):501-506.
  17. Taheri A, Firouzi-Marani S, Khoshbin M. Nonsurgical treatment of stylohyoid (Eagle) syndrome: A case report. J Korean Assoc Oral Maxillofac Surg. 2014;40(5):246-249.
  18. Wilk SJ. Surgical management of refractory craniomandibular pain using radiofrequency thermolysis: A report of thirty patients. Cranio. 1994;12(2):93-99.