According to the definition of the International Headache Society (IHS) 3rd Edition (ICHD–3), the diagnostic criteria for occipital neuralgia (ON)are as follows: A. Paroxysmal stabbing pain, with or without persistent aching between paroxysms, in the distribution of the greater, lesser, and/or third occipital nerve B. Tenderness over the affected nerve C. Pain is eased temporarily by a local anesthetic block of the nerve [1]. Hence, the diagnosis of ON mainly relies on the scope, properties, and characteristics of the pain. ON usually originates from the suboccipital region and spreads throughout the back of the head. However, the pain can involve other areas, such as the retro-orbital area, due to the convergence of the nucleus trigeminus pars caudalis [8, 9]. This pain is usually unilateral, but bilateral exits. During physical examination, tenderness is typically detected by palpation. Besides, Tinel and pillow signs may be evoked [2]. All three cases in our series fulfilled these characteristics. For example, the pain extended to the periorbital region in case 2. Tinel and pillow signs were apparent during her physical examination.
The treatment algorithm for ON should generally be in accordance with the ladder principle. That is to say, conservative therapy involving physical and drug medications should initially be considered. Patients with symptoms refractory to conservative therapy may be treated with percutaneous anesthetic injections with/without steroid medications. However, most C2 nerve blocks commonly provide short-term pain relief, with approximately 15–36% sustaining extended relief for several months [10]. In our series, the three patients had undergone C2 nerve blocks more than once in other hospitals without a satisfactory duration of pain relief. The procedure can be performed either distally at the nuchal line (commonly used method) or proximally between C1 and C2, where the C2 ganglion is located [11]. We recommend the latter before C2 ganglionectomy, given that it can more accurately reflect the effect of C2 ganglionectomy in theory. All three patients underwent C2 ganglion block, and the results were favorable.
Pulsed radiofrequency (PRF) as a treatment for occipital neuralgia has yielded promising clinical results, although evidence validating its long-term effects is lacking [12–14]. This minimally invasive surgical option may be considered for patients failing conservative and injection therapies. As a minimally invasive percutaneous technique, PRF therapy exposes the nerve to high-voltage radiofrequency pulses, which is speculated to induce an inhibitory electrical field around the sensory nerves, disrupting pain transmission and potentiation with none to minimal neurodestruction [15]. Herein, the three patients had undergone PRF in other hospitals before hospitalization in our hospital. Among them, case 2 had the longest period of pain relief, lasting around 3 months.
Occipital nerve stimulation (ONS) is an alternative treatment option for patients with medically refractory ON [16–17]. The Congress of Neurological Surgeons 2015 Guideline recommends the use of ONS for the treatment of intractable occipital neuralgia (level III) [18]. ONS affects the subcutaneous insertion of electrodes in the C1/C2 regions of the posterior cervical spine. It is attractive as a non-destructive, reversible therapeutic approach with long-term efficacy. However, several potential complications related to ONS include lead migration, electrode fractures, hardware erosions, disconnections, and sepsis [2, 19, 20]. Moreover, some other factors limit its application, such as high medical costs, lack of medical insurance coverage in some regions, and the patient's religious belief that implants are not acceptable in their body. In our three cases, we recommended ONS to all the patients and thoroughly outlined the pros and cons of this technique. Case 1 did not consent to ONS because of her religious belief, and the other two cases did not choose this approach given its exorbitant cost.
Patients with symptoms refractory to the above medical procedures may be subjected to increasingly invasive surgical modalities. The ideal surgical management remains to be elucidated. Alternative surgical methods include occipital nerve decompression [21], neuroablation [12, 13, 22], partial rhizotomy [20], peripheral neurolysis [24], and C2 to C3 root decompression [25]. However, these procedures are commonly associated with pain recurrence from aberrant postoperative neuronal regeneration and neuroma formation [2, 5, 23, 25].
C2 dorsal root ganglionectomy was developed to address the failures of the above procedures [4–6, 25]. For patients with intractable ON, C2 dorsal root ganglionectomy is a viable surgical option. Theoretically, the removal of the primary sensory neurons residing in the C2 ganglion is more conducive to preventing pain recurrence [2, 5, 25–26]. Previous pieces of literature have established that this procedure is successful in treating the majority of intractable ON.
However, traditional C2 ganglionectomy is performed under microscopy [4, 5, 25], which necessitates a wide incision due to the deep location of the C2 ganglion. The incision of the skin usually extends from the occiput to the spinous process of C3 or C4. A large amount of paraspinal muscle is stripped from the bone to expose the C1 posterior arch and C2 dorsal element [4, 5], which may result in surgery-induced neck pain owing to intraoperative iatrogenic damage and postoperative scar of muscle tissues. Occasionally, surgery-induced neck pain can often be difficult to distinguish from preoperative occipital neuralgia in patients, influencing the evaluation of the effectiveness of surgery. In addition, brisk bleeding from an exuberant venous plexus enfolding the C-2 ganglion is commonly encountered during open surgery. It usually requires meticulous and extensive electrocoagulation, which also induces damage to adjacent nerve branches.
These shortcomings may be overcome by virtue of the properties of the percutaneous full-endoscopic surgery technique. The skin incision is less than 8 mm, without the need to strip the paravertebral muscles. Through the slim working cannula, the camera’s eye with 30 view angles can be inserted millimeters away from the C2 ganglion, delivering high-definition images for the operator on a monitor. All actions are executed through the working channel under excellent endoscopic visualization without requiring a wide wound exposure. Hence, postoperative surgery-induced neck pain can be alleviated. Moreover, the surgical procedure is performed under continuous irrigation. The pressure of the irrigation fluid can help minimize bleeding from the venous plexus enfolding the C2 ganglion. Extensive cauterization is not required, reducing injury to adjacent nerve branches. In our case series, all the patients experienced a satisfactory therapeutic response. The VAS scores were maintained at low levels after a 1-year follow-up, further corroborating the validity and merits of percutaneous full-endoscopic C2 dorsal root ganglionectomy.
There are two key steps in percutaneous full-endoscopic C2 ganglionectomy. The first is to locate and confirm the location of the C2 ganglion, and the second is to effectively and safely excise the ganglion under endoscopy. In our previous study, we introduced the “pedicle guidance technique” to accomplish the first objective [7], that is, by following the superior edge of the C2 pedicle, the operator can reach the lateral atlantoaxial articulation, behind which the C2 ganglion is located. The ganglion can be identified as a large bulge about 4 mm away from the dural sac and proximal to the C2 primary rami (Fig. 3A). Endoscopic spine surgery is characterized by one hand and one instrument, unlike microscopy surgery.
Understanding the characteristic mentioned above is critical to accomplishing the second goal. Therefore, when firstly cutting the distal end of the ganglion, the distal end should not be completely excised. The reserved partial connection can maintain the ganglion in situ (Fig. 3B). Otherwise, the ganglion will float in the water flow, leading to challenges in accurate proximal resection.
Our experience has proved that percutaneous full-endoscopic C2 ganglionectomy can be successfully accomplished. It converted an open surgery into a percutaneous puncture surgery. This will make it easier for surgeons to choose among the alternative surgical options for ON while also increasing “Return on Investment”. The primary limitations of this study were its retrospective nature, the limited sample size, and its relatively short follow-up period. Despite the aforementioned limitations, we believe that our description of the operative nuances about endoscopic C2 ganglionectomy and the introduction of clinical features about ON cases will assist in clinical decision-making. In the future, a larger number of cases and more strictly designed experiments are needed to corroborate that the “returns” have definitely been increased.