- Department of Neurosurgery, University of Messina, Messina, Italy
- Department of Neuroradiology, University of Messina, Messina, Italy
Department of Neurosurgery, University of Messina, Messina, Italy
DOI:10.4103/2152-7806.150810Copyright: © 2015 Alafaci C. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Alafaci C, Granata F, Cutugno M, Marino D, Conti A, Tomasello F. Glossopharyngeal neuralgia caused by a complex neurovascular conflict: Case report and review of the literature. Surg Neurol Int 04-Feb-2015;6:19
How to cite this URL: Alafaci C, Granata F, Cutugno M, Marino D, Conti A, Tomasello F. Glossopharyngeal neuralgia caused by a complex neurovascular conflict: Case report and review of the literature. Surg Neurol Int 04-Feb-2015;6:19. Available from: http://sni.wpengine.com/surgicalint_articles/glossopharyngeal-neuralgia-caused-complex-neurovascular-conflict-case-report-review-literature/
Background:Glossopharyngeal neuralgia (GN) is a rare condition characterized by severe, paroxysmal episodes of pain mainly localized to the external ear canal, pharynx, and tongue, usually caused by a neurovascular conflict between postero-inferior cerebellar artery (PICA) and IX cranial nerve. Sometimes there is also a compression of X c.n.
Case Description:We present a case of a 71-year-old female with a 3-year history of intense pain localized in the pharynx and posterior portion of the tongue. Preoperative magnetic resonance imaging (MRI) documented a neurovascular conflict between a loop of PICA and IX left c.n. Surgery was performed through a retrosigmoid craniectomy. The intraoperative findings documented a loop of PICA compressing IX, X, and XI c.n. Microvascular decompression (MVD) of IX c.n. was performed using the interposing technique. No rhizotomy and MVD of the X c.n. was performed. Postoperative course showed the regression of all symptoms.
Conclusions:The surgical treatment of patients with GN caused by complex neurovascular conflicts can be safely performed with the classical MVD of IX c.n. A double MVD of both IX and X c.n. has a role only in patients presenting symptoms from both nerves. Rhizotomy, in our opinion, has to be avoided in all cases. The authors review the literature concerning GN caused by complex neurovascular conflicts.
Glossopharyngeal neuralgia (GN) is an uncommon disease characterized by pain and paroxysms along the branches of the ninth cranial nerve (auricular and pharyngeal).[
In clinical practice, GN is often misdiagnosed as trigeminal neuralgia, because of similar clinical features. Clusters of unilateral attacks of sharp, stabbing, and shooting pain localized in the throat radiating to the ear or vice versa are characteristic of GN. The distribution of pain is diagnostic: The pain usually starts from the pharynx, tonsil, and posterior tongue base and then rapidly involves the Eustachian tube and inner ear or spreads to the mandibular angle.[
GN is usually idiopathic, but it can be rarely associated to cerebellopontine angle masses, oropharyngeal tumors, arachnoiditis, stylohyoid ligament ossification, multiple sclerosis and vascular malformations. GN can be isolated or associated to trigeminal neuralgia, or be part of a combined hyperactive dysfunction syndrome.[
The idiopathic type of GN is, usually, caused by a compression of the postero-inferior cerebellar artery (PICA) on the glossopharyngeal nerve as it exits or enters the brainstem.
It is important to make a “clinical” differential diagnosis between idiopathic GN and secondary forms due to inflammation and tumors. In the first case, the neuralgic pain is severe, episodic, lancinating, and of short duration, whereas inflammatory or neoplastic pain is more constant, long-lasting, and of deep-seated quality. The “mapping” of the distribution of the pain has to be performed in order to evaluate if other cranial nerves are involved.
Brain Magnetic Resonance Imaging Angiography (angio-MRI) is the diagnostic procedure in idiopathic GN. Three radiological findings are important to make diagnosis of GN due to vascular compression syndrome: High-origin of PICA, the PICA making upward loop, the PICA coursing and compressing the supraolivary fossette.[
The treatment of choice of GN caused by a neurovascular conflict is microvascular decompression (MVD). Some authors advocate the possibility to perform a rhizotomy of the IX c.n., although this procedure carries the risk of causing sensorial deficits and remains a second-line therapy. The use of rhizotomy for X c.n., even if still under debate, is not considered as a valid option by many authors because of the high risk of dysphagia and vocal cord paralysis.
A 71-year-old female with a 3-year history of intense pain in ingesting solids and liquids was admitted to our Department on October 2013. The patient complained of sudden and violent pain accesses, especially localized at the left side of the pharynx, following the ingestion of solid foods and liquids. These episodes were getting worse in recent months, thus preventing the patient to lead a normal life. Preoperative neurological examination documented a mild left hypoacusia and odynophagia. No symptoms or signs related to compression of the X and XI c.n. were found.
Presurgical MRI examination was performed using a 1.5 T superconductive scanner (Magnetom Siemens Medical Solutions, Erlangen, Germany), gradient strength 26 mT/m, slew rate 200 T/m/ms and head coil. The baseline MRI protocol for neurovascular conflicts included:
Constructive interference in steady-state (CISS-3D) sequence; slice thickness 0.70 mm; two acquisitions; Magnetic resonance angiography performed by a time-of-flight (TOF-3D); slice thickness 0.88 mm; one acquisition.
Constructive interference in steady-state (CISS-3D) sequence; slice thickness 0.70 mm; two acquisitions;
Magnetic resonance angiography performed by a time-of-flight (TOF-3D); slice thickness 0.88 mm; one acquisition.
The CISS-3D and TOF-3D data were transferred to a commercially available independent workstation (Leonardo Workstation, VD 30B, Siemens, Erlangen, Germany). The data sets were processed by the “3D fusion” function of the Leonardo software. In the CISS-3D/TOF-3D fusion images, the nervous structures were automatically displayed in blue and the arterial vessels in deep red.
Presurgical MRI examination showed a significant anomaly of the PICA course. The vessel from its origin moved cranially [
Axial constructive interference in steady-state (CISS-3D) image (a, b). Axial TOF-3D source image (c, d). (a, b) Left PICA (red arrows) impacts IX, X, and XI cranial nerves at root entry or exit zone (REZ) and along the nerves intracisternal tract (black arrows). Vascular and nervous structures are all hypointense. (c, d) Only the tortuous left postero-inferior cerebellar artery (PICA) is visible (red arrows)
MVD was performed via a left suboccipital retrosigmoid approach. The intraoperative findings showed a loop of the PICA compressing the inferior surface of the IX c.n. and the superior surface of the X and XI c.n. [
GN is an uncommon painful disease, often caused by a neurovascular conflict. In such cases, the medical treatment is generally less effective in controlling pain while surgery is the gold standard procedure. However, old patients with some age-related diseases or carrying a high-risk medical status are not candidates for surgery. In such situations, many authors suggest to perform a peripheral glycerol injection, in order to control pain.[
In few cases, GN can be associated to other neurovascular compression syndromes.[
Neurovascular conflict at the level of the root exit zone of cranial nerves IX and X is believed to be the cause of this pain syndrome in most cases. Many authors suggest to perform a vagus nerve rhizotomy for cases in which vascular conflict is not evident.[
Most authors consider suboccipital retromastoid craniectomy as the best approach to surgically treat those syndromes. However, some authors prefer a midline suboccipital subtonsillar approach.[
In cases in which an exploratory surgery does not show any conflict, the rhizotomy can be considered as a curative option. In a large series by Rushton et al.[
Rushton has reported a series of 217 cases of GN treated with sectioning of the IX c.n. alone or combined with X c.n. (in 129 patients). Pain relief was obtained in 100 of these patients. The most common reported postoperative complication was difficulty in swallowing, which occurred in 25 of the 129 patients.[
In a recent review, Rey-Dios and Cohen-Gadol[
With the improvement of microsurgical, anesthesiological and neurophysiological techniques (brainstem-evoked potentials), MVD has proven to be an effective and safe available treatment and should be considered the first-line treatment in idiopathic GN.[
Stylectomy done for elongated styloid process has been promising, once the central causes of GPN have been ruled out[
Recently, various case reports have been published, which have shown beneficial effects of pulsed radiofrequency neurolysis (PRN) and gamma-knife surgery (GKS). PRN is a neuromodulatory, nonablative method to treat both idiopathic and secondary GN in which short pulses of radiofrequency energy, delivered at a constant temperature, produce central and peripheral neuromodulatory effects.[
GN is an uncommon facial pain syndrome often misdiagnosed as trigeminal neuralgia. These aspects often lead to a significant delay in diagnosis. The most important diagnostic examination is brain angio-MRI with specific sequences (CISS and TOF). The use of a software allowing a “fusion” of CISS-3D/TOF-3D images is a useful tool which well depicts the relation between cranial nerves and vascular structures in the cerebello-pontine angle. Microvascular decompression is currently the most effective strategy to treat idiopathic GN. If exploratory surgery does not identify an offending vessel, sectioning cranial nerve IX and the upper rootlets of cranial nerve X is an option. However, this maneuver can lead to dysphagia and vocal cord paralysis and has to be performed only in exceptional cases. In cases where surgery is not possible because of poor conditions of the patients, a peripheral injection of glycerol or SRS are good therapeutic options. We believe that in the typical form of GN caused by a neurovascular conflict offending both IX and X c.n., it is sufficient to perform a MVD in order to decompress the IX c.n., without decompressing also the X c.n. A careful patients selection and a safe operation allows the identification of the site of vascular compression, avoiding cranial nerve X rhizotomy, and, as a consequence, a higher rate of vagus dysfunction. This minimizes the rate of “negative” exploratory operations requiring cranial nerve IX and X rhizotomy.
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