A Case Report of Pediatric Geniculate Neuralgia Treated with Sectioning of the Nervus Intermedius and Microvascular Decompression of Cranial Nerves IX and X

2020 ◽  
Vol 55 (6) ◽  
pp. 439-443
Author(s):  
Georgios Zenonos ◽  
Michael M. McDowell ◽  
Hussam Abou-Al-Shaar ◽  
Kenan Alkhalili ◽  
Paul A. Gardner

<b><i>Background:</i></b> Classic geniculate neuralgia (GN) is a rare condition characterized by lancinating pain centered in the ear and not involving the throat. To the best of our knowledge, no case of pediatric GN has been reported in the English literature. <b><i>Case Presentation:</i></b> We present the first reported case of successfully treated GN in a child via an endoscopic approach. The patient was a 9-year-old boy who presented with a 1-year history of lancinating right ear pain. Neuroleptics resulted in a short-lived improvement in symptoms, but with significant side effects. Extensive evaluation by multiple specialties did not reveal a cause for his pain. Imaging disclosed a tortuous loop of the right posterior inferior cerebellar artery abutting cranial nerves IX and X but no other abnormalities. The patient underwent an endoscopic microvascular decompression of cranial nerves IX and X, and sectioning of the nervus intermedius through a right retromastoid craniotomy. Postoperatively, the patient reported complete resolution of his symptoms that persisted at 3 months of follow-up. At the 5-year follow-up, the patient maintained pain relief and was developing normally. <b><i>Conclusion:</i></b> GN can affect the pediatric population. In carefully selected patients with consistent clinical and radiographic presentation, sectioning of the nervus intermedius and microvascular decompression of the lower cranial nerves can be an effective treatment.

Neurosurgery ◽  
2019 ◽  
Vol 87 (5) ◽  
pp. E573-E577
Author(s):  
C Michael Honey ◽  
Marie T Krüger ◽  
Alan R Rheaume ◽  
Josue M Avecillas-Chasin ◽  
Murray D Morrison ◽  
...  

Abstract BACKGROUND AND IMPORTANCE Hemi-laryngopharyngeal spasm (HeLPS) has been recently described but is not yet widely recognized. Patients describe intermittent coughing and choking and can be cured following microvascular decompression of their Xth cranial nerve. This case report and literature review highlight that HeLPS can co-occur with glossopharyngeal neuralgia (GN) and has been previously described (but not recognized) in the neurosurgical literature. CLINICAL PRESENTATION A patient with GN and additional symptoms compatible with HeLPS is presented. The patient reported left-sided, intermittent, swallow-induced, severe electrical pain radiating from her ear to her throat (GN). She also reported intermittent severe coughing, throat contractions causing a sense of suffocation, and dysphonia (HeLPS). All her symptoms resolved following a left microvascular decompression of a loop of the posterior inferior cerebellar artery that was pulsating against both the IXth and Xth cranial nerves. A review of the senior author's database revealed another patient with this combination of symptoms. An international literature review found 27 patients have been previously described with symptoms of GN and the additional (but not recognized at the time) symptoms of HeLPS. CONCLUSION This review highlights that patients with symptoms compatible with HeLPS have been reported since 1926 in at least 4 languages. This additional evidence supports the growing recognition that HeLPS is another neurovascular compression syndrome. Patients with HeLPS continue to be misdiagnosed as conversion disorder. The increased recognition of this new medical condition will require neurosurgical treatment and should alleviate the suffering of these patients.


1991 ◽  
Vol 75 (3) ◽  
pp. 388-392 ◽  
Author(s):  
Shinji Nagahiro ◽  
Akira Takada ◽  
Yasuhiko Matsukado ◽  
Yukitaka Ushio

✓ To determine the causative factors of unsuccessful microvascular decompression for hemifacial spasm, the follow-up results in 53 patients were assessed retrospectively. The mean follow-up period was 36 months. There were 32 patients who had compression of the seventh cranial nerve ventrocaudally by an anterior inferior cerebellar artery (AICA) or a posterior inferior cerebellar artery. Of these 32 patients, 30 (94%) had excellent postoperative results. Of 14 patients with more severe compression by the vertebral artery, nine (64%) had excellent results, three (21%) had good results, and two (14%) had poor results; in this group, three patients with excellent results experienced transient spasm recurrence. There were seven patients in whom the meatal branch of the AICA coursed between the seventh and eighth cranial nerves and compressed the dorsal aspect of the seventh nerve; this was usually associated with another artery compressing the ventral aspect of the nerve (“sandwich-type” compression). Of these seven patients, five (71%) had poor results including operative failure in one and recurrence of spasm in four. The authors conclude that the clinical outcome was closely related to the patterns of vascular compression.


2020 ◽  
Vol 26 (2) ◽  
pp. 189-192
Author(s):  
Derek D. George ◽  
Thomas S. Ridder

Geniculate neuralgia or nervus intermedius (NI) neuralgia is a rare condition characterized by intermittent, severe, stabbing deep ear pain. The pain can be triggered by stimulation of the external ear and is sometimes accompanied by facial pain. The condition is thought to result, in part, from vascular compression of the NI, although other etiologies exist. To date, fewer than 150 cases have been described in the English-language literature, and only 1 case of surgically treated geniculate neuralgia with microvascular decompression (MVD) of cranial nerves VIII, IX, and X has been described in a pediatric patient. Here, the authors present the case of an adolescent boy with bilateral geniculate neuralgia treated at two different time points with sectioning of the NI and MVD.


2021 ◽  
Author(s):  
Keaton Piper ◽  
Qizhi Victoria Zheng ◽  
Robert S Heller ◽  
Siviero Agazzi

Abstract BACKGROUND AND IMPORTANCE Geniculate neuralgia is a rare condition characterized by excruciating ear pain. Surgical options for geniculate neuralgia include microvascular decompression and sectioning of the nervus intermedius. We report herein a case of bilateral geniculate neuralgia treated by nervus intermedius sectioning without prior microvascular decompression. To our knowledge, this is the first report of this treatment strategy with a subsequent description of the side effects of bilateral nervus intermedius disruption. CLINICAL PRESENTATION A 54-yr-old woman presented with bilateral geniculate neuralgia, worse on the left, refractory to medical therapy. Surgical treatment options were reviewed, including microvascular decompression and sectioning of the nervus intermedius. She opted for left nervus intermedius sectioning. The procedure was uncomplicated and no compressive vascular loop was identified during surgery. Postoperatively, she had complete symptom resolution with no discernable side effects. Three years later, the patient developed worsening geniculate neuralgia on the contralateral side. After the discussion of treatment options, she opted again for sectioning of the contralateral nervus intermedius with successful resolution of all symptoms after surgery. Following surgery, the patient identified partial impairment of lacrimation and gustation. She continued to have functional taste of the anterior two-thirds of the tongue, lacrimation, and hearing bilaterally. CONCLUSION Bilateral sectioning of nervus intermedius may provide benefit in patients with bilateral geniculate neuralgia without egregious side effects. However, lacrimatory and gustatory alterations are a potentially significant side effect with a wide range of symptomatology.


2018 ◽  
Vol 80 (S 03) ◽  
pp. S322-S322
Author(s):  
Vincent N. Nguyen ◽  
Jaafar Basma ◽  
Jeffrey Sorenson ◽  
L. Madison Michael

Objectives To describe a retrosigmoid approach for the microvascular sectioning of the nervus intermedius and decompression of the 5th and 9th cranial nerves, with emphasis on microsurgical anatomy and technique. Design A retrosigmoid craniectomy is performed in the lateral decubitus position. The dura is opened and cerebrospinal fluid (CSF) is released from the cisterna magna and cerebellopontine cistern. Dynamic retraction without rigid retractors is performed. Subarachnoid dissection of the cerebellopontine angle exposes the 7th to 8th nerve complex. A neuromonitoring probe is used with careful inspection of the microsurgical anatomy to identify the facial nerve and the nervus intermedius as they enter the internal auditory meatus. The nervus intermedius is severed. A large vein coursing superiorly across cranial 9th nerve was coagulated and cut. A Teflon pledget is inserted between a small vessel and the 5th nerve. Photographs of the region are borrowed from Dr. Rhoton's laboratory to illustrate the microsurgical anatomy. Participants The senior author performed the surgery. The video was edited by Drs. V.N. and J.B. Outcome Measures Outcome was assessed by postoperative neurological function. Results The nervus intermedius was successfully cut and the 5th and 9th nerves were decompressed. The patient's pain resolved after surgery and at later follow-up. Conclusions Understanding the microsurgical anatomy of the cerebellopontine angle is necessary to identify the cranial nerves involved in facial pain syndromes. Subarachnoid dissection and meticulous microsurgical techniques are key elements for a successful microvascular decompression.The link to the video can be found at: https://youtu.be/pV5Wip7WusE.


2020 ◽  
Vol 5 (4) ◽  
pp. 2473011420S0029
Author(s):  
Jaeyoung Kim ◽  
Jonathan Day ◽  
Woo-Chun Lee

Category: Midfoot/Forefoot; Other Introduction/Purpose: Coalition of the naviculo-medial cuneiform joint (NCJ) is a relatively rare condition among the tarsal bone coalitions. Thus, optimal treatment is still largely unknown. There is a paucity of literature, with few cases documenting arthrodesis of the NCJ in adults with varied outcomes. As the NCJ contributes to the majority of motion along the medial column of the foot, arthrodesis of the joint may cause excessive stress on adjacent joints. Furthermore, the nonunion rate of NCJ is reportedly high, ranging from 3 to 15%. The purpose of this study was to report the outcomes of simple coalition bar excision in patients with NCJ coalition. Additionally, we investigated preoperative abnormal conditions around the NCJ using weight bearing computed tomography (WBCT). Methods: We retrospectively identified 21 feet in 18 prospectively followed patients from 2010 to 2018 who underwent simple coalition bar excision of NCJ in our institution. Chart review was performed to retrieve demographic data of the patients, clinical presentation findings, and concomitant procedures with coalition bar excision. Radiographically, the location and morphological pattern of the coalition were analyzed. Several angular parameters including medial arch sag angle (MASA) were measured on weightbearing x-rays to see if there are any angular collapse at NCJ after coalition bar excision (Figure 1). The existence of abnormal conditions adjacent to the NCJ such as arthritis of the first and second tarsometatarsal joint (TMTJ) and talonavicular joint (TNJ) were assessed using WBCT (n=17). Clinically, pre- and postoperative visual analogue scale (VAS) and foot function index (FFI) were compared to assess for improvement in patient-reported outcomes. Results: The mean age of the patients was 30.9 years (range, 16-62) and the follow-up was 15.9 months (range, 12-24). Majority of the patient had fibrous coalition at the plantar-medial aspect and only one patient had bony coalition. The morphology of fibrous coalition was classified as irregular (n=8), cystic (n=1), and combined (n=11) based on CT findings. Intraoperatively, the motion of the NCJ was identified in every patient after coalition bar excision. WBCT revealed 15 feet (71.4%) having at least one abnormal finding around the NCJ (First TMTJ plantar gap; n=10, second TMTJ narrowing; n=9, first TMTJ spur & irregularity; n=2, TNJ spur; n=1). Pre- and postoperative MASA did not change significantly (p=0.932). There was significant improvement in VAS and FFI at final follow-up (p<0.001) Conclusion: A considerable proportion of patients with NCJ coalition had at least one radiographically arthritic feature at adjacent joints preoperatively, which may be caused by the restriction in motion associated with NJC coalition. Simple coalition bar excision in adults resulted in satisfactory outcomes without NC joint angular deterioration, while restoring motion at the joint.


Neurosurgery ◽  
2005 ◽  
Vol 57 (2) ◽  
pp. 415-415 ◽  
Author(s):  
Kenneth F. Casey ◽  
Peter J. Jannetta ◽  
Ricky E. Kortyna ◽  
Emily Belz

2021 ◽  
Author(s):  
Ehab El Refaee ◽  
Steffen Fleck ◽  
Marc Matthes ◽  
Sascha Marx ◽  
Joerg Baldauf ◽  
...  

Abstract BACKGROUND Microvascular decompression (MVD) is the most effective treatment option for hemifacial spasm (HFS). However, deeply located forms of compression would require proper identification to allow for adequate decompression. OBJECTIVE To describe the usefulness of endoscopic visualization in one of the most challenging compression patterns in HFS, where the posterior inferior cerebellar artery (PICA) loop is severely indenting the brain stem at the proximal root exit zone of facial nerve along the pontomedullary sulcus. METHODS Radiological and operative data were checked for all patients in whom severe indentation of the brainstem by PICA at pontomedullary sulcus was recorded and endoscope-assisted MVD was performed. Clinical correlation and outcome were analyzed. RESULTS A total of 58 patients with HFS were identified with radiological and surgical evidence proving brainstem indentation at the VII transitional zone. In 31 patients, PICA was the offending vessel to the facial nerve. In 3 patients, the PICA loop was mobilized under visualization of a 45° endoscope. A total of 31 patients had a mean follow-up duration of 52.1 mo. The mean duration between start of complaints and surgery was 7.2 yr. In the last follow-up, all patients had remarkable spasm improvement. A total of 5 patients had more than 90% disappearance of spasms and 26 patients experienced spasm-free outcome. CONCLUSION Although severe indentation of brain stem implies morphological damage, outcome after MVD is excellent. A 45° endoscope is extremely helpful to identify compression down at the pontomedullary sulcus. Deeply located compression site can easily be missed with microscopic inspection alone.


Neurosurgery ◽  
1989 ◽  
Vol 24 (2) ◽  
pp. 257-263 ◽  
Author(s):  
Aage R. Møller ◽  
Margareta B. Møller

Abstract During a 14-month period, 129 individuals underwent 140 operations for microvascular decompression to relieve hemifacial spasm, disabling positional vertigo, tinnitus, or trigeminal neuralgia at our institution. Seven patients were operated upon twice on the same side and 4 were operated upon on both sides at different times. In each case, the brainstem auditory evoked potentials were monitored intraoperatively by the same neurophysiologist. In 75 of these operations, compound action potentials were also recorded from the exposed 8th nerve. Comparison of speech discrimination scores before the operation and at the time fo discharge showed that at discharge, discrimination had decreased in 7 patients by 15% or more and increased in 4 patients by 15% or more, in 2 patients by as much as 52%. Essentially similar results were obtained when preoperative speech discrimination scores were compared with results obtained from the 87 patients who returned for a follow-up visit between 3 and 6 months after discharge. Only one patient lost hearing (during a second operation to relieve hemifacial spasm). Another patient (also operated upon to relieve hemifacial spasm) suffered noticeable hearing loss postoperatively, but had recovered nearly normal hearing by 4 months after the operation. Nine patients had an average elevation of the hearing threshold for pure tones in the speech frequency range (500 to 2000 Hz) of 11 dB or more at 4 to 5 days after the operation; 8 of these had fluid in their middle ears that most likely contributed to the hearing loss. Threshold elevations occurred at 4000 Hz and 8000 Hz in 19 and 29 ears, respectively.


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