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Katherine G. Holste, Frances A. Hardaway, Ahmed M. Raslan and Kim J. Burchiel

OBJECTIVE

Nervus intermedius neuralgia (NIN) or geniculate neuralgia is a rare facial pain condition consisting of sharp, lancinating pain deep in the ear and can occur alongside trigeminal neuralgia (TN). Studies on the clinical presentation, intraoperative findings, and ultimately postoperative outcomes are extremely limited. The aim of this study was to examine the clinical presentation and surgical findings, and determine pain-free survival after sectioning of the nervus intermedius (NI).

METHODS

The authors conducted a retrospective chart review and survey of patients who were diagnosed with NIN at one institution and who underwent neurosurgical interventions. Pain-free survival was determined through chart review and phone interviews using a modified facial pain and quality of life questionnaire and represented as Kaplan-Meier curves.

RESULTS

The authors found 15 patients with NIN who underwent microsurgical intervention performed by two surgeons from 2002 to 2016 at a single institution. Fourteen of these patients underwent sectioning of the NI, and 8 of 14 had concomitant TN. Five patients had visible neurovascular compression (NVC) of the NI by the anterior inferior cerebellar artery in most cases where NVC was found. The most common postoperative complaints were dizziness and vertigo, diplopia, ear fullness, tinnitus, and temporary facial nerve palsy. Thirteen of the 14 patients reportedly experienced pain relief immediately after surgery. The mean length of follow-up was 6.41 years (range 8 months to 14.5 years). Overall recurrence of any pain was 42% (6 of 14), and 4 patients (isolated NIN that received NI sectioning alone) reported their pain was the same or worse than before surgery at longest follow-up. The median pain-free survival was 4.82 years ± 14.85 months. The median pain-controlled survival was 6.22 years ± 15.78 months.

CONCLUSIONS

In this retrospective review, sectioning of the NI produced no major complications, such as permanent facial weakness or deafness, and was effective for patients when performed in addition to other procedures. After sectioning of the NI, patients experienced 4.8 years pain free and experienced 6.2 years of less pain than before surgery. Alone, sectioning of the NI was not effective. The pathophysiology of NIN is not entirely understood. It appears that neurovascular compression plays only a minor role in the syndrome and there is a high degree of overlap with TN.

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Zoe E. Teton, Katherine G. Holste, Fran A. Hardaway, Kim J. Burchiel and Ahmed M. Raslan

OBJECTIVE

Glossopharyngeal neuralgia (GN) is a rare pain condition in which patients experience paroxysmal, lancinating throat pain. Multiple surgical approaches have been used to treat this condition, including microvascular decompression (MVD), and sectioning of cranial nerve (CN) IX and the upper rootlets of CN X, or a combination of the two. The aim of this study was to examine the long-term quality of life and pain-free survival after MVD and sectioning of the CN X/IX complex.

METHODS

A combined retrospective chart review and a quality-of-life telephone survey were performed to collect demographic and long-term outcome data. Quality of life was assessed by means of a questionnaire based on a combination of the Barrow Neurological Institute pain intensity scoring criteria and the Brief Pain Inventory–Facial. Kaplan-Meier analysis was performed to determine pain-free survival.

RESULTS

Of 18 patients with GN, 17 underwent sectioning of the CN IX/X complex alone or sectioning and MVD depending on the presence of a compressing vessel. Eleven of 17 patients had compression of CN IX/X by the posterior inferior cerebellar artery, 1 had compression by a vertebral artery, and 5 had no compression. One patient (6%) experienced no immediate pain relief. Fifteen (88%) of 17 patients were pain free at the last follow-up (mean 9.33 years, range 5.16–13 years). One patient (6%) experienced throat pain relapse at 3 months. The median pain-free survival was 7.5 years ± 10.6 months. Nine of 18 patients were contacted by telephone. Of the 17 patients who underwent sectioning of the CN IX/X complex, 13 (77%) patients had short-term complaints: dysphagia (n = 4), hoarseness (n = 4), ipsilateral hearing loss (n = 4), ipsilateral taste loss (n = 2), and dizziness (n = 2) at 2 weeks. Nine patients had persistent side effects at latest follow-up. Eight of 9 telephone respondents reported that they would have the surgery over again.

CONCLUSIONS

Sectioning of the CN IX/X complex with or without MVD of the glossopharyngeal nerve is a safe and effective surgical therapy for GN with initial pain freedom in 94% of patients and an excellent long-term pain relief (mean 7.5 years).

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Fran A. Hardaway, Hanna C. Gustafsson, Katherine Holste, Kim J. Burchiel and Ahmed M. Raslan

OBJECTIVE

Pain relief following microvascular decompression (MVD) for trigeminal neuralgia (TN) may be related to pain type, degree of neurovascular conflict, arterial compression, and location of compression. The objective of this study was to construct a predictive pain-free scoring system based on clinical and radiographic factors that can be used to preoperatively prognosticate long-term outcomes for TN patients following surgical intervention (MVD or internal neurolysis [IN]). It was hypothesized that contributing factors would include pain type, presence of an artery or vein, neurovascular conflict severity, and compression location (root entry zone).

METHODS

At the authors’ institution 275 patients with type 1 or type 2 TN (TN1 or TN2) underwent MVD or IN following preoperative high-resolution brain MRI studies. Outcome data were obtained retrospectively by chart review and/or phone follow-up. Characteristics of neurovascular conflict were obtained from preoperative MRI studies. Factors that resulted in a probability value of < 0.05 on univariate logistic regression analyses were entered into a multivariate Cox regression analysis in a backward stepwise fashion. For the multivariate analysis, significance at the 0.15 level was used. A prognostic system was then devised with 4 possible scores (0, 1, 2, or 3) and pain-free survival analyses conducted.

RESULTS

Univariate predictors of pain-free survival were pain type (p = 0.013), presence of any vessel (p = 0.042), and neurovascular compression severity (p = 0.038). Scores of 0, 1, 2, and 3 were found to be significantly different in regard to pain-free survival (log rank, p = 0.005). At 5 and 10 years there were 36%, 43%, 61%, and 69%, and 36%, 43%, 56%, and 67% pain-free survival rates in groups 0, 1, 2, and 3, respectively. While TN2 patients had worse outcomes regardless of score, a subgroup analysis of TN1 patients with higher neurovascular conflict (score of 3) had significantly better outcomes than TN1 patients without severe neurovascular conflict (score of 1) (log rank, p = 0.005). Regardless of pain type, those patients with severe neurovascular conflict were more likely to have arterial compression (99%) compared to those with low neurovascular conflict (p < 0.001).

CONCLUSIONS

Pain-free survival was predicted by a scoring system based on preoperative clinical and radiographic findings. Higher scores predicted significantly better pain relief than lower scores. TN1 patients with severe neurovascular conflict had the best long-term pain-free outcome.

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Zoe E. Teton, Daniel Blatt, Katherine Holste, Ahmed M. Raslan and Kim J. Burchiel

OBJECTIVE

Hemifacial spasm (HFS), largely caused by neurovascular compression (NVC) of the facial nerve, is a rare condition characterized by paroxysmal, unilateral, involuntary contraction of facial muscles. It has long been suggested that these symptoms are due to compression at the transition zone of the facial nerve. The aim of this study was to examine symptom-free survival and long-term quality of life (QOL) in HFS patients who underwent microvascular decompression (MVD). A secondary aim was to examine the benefit of utilizing fused MRI and MRA post hoc 3D reconstructions to better characterize compression location at the facial nerve root exit zone (fREZ).

METHODS

The authors retrospectively analyzed patients with HFS who underwent MVD at a single institution, combined with a modified HFS-7 telephone questionnaire. Kaplan-Meier analysis was used to determine event-free survival, and the Wilcoxon signed-rank test was used to compare pre- and postoperative HFS-7 scores.

RESULTS

Thirty-five patients underwent MVD for HFS between 2002 and 2018 with subsequent 3D reconstructions of preoperative images. The telephone questionnaire response rate was 71% (25/35). If patients could not be reached by telephone, then the last clinic follow-up date was recorded and any recurrence noted. Twenty-four patients (69%) were symptom free at longest follow-up. The mean length of follow-up was 2.4 years (1 month to 8 years). The mean symptom-free survival time was 44.9 ± 5.8 months, and the average symptom-control survival was 69.1 ± 4.9 months. Four patients (11%) experienced full recurrence. Median HFS-7 scores were reduced by 18 points after surgery (Z = −4.013, p < 0.0001). Three-dimensional reconstructed images demonstrated that NVC most commonly occurred at the attached segment (74%, 26/35) of the facial nerve within the fREZ and least commonly occurred at the traditionally implicated transition zone (6%, 2/35).

CONCLUSIONS

MVD is a safe and effective treatment that significantly improves QOL measures for patients with HFS. The vast majority of patients (31/35, 89%) were symptom free or reported only mild symptoms at longest follow-up. Symptom recurrence, if it occurred, was within the first 2 years of surgery, which has important implications for patient expectations and informed consent. Three-dimensional image reconstruction analysis determined that culprit compression most commonly occurs proximally along the brainstem at the attached segment. The success of this procedure is dependent on recognizing this pattern and decompressing appropriately. Three-dimensional reconstructions were found to provide much clearer characterization of this area than traditional preoperative imaging. Therefore, the authors suggest that use of these reconstructions in the preoperative setting has the potential to help identify appropriate surgical candidates, guide operative planning, and thus improve outcome in patients with HFS.

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Fran A. Hardaway, Katherine Holste, Gulsah Ozturk, David Pettersson, Jeffrey M. Pollock, Kim J. Burchiel and Ahmed M. Raslan

OBJECTIVE

The pathophysiology of trigeminal neuralgia (TN) in patients without neurovascular compression (NVC) is not completely understood. The objective of this retrospective study was to evaluate the hypothesis that TN patients without NVC differ from TN patient with NVC with respect to brain anatomy and demographic characteristics.

METHODS

Six anatomical brain measurements from high-resolution brain MR images were tabulated; anterior-posterior (AP) prepontine cistern length, cerebellopontine angle (CPA) cistern volume, nerve-to-nerve distance, symptomatic nerve length, pons volume, and posterior fossa volume were assessed on OsiriX. Brain MRI anatomical measurements from 232 patients with either TN type 1 or TN type 2 (TN group) were compared with measurements obtained in 100 age- and sex-matched healthy controls (control group). Two-way ANOVA tests were conducted on the 6 measurements relative to group and NVC status. Bonferroni adjustments were used to correct for multiple comparisons. A nonhierarchical k-means cluster analysis was performed on the TN group using age and posterior fossa volume as independent variables.

RESULTS

Within the TN group, females were found to be younger than males and less likely to have NVC. The odds ratio (OR) of females not having NVC compared to males was 2.7 (95% CI 1.3–5.5, p = 0.017). Patients younger than 30 years were much less likely to have NVC compared to older patients (OR 4.9, 95% CI 1.3–18.4, p = 0.017). The mean AP prepontine cistern length and symptomatic nerve length were smaller in the TN group than in the control group (5.3 vs 6.5 mm and 8.7 vs 9.7 mm, respectively; p < 0.001). The posterior fossa volume was significantly smaller in TN patients without NVC compared to those with NVC. A TN group cluster analysis suggested a sex-dependent difference that was not observed in those without NVC. Factorial ANOVA and post hoc testing found that findings in males without NVC were significantly different from those in controls or male TN patients with NVC and similar to those in females (female controls as well as female TN patients with or without NVC).

CONCLUSIONS

Posterior fossa volume in males was larger than posterior fossa volume in females. This finding, along with the higher incidence of TN in females, suggests that smaller posterior fossa volume might be an independent factor in the pathophysiology of TN, which warrants further study.