Trigeminal trophic syndrome: A systematic review.

OBJECTIVES: To systematically report and document Trigeminal Trophic Syndrome (TTS), characterize its clinical presentation, diagnostic tests performed, outline management strategies, outcomes; and highlight the role of otolaryngologists in the tissue diagnosis of this rare syndrome. DATA SOURCES: PubMed/Medline, Scopus, and Cochrane databases. REVIEW METHODS: PubMed/Medline, Scopus, and Cochrane databases were systematically reviewed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines to identify all cases of TTS published with an English translation from inception to December 2020. RESULTS: A total of 142 articles describing 214 patients with TTS were included in the analysis. There was a female predominance (62.9 %) and a median age of 57 (range 1-93) years at presentation. A trigeminal neurological insult was identified in 200 (93.5 %) cases. The most common triggers for TTS were treatment for trigeminal neuralgia (35.7 %) and cerebrovascular accident (21.6 %). Self-inflicted trauma occurred in 137 (64 %) patients. Biopsy was done in 123 (57.5 %) patients. Patient education, barrier devices, and medications to address parasthesias were the most common treatment strategies. The majority of patients (72.5 %) received multimodal therapy. Surgery was performed in 35 (22.7 %) patients. Treatment outcomes were discussed in 120 (56.1 %) patients. CONCLUSIONS: TTS is a rare condition with poorly understood pathophysiology. It should be suspected in a patient with non-healing facial ulceration and altered sensation within the trigeminal nerve distribution. Biopsy of the ulcer is important to confirm the diagnosis and exclude malignancy. Treatment options include conservative and pharmacologic measures, and less frequently surgery.

Long-Term Outcome of Unilateral Acoustic Neuromas With or Without Hearing Loss: Over 10 Years and Beyond After Gamma Knife Radiosurgery.

BACKGROUND: Since the long-term outcomes of 162 patients who underwent gamma knife radiosurgery (GKS) as an initial or adjuvant treatment for acoustic neuromas (ANs) with unilateral hearing loss were first reported in 1998, there has been no report of a comprehensive analysis of what has changed in GKS practice. METHODS: We performed a retrospective study of the long-term outcomes of 106 patients with unilateral sporadic ANs who underwent GKS as an initial treatment. The mean patient age was 50 years, and the mean initial tumor volume was 3.68 cm3 (range, 0.10-23.30 cm3). The median marginal tumor dose was 12.5 Gy (range, 8.0-15.0 Gy) and the median follow-up duration was 153 months (range, 120-216 months). RESULTS: The tumor volume increased in 11 patients (10.4%), remained stationary in 27 (25.5%), and decreased in 68 patients (64.2%). The actuarial 3, 5, 10, and 15-year tumor control rates were 95.3 ± 2.1%, 94.3 ± 2.2%, 87.7 ± 3.2%, and 86.6 ± 3.3%, respectively. The 10-year actuarial tumor control rate was significantly lower in the patients with tumor volumes of ≥ 8 cm3 (P = 0.010). The rate of maintaining the same Gardner-Robertson scale grade was 28.6%, and that of serviceable hearing was 46.4%. The rates of newly developed facial and trigeminal neuropathy were 2.8% and 4.7%, respectively. The patients who received marginal doses of less than 12 Gy revealed higher tumor control failure rates (P = 0.129) and newly occurred facial or trigeminal neuropathy rates (P = 0.040 and 0.313, respectively). CONCLUSION: GKS as an initial treatment for ANs could be helpful in terms of tumor control, the preservation of serviceable hearing, and the prevention of cranial neuropathy. It is recommended to perform GKS as soon as possible not only for tumor control in unilateral ANs with hearing loss but also for hearing preservation in those without hearing loss.

Uncommon cause of trigeminal neuritis and central nervous system involvement by herpes labialis: a case report.

BACKGROUND: Trigeminal neuropathy is characterized by numbness in the region innervated by the trigeminal nerves, with or without neuropathic weakness in the muscles of mastication. Trigeminal neuritis is a form of trigeminal neuropathy in which the lesion is caused by an inflammation. Herein, we report a patient with trigeminal neuritis due to central nervous system (CNS) involvement of herpes labialis (HL) infection, which was successfully treated with anti-viral and anti-inflammatory agents. CASE PRESENTATION: A young healthy female presented with numbness in the left hemiface for two weeks. She had a preceding typical HL infection on left facial lip one week before the sensory symptom onset. Brain magnetic resonance imaging revealed high signal intensities and asymmetrical thickening with enhancement along the cisternal segment of the left trigeminal nerve. Additionally, brain MR angiography showed multifocal stenoses in the M1 segment of the middle cerebral artery and the cavernous portion of the internal carotid artery. Cerebrospinal fluid (CSF) examination showed mild pleocytosis with normal protein level, glucose ratio, but CSF polymerase chain reaction assay for specific anti-viral antibodies including herpes simplex virus was negative, and CSF culture also did not identify a specific pathogen. The results of serologic testing including tumor markers and autoimmune markers were all unremarkable. A tentative diagnosis of trigeminal neuritis as a complication of HL involving the CNS was made considering the clinical, neuroradiological, and laboratory findings of the patient. Therefore, the patient was treated with intravenous methylprednisolone and acyclovir for 10 days. After the treatments, her sensory disturbance was markedly improved. Brain MRI at the 3-month follow-up also demonstrated improvement of previously identified high signal intensity lesions and multifocal intracerebral artery stenoses. CONCLUSION: HL is usually a self-limiting, benign disease without complications, but rarely presents as trigeminal neuritis due to CNS involvement. Therefore, meticulous evaluation may be necessary if trigeminal neuritis or CNS involving symptoms occur after HL.

Etiologies and Utility of Diagnostic Tests in Trigeminal Neuropathy.

OBJECTIVE: To evaluate the utility of diagnostic studies in identifying treatable etiologies of trigeminal neuropathy (TNP). PATIENTS AND METHODS: We performed a review of consecutive patients with nontraumatic, noniatrogenic TNP seen at Mayo Clinic between January 1, 2000, and August 31, 2019. Patients were excluded if they had trigeminal neuralgia without neuropathy or if their diagnostic work-up had been completed elsewhere. Data were analyzed to determine which diagnostic studies were most useful in identifying treatable etiologies. RESULTS: In total, 439 patients were included. The mean ± SD age was 56.3±13.6 years and 285 (64.9%) were female. Among the 180 cases in which an etiology was identified (41.0%), neoplasms were causative in 76 (42.2%), while specific connective tissue diseases were implicated in 71 (39.4%). Bilateral TNP (n=83) was associated with the presence of underlying connective tissue disease (P<.01). Identification of etiology was made by magnetic resonance imaging in 88 cases (48.8%), by abnormal connective tissue disease cascades combined with rheumatology consultation in 42 (23.3%), by a previously known connective tissue disorder in 30 (16.7%), and by abnormal connective tissue disease cascades alone in 8 (4.4%). Among the 439 study patients, electromyography was performed in 211 (48.1%) and lumbar puncture in 139 (31.7%), but their diagnostic utility was low. CONCLUSION: Underlying causes of nontraumatic, noniatrogenic TNP can be identified in approximately 40% of cases. Bilateral TNP is strongly associated with underlying connective tissue disease. Careful history taking, dedicated magnetic resonance imaging, and connective tissue panels have the greatest diagnostic utility. Electromyography and cerebrospinal fluid analysis are unlikely to elucidate treatable etiologies of TNP.

Clinical Outcomes of Minimally Invasive Corneal Neurotization After Cerebellopontine Angle Neurosurgical Procedures.

PURPOSE: To report 12 patients with neurotrophic keratopathy due to the trigeminal nerve injury after intracranial tumor surgeries underwent minimally invasive corneal neurotization and evaluate the outcomes of corneal reinnervation. METHODS: Twelve patients (12 eyes) with neurotrophic keratopathy caused by the trigeminal nerve injury after intracranial surgeries received minimally invasive corneal neurotization. All the preoperative central corneal sensation was under 5 mm, and minimally invasive corneal neurotization was performed over 6 months after the intracranial surgery. Follow-up was conducted 1 week and 1 month after the surgery and then every 3 months. Twelve healthy age-matched participants were enrolled as controls. The indicators included corneal sensation, best-corrected visual acuity, corneal nerve fiber length and branch density, diameter of nerve trunk, corneal ulcer lesion ratio, and sensation of the contralateral forehead. RESULTS: Mean follow-up was 24.7 ± 7.1 months. Mean central corneal sensation rose from 0.4 ± 1.4 to 31.7 ± 21.8 mm. Corneal nerve fiber length improved from 9.56 ± 5.00 to 14.96 ± 4.65 mm/mm2 and corneal nerve branch density and diameter of nerve trunk both increased (p < .01 and p < .05, respectively). Corneal lesion ratio decreased from 0.17 ± 0.12 to 0.10 ± 0.10 (p < .01). CONCLUSIONS: Minimally invasive corneal neurotization promotes corneal reinnervation for patients with neurotrophic keratopathy induced by the trigeminal nerve injury after intracranial surgeries. The process of corneal reinnervation after minimally invasive corneal neurotization often lasts over 12 months, and it takes about 18 months to return to a higher level. Corneal sensation and corneal nerve fiber length are related to clinical outcomes such as corneal ulcer lesion and best-corrected visual acuity. The effect on the sensation of the contralateral side forehead is temporary, and most patients can restore normal forehead sensation of the contralateral side.

Multicompartmental Epidermoid Cyst Causing Chronic Parotid Gland and Masticator Space Muscle Atrophy.

Epidermoid cysts are rare benign intracranial tumors of congenital origin. They are slow-growing and are seen to insinuate between brain structures. These are commonly located in cerebello-pontine angle and parasellar regions. The symptoms produced are primarily due to mass effect. Hearing loss, facial nerve palsy, and trigeminal neuralgia are reported when cranial nerves are involved; motor palsy of trigeminal nerve is uncommon. Here, we present an interesting case of an extensive multicompartmental epidermoid cyst causing atrophy of trigeminal nerve with radiologic evidence of chronic motor trigeminal nerve palsy characterized by atrophy of masticator space muscles and parotid gland.

Long lasting trigeminal neuropathy, limbic encephalitis and abdominal ganglionitis without primary cancer: An atypical case of Hu-antibody syndrome.

OBJECTIVES: Anti-Hu antibodies (Hu-Abs) are the most frequent onconeural antibodies associated with paraneoplastic neurologic syndromes (PNS). PNS include a variety of neurological syndromes, affecting less than 1/10,000 patients with cancer. In the majority of cases, PNS will manifest before the malignancy is diagnosed. We found a case in which PNS was diagnosed without finding a primary malignancy after extensive work-up and even post-mortem autopsy. PATIENT AND METHODS: We present a case report of a 58-year-old man. This article includes extensive clinical work-up, full-body autopsy and brain autopsy with classical histochemical and myelin stainings and immunohistochemistry was performed. RESULTS: The patient developed a progressive trigeminal neuropathy over a period of 5 years, in combination with cerebellar degeneration, asymmetrical brainstem and limbic encephalitis. Serum showed repeatedly high anti-Hu antibodies. Comprehensive cancer screening could not demonstrate any primary malignancy. Therapy with corticosteroids, plasma exchange, cyclophosphamide and rituximab showed no beneficial effect. He died from the complications of enteric ganglionitis 5 years after onset of the first symptoms. A postmortem autopsy could not detect a primary malignancy either. Brain morphology is described in detail. CONCLUSION: Paraneoplastic anti-Hu encephalitis cases associated with SCLC or other primary neoplasms are well known. An adult with a progressive multifocal neurological syndrome in the presence of positive anti-Hu antibodies, but without any primary neoplasm after a follow-up over 5 years is unusual.

[Cavernous sinus syndrome as the initial symptom of Burkitt's lymphoma: a case report and literature review]. / Sindrome del seno cavernoso como inicio de un linfoma de Burkitt: a proposito de un caso y revision de la bibliografia.

INTRODUCTION: The cavernous sinus is a structure in the base of the skull that houses several nerve and vascular structures. Its compromise leads to cavernous sinus syndrome, which is a combination of oculomotor disorders and others affecting the first two trigeminal branches, often accompanied by pain or proptosis. Infiltration due to Burkitt's lymphoma is a rare cause of this syndrome. CASE REPORT: A 43-year-old male, carrier of human immunodeficiency virus, with good control of the disease, who developed a clinical picture consisting of progressive painful ophthalmoplegia in the presence of a laterocervical adenopathy. Complementary tests allowed a diagnosis of Burkitt's lymphoma with extranodal extension to the cavernous sinus. A review of the cases published in Medline was also carried out: a total of 15 cases were detected and their epidemiological characteristics, form of presentation, extracranial involvement at the time of diagnosis and clinical progression were described. CONCLUSIONS: Burkitt's lymphoma is a high-grade lymphoproliferative syndrome. Its form associated with immunodeficiency is an important cause of morbidity and mortality in this subgroup of patients. In the cases analysed in the literature, the age of presentation varied and the form of onset was a progressive painful ophthalmoplegia or numb chin syndrome. Exclusive involvement of the cavernous sinus was infrequent, but in that case it entailed a poor prognosis. It is important to rule out a primary extracranial origin and not to confuse it with an idiopathic Tolosa-Hunt syndrome that would delay the beginning of antitumour treatment.

TITLE: Sindrome del seno cavernoso como inicio de un linfoma de Burkitt: a proposito de un caso y revision de la bibliografia.Introduccion. El seno cavernoso es una estructura de la base craneal que alberga varias estructuras nerviosas y vasculares. De su afectacion se deriva el sindrome del seno cavernoso, una combinacion de alteraciones oculomotoras y de las dos primeras ramas del trigemino, frecuentemente acompañadas de dolor o proptosis. La infiltracion por un linfoma de Burkitt es una causa infrecuente de este sindrome. Caso clinico. Varon de 43 años, portador del virus de la inmunodeficiencia humana, con buen control de la enfermedad, que desarrolla un cuadro consistente en oftalmoplejia dolorosa progresiva en presencia de adenopatia laterocervical. Las pruebas complementarias permiten el diagnostico de linfoma de Burkitt con extension extranodal al seno cavernoso. Tambien se realiza una revision de los casos publicados en Medline: se recogen un total de 15 casos y se describen sus caracteristicas epidemiologicas, forma de presentacion, afectacion extracraneal en el momento del diagnostico y evolucion clinica. Conclusiones. El linfoma de Burkitt es un sindrome linfoproliferativo de alto grado. Su forma asociada a inmunodeficiencia es una importante causa de morbimortalidad en este subgrupo de pacientes. En los casos analizados en la bibliografia, la edad de presentacion fue variable y la forma de inicio fue una oftalmoplejia dolorosa progresiva o un sindrome de numb chin. Fue infrecuente una afectacion exclusiva del seno cavernoso, pero en ese caso conllevaba un mal pronostico. Es importante descartar un origen primario extracraneal y no confundirlo con un sindrome de Tolosa-Hunt idiopatico que retrasaria el inicio del tratamiento antitumoral.

Quantitative and qualitative sensory testing results are associated with numbness rather than neuropathic pain in patients with post-implant trigeminal neuropathy: a cross-sectional pilot study.

Purpose: This study aimed to characterize the sensory profile of patients with post-implant trigeminal neuropathy and identify the association between subjective symptoms and objective signs including psychophysical testing and radiographic imaging. This study further evaluated to the association between quantitative sensory testing (QST)/qualitative sensory testing (QualST) and the severity of nerve injury graded by radiographic imaging. Materials and methods: This retrospective study included 34 patients diagnosed with post-implant trigeminal neuropathy. Data on the neuropathic pain symptom inventory (NPSI), thermal and electric QST, bedside QualST, and cone beam computed tomography (CBCT) was collected and the association between these variables were analysed. Results: Numbness was the most common subjective symptom and evoked pain was the most frequent neuropathic pain. There was no significant correlation between negative and positive symptoms. Spearman's rank correlation analyses indicated that objective findings including QST/QualST correlated with a sensory loss profile rather than a gain of function profile. Moderate positive correlations between some positive symptoms and the score of QualST were observed. The Mann-Whitney U test showed that subjective symptoms did not differ according to the severity of nerve damage according to CBCT, but the electric QST and QualST was discriminative. Conclusions: This study suggests that QST/QualST associated with the severity of nerve damage according to CBCT might be useful in assessing numbness in patients with negative and positive symptoms after implant surgery, but may be of marginal utility in the evaluation of neuropathic pain within the limitation of this cross-sectional study with small sample size.

Trigeminal Neuropathy After Mandibular Fractures: Epidemiology and Neurophysiologic Diagnosis.

OBJECTIVES: To study the trigeminal nerve neuropathy prevalence after mandibular fractures and follow nerve recovery and to examine associations with clinical features. SUBJECTS: The files of 304 patients treated for 424 mandibular fractures in Hadassah Medical Center between 2001 and 2008 were analyzed. Twenty-five patients with paresthesia were examined by electro-diagnostic conduction test (quantitative sensory testing). RESULTS: The most common fracture sites were the symphysis (28.5%) and condyle (21.4%). A strong correlation between fracture severity and treatment modality was found. Nerve damage occurred in 13.4% of nondisplaced fractures and in 65.3% of >5 mm displaced fractures. Patients who were treated by open reduction internal fixation demonstrated the biggest difference in quantitative sensory testing values between the affected and nonaffected sides. C nerve fibers were the most damaged and least regenerated. There was correlation between damage perception and objective test results. CONCLUSIONS: There is a correlation between fracture type and displacement severity on neurologic deficit and prognosis. The objective neurosensory damage was manifested by higher nerve excitation threshold on the injured side. Patients should be informed on the prognosis of neurologic deficit according to fracture type.

Long-term follow-up results of stereotactic radiosurgery for vestibular schwannomas larger than 8 cc.

BACKGROUND: Accumulated stereotactic radiosurgery (SRS) experience for large vestibular schwannomas (VSs) based on over 5 years of follow-up are as yet insufficient, and chronological volume changes have not been documented. METHOD: Among 402 patients treated between 1990 and 2015, tumor volumes exceeded 8 cc in 30 patients. We studied 19 patients with follow-up for more than 36 post-SRS months or until an event. Median tumor volume was 11.5 cc (range; 8.0 to 30.6). The target volume was basically covered with 12.0 Gy. RESULTS: The median magnetic resonance imaging and clinical follow-up periods were both 98 months (range 49 to 204). Tumor shrinkage was documented in 13 patients (72%), no change in 2 (11%), and growth in the other 3 (17%). Therefore, the crude growth control rate was 83%. All three patients with tumor enlargement needed salvage treatment. Thus, the crude clinical control rate was 84%. Actuarial further procedure-free rates were 91%, 83% and 76%, at the 60th, 120th, and 180th post-SRS month. Among six patients followed chronologically, transient tumor expansion was observed in three (43%) and two cystic VSs showed rapid tumor growth. Transient trigeminal neuropathy occurred in two patients (11%). No patients experienced facial nerve palsy. None of the six patients with useful hearing pre-SRS maintained serviceable hearing. Ventricular-peritoneal shunt placement was required in three patients. CONCLUSIONS: Long-term tumor control with SRS was moderately acceptable in large VSs. In terms of functional outcome, trigeminal neuropathies and facial palsies were rare. However, hearing preservation remains a challenge. In the long term, chronological tumor volumes were generally decreased after SRS. However, caution is required regarding rapid increases in tumor size, especially for cystic type VSs. Further studies are needed to optimize clinical positioning of SRS for large VSs.

Horner's syndrome and paresthesia in the trigeminal nerve territory secondary to epidural analgesia for labor / Síndrome de Horner e parestesia do território do nervo trigêmeo secundário a analgesia peridural para trabalho de parto

Abstract Currently, epidural analgesia is a common procedure for labor analgesia. Although it is considered a safe technique, it is not without complications. Horner's syndrome and paresthesia within the trigeminal nerve distribution are rare complications of epidural analgesia. We report a case of a pregnant woman who developed Horner's syndrome and paresthesia within the distribution of the trigeminal nerve following epidural analgesia for the relief of labor pain.

Resumo A analgesia peridural é hoje em dia um procedimento comum para analgesia do trabalho de parto. Embora seja considerada uma técnica segura, não está isenta de complicações. A síndrome de Horner e a parestesia do território do nervo trigêmeo são complicações raras da analgesia peridural. Relatamos um caso de uma grávida que desenvolveu a síndrome de Horner e parestesia do território do nervo trigêmeo após analgesia peridural para o alívio da dor do trabalho de parto.

Adenoid Cystic Carcinoma of the Maxillary Sinus with Isolated Trigeminal Anesthesia.

Adenoid cystic carcinoma (ACC) is a rare malignant secretory gland tumor. It is characterized by slow growth, long clinical course, local recurrences, and distant metastases. In the sinonasal tract, it most commonly arises in the maxillary sinus. It often presents at an advanced stage with perineural spread (PNS). Our patient presented with left-sided facial numbness without other symptoms. The numbness was localized to the left cheek, left side of nose, and left upper lip. Magnetic resonance imaging (MRI) of the brain revealed an enhancing lesion involving the left maxillary sinus with orbital invasion and posterior extension into the cavernous sinus. Transnasal endoscopic exploration with tissue removal revealed ACC. 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) scan revealed no evidence of distant metastases. Presentation of sinonasal ACC (SNACC) is variable depending on the involved structures. Characteristic PNS with ACC may cause neuropathic symptoms. This case displays a unique presentation of an advanced ACC of the maxillary sinus manifesting as isolated unilateral trigeminal anesthesia without sinonasal symptoms. The patient also failed to demonstrate any ocular or oculomotor symptoms despite extensive involvement of the orbit and surrounding structures. This case highlights the importance of recognizing ACC due to its association with late symptomatic manifestations. It also reinforces the need for clinical diligence with the workup of new onset neuropathic symptoms in the maxillary distribution of the trigeminal nerve.

Corneal Subbasal Nerve Density and Sensitivity After Pars Plana Vitrectomy Using Contact or Noncontact Wide-Angle Viewing Systems.

PURPOSE: To assess corneal subbasal nerve density (SBND) and corneal sensitivity (CS) after pars plana vitrectomy (PPV) for rhegmatogenous retinal detachment (RRD) without intraoperative 360-degree laser treatment using contact or noncontact panoramic viewing systems. METHODS: This is a prospective study of 34 eyes affected by RRD, which underwent PPV using contact (18 eyes) and noncontact (16 eyes) panoramic viewing systems; 12 eyes which underwent scleral buckling, and 17 eyes which underwent cataract surgery. SBND and CS were assessed before surgery and 3 and 6 months postoperatively by in vivo confocal microscopy and Cochet-Bonnet esthesiometry. RESULTS: Compared with baseline values, at 6 months, SBND and CS decreased in both contact PPV (SBND preoperative value: 19.1 ± 3.7 mm/mm; SBND postoperative value: 3.5 ± 1.3 mm/mm; CS preoperative value: 5.1 ± 0.5 cm; CS postoperative value: 1.5 ± 0.4 cm) and in noncontact PPV groups (SBND preoperative value: 19.5 ± 3.8 mm/mm; SBND postoperative value: 8.7 ± 2.3 mm/mm; CS preoperative value: 5.3 ± 0.5 cm; CS postoperative value: 2.5 ± 0.7 cm) (P < 0.001 for all comparisons). SBND reduction was greater in the contact PPV group than in the noncontact PPV group (P < 0.001). By contrast, the scleral buckling and cataract surgery group values were unchanged (P > 0.1 for all comparisons). In multivariate analysis, no significant effect was found for cataract surgery associated with PPV, pseudophakia, surgical time, intraocular pressure, or for laser in horizontal sectors for PPV groups. SBND was highly correlated with CS (r = 0.93). CONCLUSIONS: A contact viewing system reduces SBND after PPV more than a noncontact system does.

Brainstem Dose Constraints in Nonisometric Radiosurgical Treatment Planning of Trigeminal Neuralgia: A Single-Institution Experience.

BACKGROUND: CyberKnife stereotactic radiosurgery (SRS) for trigeminal neuralgia (TGN) administers nonisometric, conformational high-dose radiation to the trigeminal nerve with risk of subsequent hypoesthesia. METHODS: We performed a retrospective, single-institution review of 66 patients with TGN treated with CyberKnife SRS to compare outcomes from 2 distinct treatment periods: standard dosing (n = 38) and reduced dosing (n = 28). Standard and reduced dosing permitted a maximum brainstem dose of 45 Gy and 25 Gy, respectively, each with a prescription dose of 60 Gy. Primary and secondary outcomes were Barrow Neurologic Institute pain and numbness scores. Maximum brainstem dose, prepontine nerve length, and treatment history were recorded for their predictive contributions by logistic regression. RESULTS: After matching, patients in the standard dosing and reduced dosing groups were followed for a median of 25 months and 19.5 months, respectively. Mean trigeminal nerve length was 8.55 mm in the standard dosing group and 9.46 mm in the reduced dosing group. Baseline rates of poorly controlled pain were 97% and 88%, respectively, which improved to 23.4% and 8.3%, respectively (P < 0.001 for both). The baseline rates of bothersome numbness were null in both groups, and increased to 25% in the standard group (P = 0.006) and to 21% in the reduced group (P = 0.07). Regression analyses suggested that reduced brainstem exposure (P = 0.01), as well as a longer trigeminal nerve (P = 0.01), were predictive of durable pain control. CONCLUSIONS: These outcomes demonstrate that a lower maximum brainstem dose can provide excellent pain control without affecting facial numbness. Longer nerves may achieve better long-term outcomes and help optimize individual plans.

Trigeminal neuropathy in vestibular schwannoma: a treatment algorithm to avoid long-term morbidity.

BACKGROUND: Trigeminal neuropathy (TGN) can occur as a presenting feature of vestibular schwannoma (VS) or as an adverse effect of radiosurgery. This study was designed to evaluate a treatment algorithm for presenting symptoms of TGN in patients with VS, and a new radiosurgery dosimetric tolerance to avoid TGN after treatment. Outcome was measured after microsurgery (MS), stereotactic radiosurgery (SRS), hypofractionated stereotactic radiotherapy (HSRT), and fractionated radiotherapy (FRT). METHODS: A prospectively held VS database was retrospectively analysed from 2011 to 2016 at a tertiary university hospital. All patients who underwent MS from 2011 and all patients who underwent radiotherapy (SRS, HSRT, FRT) from 2015 were studied. Patients on surveillance and neurofibromatosis type 2 patients were not included. Patient demographic data, tumour characteristics, presenting symptoms, and post-treatment outcomes were analysed. RESULTS: Eighty-eight patients were included in the study (43 microsurgery, 45 radiotherapy). Twenty-seven (31%) patients presented with TGN symptoms. The median age of patients included was 56.5 (range 6-72 years), with a median follow-up for MS and SRS of 38 and 20 months, respectively (range 10-80 months). All 27 patients with TGN were offered MS as per protocol. Three patients declined, or were not fit for surgery, and received FRT. Complete resolution of TGN symptoms was achieved in all 24 patients who underwent MS and 33% (1/3) of patients with FRT. Eleven patients experienced transient post-operative complications (pseudomeningocele (6), meningitis (3), venous sinus thrombosis, cerebellar haemorrhagic contusion, and posterior fossa haematoma). Of the 45 patients in the radiotherapy cohort, 36 were suitable for SRS, of which 30 patients who met the dose-volume constraints for trigeminal nerve underwent single-fraction SRS and 6 patients who did not meet the constraints received HSRT. Nine patients (20%) received FRT including three patients with pre-treatment TGN. None of the patients developed new TGN symptoms following SRS or HSRT. CONCLUSIONS: Our algorithm to select the optimal treatment modality appears to achieve comparable or better long-term outcome. Microsurgical resection in our cohort resulted in complete resolution of symptoms in all patients. None of our SRS- or HSRT-treated patients developed TGN during the follow-up period. The adherence to strict trigeminal nerve dose-volume constraints for SRS remains critical to minimise TGN post treatment. Fractionated radiotherapy is an alternative for patients who refuse surgery or those who are unfit for surgery.

Population-Based Study of Stereotactic Radiosurgery or Fractionated Stereotactic Radiation Therapy for Vestibular Schwannoma: Long-Term Outcomes and Toxicities.

PURPOSE: To examine long-term local control of vestibular schwannoma and side effects in patients treated with stereotactic radiosurgery (SRS) and fractionated stereotactic radiation therapy (SRT) in British Columbia. METHODS AND MATERIALS: From August 1998 to May 2009, 207 patients were treated with radiation therapy (RT) at British Columbia Cancer Agency. 136 (66%) received SRS, and 71 (34%) received SRT. Dose prescriptions were 50 Gy/25 fractions for SRT and 12 Gy/1 fraction for SRS. Our multidisciplinary provincial neuro-stereotactic conference recommended SRT for tumors >3 cm and for patients with serviceable hearing (Gardner-Robertson classes I and II). RESULTS: Median follow-up was 7.7 years to the last MRI and 6.4 years to the last clinical assessment. Local control for SRS versus SRT was 94% versus 87% at 5 years and 90% versus 85% at 10 years (P=.2). Five- and 10-year actuarial rates of RT-induced trigeminal nerve dysfunction were 25% and 25% after SRS, compared with 7% and 12% after SRT (P=.01). Five- and 10-year actuarial rates of RT-induced facial nerve dysfunction were 15% and 15% after SRS, versus 13% and 15% after SRT (P=.93). In the 49 patients with serviceable hearing at baseline who were treated with SRT, hearing preservation was 55% at 3 years, 37% at 5 years, and 29% at 7 years. In multivariable analysis, better pretreatment ipsilateral pure tone average was significantly associated with hearing preservation (hazard ratio 1.03; 95% confidence interval 1.00-1.07; P=.04). CONCLUSIONS: Both SRS and SRT provided excellent long-term local control of vestibular schwannoma. Stereotactic radiosurgery was associated with higher rates of trigeminal nerve dysfunction. Even with a fractionated course, hearing preservation declined steadily with long-term audiometric follow-up.

Ophthalmic Complications Following Acoustic Neuroma Resection.

BACKGROUND: Past studies have shown high rates of ocular complications with the need for ophthalmic surgery following acoustic neuroma resection (ANR). OBJECTIVE: To determine the rates of ophthalmic complications, referrals, and surgery following ANR, and the factors associated with poor outcomes. METHODS: A retrospective study of ophthalmic outcomes in patients who underwent ANR was conducted, following institutional review board approval. Surgical approach, tumor size, tumor characteristics, completeness of resection, postoperative House-Brackmann grades, ocular complications, referrals to ophthalmology, and ophthalmic treatments were recorded. RESULTS: Between 2007 and 2012, 174 patients underwent ANR. There were 3 surgical groups: retrosigmoid (n = 97), translabyrinthine (n = 59), and combined retrosigmoid and translabyrinthine (n = 18). Median tumor size was 2.2 cm. Postoperatively, 30% of patients had facial nerve dysfunction (House-Brackmann ≥3), which recovered to 19% by 1 mo and 8.6% by 1 yr following ANR. Fifty-six (32.9%) patients experienced ocular complications postoperatively, with lagophthalmos, dry eye, and blurry vision as the most common complications. Thirty-six (67.9%) of the patients who required ophthalmic treatment were managed nonsurgically, with just 13 (7.6%) patients requiring referral to an ophthalmologist. In total, only 9 (5.3%) patients received an ophthalmic procedure. Patients with tumors >2 cm, those undergoing combined retrosigmoid and translabyrinthine resection, and those with severe facial nerve dysfunction which did not improve in the first month following surgery were more likely to have poor ophthalmic outcomes. CONCLUSION: We present lower rates of ophthalmic complications following ANR than previously reported. Improved surgical technique, better postoperative eye care, and facial nerve monitoring most likely accounted for the improved ocular outcomes.