Combined open maxillectomy and endoscopic endonasal resection of a giant V2 trigeminal schwannoma.

BACKGROUND: Trigeminal schwannoma is a rare type of tumor that arises from the Schwann cells of the trigeminal nerve. METHOD: We present a case of a patient with a giant V2 trigeminal schwannoma with painful swelling in the left maxilla. A complete resection using a combined open maxillectomy and endoscopic endonasal approach was performed. CONCLUSION: This case highlights the importance of a multidisciplinary approach to perform a combined open and endoscopic approach for safe resection while preserving adequate speech and swallowing.

A novel indicator to predict the outcome of percutaneous stereotactic radiofrequency rhizotomy for trigeminal neuralgia patients: diffusivity metrics of MR-DTI.

Magnetic resonance-diffusion tensor imaging (MR-DTI) has been used in the microvascular decompression and gamma knife radiosurgery in trigeminal neuralgia (TN) patients; however, use of percutaneous stereotactic radiofrequency rhizotomy (PSR) to target an abnormal trigeminal ganglion (ab-TG) is unreported. Fractional anisotropy (FA), mean and radial diffusivity (MD and RD, respectively), and axial diffusivity (AD) of the trigeminal nerve (CNV) were measured in 20 TN patients and 40 healthy control participants immediately post PSR, at 6-months, and at 1 year. Longitudinal alteration of the diffusivity metrics and any correlation with treatment effects, or prognoses, were analyzed. In the TN group, either low FA (value < 0.30) or a decreased range compared to the adjacent FA (dFA) > 17% defined an ab-TG. Two-to-three days post PSR, all 15 patients reported decreased pain scores with increased FA at the ab-TG (P < 0.001), but decreased MD and RD (P < 0.01 each). Treatment remained effective in 10 of 14 patients (71.4%) and 8 of 12 patients (66.7%) at the 6-month and 1-year follow-ups, respectively. In patients with ab-TGs, there was a significant difference in treatment outcomes between patients with low FA values (9 of 10; 90%) and patients with dFA (2 of 5; 40%) (P < 0.05). MR-DTI with diffusivity metrics correlated microstructural CNV abnormalities with PSR outcomes. Of all the diffusivity metrics, FA could be considered a novel objective quantitative indicator of treatment effects and a potential indicator of PSR effectiveness in TN patients.

Application of Sequential Thresholding-Based Automated Reconstruction of the Trigeminal Nerve in Trigeminal Neuralgia.

OBJECTIVE: High-resolution magnetic resonance imaging (MRI) of the trigeminal nerve is indispensable for workup of trigeminal neuralgia (TN) before microvascular decompression; however, the evaluation is often subjective and prone to variability. We aim to develop and assess sequential thresholding-based automated reconstruction of the trigeminal nerve (STAR-TN) as an algorithm for segmenting the trigeminal nerve and contacting structures that will allow for a structured method for assessing neurovascular conflict. METHODS: A total of 42 patients with TN who underwent high-resolution MRI before microvascular decompression in 2022 were included in our study. Segmentation of the trigeminal nerve and contacting structures was performed on preoperative MRI scans using STAR-TN. The segmentations were then evaluated for neurovascular conflict and compared to the preoperative radiology and operative notes. Geometric features, including the area of contact and distance to conflict, were extracted. RESULTS: Of the 42 patients, 32 (76.2%) were found to show neurovascular conflict based solely on their STAR-TN segmentations and 10 (23.8%) were found to not show neurovascular conflict. Compared with the intraoperative findings, this resulted in a sensitivity of 78.0% and specificity of 100%. In contrast, assessments of neurovascular conflict by radiologists using only 2-dimensional MRI views had a sensitivity of 68.3% and specificity of 100%. Of the 32 patients with neurovascular conflict, 29 (90.9%) had conflict within the root entry zone. Overall, the patients had a median area of contact of 10.66 mm2. CONCLUSIONS: STAR-TN allows for 3-dimensional visualization and identification of neurovascular conflict with improved sensitivity compared with neuroradiologist assessments from MRI slices.

[Neurovascular decompression in trigeminal neuralgia: Functional outcome]. / Descompresión neurovascular en neuralgia trigeminal: resultado funcional.

INTRODUCTION: The most frequent pathology of the fifth cranial nerve is trigeminal neuralgia (TN), characterized by unilateral orofacial pain, of a paroxysmal nature, with distribution in one or more divisions of the trigeminal nerve. The main objective of this work is to demonstrate the efficacy and safety of neurovascular decompressive surgery (NVD). METHODS: Retrospective analytical study, patients operated on for NVD by TN (n: 155), from January 2006 - 2022, using a retrosigmoid approach and clinicalradiological follow-up, whose intraoperative recording was in 3D system (n: 42). RESULTS: 83.3% (n=35) presented classic NT and 16.7% (n = 7) idiopathic. The right side prevailed with 59.5% (n = 25) and paroxysmal pain with 81% (n=34) of representation in the entire series. Compression of arterial origin represented 76.2% (n: 32) of the cases, the superior cerebellar artery represented the first cause of neurovascular compression (NVC) in 52.4% (n: 22) in the exit zone of the trigeminal nerve. The validity of the magnetic resonance imaging (MRI) protocol selected for this series was analyzed; evidencing a high sensitivity of MRI with 97%, and a specificity of 86%. CONCLUSION: The sensitivity and specificity of highdefinition MRI studies and with special protocols for the evaluation of TN, as gold standard paraclinical, were presented. The DNV offered safe results, a high percentage of the patients were described as successful. The 3D recording allowed retrospective analysis with a vision identical to that of the surgeon during the intraoperative period, the type of NVC.

Introducción: La afección más frecuente del V nervio craneal es la neuralgia del trigémino (NT), se caracteriza por dolor orofacial unilateral, paroxístico, distribuyéndose en una o más divisiones del nervio trigémino. El objetivo de presente trabajo es demostrar la eficacia y seguridad de la cirugía descompresiva neurovascular (DNV). Métodos: Estudio analítico retrospectivo, pacientes intervenidos para DNV por NT (n: 155), desde enero de 2006 - 2022, mediante un abordaje retrosigmoideo y seguimiento clínico-radiológico, cuyo registro intraoperatorio fue en sistema 3D (n: 42). Resultados: 83.3% (n=35) presentó NT clásica y 16.7% (n = 7) idiopática. La mayoría con afectación del lado derecho 59.5% (n = 25) y el dolor paroxístico con 81% (n = 34). La compresión de origen arterial representó 76.2% (n: 32) de los casos, la arteria cerebelosa superior representó la primera causa de compresión neurovascular (CNV) en un 52.4% (n:22) en la zona de salida del nervio trigémino. Se analizó la validez del protocolo de resonancia magnética (RM) seleccionado para esta serie; evidenciando una alta sensibilidad de la RM con 97%, y una especificidad del 86%. Conclusión: Se demostró la sensibilidad y especificidad de los estudios de resonancia magnética (RM) de alta definición y con protocolos especiales para la evaluación de la NT, como paraclínico estándar de oro. La DNV ofreció resultados seguros, un alto porcentaje de los pacientes se calificaron como exitosos. El registro 3D permitió analizar en retrospectiva con una visión idéntica a la del cirujano durante el intraoperatorio, el tipo de CNV.

Dosimetric investigation of radiation-induced trigeminal nerve toxicity in parotid tumor patients.

PURPOSE: We aimed to describe the association between trigeminal nerve (TN) dose and toxicity and determine a threshold value that leads to TN toxicity in patients with parotid tumors treated with adjuvant conventional fractionated radiation therapy. METHODS AND MATERIALS: Eighteen patients who underwent adjuvant radiotherapy (RT) between 2013 and 2018 were included in this retrospective study. TN and its branches were outlined subsequently on the planning CT scans. The doses received by TN were obtained based on the dose-volume histogram. The dose and toxicity relationship was investigated over the total prescribed dose. RT-related toxicity was graded according to Common Terminology Criteria for Adverse Events V4.0 (CTCAEv4.0). RESULTS: The median follow-up was 29.5 months. After RT, 61% of patients had Grade I-II late TN toxicity divided into Grade I in 4 (22%) and Grade II in 7 (39%) patients. TN injury symptoms were as follows: loss of sensation in the chin area in 3, difficulty in jaw movements in 3, and paresthesia in 5 patients. The total RT dose (p = 0.001), Dmax (p = 0.001), PTV-TN Dmax (p = 0.001), D1cc (p = 0.004), D0.5cc (p = 0.001), and D0.1cc (p = 0.01) had a significant effect on TN toxicity. Cut-off values leading to toxicity were determined as 66, 65.5, 65.25, 63.6, and 62.7 Gy for Dmax, PTV-TN Dmax, D0.1cc, D 0.5cc, and D1cc, respectively. CONCLUSIONS: Radiation-induced TN injury in head and neck cancer patients may further be investigated in clinically prospective trials by virtue of high toxicity rates with current RT doses in our retrospectively designed dosimetric study in parotid tumors.

Current Applications of Ablative Therapies for Trigeminal Neuralgia.

Trigeminal neuralgia (TN) is a syndrome consisting of episodic neuropathic facial pain. Although the precise symptoms vary across individuals, TN is typically described as lancinating electrical shocks triggered by sensory stimuli (light touch, talking, eating, and brushing teeth) that improve with antiepileptic medication (especially carbamazepine), remit spontaneously for weeks to months (pain-free intervals), and do not involve any changes in baseline sensation. The etiology of TN has not been definitively established, but many cases are associated with compression of the trigeminal nerve by a blood vessel at the trigeminal root entry zone adjacent to the brainstem. Patients who do not respond to medical management and who are not candidates for microvascular decompression often benefit from focal therapeutic injury to the trigeminal nerve at some point along its course. Many lesions have been described, including peripheral neurectomies that target distal branches of the trigeminal nerve, rhizotomies of the Gasserian ganglion of the nerve within Meckel's cave, radiosurgery of the trigeminal nerve at its root entry zone, partial sensory rhizotomy at the root entry zone, tractotomy of the spinal nucleus of the trigeminal nerve, and DREZotomy of the trigeminal nucleus caudalis, Though the latter two interventions are seldom done for TN and more commonly performed for trigeminal neuropathic pain. This article reviews the relevant anatomy and lesioning procedures for the treatment of trigeminal neuralgia.

An unusual finding of the Porus trigeminus: Case report with histological findings.

Many anatomical variants can potentially contribute to trigeminal neuralgia. These include compression by the superior cerebellar artery and less commonly, bony compression near the trigeminal cave. Herein, we report the gross and histological findings of a cadaveric specimen found to have a bony roof of the trigeminal cave. During the routine dissection of a male cadaver, an unusual finding of the skull base was observed. Palpation over the porus trigeminus identified a completely ossified roof. The bony spicule was 1.22 cm long and 0.76 mm wide. The trigeminal nerve was noted to have an indented region just below its contact with the ossified roof of the porus trigeminus. No frank nerve degeneration was noted with histological analysis. Normal mature bone tissue was noted surrounded by a sheath of dura mater. Future radiographic research is needed to better elucidate if ossification of the roof of the trigeminal cave is related to clinical symptoms of trigeminal neuralgia (TN). However, physicians should be cognizant of radiographic ossification of the trigeminal cave as a potential cause of TN.

[Trigeminal neuromas: modern diagnosis and treatment]. / Trigeminal'nye nevrinomy: sovremennaya diagnostika i lechenie.

Trigeminal neuroma (TN) is a benign neoplasm arising from trigeminal nerve sheath. The tumor can grow from any part of the nerve from the root in posterior cranial fossa to peripheral extracranial branches. Symptoms of trigeminal neuroma are variable and usually depend on location and dimensions of tumor. We present a review devoted to the problem of TN. Surgery was the only possible option in patients with TN for a long time. However, radiotherapy became one of the options and sometimes alternative to surgical treatment since the late 1980s. Besides active management of patients with TN, follow-up with regular radiographic control of small asymptomatic tumors also seems to be reasonable. When evaluating treatment outcomes, physicians consider quality of life and return to previous work and activity in addition to resection quality, neurological impairment, relapse-free period and tumor growth control. However, assessment of these indicators after certain treatment is rare. Thus, it is difficult to determine treatment strategy with maximum ratio of effectiveness and quality of life. Therefore, optimization of TN treatment is currently an urgent problem that requires further study.

Connection between V2 and V3 parts of the trigeminal nerve at the internal cranial base.

Anatomical variations can occasionally result in unexpected findings on physical examination. Here, we report two cases of seemingly unique connections between V2 and V3 parts of the trigeminal nerve. In these two cadaveric specimens, at the foramen ovale, small neural connections, confirmed with histology, were identified joining V2 to specifically, the motor root of V3. The findings of these two cadaveric specimens and the potential clinical ramifications are discussed.

Symptomatic perineural and intraneural invasion of the trigeminal nerve and subsequent abducens Nerve palsy by cutaneous squamous cell carcinoma.

A 74-year-old woman who presented initially with trigeminal neuralgia of the left forehead and scalp was later found to have a poorly differentiated squamous cell carcinoma (SCC) with large-nerve perineural and intraneural invasion of the left supraorbital nerve. Negative histopathologic margins were achieved in three stages of permanent fixed tissue en face processing and the final defect was repaired with a large rotation flap. Approximately one month after repair, the patient presented with new-onset diplopia and was found to have a complete left cranial nerve VI palsy suspicious for continued disease spread. MRI confirmed perineural spread along the ophthalmic branch of the trigeminal nerve through the superior orbital fissure into the cavernous sinus. She was subsequently treated with radiation therapy (66Gy in 33 fractions). The involvement of two distinct cranial nerves by perineural invasion is uncommon and has mostly been described involving branches of the trigeminal and facial nerves. This case highlights the rare presentation of perineural invasion involving both the trigeminal nerve and the abducens nerve. Anatomically, this clinical presentation can be explained by the retrograde perineural spread along the ophthalmic branch of the trigeminal nerve through the supraorbital fossa into the cavernous sinus where these two nerves are in close proximity.

The Role of Preoperative Magnetic Resonance Imaging in Assessing Neurovascular Compression Before Microvascular Decompression in Trigeminal Neuralgia.

BACKGROUND: Preoperative magnetic resonance imaging (MRI) is a standard component of the preoperative clinical workup for patients before microvascular decompression (MVD). However, its ability to accurately exclude neurovascular compression of the trigeminal nerve is not well understood. METHODS: We retrospectively reviewed 1020 patients with available preoperative MRI data before microvascular decompression. General patient demographics and clinical characteristics were collected for each case. We recorded both evidence of neurovascular conflict on preoperative MRI radiology notes and intraoperative compression from operative notes. Sensitivity, specificity, positive predictive value, and negative predictive value were determined for general MRI, high-resolution MRI, and non-high resolution. RESULTS: Overall, preoperative MRI before MVD showed a sensitivity of 75.8%, specificity of 65.8%, positive predictive value of 92.4%, and negative predictive value of 33.3% in predicting neurovascular compression of the trigeminal nerve. In particular, MRI was unable to identify 21.0% cases of sole arterial compression, 42.5% cases of sole venous compression, and combined arterial and venous compression in 18.5% of cases. A total of 958 patients (93.9%) underwent high-resolution preoperative MRI with skull base sequences. This imaging showed a sensitivity of 75.6%, specificity of 66.9%, positive predictive value of 92.5% and a negative predictive value of 33.4% in predicting trigeminal nerve neurovascular compression. Non-high-resolution MRI showed a sensitivity of 78.8%, specificity of 50.0%, positive predictive value of 89.1%, and negative predictive value of 31.3%. The negative predictive values of general, high-resolution, and non-high-resolution MRIs were all <50%. CONCLUSIONS: Preoperative MRI may offer a high predictive value for neurovascular conflict and should be part of the standard preoperative care workup for patients with trigeminal neuralgia. However, lack of neurovascular conflict on preoperative imaging is not sufficient to exclude patients from undergoing MVD.

The Trigeminal Nerve: Anatomy and Pathology.

The trigeminal nerve is the fifth cranial nerve and is a sensory-motor nerve that provides the innervation to the face with its three roots. The trigeminal nerve can be affected by several diseases, such as vascular conflict, congenital malformation, inflammatory or neoplastic diseases. Magnetic Resonance Imaging plays a crucial role in its evaluation. This article aims to review the trigeminal nerve anatomy, discuss the best magnetic resonance imaging techniques to evaluate each nerve segment, and demonstrate the imaging aspect of the diseases that most commonly affect it.

Pediatric benign triton tumor of trigeminal nerve: a case report and literature review.

PURPOSE: Benign triton tumors (BTTs) in the pediatric population are extremely rare occurrences. Paucity of data on BTTs poses both diagnostic and therapeutic challenges, particularly when found intracranially. METHODS: A case report of a 10-year-old male diagnosed with incidental maxillary trigeminal (V2) BTT is presented. We discuss radiographic and histopathological interpretations. Furthermore, we provide a brief review of current literature and historical background on pediatric trigeminal BTT diagnosis, histopathology, and management. RESULTS: Successful gross total resection of the tumor was achieved via Dolenc approach to the cavernous sinus. Management options with consideration of outcomes from the few prior cases reported in the literature are presented. CONCLUSION: Treatment of trigeminal nerve tumors requires a broad differential diagnosis and understanding rare tumors is essential in the diagnosis and treatment algorithm.

Secondary trigeminal neuralgia diagnosed in orofacial pain setting as epidermoid tumor.

This case report describes the rare occurrence of an epidermoid tumor compressing the ipsilateral trigeminal nerve resulting in secondary trigeminal neuralgia. MRI is the imaging modality of choice for the diagnosis of secondary trigeminal neuralgia. The epidermoid tumor was discovered by an orofacial pain specialist after reassessing the MRI study, previously reported as normal. Clinicians encounter a diagnostic dilemma when the clinical picture is not consistent with the MRI reports, clinical presentation, and expected results of treatment. The reassessment of the MRI and discovery of the epidermoid tumor resulted in a prompt referral to a neurosurgeon with a successful treatment outcome.

Purely Endonasal Endoscopic Approaches for Extracranial Trigeminal Nerve Schwannoma.

OBJECTIVE: The aim of this study is to assess the effectiveness and safety of purely endonasal endoscopic resection of extracranial trigeminal schwannomas (TGSs), with assessment of surgical and clinical outcomes in order to identify optimal candidates for an endonasal endoscopic approach (EEA). METHODS: A retrospective review of patient's records operated for TGSs between 2008 and 2021 was conducted. Patients operated with a purely EEA were included in this study. Pictures from a frozen fresh cadaver head dissection were used to demonstrate the surgical approach and to show anatomic relationships, complexity of the surgical area and safe corridors for surgery. RESULTS: A total of 5 patients (4 females and 1 male) were operated for TGS. All patients had facial numbness (100%) as a presenting symptom, followed by facial pain in 2 patients (40%), and orbital pain in 1 (20%). Also, 3 patients (60%) had a tumor originating at the level of the foramen ovale and 2 (40%) at the foramen rotundum. The mean tumor diameter was 3,7 ± 2 cm. Gross total resection were achieved in all cases. Postoperatively, 1 patient had severe mastication problems, 1 had blurred vision, and in the long-term follow-up, 1 had frontal sinusitis. The mean follow up was 106.6 (min:49, max:132, SD: 29.82) months. No recurrences were detected. CONCLUSIONS: In cases with the extradural TGS having limited extension into Posterior Cranial Fossa, or located in the Middle Cranial Fossa, a purely EEA is possible even for tumors bigger in size. Unilateral endonasal corridors are adequate for resection in most cases.

The application of magnetic resonance imaging (MRI) for the prediction of surgical outcomes in trigeminal neuralgia.

Trigeminal neuralgia (TN) is a severe facial pain disorder that often requires surgical treatment. Neurovascular compression (NVC) has been widely accepted as the primary cause of classical TN (cTN). Vascular compression involving the near half of the cisternal segment of trigeminal nerve was the most likely cause of patient's symptoms. And severe NVC was a strong imaging predictor of an optimal surgical outcome. Operative treatments for cTN include microvascular decompression (MVD) and various ablative procedures. However, a significant proportion of cTN patients with significant NVC fail to achieve long-term pain relief after technically successful surgery. Neuroimaging using magnetic resonance imaging (MRI) provides a noninvasive method to generate objective biomarkers of eventual response to TN surgery. This paper reviewed the progress of research on the prediction of surgical outcomes in TN with MRI.

Paediatric Cavernous Malformation of the Trigeminal Nerve: Case Report and Review of the Literature.

INTRODUCTION: Intradural, extra-axial cerebral cavernous malformations (CCMs) are rare entities and are mostly reported in relation to the optic apparatus or the facial/vestibulocochlear complex. Cranial nerve CCMs tend to follow a clinically aggressive course, with a tendency to progressive neurological dysfunction following intra-lesional haemorrhage or less commonly due to the effects of subarachnoid haemorrhage. CASE PRESENTATION: We report the first case of a trigeminal CCM presenting in a child with otalgia and left-sided headaches. The patient was initially managed with radiological surveillance but required surgical management following deterioration. We describe the successful treatment of the lesion with microsurgical resection. CONCLUSION: A CCM should be considered in the differential diagnosis of mass lesions arising in the region of the trigeminal nerve. Surgical resection is recommended to prevent neurological deterioration and may result in significant symptomatic improvement.

Pathogenic Mechanism of Dry Eye-Induced Chronic Ocular Pain and a Mechanism-Based Therapeutic Approach.

Purpose: Dry eye-induced chronic ocular pain is also called ocular neuropathic pain. However, details of the pathogenic mechanism remain unknown. The purpose of this study was to elucidate the pathogenic mechanism of dry eye-induced chronic pain in the anterior eye area and develop a pathophysiology-based therapeutic strategy. Methods: We used a rat dry eye model with lacrimal gland excision (LGE) to elucidate the pathogenic mechanism of ocular neuropathic pain. Corneal epithelial damage, hypersensitivity, and hyperalgesia were evaluated on the LGE side and compared with the sham surgery side. We analyzed neuronal activity, microglial and astrocytic activity, α2δ-1 subunit expression, and inhibitory interneurons in the trigeminal nucleus. We also evaluated the therapeutic effects of ophthalmic treatment and chronic pregabalin administration on dry eye-induced ocular neuropathic pain. Results: Dry eye caused hypersensitivity and hyperalgesia on the LGE side. In the trigeminal nucleus of the LGE side, neuronal hyperactivation, transient activation of microglia, persistent activation of astrocytes, α2δ-1 subunit upregulation, and reduced numbers of inhibitory interneurons were observed. Ophthalmic treatment alone did not improve hyperalgesia. In contrast, continuous treatment with pregabalin effectively ameliorated hypersensitivity and hyperalgesia and normalized neural activity, α2δ-1 subunit upregulation, and astrocyte activation. Conclusions: These results suggest that dry eye-induced hypersensitivity and hyperalgesia are caused by central sensitization in the trigeminal nucleus with upregulation of the α2δ-1 subunit. Here, we showed that pregabalin is effective for treating dry eye-induced ocular neuropathic pain even after chronic pain has been established.

Rapid deterioration of intravascular large B-cell lymphoma with mass formation in the trigeminal nerve and multiple organ infiltration: An autopsy case report.

Intravascular large B-cell lymphoma (IVLBCL) is a rare lymphoma characterized by the selective growth of lymphoma cells within the lumen of vessels. We describe the case of a 69-year-old male who presented with marked pain in the left facial region. Gadolinium-enhanced magnetic resonance imaging revealed a swollen left trigeminal nerve (TN) and positron emission tomography/computed tomography demonstrated fluorodeoxyglucose-only uptake at the same site. The patient had high serum lactate dehydrogenase and soluble interleukin-2 receptor levels. As random skin biopsy and bone marrow biopsy detected no abnormal pathogenesis, open biopsy of the TN was performed, revealing diffuse large B-cell lymphoma (DLBCL). However, ground glass opacities rapidly developed in both lung fields with severe respiratory failure. The patient died of progressive disease before the initiation of chemotherapy. Postmortem examination revealed widespread lymphoma cells in the lumen of vessels in multiple organs, including the lungs, excluding the bone marrow and skin. Lymphoma cells formed a mass in the TN and left lumbar plexus. A diagnosis of IVLBCL was made based on the postmortem pathological analysis. DLBCL of abnormal sites, such as the peripheral nervous system, should be considered in cases of IVLBCL as a differential diagnosis.