Thirty-year clinical experience in gamma knife radiosurgery for trigeminal schwannomas.

We aimed to evaluate the radiographic and clinical outcomes after gamma knife radiosurgery (GKRS) for trigeminal schwannomas (TSs). A total of 87 patients who underwent GKRS for TSs between 1990 and 2020 were enrolled. The mean tumor volume was 4.3 cm3. The median prescribed dose for the margins of the tumor was 13 Gy. The median follow-up duration was 64.3 months (range 12.0-311.5 months). The overall local tumor control rate was 90%, and the symptom response rate was 93%. The response rate for each symptom was 88% for facial pain, 97% for facial sensory change, and 86% for cranial nerve deficits. Nineteen (22%) patients showed transient swelling, which had regressed at the time of the last follow-up. Cystic tumors were associated with transient swelling (p = 0.04). A tumor volume of < 2.7 cm3 was associated with local tumor control in univariable analysis. Transient swelling was associated with symptom control failure in both univariable and multivariable analyses (p = 0.04, odds ratio 14.538). GKRS is an effective treatment for TSs, both for local control and symptom control.

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.

Trigeminal neuropathy as presenting symptom of craniofacial venous metameric syndrome.

AIMS: Trigeminal neuropathy is more likely to suggest neuronal damage and occur due to secondary pathology than trigeminal neuralgia. Evaluation of underlying etiologies are necessary. CASE: A 29-year-old female patient presented with left sided continuous burning pain likened to pins and needles at maxillary distribution for about a year. Her examination was normal except left-sided buccal swelling without any skin or mucosal change. Cranial MRI revealed asymmetrical dilation of left Meckel's cave, bilateral cerebral developmental venous anomaly and left sided slow flow venous malformation from superior temporal fossa to masseter muscle. Cerebral angiography confirmed widespread venous return anomaly in both cerebral hemispheres and slow-flow venous malformation that does not fill in the early arterial phase in the left buccal space and superficial temporal fossa. Cerebrofacial venous metameric syndrome is diagnosed. Percutaneous sclerotherapy with alcohol is planned in three separate sessions, the first of the three planned sessions is performed yet and the patient stated that her neuropathic pain decreased by 40% afterwards. SIGNIFICANCE: Clinical manifestation of the cerebrofacial venous metameric syndrome depends on the localization of the lesions; therefore, venous anomalies in relation with the trigeminal branches can present with painful trigeminal neuropathy.

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.

Anterior Petrosal Approach for Enlarging Previously Radiated Recurrent Trigeminal Schwannoma: 2-Dimensional Operative Video.

Trigeminal nerve schwannomas (TNSs) are rare lesions that typically present with symptoms of trigeminal neuropathy or other cranial nerve palsies. These lesions classically have a dumbbell shape, with an anterior component within Meckel's cave and posterior component extending into the posterior fossa through the porus trigeminus. Surgical resection of TNSs can often be achieved via an extradural subtemporal approach to Meckel's cave without an anterior petrousectomy, even for tumors with a significant posterior fossa component, as the tumor often erodes a portion of the petrous apex.1 We present the case of a 53-yr-old female presenting to our institution with complete trigeminal neuropathy secondary to a right-sided, previously resected and radiated TNS. Serial imaging demonstrated an interval growth of significant residual tumor despite multiple adjuvant therapies, and, thus, the patient was recommended to undergo additional surgical resection. The lesion was approached through a right-sided subtemporal approach to Meckel's cave,2 with a plan to utilize an anterior petrousectomy only if difficulty resecting the posterior fossa component of the tumor was encountered. Intraoperatively, the posterior fossa component was found to be densely adherent to the adjacent brainstem, likely secondary to prior surgery and radiation therapy, and, thus, an anterior petrousectomy was performed. Postoperatively, the patient had stable trigeminal neuropathy without any new neurological deficits and a magnetic resonance imaging (MRI) confirmed a gross total resection. In the accompanying video, we hope to demonstrate the steps and nuances of both the subtemporal approach to accessing Meckel's cave and anterior petrousectomy when employed for the resection of TNSs. The patient in question provided formal consent for the making of this video.

Epidermoid Cyst Extending Along Trigeminal Nerve Pathway with Unusual Imaging Findings.

Epidermoid cysts (ECs) are benign extraaxial tumors. They frequently occur at the cerebellopontine angle and parasellar regions. However, they rarely occur in the Meckel's cave. Typically, ECs appear as a hypointense mass on T1-weighted magnetic resonance imaging (MRI) and hyperintense on T2-weighted MRI. However, ECs may occasionally present as hyperintense on T1-weighted imaging and hypointense on T2-weighted imaging. When this occurs, they are known as white epidermoid cysts. We present a case of a 25-year-old woman with a 3-month history of hypoesthesia in the distribution of the right trigeminal nerve. MRI showed a lesion located within the Meckel's cave. The MRI signal was heterogeneous, with hyperintense areas on T1-weighted images, being hypointense on T2-weighted imaging. Preoperative suspicion was trigeminal schwannoma with unusual radiologic features. Finally, the pathologic diagnosis was epidermoid cyst. Therefore to establish a proper preoperative diagnosis, one should be aware that ECs can occur in Meckel's cave and with unusual radiologic features, as occurred in the case described earlier.

Pediatric Use of Recombinant Human Nerve Growth Factor 20 µg/mL Eye Drops (Cenegermin) for Bilateral Neurotrophic Keratopathy in Congenital Corneal Anesthesia.

PURPOSE: This study aimed to present the efficacy and safety of cenegermin eye drop (Oxervate; Dompè Farmaceutici, Milan, Italy) treatment in a pediatric patient affected by neurotrophic keratopathy (NK) with Goldenhar syndrome. METHODS: This case reports an infant presenting ulceration and a small central opacity in the cornea of the right and left eyes, respectively. The NK bilaterally worsened despite the use of therapeutic contact lenses and temporary partial tarsorrhaphy. Magnetic resonance imaging showed absence and hypoplasia of the right and left trigeminal nerves, respectively. Cenegermin eye drops were administered 1 drop/each eye, 6 times daily for 8 weeks to promote corneal healing. RESULTS: Complete healing was achieved in both eyes after treatment. During the 16-month follow-up period, no epithelial defect, recurrence, or complications were noticed, whereas corneal opacities progressively became clearer, although insignificant improvements in corneal sensitivity or in the reflex tearing were observed. CONCLUSIONS: Cenegermin was effective in treating NK in an infant with Goldenhar syndrome.

Neuroaxonal Degeneration in Patients With Multiple Sclerosis: An Optical Coherence Tomography and in Vivo Corneal Confocal Microscopy Study.

PURPOSE: To investigate the effect of multiple sclerosis (MS) on corneal and retinal nerve fiber by quantifying corneal subbasal nerve fibers and retinal ganglion cells. METHODS: A total of 46 eyes of 23 patients with MS and 42 eyes of 21 healthy subjects were included in the study. All patients and healthy subjects underwent a comprehensive ocular examination. In vivo confocal microscopy with Heidelberg Retina Tomograph in association with Rostock Cornea Module (Heidelberg Engineering, Heidelberg, Germany) and a swept-source optical coherence tomography (Topcon Corporation) were performed in all patients and healthy subjects. The number of subbasal nerve fibers and the nerve fiber density were calculated. Student t test was used to compare eyes with MS with control eyes. The normal distribution was first confirmed with the Shapiro-Wilk test. RESULTS: A statistically significant (P < 0.05) decrease was found for nerve fiber number, ganglion cell-inner plexiform layer, and retinal nerve fiber layer in patients with MS compared with those of healthy subjects. Moreover, an inverse correlation was found between retinal nerve fiber layer (r = -0.32), nerve fiber number (r = -0.47), and ganglion cell-inner plexiform layer (r = -0.51) and Expanded Disability Status Scale. A direct correlation between Expanded Disability Status Scale and optic neuritis frequency was found (r = 0.322). CONCLUSIONS: In vivo confocal microscopy showed a difference in corneal morphological parameters and retinal damage; moreover, these changes seemed to be related to the degree of neurological disability. Both retinal ganglion and trigeminal cell atrophy measurements could become affordable and accessible biomarkers for clinical trials in progressive disease.

Diagnostic Values of Clinical and Magnetic Resonance Findings in Presumptive Trigeminal Neuropathy: 49 Dogs.

The goal of this retrospective, cross-sectional study was to describe the different etiologies of trigeminal neuropathy based on clinical and MRI findings and to evaluate the significance of associated concomitant disorders. MRI studies of 49 dogs with trigeminal neuropathy were blindly reviewed and were classified into the following three groups: neoplasia, neuritis, or idiopathic trigeminal neuropathy (ITN). Thirty-one percent were suspected to have neoplasia (all unilateral), 16% to have neuritis (1 bilateral and 7 unilateral), and 53% to have ITN (4 unilateral and 22 bilateral). Dogs with clinical bilateral trigeminal dysfunction were most likely to have a diagnosis of ITN (predicted probability 95.7%). Unilateral clinical signs were significantly associated with neoplasia or neuritis compared with ITN (P < .001 and P = .002, respectively). Even with marked brainstem neoplastic involvement, central neurological deficits may be absent. Sensory impairment was significantly associated with either neoplasia or neuritis compared with ITN (P = .007 and P = .03, respectively). Ipsilateral noninfectious middle ear effusion was only seen in dogs with neoplasia (33%). Horner's syndrome was present in 12% of all dogs (2 dogs in each group). Dogs with neoplasia were significantly older than dogs with neuritis (P = .02) and ITN (P = .002). JAAHA-MS-6997.

A Trigeminal Schwannoma Masked by Solely Vestibulocochlear Symptoms.

BACKGROUND: Intracranial schwannomas are most commonly associated with the vestibulocochlear nerve, often leading to hearing loss, tinnitus, and vestibular dysfunction. Much less often, a schwannoma can arise from the trigeminal nerve which can lead to facial pain, numbness, and weakness. PURPOSE: We explored a case of a patient with an magnetic resonance imaging (MRI)-confirmed trigeminal schwannoma that was mistaken for a vestibulocochlear schwannoma because of a myriad of ipsilateral vestibulocochlear symptoms. RESEARCH DESIGN: This is a retrospective chart review and case study, with no statistics applied. RESULTS: This diagnostic error led to clinical confusion and inaccurate medical record-keeping. Radiologists and radiation oncologists deemed the patient's symptoms to be unrelated to the asymptomatic trigeminal schwannoma, and she was referred to an otolaryngologist following complaints of ear fullness, ear pain, and hearing loss. The patient's audiogram showed ipsilateral, asymmetric sensorineural hearing loss, and she was diagnosed with concurrent Meniere's disease. Alternative explanations, such as an additional schwannoma or external compression of the vestibulocochlear nerve, were considered, but not apparent on MRI. CONCLUSIONS: From this case, we see that symptoms do not always concur with imaging results and that multiple etiologies, especially when one is rare, can confuse a clinical picture.

Alterations in grey matter density and functional connectivity in trigeminal neuropathic pain and trigeminal neuralgia: A systematic review and meta-analysis.

BACKGROUND: Various studies reported changes in grey matter volumes and modifications in functional connectivity of cortical and subcortical structures in patients suffering from trigeminal neuralgia (TN) and trigeminal neuropathic pain (TNP). This study meta-analyzed the concordant structural and functional changes in foci and provide further understanding of the anatomy and biology of TN/TNP. METHODS: Relevant articles on magnetic resonance imaging (MRI) and functional MRI in TN/TNP, published before August 2018, were searched for on PubMed and Embase. Following exclusion of unsuitable studies, a meta-analysis was performed using activation likelihood estimation (ALE). RESULTS: In total, 322 paper were identified, 11 of which could be included based on the predefined inclusion and exclusion criteria. Eight papers, totaling 279 subjects, discussing structural changes and four papers, totaling 102 subjects, discussing functional changes were included (i.e., one paper investigated both structural and functional alterations). ALE analysis showed that in TN/TNP, grey matter decreases are found in the thalamus, (anterior) cingulate gyrus, bilateral striatum, the superior-, middle- and transverse temporal gyrus, subcallosal gyrus, the bilateral insular cortex, the pre- and postcental gyrus, the middle frontal gyrus bilaterally and the anterior cerebellar lobe. Grey matter increases were seen in the periaqueductal grey (PAG). Increased resting state functional organization was found within the bilateral middle- and superior frontal gyri, the (posterior) cingulate cortex and the thalamus/pulvinar. CONCLUSIONS: Structural and functional changes meta-analyzed in this paper may contribute to elucidating the central pathophysiological mechanisms involved in TN/TNP. These results may be used as biomarkers to predict the response to medication and, ideally, in the future to offer personalized treatments.

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.

Trigeminal Nerve Compression Without Trigeminal Neuralgia: Intraoperative vs Imaging Evidence.

BACKGROUND: While high-resolution imaging is increasingly used in guiding decisions about surgical interventions for the treatment of trigeminal neuralgia, direct assessment of the extent of vascular contact of the trigeminal nerve is still considered the gold standard for the determination of whether nerve decompression is warranted. OBJECTIVE: To compare intraoperative and magnetic resonance imaging (MRI) findings of the prevalence and severity of vascular compression of the trigeminal nerve in patients without classical trigeminal neuralgia. METHODS: We prospectively recruited 27 patients without facial pain who were undergoing microvascular decompression for hemifacial spasm and had undergone high-resolution preoperative MRI. Neurovascular contact/compression (NVC/C) by artery or vein was assessed both intraoperatively and by MRI, and was stratified into 3 types: simple contact, compression (indentation of the surface of the nerve), and deformity (deviation or distortion of the nerve). RESULTS: Intraoperative evidence of NVC/C was detected in 23 patients. MRI evidence of NVC/C was detected in 18 patients, all of whom had intraoperative evidence of NVC/C. Thus, there were 5, or 28% more patients in whom NVC/C was detected intraoperatively than with MRI (Kappa = 0.52); contact was observed in 4 of these patients and compression in 1 patient. In patients where NVC/C was observed by both methods, there was agreement regarding the severity of contact/compression in 83% (15/18) of patients (Kappa = 0.47). No patients exhibited deformity of the nerve by imaging or intraoperatively. CONCLUSION: There was moderate agreement between imaging and operative findings with respect to both the presence and severity of NVC/C.

Case Report: Metastasis of a Trigeminal Malignant Peripheral Nerve Sheath Tumor to the Corpus Callosum.

BACKGROUND AND IMPORTANCE: Malignant peripheral nerve sheath tumors (MPNST) are relatively rare tumors of peripheral nerves that are notable for their locally aggressive nature, ability to metastasize, poor prognosis, and association with Neurofibromatosis type I. We present the case of a patient with a trigeminal nerve MPNST who developed an unusual metastasis to the corpus callosum, in the absence of any other central nervous system or systemic metastatic disease. We review the pathology and presentation of MPNST. CLINICAL PRESENTATION: A 53-yr-old woman presented with a 1-yr history of paroxysmal facial pain and dysesthesias in the right V1 and V2 distributions of the trigeminal nerve. She was initially diagnosed with trigeminal neuralgia although further imaging showed a cavernous sinus mass extending along the trigeminal nerve. She later developed an isolated lesion in the corpus callosum that was biopsied and consistent with MPNST. CONCLUSION: This case reviews the pathology and aggressive nature of MPNST and demonstrates an unusual site of metastasis. Clinicians should remain aware that MPNST can metastasize to sites in the central nervous system as well as systemically. Furthermore, clinicians should have a high index of suspicion for secondary causes of trigeminal neuralgia in cases with atypical features.

Clinical findings and outcome of dogs with unilateral masticatory muscle atrophy.

BACKGROUND: Little is known about the spectrum of underlying disorders in dogs with unilateral masticatory muscle (MM) atrophy. OBJECTIVES: To evaluate the clinical presentation, magnetic resonance imaging (MRI) findings, and outcome of dogs with unilateral MM atrophy. ANIMALS: Sixty-three client-owned dogs. METHODS: The medical database was retrospectively reviewed for dogs that underwent MRI for evaluation of unilateral MM atrophy. Imaging studies were reviewed and follow-up information was obtained from telephone interviews. RESULTS: Presumptive trigeminal nerve sheath tumor (pTNST) was diagnosed in 30 dogs (47.6%); survival time varied from 1 day to 21 months (median, 5 months). Other extra-axial mass lesions were observed in 13 dogs (20.6%); survival time varied from 6 days to 25 months (median, 2.5 months). In 18 dogs (28.6%), no abnormalities were observed on MRI; neurological signs only progressed in 1 dog. Diagnosis had a significant influence on the type of neurological abnormalities, with additional neurological deficits observed in most dogs with pTNST and in all dogs with other extra-axial mass lesions. Diagnosis had a significant effect on euthanasia at the time of diagnosis and likelihood of neurological deterioration. Dogs with mass lesions were more likely to be euthanized or experience neurological deterioration, whereas these outcomes occurred less often in dogs in which no causative lesion could be identified. CONCLUSIONS AND CLINICAL IMPORTANCE: Trigeminal nerve sheath tumors should not be considered the only cause of unilateral MM atrophy. Our results illustrate the importance of performing a neurological examination and MRI when evaluating dogs with unilateral MM atrophy.

Neuroendoscopic Resection of Trigeminal Schwannoma in the Pterygopalatine/Infratemporal Fossa via the Transnasal Perpendicular Plate Palatine Bone or Transnasal Maxillary Sinus Approach.

BACKGROUND: Both the pterygopalatine fossa (PPF) and the infratemporal fossa (ITF) lie outside the midline of the skull base. Lesions in the PPF or ITF include trigeminal schwannoma (trigeminal schwannoma, TS), which originates from the second or third branch of the trigeminal nerve (maxillary nerve or mandibular nerve). Due to their typically deep anatomic location, lesions in the PPF or ITF can be difficult to treat using traditional surgical approaches. In recent years, because of their advantages, which include the fact that they allow the problem to be observed close up, neuroendoscopic techniques are increasingly being applied in skull base surgery, especially in treatment of lesions around the midline of the base of the skull. This study aims to 1) evaluate the neuroendoscopic treatment of lesions in PPF or ITF via the transnasal palate bone perpendicular plate or transnasal maxillary sinus approach and 2) analyze the clinical significance of this approach. METHODS: We retrospectively analyzed 3 cases of PPF TSs and 1 case of ITF TS treated between January 2015 and May 2017. All of the cases underwent neuroendoscopic resection of TSs located in the PPF via the nasal perpendicular plate palatine bone (or nasal maxillary sinus) approach. RESULTS: Two cases of PPF TSs were characterized by a thin palate bone perpendicular plate due to oppressed absorption of the tumor. Therefore the endoscopic transnasal palate bone perpendicular plate approach was employed. Additionally, 1 case of PPF TSs and 1 case of ITF TS were resected via the transnasal maxillary sinus approach. All 4 patients received total resection under endoscopy and recovered well after their respective operations without cerebrospinal fluid leakage, although 1 patient experienced postoperative dry eye symptoms and 1 other patient showed no improvement in facial numbness before and after the operation. CONCLUSIONS: Neuroendoscopic surgery performed via the transnasal perpendicular plate palatine bone or transnasal maxillary sinus approach has its own unique advantages in removing TSs in PPF and in ITF: Notably, the tumor can be exposed and dealt with under direct vision, which prevents damage to important structures, such as the internal carotid and maxillary nerves, while at the same time helping to achieve total removal of TSs. Furthermore, by adopting this approach versus traditional skull base surgery, postoperative trauma can be reduced significantly, which should be advocated for in this time of minimal invasive surgery.

Posttraumatic Trigeminal-Cavernous Fistula.

INTRODUCTION: Persistent trigeminal artery is the most frequent embryonic communication between the vertebrobasilar and carotid systems. To the best of our knowledge, posttraumatic trigeminal-cavernous fistula is rarely reported in the literature. CASE PRESENTATION: We present a 47-year-old man with posttraumatic trigeminal-cavernous fistula, which we treated using Onyx embolization. CONCLUSION: Even though preservation of the parent artery is generally considered a desirable goal in fistula treatment, in special cases such as Salzmann type 2 anatomy plus a unique fistulous orifice accompanied by external carotid-cavernous fistula, it is required to embolize the persistent trigeminal artery and cavernous sinus.

Malignant Peripheral Nerve Sheath Tumor of the Trigeminal Nerve Involving the Middle and Posterior Cranial Fossa.

BACKGROUND: Although benign trigeminal schwannomas are uncommon, malignant peripheral nerve sheath tumors (MPNSTs) of the trigeminal nerve are extraordinarily rare. CASE DESCRIPTION: A 56-year-old female presented with a 2-month-long history of numbness of the right face and progressive weakness of the left limbs. Preoperative neuroimages indicated a giant tumor involving the middle and posterior cranial fossa with similar radiologic characteristics to benign trigeminal schwannomas. However, histopathologic and immunochemical examinations confirmed the tumor to be an MPNST. A nearly gross total resection was obtained with a combined frontotemporal extradural and subtemporal anterior petrosal approach. The postoperative course was uneventful, and the patient received adjuvant radiotherapy subsequently. There was no recurrence of the tumor with a 6-month-long follow-up. CONCLUSION: MPNSTs of the trigeminal nerve are exceedingly rare. This study described the 21st case of MPNSTs of the trigeminal nerve. MPNSTs of the trigeminal nerve showed similar radiologic characteristics to benign trigeminal schwannomas, and accurate diagnosis depended on pathologic and immunochemical examinations. Gross total resection followed by radiotherapy is the usual treatment.