Endolymphatic sac tumor: An urgent case presenting acute intracranial hypertension successfully treated with suboccipital decompressive craniectomy - 8 years of follow-up.

Background: Endolymphatic sac tumor (ELST) is a rare lesion. It may be sporadically or associated with Von Hippel-Lindau syndrome. Progressive audiovestibular symptoms characterize the typical clinical presentation. Here, we report a unique case of ELST with acute intracranial hypertension (IH) due to tumor compression, successfully treated with an urgent suboccipital decompressive craniectomy (SDC). Case Description: A 33-year-old woman previously underwent a biopsy and ventriculoperitoneal shunt. The histopathological finding revealed an ELST. One year later, she developed headache, vomiting, and somnolence due to brainstem compression. An urgent SDC was performed. One month later, preoperative endovascular embolization and partial tumor resection were carried out. After 6 months adjuvant radiotherapy (RT) therapy was administered. She has been under follow-up for 8 years since the last surgical procedure, and the tumor remains stable. Conclusion: ELST generally has a progressive clinical course. This is a unique case with acute IH due to tumor compression. The tumor's high vascularity and the unavailability of endovascular embolization precluded its resection. SDC was an alternative approach. The final treatment included tumor embolization, surgical resection, and RT. No progression was observed for 8 years after the last procedure, and long-term follow-up is warranted.

Isolated hypertelorism: Late surgical correction using the box osteotomy technique.

Background: Orbital hypertelorism is a rare congenital condition caused by craniofacial malformations. It consists of complete orbital lateralization, characterized by an increase in distance (above the 95th percentile) of the inner canthal (ICD), outer canthal, and interpupillary distances. It can be approached surgically, and the main techniques are box osteotomy and facial bipartition. The surgical procedure is usually performed before the age of 8. We describe here two patients who underwent late surgical correction using the box osteotomy technique. Case Description: Patient 1: A 13-year-old female presenting isolated hypertelorism with 5 cm ICD and left eye amblyopia. Patient 2: A 15-year-old female with orbital hypertelorism, 4.6 cm ICD, and nasal deformity. Both patients underwent orbital translocation surgery and had no neurological disorders. Conclusion: The article reports two cases of isolated hypertelorism treated late with the box osteotomy technique. Both surgeries were successful, with no postoperative complications. It appears that it is possible to obtain good surgical results even in patients who have not been able to undergo surgery previously.

Temporal bone squamous cell carcinoma: Aggressive behavior coursing with cerebellar invasion and hydrocephalus.

Background: Temporal bone squamous cell carcinoma (TBSCC) is a very rare condition. The prognosis is dismal for advanced tumors. Due to its rarity, information in the literature is scarce. Here, we report a unique case of TBSCC with cerebellar invasion and hydrocephalus. Case Description: A 46-year-old reported right-sided hearing loss and a painful right retroauricular mass for 4 months. Magnetic resonance imaging revealed a 8.7 × 7.6 × 6.4 cm mass invading the right temporal and occipital bones. After a biopsy and 3 surgical procedures over 6 months, the diagnosis of TBSCC was obtained. Due to invasion of the cerebellar tissue and obstructive hydrocephalus, a ventriculoperitoneal shunt was performed. The patient was referred for adjuvant radiotherapy. However, palliative care was initiated due to tumor progression. Conclusion: We report a case of advanced TBSCC with poor prognosis despite surgical treatment and radiotherapy. More data are necessary to provide new and better treatment to these patients.

Bovine Pericardium Treated with Polyethylene Glycol and Ethanol Versus Pericranium for Duraplasty: A Pilot Study in Supratentorial Neurosurgery.

BACKGROUND: Watertight closure of dura mater after intracranial surgery can avoid cerebrospinal fluid leakage and central nervous system infection and herniation. When primary closure is not possible, the pericranium is the preferential choice. When it is not available, a dural substitute becomes necessary. Bovine pericardium treated with polyethylene glycol and ethanol is herein tested as a dural substitute. METHODS: A pilot study comparing bovine pericardium with pericranium in supratentorial neurosurgery was performed. RESULTS: Twenty patients were initially allocated into a bovine pericardium group (group 1) or a pericranium group (group 2). Three patients from group 1 and 2 from group 2 had a loss of follow-up, being excluded. In the remaining 15 patients, epidemiological analysis demonstrated a male:female ratio of 3:4 and 4:4 for groups 1 and 2. Ages varied from 28 to 68 (Mean = 49.6) in group 1 and 40-80 (Mean = 61.2) in group 2, with a mean difference of 11.68 years (P = 0.09). Two cases of surgical site infection and 1 of hydrocephalus were observed. Although the calculated relative risk for complications was higher in group 1 (Relative Risk = 1.08), Fisher exact test demonstrated no statistically significant difference between groups (P = 1.00). Procedure mean time was 23 minutes and 11 seconds in group 1 versus 27 minutes and 55 seconds in group 2 (P = 0.47). Mean graft area was 13.17 and 6.23 cm2 in groups 1 and 2 (P = 0.02). CONCLUSIONS: Bovine pericardium treated with polyethylene glycol and ethanol was comparable to pericranium as a dural substitute. More studies are encouraged to certify our findings.

Endoscopic endonasal transsphenoidal approach during COVID-19 pandemic in Brazil.

Background: Endoscopic endonasal transsphenoidal approach (EETA) is a well-established technique for sellar tumor resection. However, this route causes aerosol dispersion from the nasal cavity. In the context of the coronavirus (COVID-19) pandemic, new measures were taken aiming at the safety and protection of patients and health-care professionals. Herein, we present a Brazilian experience with EETA during COVID-19 pandemic. Methods: This study was based on the review of medical records and observation in the operating room of the patients undergoing endoscopic surgery in the period from May 2020 to July 2022. All patients were tested by real-time polymerase chain reaction (RT-PCR) COVID-19 before and after surgery. Since September 2021, it has been mandatory to present vaccination cards for adults (over 18 years old). Results: This case series included 28 patients and 35 surgical procedures using the EETA, who presented of nonfunctioning macroadenomas (19 cases - 67.8%), GH-secreting tumor (three cases - 10.8%), ACTH-secreting tumor (three cases - 10.8%), meningiomas (two cases - 7.1%), and Rathke's cleft cyst (one case - 3.5%). There were eight cases of diabetes insipidus (28.5%), five cases of cerebrospinal fluid leak (17.8%), and one case of meningitis (3.5%). Three patients died due to meningitis (one case), carotid occlusion (one case), and COVID-19 complications (one case). Conclusion: A simple protocol was established to perform EETA during the COVID-19 pandemic. The pituitary surgeries were maintained to treat critical cases. To date, the protocol should be continually updated to improve the procedure's safety.

Cavum septum pellucidum and vergae cyst: A symptomatic case with intracranial hypertension and multiple nerve involvement.

Background: Cavum septum pellucidum (CSP) and cavum vergae (CV) are normal anatomical variations present in some children, adolescents, and adults. When the cavity is larger than normal, it is called a cyst. Symptomatic cases of CSP and CV cyst are rare, and the clinical presentation is varied. A case with multiple nerve involvement is described. Case Description: A healthy 17-year-old female presented a sudden headache, right cervicobrachialgia, right ptosis, visual changes, and left facial paralysis over 10 days. Head magnetic resonance imaging revealed CSP and CV cyst. Inflammation, infection, and vascular disorders were ruled out. We decided to perform a right transfrontal endoscopic intraventricular septostomy and a right Monro foraminoplasty, which were successful. One month after surgery, the patient had no more signs or symptoms. She has remained asymptomatic for the past year. Conclusion: Multiple nerve involvement was directly related to CSP and CV cyst. The cyst bilaterally occluded the foramen of Monro causing intracranial hypertension. It was possible to obtain complete resolution of the clinical features through neuroendoscopic fenestration and foraminoplasty.

Spinal dural arteriovenous fistula rupture after Rathke's cleft cyst endoscopic resection: Case report and literature review.

BACKGROUND: Spinal dural arteriovenous fistula (SDAVF) is the most frequent vascular malformation of the spine and accounts for approximately 70% of all vascular spinal malformations. In rare cases, SDAVF rupture and subsequent subarachnoid hemorrhage or intramedullary hematoma may occur. The aim of this article is to present a fatal case of SDAVF rupture after a Rathke's cleft cyst (RCC) endoscopic resection. CASE DESCRIPTION: An 80-year-old female was referred to our hospital with a clinical presentation of bilateral reduction in visual acuity, bitemporal hemianopsia, and sellar magnetic resonance imaging (MRI) highly suggestive of RCC. After the first endonasal endoscopic surgery, the cyst was partially removed and vision improved. No signs of cerebrospinal fluid (CSF) leak were observed. After 1 year, the patient returned because of RCC recurrence and decreased visual acuity. In the second procedure, the lesion was totally resected and CSF leak was observed. A nasoseptal flap was rotated to cover the skull base defect. The patient developed subtle paraparesis followed by paraplegia on the 4th postoperative day. The dorsal spine MRI revealed a T3-T4 intramedullary hematoma. A dorsal laminectomy was performed and a SDAVF was observed. During microsurgery, at the right T3 nerve root level, an arteriovenous shunting point was identified, coagulated, and divided. The intramedullary hematoma was evacuated. The patient developed neurogenic and septic shock and died. CONCLUSION: Venous hypertension, venous wall fragility, and venous thrombosis seem to be the main factors involved in SDAVF rupture. In this particular case, reduction of the extravascular pressure and sudden variation in the pressure gradient caused by sustained CSF leak, also appeared to play an important role in SDAVF rupture. It may represent one more complication related to radical resection of RCC.

Ganglioglioma of the cervicothoracic spinal cord in a patient with neurofibromatosis type 1: A case report.

BACKGROUND: Gangliogliomas are rare tumors of the central nervous system. They are usually located intracranially and rarely in the spinal cord. There is no clear correlation between this tumor and neurofibromatosis type 1 (NF1) with only four cases described. The aim of this article is to describe one more case and add data to the literature regarding this rare association. CASE DESCRIPTION: An 8-year-old boy with NF1 presented progressive asymmetrical paraparesis (Grade 4 medical research council scale on the right leg and Grade 3 on the left leg). The cervicothoracic spinal magnetic resonance imaging demonstrated an intramedullary lesion from C4 to T4 vertebrae. The patient underwent a microsurgical resection. A partial resection was performed due to a drop in the motor evoked potential signal amplitude during dissection. Pathology report revealed a ganglioglioma (World Health Organization Grade I). Postoperatively, the patient evolved with worsening of the paraparesis. A few weeks later, he has improved his preoperative functional neurological state (better strength and gait). Adjuvant radiotherapy was not used. The patient is being followed up at the neurosurgery outpatient clinic. CONCLUSION: This is another case of spinal ganglioglioma associated with NF1. The tumor must be included in the differential diagnosis of patients with NF1 and spinal lesions. Complete microsurgical resection remains the standard treatment for spinal gangliogliomas, however, in this specific case, it was decided to leave a portion of the tumor to prevent neurological damage. The prognosis and treatment of this condition associated with NF1 remains to be determined.

Removal of the Retained Ventricular Catheter Using the Endoscopic Monopolar Instrument.

BACKGROUND: Ventriculoperitoneal shunt (VPS) remains the main treatment for hydrocephalus. However, VPS revision surgery is very common. Here, we present a case in which the retained ventricular catheter was removed using the endoscopic monopolar instrument. METHODS: We report a case of a 28-year-old female who presented with VPS obstruction. She had two previous shunt revision surgeries due to shunt obstruction. Eleven years after the last one, she presented an abdominal pseudocyst that indicated a total system removal. During VPS revision surgery, a retained ventricular catheter was observed. The endoscopic monopolar instrument was introduced into the retained catheter under direct view. Coagulations in a back-and-forth movement were applied to release inner catheter adhesions. After these steps, the catheter was removed, and a new one was placed through the same route. RESULTS: The catheter was removed without complications, confirmed by the postoperative cranial computed tomography. The patient remained asymptomatic. CONCLUSION: The described technique was effective and avoided ventricular bleeding. Further studies are necessary to validate this method.

Measurement of Active Motor Threshold Using a Dynamometer During Navigated Transcranial Magnetic Stimulation in a Patient with Postoperative Brain Tumor: Technical Note.

BACKGROUND: Navigated transcranial magnetic stimulation (nTMS) is being used for different purposes in patients with brain tumors. However, the procedure requires a positive electrophysiological response. For patients with negative response in rest conditions, active motor threshold (AMT) may be used. However, sometimes it is difficult to obtain AMT measures owing to inability of the patient to sustain steady muscle contraction. Herein, we describe a simple method by using a hand dynamometer to obtain AMT measures during nTMS session. CASE DESCRIPTION: A woman aged 68 years underwent total removal of a right frontal lobe oligodendroglioma World Health Organization grade II 15 years ago. Cranial magnetic resonance imaging during follow-up revealed local recurrence. In the postoperative period, she developed left upper limb paresis. A postoperative nTMS session was performed for motor electrophysiological evaluation. However, using the standard technique for AMT measurement, the patient was unable to perform sustained muscle contraction as required. A hand dynamometer was used. It allowed sustained muscle contraction for AMT measurement. A counter force for the index finger flexion, the hand support to stabilize hand joints, and a numerical screen serving for both the examiner and the patient as a feedback parameter may explain the success obtained with this simple device. CONCLUSIONS: Although more studies are necessary to validate the method, the hand dynamometer should be considered for patients unable to sustain muscle contraction during AMT measurement.

Computed tomography-guided navigated transcranial magnetic stimulation for preoperative brain motor mapping in brain lesion resection: A case report.

BACKGROUND: Navigated transcranial magnetic stimulation (nTMS) is a well establish a noninvasive method for preoperative brain motor mapping. We commonly use magnetic resonance imaging (MRI) to supply the nTMS system. In some cases, MRI is not possible or available, and the use of computed tomography (CT) is necessary. We present the first report describing the association of CT and nTMS motor mapping for brain lesion resection. CASE DESCRIPTION: CT imaging of a 59-year-old man suffering from acquired immune deficiency syndrome for 17 years, presenting with seizure and right hemiparesis, revealed a small single hypodense ring-enhancing lesion in the left central sulci suggesting cerebral toxoplasmosis. After 3 weeks of neurotoxoplasmosis treatment, due to four consecutive tonic-clonic seizures, a new CT scan was performed and showed no lesion changes. MRI was in maintenance at that time. Infectious diseases department suggested a brain lesion biopsy. Due to lesion's location, we decided to perform a presurgical nTMS motor mapping. After a small craniotomy, we could precisely locate and safely totally remove the lesion. The pathology report revealed a high suspicious toxoplasmosis pattern. The patient was discharged after 2 days and continued toxoplasmosis treatment. After 6 months follow-up, he showed no signs of any procedure-related deficits or radiological recurrence. CONCLUSION: We report the feasibility and applicability of nTMS motor mapping using CT scan as an image source. It gives neurosurgeons another possibility to perform motor mapping for brain lesion removal, especially when MRI is not available or feasible.

Microsurgical Treatment of Intraventricular Cavernoma with Prior Planning Neuroendoscopy.

Microsurgery remains the gold standard treatment for intraventricular lesions, but it is not without limitations. The cerebral ventricles approach is considered a challenge for neurosurgeons because of their deep location and their intimate association with critical areas of the brain. It has been shown that direct endoscopic view is superior to magnetic resonance imaging (MRI) in identifying intraventricular structures and lesions. Also, during neuroendoscopy, there is no cerebrospinal fluid leak causing brain collapse as seen in microsurgery. Different surgical strategies should be shared and may improve and facilitate intraventricular lesions resection. Herein, a case of a successful microsurgical cavernoma resection with prior ventriculoscopy is described. A 28-year-old woman was admitted with intense holocranial headache. A mild stiff neck was observed. MRI showed a 3-cm lesion with heterogeneous signal intensity and no contrast enhancement suggestive of cavernoma. Initially, a ventriculoscopy was done to inspect the lesion and the surrounding ventricular structures. After that, a conversion technique from endoscopy to microsurgery was performed. The initial corticectomy was extended and the endoscope pathway was followed into the ventricular cavity. Then, the cavernoma was completely removed by microsurgical technique. No complications were observed. Control MRI showed total tumour removal. The patient remained asymptomatic with no recurrence after 1 year. This case illustrates a successful prior endoscopic approach followed by microsurgery as an alternative to a direct microsurgical approach for intraventricular cavernoma. A previous direct endoscopic view of the lesion and its relation with intraventricular structures may ensure a safe and complete resection by microsurgery.

Conversion technique from neuroendoscopy to microsurgery in ventricular tumors: Technical note.

BACKGROUND: Ventricular tumors represent a major neurosurgical challenge, making endoscopic approach an invaluable tool as it gained importance due to technological advances. Nevertheless, the method is not exempt of risk and limitations, sometimes requiring an open surgery. Thus, initial measurements must be adopted in order to simplify an eventual need for conversion to open craniotomy. METHODS: Here, we describe a series of 6 patients with ventricular tumors approached by neuroendoscopy where the conversion to microsurgery turned out to be necessary. Patients' average age was 59.5 years (39-75 years). Average tumoral size was 17.8 mm (15-21 mm). There were 2 cases of lateral ventricle subependymoma and 4 cases of third ventricle colloid cysts. A standard surgical incision was performed in the coronal direction, allowing lateral expansion to 10 cm. Moreover, the endoscopic burr hole was enlarged to a 5 cm craniotomy. A small enlargement of the endoscopic cortical access was performed to gain a transcortical microsurgical corridor to the ventricular cavity. The need for conversion arose due to high consistency of the tumor (3 cases), technical problems (2 cases), and cortical collapse (1 case). RESULTS: There was one case of cerebrospinal fluid fistula and infection and one case of transitory memory disturbance. In both the cases, we obtained a complete functional recovery. Clinical and radiological follow-up showed total tumor removal with no recurrences. CONCLUSIONS: The technique herein described was easy to perform, promptly bypassed the endoscopic limitations, and gathered excellent surgical results. The possibility of adapting the method to other tumor locations may be considered.

Late obstruction of an endoscopic third ventriculostomy stoma by metastatic seeding of a recurrent medulloblastoma.

Endoscopic third ventriculostomy (ETV) has been established as an effective method in the treatment of tumoral obstructive hydrocephalus. Delayed closure of the ETV stoma has been attributed to scarring involving the third ventricular floor. Secondary obstruction of the stoma due to intraventricular tumor seeding is an extremely rare condition, with only one case described to date. The authors report on a unique case of late closure of the ETV stoma caused by metastatic seeding of a recurrent medulloblastoma 9 years after the initial treatment. The patient was submitted to a second endoscopic procedure in which a reddish mass located just at the third ventricular floor was resected, leading to reopening of the previous ETV stoma. To the best of the authors' knowledge, this case is the first report of an ETV obstruction due to metastatic CSF seeding of a medulloblastoma. In such cases of late failure of the ETV stoma because of tumor obstruction, a second ETV can be safely performed and lead to adequate function of the stoma, even if limited by the aggressive nature of the disease.

Acometimento do gânglio trigeminal na sarcoidose: relato de caso / Trigeminal ganglion involvement in sarcoidosis: case report

A sarcoidose é uma doença de causa desconhecida, vista comoinvasão tecidual por infiltrado granulomatoso não-caseoso.O sistema nervoso é afetado em casos raros, geralmente comodisseminação da doença situada em outros órgãos, principalmenteos pulmões. Apresentamos um caso no qual a pacienteapresentava apenas acometimento do sistema nervosocentral, exibindo uma lesão expansiva ao nível do gânglio deGasser direito e outra lesão no córtex parietal direito, observadasna ressonância magnética. O diagnóstico foi dado porestudo histopatológico da lesão, sendo que paciente obtevemelhora dos sintomas com terapia com corticosteróide.

Esclerose múltipla e atrofia muscular: relato de caso / Multiple sclerosis and muscle atrophy: a case report

Os autores relatam um caso de atrofia muscular em paciente com esclerose multipla e tentam correlacionar este achado atraves de estudos eletromiograficos e biopsia de musculo e nervo