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.

Decompressive Hemicraniectomies as a Damage Control Approach for Multilobar Firearm Projectile Injuries: A Single-Center Experience.

BACKGROUND: While firearms projectile injuries to the head carry a high rate of morbidity and mortality, current literature in clinical management remains controversial. Decompressive hemicraniectomy (DHC) has been previously described in the neurosurgical literature for traumatic brain injuries, with positive results in the reduction of mortality. Here we aim to assess DHC as a damage control approach for multilobar firearm injuries to the head and compare our results with what is present in the literature. METHODS: A retrospective review of patients who sustained multilobar firearm injuries to the head admitted to our center from January 2009 to April 2021 was performed. Exclusion criteria were a Glasgow Coma Scale (GCS) score <5, and/or brain stem dysfunction that persisted despite stabilization and medical therapy for intracranial hypertension. RESULTS: A total of 20 patients were analyzed, with an average GCS on admission of 8.35. The 60-day mortality rate for all 20 patients was 20% with a total of 4 deaths, 1 of which was due to pulmonary sepsis in the critical postoperative care unit. The mean hospital stay of surviving patients was 22 days. CONCLUSIONS: DHC should be considered as a damage control strategy for young patients with multilobar firearm injuries and GCS >5, having yielded favorable results in this study when compared to current literature.

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.

Resection arthroplasty for isolated costotransverse joint osteoarthritis: A case report and literature review.

BACKGROUND: Symptomatic isolated costovertebral joint (CVJ) osteoarthritis is rare, and establishing this diagnosis is often difficult. There are few reports in the literature about how to surgically manage these lesions. Our aim was to describe a case of isolated osteoarthritis of the costotransverse joint (CTJ) successfully treated with a resection arthroplasty. CASE DESCRIPTION: A 51-year-old female presented with 3 years of the right paravertebral T 10-level back and radiating pain. No conservative treatment modality effectively resolved this pain (i.e., these included anti-inflammatory medications, physiotherapy, and joint blockages). MRI, CT, and technetium-99m methylene diphosphonate bone scintigraphy demonstrated inflammatory changes involving the right T10 CTJ. Following resection arthroplasty, the patient's symptoms abated. CONCLUSION: Symptomatic osteoarthritis of the right T10 CVJ successfully resolved following costotransversectomy for joint resection arthroplasty.

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.