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.

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.

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.

Tetraventricular noncommunicating hydrocephalus: Case report and literature review.

BACKGROUND: Tetraventricular hydrocephalus is a common presentation of communicating hydrocephalus. Conversely, cases with noncommunicating etiology impose a diagnostic challenge and are often neglected and underdiagnosed. Herein, we present a review of literature for clinical, diagnostic, and surgical aspects regarding noncommunicating tetrahydrocephalus caused by primary fourth ventricle outlet obstruction (FVOO), illustrating with a case from our service. METHODS: We performed a research on PubMed database crossing the terms "FVOO," "tetraventriculomegaly," and "hydrocephalus" in English. Fifteen articles (a total of 34 cases of primary FVOO) matched our criteria and were, therefore, included in this study besides our own case. RESULTS: Most cases presented in adulthood (47%), equally divided between male and female. Clinical presentation was unspecific, commonly including headache, nausea, and dizziness as symptoms (35.29%, 21.57%, and 9.80%, respectively), with ataxic gait (65%) and papilledema (40%) being the most frequent signs. MRI and CT were the imaging modalities of choice (11 patients each), often associated with CSF flow studies, such as cine MRI and CT ventriculogram. Endoscopic third ventriculostomy (ETV) was both the most popular and effective surgical approach (50.85% of cases, with 18.91% of recurrence) followed by ventricle-peritoneal shunt (16.95% of patients, 23.0% of recurrence). CONCLUSION: FVOO stands for a poorly understood etiology of noncommunicating tetrahydrocephalus. With the use of ETV, these cases, once hopeless, had its morbimortality and recurrence reduced greatly. Therefore, its suspicion and differentiation from other forms of tetrahydrocephalus can improve its natural course, reinforcing the importance of its acknowledgment.

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.

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.