Intracranial hypertension secondary to unruptured pial arteriovenous malformation. Suitability of isolated endovascular treatment with ethylene vinyl alcohol/dimethyl sulfoxide (Onyx®). Case report and literature review.

A 36-year-old male presented to the Emergency Department with clinical symptoms of blurred vision of progressive onset of two years of evolution. The ophthalmological examination revealed the existence of bilateral papilledema. Using cranial computed tomography and magnetic resonance imaging, the presence of a right occipital pial arteriovenous malformation was certified. Arteriographically, pial arterial contributions dependent on the right middle cerebral artery and the right posterior cerebral artery were identified. Venous drainage was located at the level of the superior sagittal sinus. An associated right transverse sinus stenosis was also identified. The existence of secondary intracranial hypertension was corroborated by monitoring with an intracranial pressure sensor. An interventional procedure was carried out consisting of embolization of the arterial supplies of the lesion using Onyx®. The clinical-radiological findings after the procedure were favorable: the papilledema disappeared and complete exclusion of the malformation was achieved. A new intracranial pressure measurement showed resolution of intracranial hypertension. Subsequent regulated radiological controls showed complete exclusion of the malformation up to 5 years later.

Lateral micro-orbitotomy as the technique of choice for orbital pseudotumor in IgG4-RD. Case report and review of the literature.

Immunoglobulin G4-related disease (IgG4-RD) is characterized by a systemic fibroinflammatory infiltrate that often involves the orbit in addition to other tissues. Thus it has to be considered in the differential diagnosis of orbital tumors. We report the clinical case of a 64-year-old woman who presented with right mydriasis, progressive proptosis and paralysis of the third cranial nerve of 1 year of evolution. Cranial MRI identified an intraconal lesion of the right orbit, located between the external and inferior rectus muscles and the optic nerve, and she was scheduled for surgery by transcranial approach with lateral micro-orbitomy. A satisfactory macroscopic excision was achieved with no remarkable complications and a definitive deferred histological result of pseudotumor by IgG4-RD. Follow-up for 24 months showed no tumor recurrence, and the patient clinically improved from ophthalmoplegia. This case highlights the efficacy of lateral orbitotomy in the etiologic diagnosis and successful therapeutic outcome of complex orbital lesions associated with IgG4-RD pseudotumor.

Neuroendoscopic access to the third ventricle in patients with narrow foramen of monro without stenosis/obstruction: role of foraminoplasty.

OBJECT: One of the critical steps for the success of intraventricular neuroendoscopic procedures is the entry into the third ventricle and passage of the endoscopy system through the foramen of Monro (FM). A diameter larger than that of the instrument used is considered a prerequisite for safely performing the technique, as damage to this structure can lead to alterations in the fornix and vascular structures. When the foramen diameter is narrow and there is no obstruction/stenosis, the role of foraminoplasty in reducing the risk of complications has not been adequately assessed in the literature. METHODS: A review of endoscopic procedures conducted at our center since 2018 was undertaken. Cases in which preoperative imaging indicated a FM diameter < 6 mm and foraminoplasty technique was applied were examined to determine the technical and functional success of the procedure. The technical success was determined by completing the neuroendoscopic procedure with the absence of macroscopic lesions in the various structures comprising the foramen and without complications in the follow-up imaging tests. Functional success was defined as the absence of cognitive/memory alterations during the 3-month postoperative follow-up. Additionally, a review of the various forms of foraminoplasty described in the literature is conducted. RESULTS: In our cohort, six patients were identified with a preoperative FM diameter < 6 mm without obstruction or stenosis. Foraminoplasty was planned for these cases to facilitate various intraventricular neuroendoscopic procedures. In all instances, the technique was successfully performed without causing macroscopic damage to the structures comprising the foramen. Follow-up visits included various cognitive tests to assess potential sequelae related to microscopic damage to the fornix. None of the patients exhibited anomalies. CONCLUSION: Foraminoplasty in patients with a narrow FM without signs of stenosis/obstruction is a useful technique to reduce the risk of complications during the passage of the endoscopy system through this structure, enabling the safe performance of neuroendoscopic procedures.

Improved lipid-lowering treatment and reduction in cardiovascular disease burden in homozygous familial hypercholesterolemia: The SAFEHEART follow-up study.

AIM: We aimed to describe clinical and genetic characteristics, lipid-lowering treatment and atherosclerotic cardiovascular disease (ASCVD) outcomes over a long-term follow-up in homozygous familial hypercholesterolemia (HoFH). METHODS: SAFEHEART (Spanish Familial Hypercholesterolaemia Cohort Study) is a long-term study in molecularly diagnosed FH. Data analyzed in HoFH were prospectively obtained from 2004 until 2022. ASCVD events, lipid profile and lipid-lowering treatment were determined. RESULTS: Thirty-nine HoFH patients were analyzed. The mean age was 42 ± 20 years and nineteen (49%) were women. Median follow-up was 11 years (IQR 6,18). Median age at genetic diagnosis was 24 years (IQR 8,42). At enrolment, 33% had ASCVD and 18% had aortic valve disease. Patients with new ASCVD events and aortic valve disease at follow-up were six (15%), and one (3%), respectively. Median untreated LDL-C levels were 555 mg/dL (IQ 413,800), and median LDL-C levels at last follow-up was 122 mg/dL (IQR 91,172). Most patients (92%) were on high intensity statins and ezetimibe, 28% with PCSK9i, 26% with lomitapide, and 23% with lipoprotein-apheresis. Fourteen patients (36%) attained an LDL-C level below 100 mg/dL, and 10% attained an LDL-C below 70 mg/dL in secondary prevention. Patients with null/null variants were youngers, had higher untreated LDL-C and had the first ASCVD event earlier. Free-event survival is longer in patients with defective variant compared with those patients with at least one null variant (p=0.02). CONCLUSIONS: HoFH is a severe life threating disease with a high genetic and phenotypic variability. The improvement in lipid-lowering treatment and LDL-C levels have contributed to reduce ASCVD events.

Transcortical transcatheter ultrasound-assisted technique for deep-seated brain tumors. Technical note.

PURPOSE: Surgery of deep-seated brain tumors can be challenging. Several methods have been described to facilitate transcortical approaches, including ultrasound-assisted resection. Ultrasound-guided placement of a standard ventricular catheter is a widely reported technique and has been used to approach these lesions via the transcortical route. We describe how we usually perform this useful technique to assist and enhance the transcortical resection of some deep-seated brain tumors. METHODS: Standard electromagnetic frameless navigation (S8 Neuronavigation System, Medtronic, Minneapolis, USA) was employed to focus the craniotomy and to plan the trajectory of the ventricular catheter. After dural opening, an ultrasound device (Arietta 850, Hitachi-Aloka Medical, Tokyo, Japan) was used for intraoperative ultrasound (IOUS) assessment. A ventricular catheter was placed from the cortex to the lateral wall of the tumor under direct real-time IOUS visualization to guide the further transcortical dissection. RESULTS: Transcortical transcatheter ultrasound-assisted technique involved minimal time and infrastructure requirements. There were no major technical difficulties during its use, providing confidence and improving subcortical white matter dissection by guiding the route to the tumor. CONCLUSIONS: Recent improvement of IOUS image-quality devices offers several attractive options for real-time navigation. The combination of conventional neuronavigation systems with real-time IOUS assessment during the intradural step provides a higher degree of control by improving the execution of the surgery. We hope this description may be a useful tool for some selected cases and contribute to the further enhancement and improvement of this widely used technique.

Microsurgical Fenestration of Middle Fossa Arachnoid Cyst in a Pediatric Patient Through a Keyhole Approach with Endoscopic Inspection. 2-Dimensional Operative Video.

In recent decades, the management of middle fossa arachnoid cysts in pediatric patients has evolved significantly through the integration of novel techniques, such as the utilization of endoscopy systems1 and implementation of minimally invasive approaches like keyhole craniotomy.2,3 These cystic formations, occurring within the arachnoid membrane, may lead to neurologic impairments and raised intracranial pressure if left untreated.4 The utilization of endoscopy to aid microsurgical techniques or as a complement to them provides a level of visualization and manipulation of the cyst walls that is significantly more precise than the isolated use of a microscope.1 The keyhole craniotomy allows for reduced surgical trauma, smaller incisions, and quicker recovery times.5 In Video 1, we present the case of a 2-year-old patient with bilateral middle fossa arachnoid cysts exerting mass effect on the adjacent parenchyma. The patient was referred to our institution due to developmental delay and cognitive issues related to language and social interactions. On the basis of imaging findings and clinical correlation, we opted for a microsurgical fenestration with endoscopic inspection using a keyhole craniotomy to minimize complications and enhance the benefits of both techniques. Throughout the surgical video, tricks and considerations that contribute to the combined procedure's efficiency and ease of execution are highlighted and discussed. Postoperative images showed no complications, and the patient was discharged 3 days after surgery.

Dorsum Sellae as Key Landmark in ETV With Disminished Prepontine Cistern: Technical Note and Case Series.

BACKGROUND AND OBJECTIVES: One of the key aspects in the surgical technique of endoscopic third ventriculostomy (ETV) is the perforation of the floor of the third ventricle because of the high risk of injuring vital structures located in that region. According to the standard technique, this perforation should be performed in the midline halfway between mammillary bodies and the infundibular recess to avoid damage to the structures. This can be performed without excessive complications when the diameter of the prepontine cistern is wide. However, in situations where the diameter is reduced (defined in the literature as having a prepontine interval [PPI] ≤1 mm), the probability of complications increases exponentially.In this article, we propose using dorsum sellae as a key point to safely perform ETV in patients with a decreased PPI, guiding the trajectory and its marking using neuronavigation. METHODS: A review was conducted on the latest 100 ETV procedures performed by our team in the past 5 years. The measurement of the PPI was conducted using archived preoperative MRI imaging studies, specifically between the dorsum sellae and the basilar artery. In cases where the PPI was ≤1 mm and, therefore, the use of the dorsum sellae was applied as a reference point, the technical results and procedural functions were documented. RESULTS: In the cohort, 7 patients with a PPI ≤1 mm were identified. In all 7 cases, fenestration of the tuber cinereum was successfully performed without causing vascular damage or associated complications. ETV was successful in 6 patients, with only one experiencing ETV failure necessitating the placement of a ventriculoperitoneal shunt. CONCLUSION: The utilization of the dorsum sellae as a reference point to perform ETV in reduced PPI constitutes a safe alternative to the classical technique.

Endoscopic extended transforaminal approach (medial subchoroid) as an alternative to the classical transchoroidal approach: Technical note.

The extended transforaminal endoscopic approaches allows visualization and manipulation of the middle and posterior third of the III ventricle. In selected cases where the venous anatomy is favorable, the medial subchoroidal approach can be performed as an alternative to the classic transchoroidal approach (via trans-taenia fornicis) with increased protection over the fornix and without the need to sacrifice the septal vein. We present a 14-year-old male with history of Neurofibromatosis type 1 referred for two weeks of clinical evolution with headache, dizziness, gait instability and appearance of a right VI nerve palsy. Magnetic resonance imaging showed obstructive tri-ventricular hydrocephalus due to stenosis of the aqueduct of Sylvius with suspicion of an underlying tumor. An endoscopic surgical procedure was performed through a single approach with III cisternostomy and resection of the tissue that produced the stenosis. The anatomopathological diagnosis showed reactive glial tissue with no signs of malignancy. In conclusion, the medial subchoroidal approach is a plausible alternative in the endoscopic approach to the III ventricle structures in a safe and comfortable manner.

Neuronavigated endoscopic aqueductoplasty with panventricular stent plus septostomy for isolated fourth ventricle in complex hydrocephalus and syringomyelia associated with myelomeningocele: how I do it.

BACKGROUND: Isolated fourth ventricle (IFV) is a challenging entity to manage. In recent years, endoscopic treatment for aqueductoplasty has been on the rise. However, in patients with complex hydrocephalus and distorted ventricular system, its implementation can be complex. METHODS: We present a 3-year-old patient with myelomeningocele and postnatal hydrocephalus treated by ventriculoperitoneal shunt. In follow-up, a progressive IFV and isolated lateral ventricle with symptoms of the posterior fossa developed. An endoscopic aqueductoplasty (EA) with panventricular stent plus septostomy guided with neuronavigation was decided due to the complexity of the ventricular system. CONCLUSION: In IFV associated with complex hydrocephalus with distortion of the ventricular system, navigation can be of great help for planning and as a guide for performing EA.

ETV as treatment for obstructive hydrocephalus in an aneurysmal malformation of the vein of Galen in infants: case report and review of literature.

INTRODUCTION: A vein of Galen aneurysmal malformation (VGAM) is a high-flow arteriovenous malformation that typically presents in early childhood. Complications associated with this condition include heart failure and hydrodynamic disorders resulting from high blood flow. Hydrocephalus is one of the hydrodynamic disorders. It can present in a chronic form due to a defect in the absorption of cerebrospinal fluid (CSF) resulting from venous hypertension or in an acute/subacute form due to obstruction of normal circulation routes caused by the mass effect of the VGAM. Currently, endovascular closure of the lesion is considered the treatment of choice. However, in acute/subacute presentations of obstructive hydrocephalus, progression may occur despite endovascular treatment, necessitating a cerebrospinal fluid diversion procedure. Prior to the advent of endoscopic treatment, ventricular shunts were utilized, but outcomes were poor due to the pathological hydrodynamic parameters associated with the malformation. The role of endoscopic third ventriculostomy as a treatment option in these situations remains unclear, and there is limited literature available. Therefore, we present the case of a patient with a vein of Galen aneurysmal malformation and obstructive hydrocephalus, which was treated via ETV. Furthermore, a literature review was conducted. CASE DESCRIPTION: We present a 5-month-old infant who was diagnosed at birth with VGAM. At 2 months of age, a partial closure of the malformation was performed via endovascular. Subsequently, the patient presented at the emergency department with symptoms of irritability, lethargy, vomiting, feeding refusal, and "setting sun" sign of several days' duration due to obstructive hydrocephalus. The patient was successfully treated via ETV. Following this, a new arteriography was performed and revealed occlusion of the VGAM. The child clinically improved and currently exhibits mild psychomotor impairment after 1-year follow-up. CONCLUSION: In the literature, there are a limited number of reports on the treatment of obstructive hydrocephalus associated with VGAM via ETV. These reports have demonstrated good outcomes with minimal surgical complications. Our patient also exhibited good results. As such, ETV is a viable and safe option for the treatment of obstructive hydrocephalus associated with VGAM in pediatric patients. However, more research is needed to establish the effectiveness of ETV in comparison to other treatment options and to understand the long-term outcomes.