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.

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.

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.

Intraoperative ultrasound-assisted surgery in orbital apex tumours: technical note.

BACKGROUND: Since the introduction of ultrasonography (US) in the operating theatre in the early 1970s, intraoperative ultrasound (IOUS) has become a very useful tool for real-time neurosurgical procedures. The main limitation of US is its innately reduced capacity to penetrate the intact skull. This is the reason why most IOUS-assisted procedures are usually performed via transfontanellar or after a craniotomy or laminotomy is done. OBJECTIVE: We present a 54-year-old woman with a right optic nerve sheath meningioma, who was operated on by a right cranio-orbital approach and IOUS-assisted tumour removal. METHODS: Data concerning the anamnesis and complementary studies were obtained from the clinical history. Surgical images were obtained in the operating room during surgery. Images obtained by US were compared with the preoperative and postoperative imaging scans. IOUS imaging was also compared with the intraoperative findings. RESULTS: The correlation between US and both preoperative and postoperative studies was very high. During surgery, both transpalpebral and transperiorbital IOUS facilitated the resection with no specific technical difficulties or significant time consumption. CONCLUSION: Recent improvements in the image quality of IOUS devices offer several attractive options for real-time neuronavigation. We describe our initial experience with the IOUS-assisted technique for orbital apex tumours. In our patient transpalpebral and transperiorbital IOUS provided an excellent source of control over location and over the extent of tumour resection. We hope that this description of how we usually perform this procedure may be useful for some selected cases and contribute to the further enhancement and improvement of the technique.

Neuroendoscopic training in neurosurgery: a simple and feasible model for neurosurgical education.

BACKGROUND: The development of high levels of technical competence and excellent decision-making skills are key goals of all neurosurgical residency training programs. This acquisition of technical skills is becoming increasingly difficult due to many factors including less exposure to operative cases, demand for more time and cost-effective practices, and resident work hour restrictions. We describe a step-by-step method for how to build a low-cost and feasible model that allows residents to improve their neuroendoscopic skills. METHODS: The bell pepper-based model was developed as an endoscopic training model. Using continuous irrigation, several hands-on procedures were proposed under direct endoscopic visualization. Endoscope setup, endoscopic third ventriculostomy, septostomy, and tumor biopsy procedures were simulated and video recorded for further edition and analysis. RESULTS: The model can be setup in less than 15 min with minimal cost and infrastructure requirements. A single model allows simulation of all the exercises described above. The model allows exposure to the camera skills, instrument handling, and hand-eye coordination inherent to most neuroendoscopic procedures. CONCLUSION: Minimal infrastructure requirements, simplicity, and easily setup models provide a proper environment for regular training. The bell pepper-based model is inexpensive, widely available, and a feasible model for routine training. Neurosurgery residents may benefit from the use of this model to accelerate their learning curve and familiarize themselves with the neuroendoscopic core principles in a risk-free environment without time or resource constraints.

Rotation flap closure of a giant dorsolumbar myelomeningocele: technical note.

PURPOSE: Myelomeningocele (MMC) is the most prevalent form of spina bifida aperta. Here we present the surgical repair technique of a dorsolumbar myelomeningocele with a large associated soft tissue defect. METHODS: Data concerning the anamnesis and complementary studies were obtained from the clinical history. Surgical images were obtained in the operation room during surgery. RESULT: We describe the case of a newborn with a dorsal myelomeningocele with a placode in the central area and a large associated skin defect of 12 x× 12 cm. Urgent intervention was carried out on the day of birth with placode reconstruction. Dorsal and gluteus maximus muscle rotation and skin rotation flap were performed in order to achieve complete closure of the myocutaneous defect. The patient presented a good recovery with no new neurological deficit. CONCLUSION: Myelomeningocele may be associated with large myocutaneous defects that can be difficult to correct by primary closure. In these cases, rotation flaps may be necessary to achieve complete closure.

Different radioinducid tumors synchrony in an acute lymphoblastic leukemia survivor. / Sincronía de diferentes tumores radioinducidos en superviviente de leucemia linfoblástica aguda.

The cranial radiation-induced tumors appearance in pediatric patients treated for hematological diseases such as leukemia/T-lymphoblastic lymphoma (T-ALL/LBL) is a known phenomenon that may include lesions of different malignant degrees and require neurosurgical treatment. We present the case of a 38-year-old man referred to our department for a sudden diplopia with 6-month progressive left hemiparesis and frequent falls. After imaging tests, different intra and extraxial lesions with different radiological behavior were observed, performing a single surgical approach for their resection. The pathological anatomy confirms four histological variants: meningioma (grade 1 and 2 [atypical]), subependymoma, and cavernoma. We discuss the teratogenic role of ionizing radiation.

Mature filum terminale teratoma. Excision guided by direct stimulation of nerve roots. / Teratoma maduro del filum terminale. Extirpación guiada por estimulación directa de raíces nerviosas.

A 45-year-old male patient, with no history of interest, consulted in emergency department due to a progressive loss of strength in the lower limbs at the distal level with impossibility for dorsal flexion of the left foot, as well as neurogenic bladder. The MRI revealed a mass at the filum terminale with completed occupation of transverse diameter of the dural sac. The radiological diagnosis was lipoma of filum terminale. The patient underwent surgery with intraoperative neurophysiological monitoring using direct stimulation of nerve roots, which allowed the removal of a tumour composed of sebaceous material, fat and hair. The anatomopathological study classified it as a mature cystic teratoma. Postoperative evolution was favorable.

Anterior Unilateral Plagiocephaly in Patient with Alagille Syndrome: Case Report.

BACKGROUND: The polymalformative syndromes and craniofacial anomalies association is a well-known phenomenon in patients with Crouzon, Pfeiffer, Apert, or Muenke disease. Recently, other less frequent pathologies, such as Alagille syndrome, have shown an association with alterations in the development of cranial sutures, resulting in serious cosmetic defects and neurologic disorders. CASE DESCRIPTION: We report an exceptional case of a 30-month-old girl, a nephroblastoma survivor diagnosed with Alagille syndrome, who was referred to our department with progressive anterior plagiocephaly and premature left coronal suture closure associated with a large compensating right bossing. Despite the patient's age, we offered aggressive surgical treatment performing a new forehead harvested from the skull vertex with orbital rim reconstruction. CONCLUSIONS: Alagille syndrome is a complex multisystem pathology with a poor craniosynostosis association and only 3 cases have been described in the literature.

Risk of Recurrence in Operated Parasagittal Meningiomas: A Logistic Binary Regression Model.

BACKGROUND: Parasagittal meningiomas arise from the arachnoid cells of the angle formed between the superior sagittal sinus (SSS) and the brain convexity. In this retrospective study, we focused on factors that predict early recurrence and recurrence times. METHODS: We reviewed 125 patients with parasagittal meningiomas operated from 1985 to 2014. We studied the following variables: age, sex, location, laterality, histology, surgeons, invasion of the SSS, Simpson removal grade, follow-up time, angiography, embolization, radiotherapy, recurrence and recurrence time, reoperation, neurologic deficit, degree of dependency, and patient status at the end of follow-up. RESULTS: Patients ranged in age from 26 to 81 years (mean 57.86 years; median 60 years). There were 44 men (35.2%) and 81 women (64.8%). There were 57 patients with neurologic deficits (45.2%). The most common presenting symptom was motor deficit. World Health Organization grade I tumors were identified in 104 patients (84.6%), and the majority were the meningothelial type. Recurrence was detected in 34 cases. Time of recurrence was 9 to 336 months (mean: 84.4 months; median: 79.5 months). Male sex was identified as an independent risk for recurrence with relative risk 2.7 (95% confidence interval 1.21-6.15), P = 0.014. Kaplan-Meier curves for recurrence had statistically significant differences depending on sex, age, histologic type, and World Health Organization histologic grade. A binary logistic regression was made with the Hosmer-Lemeshow test with P > 0.05; sex, tumor size, and histologic type were used in this model. CONCLUSIONS: Male sex is an independent risk factor for recurrence that, associated with other factors such tumor size and histologic type, explains 74.5% of all cases in a binary regression model.

[Chondroma adjacent to Meckel's cave mimicking a fifth cranial nerve neurinoma. A case report]. / Condroma adyacente al cavum de Meckel simulando un neurinoma del quinto par craneal. A propósito de un caso.

Cranial chondromas are tumours arising from chondrocyte embryonic remnants cells that usually appear in the skull base synchondrosis. In contrast to the rest of the organism, where chondroid tumours are the most common primary bone tumour just behind the haematopoietic lineage ones, they are a rarity at cranial level, with an incidence of less than 1% of intracranial tumours. The case is reported on a 42 year-old male referred to our clinic due to the finding of an extra-axial lesion located close to the Meckel's cave region, with extension to the posterior fossa and brainstem compression after progressive paraparesis of 6 months onset. With the diagnosis of trigeminal schwannoma, a subtotal tumour resection was performed using a combined supra-infratentorial pre-sigmoidal approach. The postoperative histopathology report confirmed the diagnosis of cranial chondroma.

[Paradoxical transtentorial herniation, extreme trephined syndrome sign: A case report]. / Herniación paradójica transtentorial, manifestación extrema del síndrome del craniectomizado. Caso clínico.

The current increasing use of decompressive craniectomy carries the implicit appearance of complications due to alterations in both intracranial pressure and in the hydrostatic-hemodynamic equilibrium. Paradoxical transtentorial herniation represents a rare manifestation, included in "trephine syndrome", extremely critical but with relatively simple treatment. We present the case of a 56-year-old woman with no interesting medical history, who, after an olfactory groove meningioma surgery, presented a haemorrhage located in the surgical area with an important oedema. The patient required a second emergency surgery without any chance of conserving the cranial vault. During the post-operational period, great neurological deterioration in orthostatic position was noticed, which resolved spontaneously in decubitus. This deficit was resolved with bone replacement afterwards. We discuss possible predisposing factors and aetiologies of this pathology.