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Background: Arteriovenous malformations (AVMs) are relatively uncommon congenital vascular anomalies, and only 7–15% of AVMs occur in the posterior fossa. Most posterior fossa AVMs clinically present with hemorrhage and are associated with a high risk of neurological deficits and mortality. These malformations are associated with a high incidence of flow-related aneurysms. Endovascular treatment of infratentorial AVMs is challenging in pediatric patients. Case Description: We describe an 11-year-old female adolescent with cerebellar syndrome [Video 1], who was diagnosed with a cerebellomesencephalic fissure AVM. We observed a sequential increase in the size of the AVM after multiple sessions of endovascular treatment and performed successful microsurgical resection of the lesion. Conclusion: This illustrative video highlights the role of microsurgery as a feasible therapeutic strategy for complete resection of cerebellar AVMs after endovascular embolization.

The association between cardiac rhabdomyoma and intraventricular tumors and/or subcortical nodules is characteristic of tuberous sclerosis complex (TSC). Patients with TSC may have refractory seizures, autistic behavior, and cognitive decline. The patient received the fetal diagnosis of TSC at the age of 19 weeks of gestations, where presented at prenatal ultrasound cardiac and brain tumors. Fetal MRI showed a lesion in the right and left lateral ventricles near the foramen of Monro associated with subependymal lesions along the entire ependyma of the lateral ventricles and several subcortical tubercles, and the fetal Doppler echocardiogram revealed three cardiac lesions. The fetus underwent intrauterine treatment with everolimus and presented regression and subsequent stabilization of the cardiac and brain lesions; additionally, the patient did not develop seizures or autism and presented good neuropsychomotor development. It is the first evidence that mTOR inhibitors may help to prevent neurological complications associated with TSC.

Medullary neuroschistosomiasis is a severe complication of gastrointestinal infection by Schistosoma. There are several endemic areas, wherein the only causative species present is Schistosoma mansoni, which is responsible for the clinical manifestations of all cases in those areas. We report the case of a 13-year-old female with lumbar pain and progressive lower limb weakness, with a delayed diagnosis of medullary involvement by the parasite. We also reviewed the literature on the disease. Although it is related to the less severe forms of schistosomiasis, one should pay attention to the diagnosis of neuroschistosomiasis in cases of transverse myelitis in patients who traveled to endemic areas. The delay in diagnosis and, consequently, the introduction of treatment may result in irreversible neurological sequelae.

A scientific journal’s greatest responsibility is to ensure that all contributions accepted for publications are rigorously but fairly reviewed. The Editor-in-Chief and Archives of Pediatric Neurosurgery gratefully acknowledge the qualified and regular collaboration of renowned medical doctors and scientists who kindly devoted their time to constructively review the submitted articles. Their expertise and invaluable assistance contributed to maintain a high scientific standard for APN. We are thus indebted to the Editorial Board members and the following experts who reviewed papers and completed the peer-reviewing process within 2020.

Posterior fossa arteriovenous malformations (AVMs) can be a challenging disease, especially those large in size. AVMs can be treated with a combination of endovascular treatment and microsurgery. Here, the authors present the case of a 16-year-old female patient with progressive dizziness and episodic syncope. The workup of the patient showed a hemispheric cerebellar AVM, Spetzler-Martin grade IV. She underwent combined treatment (endovascular and microsurgery) with no complications and cure of the malformation.The video can be found here: https://youtu.be/rNw_Kyd76Mg

Background: Giant brain aneurysms account for approximately 5% of all intracranial aneurysms, often presenting with intraluminal thrombosis that causes a mass effect in surrounding neural structures. Although its exact growing mechanism remains unknown, they have to be treated. Despite the most recent advances in neurosurgical fields, the best treatment modality remains unknown and surgery of giant superior cerebellar artery (SCA) aneurysms still is a challenge even for the most experienced neurosurgeons, due to their deep location, surrounding perforating vessels, and intraluminal thrombosis. Case Description: In this video, we present the case of a 65-year-old woman with progressive hemiparesis and paresis of low cranial nerves. The symptoms were caused by a giant aneurysm located in the origin of the SCA. Despite endovascular embolization of the aneurysm and placement of a flow diverter stent, the aneurysm increased in size causing symptoms progression. In that scenario, we decided to perform a microsurgical decompression of the aneurysm thrombus and coagulation of the vasa vasorum, to reduce the mass effect and prevent the aneurysm from keep growing. Conclusion: Through an extensive description of the surgical anatomy, we illustrate an interhemispheric transcallosal transforaminal approach, with the removal of anterior thalamic tubercle to widely expose the aneurysm dome. The surgery was successfully performed, and the patient symptoms improved. The patient signed the Institutional Consent Form, which allows the use of her images and videos for any type of medical publications in conferences and/or scientific articles.

Cavernous malformations (CM) affect approximately 0.5% of the population, with only a limited portion being located in the optic nerve and chiasma. The clinical presentation is determined by their locations. In the optochiasmatic CM, the acute visual disturbance is the most common presentation. Chronically, many show a progressive visual loss, chronic headache, and pituitary disturbances. The differential diagnosis includes optic glioma, arteriovenous malformations, aneurysm, craniopharyngioma, pituitary apoplexy, and inflammatory conditions. In Video 1 , we present the case of a 39-year-old woman with a history of a hemorrhagic optochiasmatic cavernoma in 2016, who started using propranolol to reduce the lesion and symptoms of visual loss. Moreover, the first microsurgical resection of the cavernoma and evacuation of the hematoma were performed in the same year. Owing to evolvement from a partial to a total vision loss in the left eye and presentation of new symptoms in the right eye, the patient underwent microsurgical resection. The surgery was performed sequentially. An awake craniotomy was performed to monitor the chiasma and right optic nerve. The postoperative magnetic resonance imaging showed complete resection of the CM, and the patient fully recovered. The patient signed the institutional consent form, stating that he or she accepts the procedure and allows the use of his or her images and videos for any type of medical publications in conferences and/or scientific articles.

Cerebral cavernous malformations, also known as cavernomas, are vascular abnormalities of the brain that are clinically associated with a variety of neurologic symptoms that may include hemorrhagic strokes. They are the most common vascular abnormality, representing 10%–25% of all vascular malformations. 1 Lesions associated with cavernomas include developmental venous anomalies, capillary telangiectasias, and other vascular malformations 2 but not intracranial aneurysms. The latter association is extremely rare; in fact, there is only 1 case reported in the literature, in which the cavernoma was obscured by the presence of a cerebral hemorrhage and an unruptured aneurysm, which was presumed to be the primary cause of the bleeding, thereby misleading the surgeons to treat only the aneurysm. 2 There are different alternatives for the management of different types of lesions. 3 , 4 , 5 In this 3-dimensional operative video ( Video 1 ), we present a case of a cavernoma associated with hemorrhage coexisting with an unruptured aneurysm in which we achieved complete resolution of both with microsurgical treatment through a pterional approach. 6 The patient consented to publication of images.

Background Preoperative recognition of the anatomic individualities of each patient can help to achieve more precise and less invasive approaches. It also may help to anticipate potential complications and intraoperative difficulties. Here we describe the use, accuracy, and precision of a free tool for planning microsurgical approaches using 3-dimensional (3D) reconstructions from magnetic resonance imaging (MRI). Methods We used the 3D volume rendering tool of a free open-source software program for 3D reconstruction of images of surgical sites obtained by MRI volumetric acquisition. We recorded anatomic reference points, such as the sulcus and gyrus, and vascularization patterns for intraoperative localization of lesions. Lesion locations were confirmed during surgery by intraoperative ultrasound and/or electrocorticography and later by postoperative MRI. Results Between August 2015 and September 2016, a total of 23 surgeries were performed using this technique for 9 low-grade gliomas, 7 high-grade gliomas, 4 cortical dysplasias, and 3 arteriovenous malformations. The technique helped delineate lesions with an overall accuracy of 2.6 ± 1.0 mm. 3D reconstructions were successfully performed in all patients, and images showed sulcus, gyrus, and venous patterns corresponding to the intraoperative images. All lesion areas were confirmed both intraoperatively and at the postoperative evaluation. Conclusions With the technique described herein, it was possible to successfully perform 3D reconstruction of the cortical surface. This reconstruction tool may serve as an adjunct to neuronavigation systems or may be used alone when such a system is unavailable.

The cerebellopontine angle (CPA) is a subarachnoid space in the lateral aspect of the posterior fossa. In this study, we propose a complementary analysis of the CPA from the cerebellopontine fissure.We studied 50 hemi-cerebelli in the laboratory of neuroanatomy and included a description of the CPA anatomy from the cerebellopontine fissure and its relationship with the flocculus and the 5th, 6th, 7th, and 8th cranial nerves (CN) origins.The average distance from the 5th CN to the mid-line (ML) was 19.2 mm, 6th CN to ML was 4.4 mm, 7-8 complex to ML was 15.8 mm, flocculus to ML was 20.5 mm, and flocculus to 5th CN was 11.5 mm, additionally, and the diameter of the flocculus was 9.0 mm. The angle between the vertex in the flocculus and the V CN and the medullary-pontine line was 64.8 degrees.The most common access to the CPA is through the retrosigmoid-suboccipital region and this approach can be done with the help of an endoscope. The anatomy of origins of neural structures tends to be preserved in cases of CPA lesions.Knowledge of the average distances between the neural structures in the cerebellar-pontine fissure and the angular relationships between these structures facilitates the use of surgical approaches such as microsurgery and endoscopy.

Background: Infectious complications of the central nervous system secondary to endovascular procedures have rarely been reported. However, the number of complications has grown exponentially owing to the popularization of these procedures. The success rate of these procedures varies with the pathology, the patient, and surgical performance. Although brain abscesses have been extensively reported, their presence after endovascular procedures has not been described in detail in the literature. We present a case of brain abscess induced by embolization of an arteriovenous malformation (AVM), discuss the main indications, techniques, procedural complications, and review the associated literature. Case Description: A 13-year-old boy presented to us with a history of hemorrhagic stroke secondary to a cerebral AVM rupture. He underwent incomplete AVM resection (2014), with subsequent incomplete embolization (2017), and permanence of the endovenous catheter as a procedural complication. Physical examination revealed purulent exudate through the cervical surgical wound. We performed cervicotomy to remove the catheter but had no success in removing the intracranial material segment. The patient was subsequently diagnosed with a brain abscess (2018) and treated with antibiotics. Our team performed resection of the residual AVM, abscess, and the catheter-associated with the region. Conclusion: The patient showed significant clinical improvement after surgical resection of the malformation. No residual lesions were observed in the imaging examinations. Further, we reviewed the literature to find other cases of similar complications and their association with the endovascular procedure. We did not find complications in patients younger than the one presented in this case. Factors such as incomplete embolisation increase the risk of unfavourable outcomes.

Pediatric craniopharyngioma is a rare tumor with excellent survival but significant long-term morbidities due to the loco-regional tumor growth or secondary to its treatment. Visual impairment, panhypopituitarism, hypothalamic damage and behavioral changes are amongst the main challenges. This tumor should be managed under the care of a multidisciplinary team to determine the optimum treatment within the available resources. This is particularly important for low middle-income countries (LMICs) where resources are variable. We provide a risk-stratified management guideline for children diagnosed with craniopharyngioma in a resource limited setting based on the service levels describing the facilities and personnel required for management as previously specified by the Pediatric Oncology in Developing Countries (PODC) committee of The International Society of Pediatric Oncology (SIOP). A multi-disciplinary group of neurosurgeons, radiation and pediatric oncologists, radiologists, pediatric endocrinologists and an ophthalmologist with experience in managing children with craniopharyngioma in LMIC setting was formed and carried online meetings to form a consensus guideline. The clinical characteristics (including the visual and endocrine presentations), suggestive radiological features as well as potential treatment options including surgery, radiotherapy and intra-cystic therapies were discussed in depth and in relation to available resources. In addition, hormonal management, pre- and post-operative PICU care and expected future complications related to craniopharyngioma and to follow up these children were discussed and documented in the guideline. We believe this guideline is a useful reference for health care providers in LMIC.

OBJECTIVEIn this study, the authors retrospectively reviewed two cohorts of patients with occipital encephalocele (OE) and demonstrate the technical aspects of an innovative and unprecedented method for fetal OE correction.METHODSThis was a cross-sectional retrospective study of 22 patients who underwent surgery from July 2012 to July 2018. The inclusion criteria for participants were a gestational age between 19 weeks and 27 weeks, a maternal age ≥ 18 years, a normal fetal karyotype, the presence of microcephaly, and a cystic hernial sac with the cystic component accounting for more than 80% of the total volume, which was larger than 10 ml. The fetuses with OE and microcephaly underwent intrauterine repair. The exclusion criteria were the presence of a fetal anomaly not associated with OE, chromosomopathy, presence of the brainstem inside the hernial sac or venous sinuses inside the herniated content, the risk of premature birth, placenta previa, and maternal conditions that would constitute an additional risk for fetal and maternal health, as well as refusal for fetal surgery. The authors reviewed the potential for microcephaly reversal and the long-term neurocognitive development.RESULTSThe authors report the cases of 9 fetuses with OE and microcephaly who underwent intrauterine OE repair at gestational ages of less than 27 weeks (fetal group). One case selected for fetal surgery also presented with placental abruption. All patients who underwent the fetal operation had microcephaly reversal, and 3 patients required ventriculoperitoneal shunting in the 1st year of life. The authors reviewed the cases of 12 patients who underwent postnatal repair. In 10 cases, fetal surgery was refused (postnatal group), and in 2 cases, the inclusion criteria for fetal surgery were not met. The authors evaluated the neurocognitive development of the patients with the Bayley Scales of Infant Development II. The median score for the fetal group was 98.7, and that for the postnatal group was 27.8.CONCLUSIONSThe intrauterine repair of OE may stop the progression of encephalocele sac herniation and result in microcephaly reversal. The fetal group had a better cognitive outcome than the postnatal group. The technique required to correct this defect is feasible for those with previous experience in the correction of fetal myelomeningocele. However, more studies are needed to ensure the efficacy of this procedure.

The aim of this study was to analyze the skull base anatomy of patients who underwent intrauterine or postnatal myelomeningocele repair and to determine its relationship with hydrocephalus. This was a retrospective cross-sectional study that analyzed three groups: the postnatal group, 57 patients who underwent myelomeningocele repair up to 48 h after birth; the fetal group, 70 patients who underwent myelomeningocele repair between 19 and 27 weeks of gestation; and a control group (65). We compared the rate of hydrocephalus treatment, the clivus-supraocciput angle (CSA), and the Welcher angle. The mean CSA in the fetal group was 87.6°, and the postnatal group was significantly different at 78.3° (p

Pediatric craniopharyngioma is a rare tumor with excellent survival but significant long-term morbidities due to the loco-regional tumor growth or secondary to its treatment. Visual impairment, panhypopituitarism, hypothalamic damage, and behavioral changes are among the main challenges. This tumor should be managed under the care of a multidisciplinary team to determine the optimum treatment within the available resources. This is particularly important for low middle-income countries where resources are variable. This report provides risk-stratified management guidelines for children diagnosed with craniopharyngioma in a resource-limited setting.

Background: Colloid cyst treatment with purely endoscopic surgery is considered to be safe and effective. Complete capsule removal for gross total resection is usually recommended to prevent recurrence but may not always be safely feasible. Our objective was to assess the results of endoscopic surgery using mainly aspiration and coagulation without complete capsule resection and discuss the rationale for the procedure. Methods: A retrospective review was conducted of 45 consecutive symptomatic patients with third ventricle colloid cysts that were surgically treated with purely endoscopic surgery from 1997 to 2018. Results: Mean age was 35.4 years. Male-to-female ratio was 1:1. Clinical presentation included predominantly headache (80%). Transforaminal was the most used route (71.1%) followed by transeptal (24.5%) and interforniceal (4.4%). Capsule was intentionally not removed in 42 patients (93.3%) and cyst remnants were absent on postoperative MRI in 36 (85%). Mild complications occurred in 8 patients (17.8%). Surgery was statistically associated with cyst volume and ventricular size reduction. There were no serious complications, shunts or deaths. Follow-up did not show any recurrence or remnant growth that needed further treatment. Conclusion: Gross total resection may not be the main objective for every situation. Subtotal resection without capsule removal seems to be safer while preserving good results, especially in a limited resource environment. Remnants left behind should be followed but tend to remain clinically asymptomatic for the most part. Surgical planning allows the surgeon to choose among the different resection routes and techniques available. Decisions are predominantly based on preoperative imaging and intraoperative findings.

The bobble-head doll syndrome (BHDS) is a rare acquired head movement disorder characterized by up and down or side-to-side movement, most commonly seen in the first decade of life. The syndrome occurs more often in lesions causing third ventricle dilatation such as suprasellar or third ventricle cyst, but it is also found in other pathologies associated with hydrocephalus like shunt dysfunctions, trapped fourth ventricle, congenital aqueductal stenosis, Dandy-Walker syndrome, and cerebellar malformations. The pathophysiology of this head movement has different origins theories; one states that this stereotyped movements empties the cyst and move the dome away from the foramina of Monro, which relieves the symptoms of hydrocephalus; the other suggests that the extrapyramidal tracts (rubrotegmentospinal and reticulospinal) are stimulated by the compression of dorsomedial nucleus of the thalamus by the cyst, whose tracts innervate the neck muscles resulting in the bobbling head movements. This video (Video 1) presents a clinical case of BHDS caused by suprasellar cyst in a 10- year-old boy treated by endoscopic procedure. A ventricular-cyst-cisternostomy was performed resulting in complete improvement of the head movements and uneventful recovery. Postoperative images demonstrate decreasing of the cyst lesion and resolution of the hydrocephalus.

The anterior temporal lobectomy (ATL) and selective amygdalohippocampectomy (SelAH) have been used for surgical treatment of mesial temporal lobe epilepsy. We examined the comprehensive white matter tract anatomy of the temporal lobe to gain an insight into the trans-middle temporal gyrus, a lateral approach which has been commonly used. The transmiddle temporal gyrus approach was performed in a stepwise manner on cadaveric human heads to examine the traversing white matter pathways through it and the structures located in the temporal horn. We reviewed the literature to compare the trans-middle temporal gyrus approach with other SelAH techniques based on surgical outcomes. There does not appear to be a significant difference in seizure outcome between SelAH and ATL. However, the SelAH provides a better neuropsychological outcomes than the ATL in selected patients. Each SelAH approach has individual advantages and disadvantages. Based on our anatomical study, in the transcortical amygdalohippocampectomy technique through the middle temporal gyrus the white matter pathways to be encountered. In the temporal horn, the collateral eminence, hippocampus, lateral ventricular sulcus, choroidal fissure, inferior choroidal point, choroid plexus, fimbria of the fornix, and amygdala are exposed. The subpial dissection is performed along the lateral ventricular sulcus from the collateral eminence on lateral side and from the choroidal fissure on medial side by microdissector for en bloc resection of the hippocampus proper. The trans-middle temporal gyrus approach is commonly used in treatment of mesial temporal lobe epilepsy patients. A better anatomical and functional understanding of the structures of the temporal lobe is crucial for safer and more accurate surgery.

Arachnoid cysts are responsible for 1% of expansive lesions in the central nervous system. Usually, they do not cause neurologic symptoms unless they have expansion or hemorrhage. Intracystic bleeding is caused by trauma or may be spontaneous. There are few cases in the literature of spontaneous hemorrhage of arachnoid cysts. This 2-dimensional video (Video 1) demonstrates the case of a 6-year-old boy who presented with headache and diplopia. At the physical examination, he exhibited right sixth nerve palsy. The complementary examinations revealed a left middle fossa arachnoid cyst classified as Galassi 2. An urgent procedure was planned to fenestrate the cyst. Informed written consent was obtained from the patient's family. Due to the team experience, endoscope-controlled microsurgery was performed. The patient experienced remission of the headache and the diplopia. A CT scan was performed on the first postoperative day and revealed a lamina of acute blood at the subdural space. It was an asymptomatic thin lamina of blood; we opted to watch and follow. One month later, a control MRI revealed a left frontoparietal subdural hematoma. Despite being asymptomatic, the hematoma was determined to have significant mass effect; thus, it was evacuated by a parietal burr-hole. After 1 month, another MRI showed resolution of the frontoparietal hematoma and significant reduction in the arachnoid cyst. There are few cases described of spontaneous rupture of arachnoid cyst; beyond that, we would like to illustrate a step-by-step procedure that is not widely available as a video article.

Background: Intracranial aneurysms are common vascular malformation occurring in 1-2% of the population and accounting for 80–85% of nontraumatic subarachnoid hemorrhages. About 10% of the ruptured aneurysm causing subarachnoid hemorrhage (SHA) develop intraventricular hemorrhage (IVH). In this scenario, the external ventricular drain (EVD) is a usual treatment for IVH. To reduce the time for the clot absorption, the neuroendoscopy with clot removal and ventricular irrigation is a feasible option, although not routinely used. Case Description: This 2D video shows a case of a 60-year-old female, with sudden headache associated with nausea and vomit. The brain angiotomography revealed aneurysm in the communicating segment of the left internal carotid artery, with 10.5 mm of diameter; also showed intraparenchymal, subarachnoid, and IVH, with a Fisher Modified Grade of 4 and a prompt aneurysm clipping and EVD were performed. Two days after the first surgical procedure, a neuroendoscopy was performed to remove the ventricular clots and improve the patient outcomes. Conclusion: In the presented case, at the 6th postoperative month, the patient was Grade 1 in the Rankin Modified Scale and without hydrocephalus. This procedure can be used routinely as an additional tool to microsurgical clipping to improve patients outcome.

Late radiation necrosis is a rare entity presenting in 2.2% to 9% of radiation-treated arteriovenous malformations (AVMs). It occurs by a mean of 3 years following treatment. There are few reports in the literature of radionecrosis and solid lesions treated with surgery. 1 , 2 , 3 , 4 To the authors' knowledge, this case has the longest interval between radiosurgery and the presentation of cerebral necrosis. In this surgical video, we present the case of a 51-year-old female with a left supramarginal gyrus AVM that received radiosurgery with gamma knife; after 20 years, she began having seizures and aphasia. The magnetic resonance imaging scan revealed a lesion simulating an intra-axial tumor causing important edema and mass effect. Medical treatment was given including high-dose steroids without success, therefore microsurgery was performed. The surgery was presented in a step-by-step basis and correlation was performed with the involved adjacent anatomy, to illustrate the anatomy of the approach and surgical landmarks. The patient's symptoms resolved completely, and the postoperative magnetic resonance imaging scan showed complete resection and resolution of the edema. The histopathological findings were consistent with a radionecrosis and AVM. The patient signed the Institutional Consent Form, which states that she accepts the procedure and allows the use of her images and videos for any type of medical publications in conferences and/or scientific articles.

Objectives This study aims to analyze our series of pediatric patients who underwent craniopharyngioma resection using the endoscopic endonasal transsphenoidal approach (EETA). Methods We collected clinical and surgical data from the charts of 20 children who underwent craniopharyngioma removal surgery using the EETA from 2007 to 2017. From the charts, we collected demographic information, results of imaging tests (size and extension of the tumor), and information regarding the surgical procedure and postoperative complications. Results From the 20 patients included in this series (12 women and eight men), 17 underwent EETA as a primary procedure, and the remaining three underwent EETA as a secondary procedure due to a relapsing tumor following previous transcranial surgery. The mean age of the patients at the time of the surgical procedure was 7.5 years (range 3–18 years). Regarding their location, 12 tumors were in the sellar and suprasellar regions, three extended into the third ventricle, and five were exclusively intrasellar. We achieved a gross total resection (GTR) of the tumor in 14 patients (70%), subtotal in five (25%), and partial in one (5%). One patient (5%) developed a cerebrospinal fluid fistula after the surgical procedure. In the postoperative follow-up period (mean time = 5.3 years; range = 2–9 years), 11 (55%) patients developed panhypopituitarism, and a relapsing tumor was later found in three (15%) patients. Regarding visual impairment, four patients had visual abnormalities preoperatively (amaurosis, n = 2; bilateral visual acuity decrease, n = 1; bilateral visual field defect, n = 1), and those did not improve or worsened postoperatively. None of the patients who did not have vision problems before the surgery developed those postoperatively. Conclusion Our results showed that the EETA is a safe and effective approach for removing craniopharyngiomas in children, as it associated with low operative morbidity and complication rates. Also, our data demonstrated that the EETA may be performed regardless of the size of the nasal cavity, pneumatization of the sphenoid sinuses, and location or extension of the tumors.

Basilar bifurcation is a challenging site for aneurysm clipping. Anatomical factors such as size and projection of the aneurysm, distance between the aneurysm neck and the dorsum sellae, and location of the basilar bifurcation contribute to surgical complexity. Endovascular treatment has been used more frequently than microsurgical clipping, especially for posterior circulation lesions. Thus, the upcoming generation of neurosurgeons will have increasingly less access to the microsurgical treatment of such lesions. We present the case of a 45-year-old female patient who presented sudden mental confusion characterized by disorientation in time, space, and person. Investigative acute cerebral magnetic resonance imaging revealed diffusion restriction in the left posterior cerebral and superior cerebellar arteries. The clinical and cardiologic investigations revealed no abnormalities, but computed tomographic angiography and digital arteriography revealed a low-riding basilar bifurcation aneurysm and a very small aneurysm in the right internal carotid artery. The wide neck precluded coil embolization, and the appropriate stent was not covered by our public health insurance. Considering the young age, surgical treatment was proposed. Microsurgical clipping was performed using the right pre-temporal approach. In this two-dimensional video, we show the steps to reach the low-riding basilar bifurcation aneurysm neck. The positioning, transzygomatic pterional craniotomy, intradural anterior clinoidectomy, and posterior cavernous sinus opening are shown, and the surrounding anatomy is illustrated.

The insula plays a crucial role in speech planning due to its connections with cortical and subcortical areas. Surgical management of cavernous malformation (CM) of the insula consists of total resection of the lesion and the surrounding gliosis to avoid or reduce seizures. When located in the dominant hemisphere, an awake craniotomy with intraoperative mapping reduces the risk of functional damage. The insula is covered by the operculum and has a relationship with the middle cerebral artery and its branches that run along its lateral surface. Therefore high expertise is required to manage the exposure of the insula and its complex anatomy. This video demonstrates the surgical management of a large left insular CM. A 29-year-old female with multiple CM and 7 years of partial seizures and recent onset of short memory loss. Neuroimaging showed a large left insular and planum polare CM with important mass effect and hemorrhage signs. The patient consented to surgery, and an awake pretemporal craniotomy was carried out with continuous motor evoked potential monitoring. No language function was localized in the superior temporal gyrus; therefore corticectomy of the middle portion was performed to expand the operative corridor. The vessel manipulation during wide opening of the sylvian fissure increased the risk of postoperative vasospasm and blood drain into the surgical field. The CM was exposed and completely removed without functional damage. The patient recovered from surgery without complications, and no seizures occurred at 2 months' follow-up. Postoperative imaging showed complete removal of the CM.

OBJECTIVEA cerebral arteriovenous malformation (cAVM) can change over time and cause symptoms, but clinical studies tend to define only the patients with ruptured cAVMs as symptomatic and do not consider neurocognitive aspects prior to neurosurgical intervention. The objective of this study was to describe the neurocognitive function of patients with ruptured and unruptured cAVMs according to the Spetzler-Martin (SM) grade, flow status, and anatomical topography.METHODSIn this blinded cross-sectional study, 70 patients of both sexes and ages 18–60 years were evaluated using the Brazilian Brief Neuropsychological Assessment Battery Neupsilin.RESULTSOf the 70 patients with cAVMs, 50 (71.4%) demonstrated deficits in at least one of the eight neurocognitive domains surveyed, although they did not exhibit neurological deficits. cAVMs in the temporal lobe were associated with memory deficits compared with the general population. The SM grade was not significantly associated with the results of patients with unruptured cAVMs. However, among patients with ruptured cAVMs, there were deficits in working memory in those with high-grade (SM grade) cAVMs and deficits in executive function (verbal fluency) in those with low-grade cAVMs (p < 0.001).CONCLUSIONSThis study indicates that patients with untreated cAVMs, either ruptured or unruptured, already exhibit neurocognitive deficits, even the patients without other neurological symptoms. However, the scales used to evaluate disability in the main clinical studies, such as A Randomized Trial of Unruptured Brain Arteriovenous Malformations (ARUBA), do not assess neurocognitive alterations and therefore disregard any deficits that may affect quality of life. The authors’ finding raises an important question about the effects of interventional treatment because it reinforces the hypothesis that cognitive alterations may be preexisting and not determined by interventions.

INTRODUCTION: Management of brain tumors in neonates and infants is very challenging. OBJECTIVE: To analyze the several treatment strategies and outcomes of brain tumors in the first year of life in a single institution. METHODS: The authors retrospectively evaluated 63 infants with brain tumors treated between 2007 and 2017, at IOP/GRAACC/UNIFESP. Data regarding initial clinical presentation, treatment modalities and outcomes were collected. RESULTS: From 63 patients treated, 61 were eligible for evaluation and 2 were excluded for loss of follow-up. Thirty were girls and 31 boys. The mean age at treatment was 6 months (range: 1 day-12 months). Twenty-nine babies presented with signs and symptoms of intracranial hypertension, 11 babies with epileptic seizures and 8, cranial nerves deficits. Forty-five tumors were located in the supratentorial compartment and 16 were infratentorially. Nine patients were diagnosed with tuberous sclerosis, 2 with neurofibromatosis type 1, 2 diagnosed with Li Fraumeni and 1 with Gorlin syndrome. The most common histological types were: 11 rhabdoid teratoid, 9 low grade astrocytoma, 5 choroid plexus carcinoma and 5 glioblastoma. Surgery is the treatment of choice. Ten patients underwent more than one surgery (2 to 6 resections) Eight deaths occurred. The mean follow-up was 3y10m (range: 2 days- 7y9m). CONCLUSIONS: Gross total resection is the goal of surgical treatment, but sometimes it is impossible in the first approach. To decrease the high intraoperative mortality of malignant tumors, these patients can undergo as many surgeries as necessary for total tumor resection, alternating with cycles of chemotherapy.

INTRODUCTION: Management of patients with craniopharyngioma is still controversial. Currently, quality of life is the goal for all treatment modalities. OBJECTIVE: To analyze the several treatment strategies and outcomes of craniopharyngioma patients in 15 years of experience in a single institution. METHODS: The authors retrospectively evaluated 110 craniopharyngeoma patients treated between 2002 and 2017, at IOP/GRAACC/UNIFESP. Data regarding initial clinical presentation, body mass index (BMI), endocrinological complications, treatment modalities and outcomes were collected. RESULTS: From 110 patients, 95 patients were eligible for evaluation. Fifty-five were boys and 40 girls. The mean age at treatment was 10,5 years. Fifty-six percent of children presented with signs and symptoms of intracranial hypertension, 24% with visual impairment and 38,5% with endocrinological disturbances. At the time of diagnosis, the mean BMI was 20,48kg/m(2). Thirty-eight children with cystic tumors were treated with intralesional interferon-alpha, 13 progressed to conventional treatment. Among the remaining 57 patients, 9 were treated with microsurgery, 29 with microsurgery + radiotherapy, 12 with endoscopic transesphenoidal surgery + radiotherapy and 7 recurrent patients with intracystic interferon-alpha. The mean BMI of patients treated exclusively with interferon-alpha was 22,88kg/m(2) (ideal weight) and with microsurgery + radiotherapy was 29,65kg/m(2) (overweight). After treatment, 61 cases (65%) progressed to panhypopituitarism. The mean follow up was 7,13 years. Eleven deaths occurred. Currently 41 patients are out of treatment. CONCLUSION: The interferon-alpha intracystic therapy has been shown to be effective in the control of predominantly cystic tumors, decreasing or delaying the morbidity from conventional treatment.

Introduction Gangliogliomas are tumors commonly found in the temporal lobe and related to seizures; their appearance in the pineal region is rarely described. This report characterizes the first case of anaplastic ganglioglioma of the pineal region. Case Report The authors describe the case of a 32-year-old woman that developed progressive headache. An MRI investigation revealed a pineal tumor. The patient tested negative for biomarkers and underwent surgery through supracerebellar infratentorial approach and achieved gross total resection of the tumor in a challenging location. Pathological analysis revealed a biphasic neoplasm with the following two distinct phenotypes in separate fields: an immature neuronal component with several atypical mitoses and a mature astrocytic component with bipolar cells, microcysts, and eosinophilic bodies. The Ki67/MIB1 proliferation index was 20–30% in localized hotspots. Based on the pathological findings, the tumor was defined as an anaplastic ganglioglioma World Health Organization (WHO) grade III. Discussion/Conclusion Gangliogliomas are classified as glioneural neoplasms based on the histologic findings described as a mixture of neoplastic astrocytes and neurons. Moreover, these tumors represent 0.4–1.3% of tumors of the central nervous system. Authors describe de novo anaplastic ganglioglioma as 1% of the largest series. Gross total resection and adjuvant treatment may play important role in patients' prognostic. In this case, due to the malignant anaplastic component of her tumor, the patient received treatment with temozolamide and radiotherapy after gross total resection of the lesion.

The anatomical characterization of the orbitofrontal cortex in human is limited in literature instead of many functional and clinical studies involving it. Objective Anatomically define the orbitofrontal region aiming to possible neurosurgical treatments and unify the scientific nomenclature as well. Method We analyze eighty four human hemispheres using a surgical microscope. Then we chose four hemispheres and dissect them according to Klinger’ technique. Results We found five main sulcus: olfatory sulcus, orbital medial sulcus, orbital lateral sulcus, orbital transverse sulcus and orbital intermediate sulcus. These sulcus, excluding the intermediate sulcus, delimit five gyrus: rectus gurys, orbital medial gyrus, orbital anterior gyrus, orbital lateral gyrus and orbital posterior gyrus. The main sulcal configuration can be divided on four more frequently patterns. Conclusion Orbitofrontal cortex is associated with many psychiatric disorders. Better anatomical and functional characterization of the orbitofrontal cortex and its connections will improve our knowledge about these diseases. A caracterização do córtex orbitofrontal em humanos é limitada na literatura, a despeito de estudos funcionais e clínicos envolvendo-o. Objetivo Definir anatomicamente o córtex orbitofrontal visualizando possíveis tratamentos neurocirúrgicos e unificar a nomenclatura científica. Método Analisamos oitenta e quatro hemisférios cerebrais humanos usando um microscópio neurocirúrgico. Escolhemos quatro hemisférios e os dissecamos de acordo com a técnica de Klinger para expor a substância branca subcortical. Resultados Encontramos cinco principais sulcos: sulco olfatório, sulco orbital medial, sulco orbital lateral, sulco orbital transverso e sulco orbital intermediário. Estes sulcos, excluindo o sulco orbital intermediário, delimitaram cinco giros: giro reto, giro orbital medial, giro orbital anterior, giro orbital lateral e giro orbital posterior. A conformação de sulcos e giros orbitais pôde ser dividida em quatro padrões mais frequentes. Conclusão O córtex orbitofrontal é associado com várias desordens psiquiátricas. Uma melhora caracterização anatômica, funcional e de suas conexões melhorará nosso conhecimento destas desordens.

The vasogenic edema in structures of posterior fossa secondary to elevated hydrostatic pressure can cause obstructive hydrocephalus, a condition called “reversible obstructive hydrocephalus from hypertensive encephalopathy.” A case of a 27-year-old woman with arterial hypertension and sign of raised intracranial pressure is reported. Her radiologic studies have showed vasogenic cerebellar edema without structural lesion. This edema leads to obstruction of the cerebral aqueduct. We discuss the clinical and therapeutic aspects of this condition. In this clinical situation, the accurate control of blood pressure is the main goal on medical care, and the need of permanent ventricular shunt is quite infrequent.

Aim Dissection of white fibers is important in identifying detailed neuroanatomical relationships. With tractrography it is possible to transport and apply this knowledge in a practical way to treat many diseases involving the white matter. Material and methods The Klingler method, subsequently disseminated by Ture with slight modifications was used. Results We review some historical aspects of white fibers and provide a guide for dissection of the internal capsule. The removal of gray matter allowed us to obtain a view of the white matter. We removed all U-shaped fibers to expose the insular cortex. The cortex of the insular lobe was removed, which exposed the extreme capsule. The removal of the claustrum exposed the external capsule, which covers the lentiform nucleus, specifically the putamen. During dissection, removing some fibers of the external capsule produced windows in which the putamen could be medially visualized. Since the internal capsule lies medial to the lentiform nucleus, it was necessary to remove the nucleus in order to expose the internal capsule. We identified five regions of the internal capsule: the anterior limb, genu, posterior limb, and sublenticular and retrolenticular parts. Finally, we determined that the fibers of the corona radiata condense into the internal capsule at the level of the superior border of the lentiform nucleus. Conclusion Knowledge gained with the cadaveric fiber dissection technique can be applied in microsurgical practice and can be used to evaluate the surgical treatment for different tumors and vascular malformations.

Schizencephaly is a rare congenital cerebral malformation associated with serious neurological manifestations. The number of studies regarding schizencephaly is limited.We conducted a literature review and extracted data from the case reports. Of 199 articles retrieved, 156 articles (734 patients) met our inclusion criteria.Patient characteristics included microcephaly (41.5% of patients), seizures (74.1%), bilateral cleft (41.4%), open lip (61.3%), septo-optic dysplasia (69.1%), and ventricular dilation (60.5%). The majority of clefts were in the frontal and parietal lobes. When these potential association factors were assessed by univariate logistic regression microcephaly (OR = 21.75, P0.001), corpus callosum agenesis (OR = 9, P0.001), motor impairments (OR = 6.21, P0.001), and bilateral clefts (OR = 6.31, P0.001) seems to have the strongest association, but also age at diagnosis10 years (OR = 1.05, P0.001), right (OR = 1.85, P = 0.001) or left (OR = 2.71, P0.001) side clefts and septum pellucidum (OR = 3.7, P = 0.002) agenesis were associated with neurocognitive dysfunctions.We describe novel findings with practical implications for predicting neurocognitive outcomes in patients with schizencephaly. Most patients had neurological impairments including motor (90.0%) or cognitive (77.5%) dysfunctions. Bilateral clefts, motor impairment, microcephaly, and corpus callosum agenesis were strongly associated with neurocognitive impairment. A lack of large cohorts of patients with schizencephaly prevented comparison of our results; most previous studies are case reports or small case series.

In the last years, a shift from the microsurgical treatment to an endovascular therapy in patients with basilar apex aneurysm has been settled, part of this phenomenon is related to the significant tendency of vital perforators to be involved in the aneurysm dissection and clipping, which can implicate unfavorable outcomes. Nevertheless, microsurgical treatment remains the treatment that can provide the superior rates of stable and durable aneurysm occlusion, which is most important to young patients.In this video, we present the case of a 45-yr-old female patient who complained of a sudden and severe headache and presented with progressive lethargy during the following 3 d.At admission, computed tomography did not show abnormal findings. However, cerebrospinal fluid analysis showed erythrocytes and corroborated the clinical suspicion of spontaneous subarachnoid hemorrhage. The patient signed the Institutional Consent Form, which allows the use of his/her images and videos for any type of medical publications in conferences and/or scientific articles.Angiography and magnetic resonance imaging revealed a saccular basilar apex aneurysm. It showed a wide neck as well as a lobulated dome with upward and slightly left projection. The aneurysm did not involve angiographically visible thalamoperforator arteries, which allowed the microsurgical treatment by the fronto-orbitozygomatic approach. However, during the interpeduncular cistern dissection, an intraoperative rupture of the aneurysm occurred. This video exemplifies the steps required to manage an intraoperative rupture of a basilar apex aneurysm.

OBJECTIVEThe authors report a novel surgical route from a superior anatomical aspect—the contralateral anterior interhemispheric-transcallosal-transrostral approach—to a lesion located in the subcallosal region. The neurosurgical approach to the subcallosal region is challenging due to its deep location and close relationship with important vascular structures. Anterior and inferior routes to the subcallosal region have been described but risk damaging the branches of the anterior cerebral artery.METHODSThree formalin-fixed and silicone-injected adult cadaveric heads were studied to demonstrate the relationships between the transventricular surgical approach and the subcallosal region. The surgical, clinical, and radiological history of a 39-year-old man with a subcallosal cavernous malformation was retrospectively used to document the neurological examination and radiographic parameters of such a case.RESULTSThe contralateral anterior interhemispheric-transcallosal-transrostral approach provides access to the subcallosal area that also includes the inferior portion of the pericallosal cistern, lamina terminalis cistern, the paraterminal and paraolfactory gyri, and the anterior surface of the optic chiasm. The approach avoids the neurocritical perforating branches of the anterior communicating artery.CONCLUSIONSThe contralateral anterior interhemispheric-transcallosal-transrostral approach may be an alternative route to subcallosal area lesions, with less risk to the branches of the anterior cerebral artery, particularly the anterior communicating artery perforators.

Myelomeningocele (MMC) is a costly lifetime disease with many comorbidities, including sensory and motor lower limb disability, bladder/bowel dysfunction, scoliosis, club foot, and hydrocephalus. MMC treatment options have changed over time because routine use of fetal ultrasonography and MRI has provided prenatal diagnosis and the potential for fetal surgery. There is still no consensus on how to treat the MMC diagnoses prenatally, mainly related to the infrastructure required to operate on pregnant patients. This article provides an overview of prenatal and postnatal MMC repair and the features in the prenatal diagnosis.

The advance in the imaging tools during the pregnancy (ultrasound and magnetic resonance) allowed the early diagnose of many fetal diseases, including the neurological conditions. This progress brought the neurosurgeons the possibility to propose treatments even before birth. Myelomeningocele is the most recognized disease that can be treated during pregnancy with a high rate of success. Additionally, this field can be extended to other conditions such as hydrocephalus and encephaloceles. However, each one of these diseases has nuances in the diagnostic evaluation that should fit the requirements to perform the fetal procedure and overbalance the benefits to the patients. In this article, the authors aim to review the neurosurgical aspects of the antenatal management of neurosurgical conditions based on the experience of a pediatric neurosurgery center.

Medial temporal basal arteriovenous malformations (AVMs) have complex anatomy. They usually drain to the basal vein of Rosenthal, and arterial feeders can arise from the anterior choroidal artery and its branches, or from the posterior cerebral artery. If the AVM is more posterior in the parahippocampal gyrus, there is a predominance of arterial feeders arising from P2P or P3 segments of the posterior cerebral artery. As posterior AVMs are difficult to reach using anterior approaches, the supracerebellar transtentorial approach provides a direct pathway to the malformation, allowing better visualization and exposure of the vascular anatomy. In this video, we present a 29-yr-old woman with a left parahippocampal AVM with P2P arterial feeders and Rosenthal basal vein drainage. The patient had three months of moderate headache and two abrupt seizures before admission. Emergency computed tomography showed intraventricular hemorrhage. Magnetic resonance imaging and cerebral angiography revealed an AVM located in the parahippocampal gyrus, posterior to pulvinar thalamus. The patient underwent microsurgical treatment in semi-sitting position using a supracerebellar and infratentorial approach with transtentorial resection. The AVM was completely removed, and the patient recovered without neurological deficits. The authors present a 3-dimensional video of the microsurgical steps required to perform a transtentorial approach for AVM resection in the parahippocampal gyrus. The patient signed the Institutional Consent Form, which allows the use of his/her images and videos for any type of medical publications in conferences and/or scientific articles.

Giant brain aneurysms account for approximately 5% of all intracranial aneurysms. Although treatment modalities can vary widely, none is ideal for every patient. Endovascular treatment is usually preferred, especially when the large size of the aneurysm limits visualization of the brain parenchyma and parent vessels that arise from the aneurysm, making surgical clip placement across the neck a difficult task. However, despite the higher chances of morbidity, microsurgery is an effective treatment modality due to lower recurrence rates. Surgically, a wide neck, calcifications, or atheroma are complicating factors to be considered while planning the best treatment. Thus, with an appropriate case selection, a favorable outcome is feasible in most cases. Here, we present the case of a 27-yr-old female who presented with a severe headache for 7 mo and 3 mo of progressive left temporal vision loss, which was confirmed by visual field perimetry using the Humphrey visual field analyzer. Magnetic resonance angiography and digital subtraction cerebral angiography showed an anterior communicating artery complex inferiorly and medially oriented aneurysm measuring 25.4 × 16.5 mm, with a 3 mm neck. It was fed by the right A1, associated with a hypoplastic left A1, incorporating the proximal right and left A2 segments, with an intraluminal thrombus and causing mass effect on the optic chiasm and hypothalamus. This video demonstrates the microsurgical steps required to perform this operation, through a right orbitozygomatic craniotomy. At a 3-mo follow-up, the patient was neurological intact without complaints. The patient signed the Institutional Consent Form, which allows the use of his/her images and videos for any type of medical publications in conferences and/or scientific articles.

The distal posterior inferior cerebellar artery (PICA) is a rare site of aneurysm formation. Only small case series and case reports regarding surgical treatment are found in the literature.The PICA is divided into 5 segments (anterior medullary, lateral medullary, tonsilomedullary, telovelotonsillary, and cortical), and the distal ones represent the most complex, due to anatomic variations. We present a case of a 69-yr-old female patient who has suffered from a sudden and intense occipital headache, associated with nausea and vomiting. CT scan showed intraventricular hemorrhage, and further investigation with MRI and MR Angiography revealed a small distal PICA aneurysm, at the superior part of the medial aspect of the left cerebellar tonsil. Digital angiography has demonstrated the aneurysm at the tonsilomedullary segment of the PICA. In this 3-dimensional video, the authors show the microsurgical clipping of a saccular distal PICA aneurysm in the close relation to a choroidal branch, performed by median suboccipital craniotomy. Step-by-step of the dissection, relevant surrounding anatomy and aneurysm clipping is demonstrated. The patient signed the Institutional Consent Form, which allows the use of his/her images and videos for any type of medical publications in conferences and/or scientific articles.

We present a 14-yr-old male with a history of traumatic brain injury in March 2016, secondary to clonic tonic generalized seizures. CT scan showed hemorrhage at mesial temporal region in the body of right hippocampus, intraventricular hemorrhage at the level of lateral ventricles (right and left side) and fourth ventricle. After this the patient presented with pulsating right temporal headache of high intensity (VAS 10/10) that improved with common analgesics, dizziness, and clonic tonic generalized seizures despite taking Phenobarbital 100 mg/24 h. Neuropsychological assessment reveal major deficits regarding executive functions: working memory, verbal fluency, and planning abilities. Brain MRI and angiography showed AVM at the right level of hippocampus body. An intranidal aneurysm was also observed. Venous drainage was through the basal vein of Rosenthal. We planned for surgery and resection of the hippocampal AVM through the trans-T2 approach. Postoperatively, the patient was without medical complications. We present a 3-dimensional video of the microsurgical treatment for right hippocampal AVM performed through a trans-T2 approach. The patient signed the Institutional Consent Form, which allows the use of his/her images and videos for any type of medical publications in conferences and/or scientific articles.

Background Neurofibromas are benign nerve sheath tumors that usually affect peripheral nerves and are related to neurofibromatosis type 1; however, they have not been described as a cause of intraparenchymal brain tumor. Case Description We report a case of intracranial myxoid neurofibroma in a 19-year-old female patient manifested as an intense and progressive cephalea, followed by nausea, vomiting, photophobia, and phonophobia. Computed tomography and magnetic resonance imaging showed an extant, expansive left frontoparietal parafalcine/parasagittal tumor. Histopathologic examination determined S-100 protein and CD34 positivity, as well as sparse expression of Ki67 protein, and indicated Schwann cells with characteristic wavy nuclei and intraneural fibroblasts in a myxoid background. Together, these observations characterized the tumor as myxoid neurofibroma. The tumor was excised, and the patient recovered without deficits and with no signs of recurrence after 6 years of follow-up. Conclusions This is a novel presentation of a myxoid neurofibroma. The tumorigenesis mechanisms are likely complex and possibly involve the differentiation of Schwann cells present in adrenergic autonomic nerves in the subarachnoid arterial branches or in trigeminal nerves present in the meningeal convexity.