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Magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) is a new minimally invasive treatment for tuberous sclerosis complex (TSC)-associated epilepsy in children. This video describes a case of a 17-year-old girl with TSC-associated drug-resistant epilepsy treated with robotic-assisted MRgLITT. In our case, MRgLITT was safe and effective in simultaneous targeting of multiple epileptic tubers in 1 single procedure, leading to a marked decrease in seizure frequency. MRgLITT could be a promising and more appealing treatment option for children who may need multiple surgeries over their lifetime as a result of the progressive nature of TSC., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Tectal plate tumors are a rare subset of midbrain tumors in pediatric populations. They are slow growing and low grade, with indolent and subtle manifestation unless they cause hydrocephalus. 1-5 We present a tectal tumor in an 8-year-old girl (Video 1). Her clinical onset occurred with headache, vomiting, and seizure secondary to intracranial hypertension. MRI revealed a ring-enhanced, roundish lesion of tectal plate, with posterior displacement of quadrigeminal corpora determining aqueductal stenosis and obstructive triventricular hydrocephalus. First, to restore CSF circulation, we performed an endoscopic third-ventriculostomy without lesion's biopsy, since there was normal brain tissue within the third ventricle and the tumor. Tumoral marker values were in the average range, so surgery was planned to remove the tumor. The patient, after the positioning of external lumbar drainage, underwent an occipital posterior interhemispheric retrocallosal transtentorial approach, which represents a direct surgical corridor to quadrigeminal cistern and quadrigeminal plate. Arachnoidal dissection of mesencephalic cistern and cutting of the tentorium are mandatory to obtain adequate exposure of quadrigeminal region, control over vascular structures, and lesser brain retraction. Identification of anatomical components and relationships between quadrigeminal plate with vein of Galen, PCA branches, tentorium, and medial occipital veins represents a crucial point. When this approach is selected, the venous anatomy must be navigated with caution. Angiography's venous phase may provide additional planning information. 6 Coagulation of vascular structures such as occipital veins is carefully avoided since it creates risk of venous infarction, leading to visual loss. Histologic examination revealed a rosette-forming glioneuronal tumor (grade I WHO 2021 7 )., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Symptomatic cysts of the septum pellucidum (CSP) are extremely rare in children and surgical indications are not well defined. A very careful clinical and neuroradiologic evaluation is necessary to consider a patient for surgical indication., Methods: We present a surgical series of 7 pediatric patients. Clinical and radiological features of the patients, including clinical presentation, previous treatment, pre, and post-operative MRI, immediate postoperative, neuropsychiatric assessment, and outcomes were reviewed., Results: There were 5 males and 2 females (mean age 8 yrs). Five patients presented a history of severe intermittent headaches, two of them were admitted with acute symptoms of raised intracranial pressure. One patient presented Epilepsy and ADHD and one patient had severe psychosis. Overall, psychiatric disorders were diagnosed in six patients, three patients had Intellectual Disability (ID). In all cases, the cyst presented a ballooning feature, with a mean volume of 18,36 cm3 (range 10,62-28,5) and significant lateral bulging of both layers. All were operated on endoscopically without complications. After surgery, a very significant decrease in cyst volume was observed (mean volume 5,68 cm3; range 3,18-10,1) with complete disappearance of the ballooning aspect. Headaches resolved in all patients. In two patients operated in emergency papilloedema and vision improved in the first week after surgery. No recurrence of the cysts was noted during follow-up in all patients., Conclusions: CSP may be associated with behavioral or psychiatric problems also in children. Neuroendoscopic surgery is a safe and effective therapeutic modality to treat CSP presenting with symptoms and signs of intracranial hypertension with good clinical results., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Objective: Moyamoya disease (MMD) is characterized by a progressive spontaneous occlusion of distal internal carotid arteries and its main branches. It is necessary to promptly diagnose and treat this condition, especially in children, because of the high risk of stroke and consequent severe disability. In this study, we examine the role of multiple burr-hole surgery (MBHS) in the treatment of pediatric patients with MMD and quasi-MMD and the role of perfusion magnetic resonance imaging (MRI) in the surgical indication and during follow-up., Methods: We reviewed preoperative, early postoperative, and late postoperative MRI perfusion and digital subtraction angiography images together with clinical and surgical outcomes in 10 children with MMD treated by MBHS., Results: Fourteen MBHS procedures (6 unilateral, 2 bilateral, and 2 single-setting bilateral) were performed in 10 children. Clinical and radiologic follow-up for all patients ranges from 16 months to 7 years. No ischemic events (transient ischemic attacks or stroke) occurred during the follow-up period. Postoperative digital subtraction angiography showed good revascularization around the burr-hole sites in all patients. The functional efficacy of the surgery was confirmed by dynamic susceptibility contrast perfusion MRI studies in 8 patients., Conclusions: Our data underline the good rate of success of MBHS in pediatric MMD and quasi-MMD. This technique results in good collateral revascularization, improved cerebral perfusion and excellent short and long-term symptoms control, with low perioperative risk. Postoperative changes of perfusion parameters and ivy sign MRI finding seem to show a positive correlation and mainly occur in posterior middle cerebral artery territory., (Copyright © 2019. Published by Elsevier Inc.)

We present an unusual association between Noonan syndrome and tetraventricular hydrocephalus, caused by fourth ventricle outlet obstruction, in a 5-year-old boy. Magnetic resonance imaging showed a diverticular enlargement of the left foramen of Luschka, with compression of the facial nerve that resolved following treatment of hydrocephalus by endoscopic third ventriculostomy., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Interhypothalamic adhesions (IHAs) are horizontally oriented parenchymal bands of tissue connecting the medial hypothalamic regions across the third ventricle. They can be assessed with high-resolution magnetic resonance (MR) techniques., Case Description: We report MR and neuroendoscopic features of IHA in a 3-year-old boy without symptoms referable to the hypothalamus. He presented with obstructive hydrocephalus secondary to posterior fossa tumor. An endoscopic third ventriculostomy (ETV) was attempted but not performed because of the presence of a thick IHA, which prevented an approach to the floor of the third ventricle. During the procedure the patient also experienced supraventricular tachycardia. The procedure was aborted, and an external ventricular drainage was left in the ventricles until resolution of hydrocephalus after posterior fossa surgery. To the best of our knowledge, no study has previously described in detail endoscopic images of IHA., Conclusions: MR imaging allows one to preoperatively identify most anatomic anomalies of the ventricular system and the floor of the third ventricle. However, an IHA that may prevent an approach to the floor of the third ventricle due to his thickness may be missed. This can be recognized only on direct vision, underlining the importance of endoscopy in neurosurgery., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Hydrocephalus in patients with neurofibromatosis (NF) type 1 is usually obstructive and may arise secondary to tumoral or nontumoral causes. Treatment of hydrocephalus in these patients is often challenging owing to combined pathologies and unique anatomic changes. The use of endoscopic third ventriculostomy (ETV) as treatment has rarely been described in this group. We aimed to characterize indications, considerations, and outcome of ETV in patients with NF 1 gathered in a multicenter international cohort., Methods: Five centers participated in this retrospective study. Following institutional review board approval, data and images were collected. Patients of all ages with NF 1 who underwent ETV for treatment of obstructive hydrocephalus were included. Patients who had no postoperative radiologic or clinical follow-up were excluded. ETV failure was defined as recurrent clinical or radiologic signs of hydrocephalus., Results: The study included 42 patients. Common etiologies for hydrocephalus were aqueductal/tectal tumor (31%), aqueductal web (26%), and aqueductal stenosis owing to NF-related changes (14%). Ten patients had a preoperative diagnosis of optic pathway glioma. ETV failures were identified in 6 patients within 1 month, in 3 patients within 9 months, and in 1 patient within 4 years. ETV was successful in 32 patients (76%) with a mean follow up of 59.4 months ± 50.9 (range, 4 months to 15 years)., Conclusions: ETV is a safe treatment for selected patients with NF 1 and obstructive hydrocephalus. Individual anatomic and pathologic aspects should be taken into consideration., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: Endoscopic third ventriculostomy (ETV) is a safe and less-invasive treatment strategy for patients with obstructive hydrocephalus and provides excellent outcome. Nevertheless, repeat ETV in cases of ETV failure is a controversial issue., Methods: Between 1993 and 1999, 113 patients underwent a total of 126 ETVs at the Department of Neurosurgery, Mainz University Hospital. Obstructive hydrocephalus was the causative pathology in all cases. A very long-term follow-up of up to 16 years could be achieved. All medical reports of patients who received ETV were reviewed and analyzed with focus on ETV failure with following repeat ETV and its initial as well as very long-term success., Results: Thirty-one events of ETV failure occurred during the follow-up period. Thirteen patients underwent repeat ETV: 3 patients during the first 3 months (early repeat ETV), the other 10 patients after 7-78 months (late repeat ETV, mean 33 months). All repeat ETV were performed without complications. Follow-up evaluation after successful repeat ETV ranged from <1 month up to 14 years (mean 7 years). Of the 3 early revisions, 2 failed and 1 other patient died during follow-up whereas only 2 of the late repeat ETV failed. Very long-term success rate of late repeat ETV up to 14 years yielded 80%., Conclusions: Repeat ETV in cases of late ETV failures represents an excellent option for cerebrospinal fluid circulation restoration up to 14 years of follow-up. Repeat ETV in early ETV failure in contrast is not favored by the performing surgeons; and factors of ETV failure should be analyzed very carefully before a decision for repeat ETV is made., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Persistent or recurrent shunt infections can be secondary to predisposing factors, such as isolated compartments, cerebrospinal fluid leaks, or foreign bodies., Case Description: A 5-year-old girl experienced several episodes of shunt infections. After careful reevaluation of all neuroradiologic records of the patient, a foreign body in the left frontal horn of the lateral ventricle was suspected. An endoscopic approach was used to identify and remove a small fragment of bone wax., Conclusions: To our knowledge, this is the first report of intraventricular bone wax causing persistent CSF infection. The diagnosis was difficult because wax resembles air on computed tomography and on magnetic resonance imaging, and so it had been not noticed for months. Only its persistence on several images raised the suspicion of foreign body inside the ventricular system., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Grisel syndrome is a nontraumatic rotatory subluxation of the atlantoaxial joint, following nasopharyngeal inflammation or ear, nose, and throat (ENT) procedures. The syndrome should be suspected in cases of persistent neck pain and stiffness, especially after ENT surgical procedures. The primary treatment of early detected Grisel syndrome is conservative. If conservative treatment fails to achieve a stable reduction or it is followed by neurologic symptoms, arthrodesis of the first and second cervical vertebrae is indicated. We report the case of a 9-year-old boy who developed Grisel syndrome after adenoidectomy and was treated with C1-C3 internal fixation and fusion., Case Description: A 9-year-old boy was referred to our hospital with a 3-month history of painful torticollis, which appeared 4 days after adenoidectomy. The patient underwent a neuroimaging study that documented the presence of atlantoaxial rotatory subluxation. The patient underwent C1-C3 internal fixation and fusion, using lateral masses and laminar and pars interarticularis screws. On the third postoperative day he was mobilized with a rigid collar. Postoperative computed tomography scans showed the resolution of rotational deformity and a solid fusion., Conclusion: Early treatment of Grisel syndrome is of utmost importance to avoid neurologic complications and surgical intervention. In a patient with torticollis following ENT procedures, Grisel syndrome should be always suspected. In case of failure of conservative treatment or in case of delayed diagnosis, rigid C1-C2 or C1-C2-C3 fixation is a straightforward and valid surgical technique, even in children, because it provides immediate spinal stability in all planes at the atlantoaxial complex, avoiding the need for prolonged rigid external bracing., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: During the past decade, endoscopic intraventricular and skull base operations have become widely used for a variety of evolving indications. A global survey of practicing endoscopic neurosurgeons was performed to characterize patterns of usage regarding endoscopy equipment, instrumentation, and the indications for using image-guided surgery systems (IGSs)., Methods: An online survey consisting of 8 questions was completed by 235 neurosurgeons with endoscopic surgical experience. Responses were entered into a database and subsequently analyzed., Results: The median number of operations performed per year by intraventricular and skull base endoscopic surgeons was 27 and 25, respectively. Data regarding endoscopic equipment brand, diameter, and length are presented. The most commonly reported indications for IGSs during intraventricular endoscopic surgery were tumor biopsy/resection, intraventricular cyst fenestration, septostomy/pellucidotomy, endoscopic third ventriculostomy, and aqueductal stent placement. Intraventricular surgeons reported using IGSs for all cases in 16.6% and never in 24.4%. Overall, endoscopic skull base surgeons reported using IGSs for all cases in 23.9% and never in 18.9%. The most commonly reported indications for IGSs during endoscopic skull base operations were complex sinus/skull base anatomy, extended approaches, and reoperation., Conclusions: Many variations and permutations for performing intraventricular and skull base endoscopic surgery exist worldwide. Much can be learned by studying the patterns and indications for using various types of equipment and operative adjuncts such as IGSs., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: Idiopathic aqueductal stenosis is a cause of noncommunicating hydrocephalus, which actual treatment with endoscopic third ventriculostomy (ETV) could assess without any interference with the etiology. The results of ETV in this indication therefore could be interpreted as the result of the surgical procedure alone, without any additional factors related to the etiology of the cerebrospinal fluid pathway obstruction, such as hemorrhage, infection, brain malformations, or brain tumors or cysts., Methods: After a brief description of pathogenesis of hydrocephalus in aqueductal stenosis, the authors review the literature for studies on ETV, extrapolating patients with idiopathic aqueductal stenosis in infancy, childhood, and adulthood. Differences in outcome between patients treated with ETV and patients treated with ventriculoperitoneal shunt (VPS) are also reviewed., Results: The overall success rates of ETV range between 23% to 94%, with a mean of 68%; when only patients affected by obstructive triventricular hydrocephalus secondary to aqueductal stenosis are considered, the success rate is actually quite homogeneous and stable, being above 60% at any age, even if a trend in lower success rate in very young infants (younger than 6 months of age) is noticeable. The few reports on intellectual outcome failed to demonstrate differences between ETV and VPS., Conclusions: Several issues, such as the cause of failures in well-selected patients, long-term outcome in infants treated with ETV, effects of persistent ventriculomegaly on neuropsychological developmental, remain unanswered. Larger and more detailed studies are needed., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: Interhemispheric and quadrigeminal cysts are rare lesions, similar in their propensity to present in young babies and to be associated with other central nervous system malformations, such as corpus callosum agenesia, holoprosencephaly, encephalocele, and neuronal heterotopias. Recently endoscopy has become increasingly popular in the treatment of arachnoid cysts, but experience with cysts located in the interhemispheric fissure and in the quadrigeminal cistern is limited., Methods: This study reviews the specific anatomy of interhemispheric and quadrigeminal cysts and their relationship with the ventricular system and subarachnoid cisterns to select the most appropriated treatment. It also reviews the literature on endoscopic treatment of interhemispheric and quadrigeminal cysts., Results: Interhemispheric and quadrigeminal cysts are not homogeneous, they have different extensions toward surrounding regions. In most cases it is presented as an area of contiguity between the cyst and ventricular system and/or subarachnoid cisterns, making endoscopic treatment feasible. The success rate for endoscopic treatment is not different from that reported in large series of arachnoid cysts elsewhere., Conclusions: Endoscopic treatment should be considered the first-line option in the treatment of such lesions, even if some complications, such as subdural collections due to thinness of the cerebral mantle or subcutaneous CSF collections due to multifactorial associated hydrocephalus, must be expected., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: Endoscopic third ventriculostomy (ETV) is the treatment of choice for noncommunicating hydrocephalus. In the last decade, its routine use also has taken place in patients who have previously undergone shunt placement (secondary ETV)., Methods: This study reviews the specific anatomy of the ventricular system in patients presenting with shunt malfunction, the criteria for patient selection, and the results of ETV performed as an alternative to shunt revision in cases of shunt malfunction or infection., Results: The success rate of secondary ETV in well-selected cases is not different from that of primary ETV; it should be reasonable to offer all patients with blocked shunts and suitable anatomy indicating an obstructive cause the opportunity for shunt independence regardless of the original cause of the hydrocephalus., Conclusions: Shunt infection should not be considered a contraindication to ETV, even if the success rate may be lower. Considering the higher complication rate and higher risk of intraoperative failure, secondary ETV should be performed by expert neuroendoscopists., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Unlabelled: Meningitis due to Escherichia coli is rare, and generally observed in very particular circumstances, such as neonatal period, anatomical anomalies or in immune-deficient patients., Case Report: A 16-month-old male infant was admitted for acute meningitis. E coli was detected in the cerebro-spinal fluid (CSF). As appropriate antibiotic treatment proved inefficient, a cerebral computerised tomography (CT) scan was performed, revealing an epidermoid cyst of the posterior fossa. The cyst was resected after CSF sterilisation. Postoperative recovery was satisfactory., Conclusion: When an unusual bacterial species such as E coli is detected in CSF, the authors suggest consideration of a cutaneous or ETN focus, or a congenital malformation with communication between cutaneous and meningeal structures.

Aims: The authors analyse a series of patients with oxycephaly in order to detail the definition of this craniosynostosis and its functional prognosis., Patients and Methods: The medical records of 129 oxycephalic patients were reviewed. Skull X-rays, ophthalmologic examination, mental level assessment, intracranial pressure monitoring and CT scan were analysed. The more recent patients were also analysed by MRI. Operated on or not, the patients were followed-up, particularly as far as the mental evolution is concerned. Mean follow-up was 3 years 7 months., Results: One third of the patients came from North Africa, where oxycephaly seems predominant. Mean age at diagnosis was 6 years. Past history of rickets was found in 15% of the patients. On X-rays, the vast majority of the patients presented with multisutural synostosis involving both coronal and sagittal sutures, and diffuse digital prints. At the first mental assessment, one third of the patients had an IQ below 80. Papilledema was found in 17%. The monitoring of intracranial pressure showed an increased pressure in almost two thirds of the patients. Sixty-four percent of the patients with increased intracranial pressure had a normal fundoscopy. Out of 16 patients explored by MRI, 12 had a Chiari I malformation. Postoperatively, all papilledemas disappeared, and the intracranial pressure returned to normal in all cases with preoperative increased intracranial pressure. The mental level seemed to stabilize, the mean postoperative IQ being strongly correlated with the preoperative level. In non-operated patients, the mental level worsened significantly., Conclusions: Oxycephaly is a late-appearing craniosynostosis, with a high risk of ophthalmologic and mental complications. Based on the present series, the operation seemed effective in preventing these complications.

Antley-Bixler syndrome was first described in 1975, and to date, 20 cases have been reported. In addition to brachycephaly, the syndrome is associated with midface hypoplasia often with choanal stenosis or atresia, bilateral radiohumeral synostosis, multiple joint contractures, femoral bowing and long bone fractures, "pear-shaped nose", dysplasic ears, and occasionally urogenital or cardiac defects. Survival is closely linked to upper airway obstruction, which also affects (with craniosynostosis) mental prognosis. Association and severity of malformations are variable, and while numerous children have died early from respiratory distress, one third of them are alive, and have had quite satisfactory development. With early and effective prevention of respiratory complications, and early treatment of craniosynostosis, overall prognosis can be favorable. The mode of inheritance is probably autosomal recessive and midtrimester prenatal diagnosis is feasible; genetic counseling depends on accurate prognostic and therapeutic data. We describe 2 further cases. The first a 4 years old male, with unilateral coronal synostosis and radiohumeral synostosis predominating on the same side. The second an 18 months old female, with brachycephaly and an imperforate anus.

Background: Mental retardation, considered as common in Apert syndrome could be in part due to associated brain abnormalities., Population and Methods: Sixty patients (32 males, 28 females) were included in the study. Patient age at the last examination was over 3 years (mean 10 years, range 3-28 years) in 38 patients. IQ was assessed from psychometric tests adapted for age. Brain anatomy was studied by MRI. Age at operation and quality of familial environment were also evaluated., Results: The IQ was over 70 in 12 patients (32%), over 90 in five (13%) and the mean IQ was 62 (10-114). Thirty percent of patients had abnormalities of the corpus callosum, 43% of the cerebral ventricles and 55% of the septum pellucidum. There was no anomaly in 28% of the patients. One or more operations were performed in 53 patients, before one year of age in 37. Ten children were institutionalized or in deleterious family situation. The main factor influencing the mental prognosis was the age at operation: the final IQ was over 70 in 50% of the children operated on before one year of age versus 8% in those operated on later (P = 0.01). Only the anomalies of the septum pellucidum seemed to play a role: 50% of the patients with normal septum had an IQ > 70 compared to 18% in those with septum anomalies (P < 0.04). The quality of the familial environment also influenced the mental development: 12.5% of the patients who were institutionalized or in difficult familial situation had an IQ > 70 compared to 39% of those who live in a normal family., Conclusions: Careful investigation including MRI is necessary for detecting associated brain abnormalities. The patients must be operated on early, if possible before the age of nine months. Attention has also to be paid to quality of the sociofamilial environment.

Background: Use of MRI makes the association Chiari malformation-cranio-facial synostosis more frequent than expected. The aim of this work was to ascertain the true incidence of Chiari malformation and to understand the reasons of the association between a bone pathology and a CNS malformation., Patients and Methods: The anatomy of the posterior cranial fossa in Crouzon syndrome was retrospectively studied on MRI scan in 49 patients., Results: A chronic tonsillar herniation, similar to a Chiari malformation, was observed in 71.4% of the cases. All the patients with Crouzon syndrome and progressive hydrocephalus had a Chiari malformation, but only 19 out of the 33 patients without associated hydrocephalus had a Chiari malformation (57.6%). Significant differences between the pattern of lambdoid suture closure were founded between both groups, with and without Chiari malformation, the lambdoid closure appearing earlier in patients with Chiari malformation., Conclusion: The high incidence of chronic tonsillar herniation in Crouzon syndrome seems related to the premature synostosis of the lambdoid suture.

The real-time B-mode echotomographies of 100 patients with transient visual symptoms have been reviewed and their findings have been compared with those of other 100 patients with transient ischemic attacks, who never had visual disturbances in their clinical history. The rate of pathologic echotomographies was higher in the group of patients with transient visual symptoms (72%), who also had a higher percentage (50% of the positive cases) of small or mild plaques without significant hemodynamic effects. The amaurosis fugax, particularly when associated with hemispheric ischemic symptoms, should suggest a carotid disease. The non-invasive exploration by real-time B-mode echotomography and Doppler with spectral analysis is the method of choice for the diagnosis of patients with transient visual symptoms.

This study reports a selected group of 45 patients with symptomatic low-grade stenoses of the carotid bifurcations, detected by real-time B-mode echotomography, and correlates the results of this technique with those of the Doppler flow study and carotid angiography. The Doppler study with spectral analysis was negative in all cases, whereas a correlation between the echotomographic and angiographic results was observed in 20 among 27 patients explored by angiography (74%). The causes of failure of angiography in some cases are briefly discussed. The almost absolute sensitivity of high resolution B-mode echotomography in detecting these early, sometimes symptomatic atherosclerotic changes of the carotid artery wall is emphasized.