Unilateral deep brain stimulation (DBS) of nucleus ventralis intermedius thalami (Vim) for the treatment of post-traumatic tremor in children: a multicentre experience.

PURPOSE: Deep brain stimulation (DBS) of nucleus ventralis intermedius thalami (Vim) is a validated technique for the treatment of essential tremor (ET) in adults. Conversely, its use for post traumatic tremor (PTT) and in paediatric patients is still debated. We evaluated the efficacy of Vim-DBS for lesional tremor in three paediatric patients with drug-resistant post-traumatic unilateral tremor. METHODS: We retrospectively collected data regarding three patients with unilateral tremor due to severe head injury, with no MRI evidence of basal ganglia lesions. The three patients underwent stereotactic frame-based robot-assisted DBS of Vim contralateral to the tremor side. RESULTS: Mean follow-up was 48 months (range: 36-60 months). Tremor was reduced in all patients with a better control of voluntary movements and improvement of functional status (mean FIM scale improvement + 7 points). No surgical complications occurred. CONCLUSION: Unilateral contralateral DBS of Vim could be efficacious in post-traumatic tremor, even in paediatric patients and should be offered in PTT drug-resistant patients.

Rare Onset of Erdheim-Chester Disease in Children and Young Adults: A Case Series and Review of the Literature.

INTRODUCTION: Erdheim-Chester disease (ECD) is a rare histiocytic neoplasm that affects patients, predominantly males aged 40-70 years, with very heterogeneous clinical presentation and prognosis. In 2020, Goyal et al. proposed consensus recommendations for the management of patients with ECD, remarking on the exceptional presentation of the disease in the pediatric population. CASE PRESENTATION: The first patient, a 20-year-old male, underwent cervical laminectomy and partial removal of a cervical spine lesion, initially apparently consistent with cervical schwannomas. The second patient, a 9-year-old female, received surgery for an extra-axial lesion of the greater sphenoid wing, radiologically consistent with a meningioma. CONCLUSION: At present, 15 pediatric cases have been reported in the literature with involvement of the central nervous system, with no consensus on the diagnostic and therapeutic management, as Pegoraro et al. evidenced in their pediatric multicenter case series. The present article adds two new cases of ECD with onset in childhood and young adulthood, who received the diagnosis after neurosurgical procedures.

Loculated hydrocephalus: is neuroendoscopy effective and safe? A 90 patients' case series and literature review.

INTRODUCTION: Loculated hydrocephalus is a complex condition in which different non-communicating compartments form within the ventricular system due to different etiology, mainly intraventricular hemorrhage and infection. Since the end of the twentieth century, neuroendoscopy has been explored as a therapeutic option for loculated hydrocephalus with non-univocal results. METHODS: We performed a retrospective analysis of 90 patients who underwent endoscopic treatment for loculated hydrocephalus from January 1997 to January 2021 (mean age: 2 years, range 7-21). We included 37 (41.1%) children with multiloculated hydrocephalus, 37 (41.1%) with isolated lateral ventricle, 13 (14.4%) with excluded temporal horn, and 3 (3.3%) with isolated fourth ventricle. We compared our results with those available in literature. RESULTS: A mean of 1.91 endoscopic procedure/patient were performed (only one endoscopy in 42.2% of cases). Complications of neuroendoscopy and of shunt surgeries were recorded in 17 (18.9%) and 52 (57.8%) children, respectively. Twenty-six (28.9%) children were shunt-free at the last follow-up, 47.8% have only one shunt. DISCUSSION: The first goal of neuroendoscopy is to increase the rate of shunt-free patients but, when it is not possible, it aims at simplifying shunt system and reducing the number of surgical procedures. In our series, neuroendoscopy was able to achieve both these goals with an acceptable complication rate. Thus, our results confirmed neuroendoscopy as a valid tool in the long-term management of loculated hydrocephalus. Neuronavigation and intraoperative ultrasound could increase the success rate in cases with distorted anatomy.

Robot-assisted transcerebellar stereotactic approach to the posterior fossa in pediatric patients: a technical note.

PURPOSE: During the last decade, there has been renewed interest in stereotactic approaches to diffuse intrinsic pontine gliomas (DIPGs) in children, due to the development of new concepts in molecular biology and management, and subsequent need for tissue sampling. Stereotactic frame-based and robot-assisted techniques are associated with reduced target error and have been incorporated into standard practice at our institution. METHODS: Four children (age 2-7 years) underwent a robot-assisted frame-based transcerebellar approach using the Leksell G frame coupled with Renishaw's neuromate® stereotactic robot. The procedures included 3 biopsies (two brainstem tumors and one cerebellar hemispheric lesion) and 1 depth electrode implantation into a low-grade tumor remnant (ganglioglioma) of the middle cerebellar peduncle causing drug-resistant epilepsy in a young girl. Targeting was based on MRI, and in one case, 18F-FET-PET was coregistered to MRI to improve sampling accuracy. The frame was applied 180° rotated compared to standard orientation, and patients were positioned prone during surgery and stereotactic preoperative CT scan. Postoperative CT scan ruled out complications and was coregistered to preoperative MRI to check the target accuracy. RESULTS: No complications occurred, and targeting was accurate in all cases. All tissue samplings provided proper histology; depth electrode EEG exploration was diagnostic and led subsequent resective surgery. CONCLUSIONS: According to our experience, the transcerebellar frame-based robotic stereotactic approach to the cerebellum and the brainstem is feasible, safe, and effective even in young children.

Airgun Wound with Superior Sagittal Sinus Involvement in a Child: Case Report and Review of the Literature.

INTRODUCTION: Although still considered quite harmless, nonpowder guns (NPG) may cause severe head injuries in children. We present the case of a depressed skull fracture with superior sagittal sinus involvement following NPG injury. Decision-making and surgical management are discussed, and the current literature concerning NPG-related pediatric head injuries is reviewed. CASE PRESENTATION: A 4-year-old boy hit by a compressed-air rifle came to our center. CT scan showed a depressed skull fracture of the occipital bone on the midline and intracranial penetration of some fragments of the pellet. CT angiography documented a compression of the superior sagittal sinus without thrombosis. Soon after hospital admission, the patient showed deterioration of the neurological status suggesting intracranial hypertension. Surgery was performed with fracture elevation, removal of metal fragments, and wound debridement. The patient had a full recovery without subsequent neurological deficits. DISCUSSION/CONCLUSION: Modern airguns may produce severe penetrating head injuries in children. Parents and physicians should be aware of the danger of NPG. Depressed skull fracture and dural venous sinus involvement can occur, and even a stable neurological condition may worsen. In such instances, a thorough preoperative assessment including vascular imaging is mandatory. The surgical risk is not negligible due to the possible injury of the sinus wall and subsequent intraoperative bleeding. This has to be weighted against potential complications of the penetrating injury, such as infection, sinus thrombosis, and intracranial hypertension. Our case suggests that prompt surgical treatment can be a safe and effective option.

Calcified Epidural Hematoma after Conservative Treatment of Acute Epidural Hematoma in the Pediatric Population: A Systematic Review.

BACKGROUND: Acute traumatic epidural hematoma (EDH) is a complication in 2-3% of pediatric head injuries. Surgery is mandatory in symptomatic cases; otherwise, conservative treatment is a valid approach, especially in the pediatric population. Ossified epidural hematomas (OEHs) have been reported in the pediatric population as a rare complication of conservative EDH management, although the exact incidence remains unknown. The progressive increase in conservative management may lead to increases in the OEH incidence over the next few years. Our study aimed to systematically review OEH incidence, management strategies, characteristics (thickness, inner/outer calcifications), complication rates, time to surgery after the EDH diagnosis, and clinical outcomes. SUMMARY: A systematic review was conducted in accordance with the PRISMA guidelines. Studies reporting diagnoses and clear descriptions of OEH after EDH in pediatric patients were considered eligible. Sixteen studies, including 18 pediatric patients aged 0-18 years, were included. Head trauma was the most common cause of OEH. Seven (38.8%) OEHs were treated less than 1 month after EDH diagnosis. Surgery was performed in 17 cases (94.44%), while 1 asymptomatic case (5.56%) was managed conservatively. KEY MESSAGES: Surgery was the most commonly used treatment for OEH. Data for conservative treatment of OEH are limited. Magnetic resonance imaging or ultrasound within the first 2 months, to check for EDH resolution, may be crucial to rule out complications in pediatric patients.

Ventriculoatrial and ventriculopleural shunts as second-line surgical treatment have equivalent revision, infection, and survival rates in paediatric hydrocephalus.

PURPOSE: Ventriculoatrial (VA) and ventriculopleural (VPL) shunts are used as alternatives when CSF diversion to the peritoneal compartment with a ventriculoperitoneal (VP) shunt is not possible. The objective of this study is to compare directly the shunt survival and complications for both procedures in this setting in children. METHODS: A retrospective analysis of 54 consecutive patients who underwent VA (36) or VPL (18) shunt insertion between January 2002 and December 2017 was conducted. RESULTS: The overall mean follow-up was 4.1 (SD 4.3) years, 2.8 (SD 4.1) for VPL and 4.7 (SD 4.4) for VA shunts, respectively (p = 0.11). Twenty-four (66.7%) patients in the VA group and 9 (50.0%) in the VPL group underwent shunt revision (p = 0.236); mean number of revisions was 2.2 (SD 3.0) and 0.94 (SD 1.4) in the VA and VPL groups (p = 0.079). Median time to failure was 8.5 (IQr 78, range 0-176) months for VA and 5.50 (IQr 36, range 0-60) for VPL shunts (log rank (Mantel-Cox) 0.832). Shunt survival at 3, 6, 12 and 30 months was 60.6, 51.5, 36.4 and 27.3%, respectively, for VA and 56.3, 43.8, 37.5 and 37.5% for VPL shunts (log rank (Mantel-Cox) test value 0.727). The infection rate was 13.8% for VA and 5.6% for VPL shunts (p = 0.358). Four patients with VPL shunts (22.2%) developed pleural effusions. Fourteen deaths (25.9%) were recorded during follow-up, 8 (22.2%) in the VA and 6 (33.3%) in the VPL group (p = 0.380); two of the deaths in the VA group were shunt-related. CONCLUSION: This study demonstrates that the outcomes of VA and VPL shunts, when used as second-line surgical treatment in paediatric hydrocephalus, were similar, as were the revision, infection and survival rates. The shorter longevity of these shunts compared with the general shunted population may reflect the complex nature of these children.

Exclusive Neurogenic Bladder and Fecal Incontinency in an Achondroplasic Child Successfully Treated with Lumbar Foraminal Decompression.

INTRODUCTION: Achondroplasia is a genetic disorder characterized by defects in the development of endochondral bone resulting in skeletal abnormalities like stenosis of the foramen magnum and of the spine, shortened limb bones, and macrocephaly. Congenital spinal stenosis is frequent and due to premature fusion of the pedicles to the laminae. CASE PRESENTATION: We report a case of neurogenic bladder and fecal incontinence due to lumbar stenosis successfully treated with L1-L5 partial laminectomy and foraminotomy in a 7-year-old achondroplasic child. DISCUSSION/CONCLUSION: To our knowledge, this is the first case report of exclusive neurogenic bladder and fecal incontinence in an achondroplasic child. Neurogenic bladder and fecal incontinence without motor impairment may be early and exclusive clinical findings of lumbar stenosis in children with achondroplasia.

Post-mortem histopathology of a pediatric brain after bilateral DBS of GPI for status dystonicus: case report and review of the literature.

PURPOSE: To investigate the effects of deep brain stimulation (DBS) electrodes on the brain of a dystonic pediatric patient submitted to bilateral DBS of the globus pallidus internus (GPI). METHODS: An 8-year-old male patient underwent bilateral DBS of GPI for status dystonicus. He died 2 months later due to multiorgan failure triggered by bacterial pneumonia. A post-mortem pathological study of the brain was done. RESULTS: At visual inspection, no grossly apparent softening, hemorrhage, or necrosis of the brain adjacent to the DBS lead tracts was detected. High-power microscopic examination of the tissue surrounding the electrode trajectories showed lymphocyte infiltration, astrocytic gliosis, microglia, macrophages, and clusters of multinucleate giant cells. Significant astrocytosis was confirmed by GFAP staining in the electrode site. The T cell lymphocyte activity was overexpressed with activated macrophages detected with CD3, CD20, CD45, and CD68 stains respectively. There was no gliosis or leukocyte infiltration away from the surgical tracks of the electrodes. CONCLUSION: This is the first post-mortem examination of a child's brain after bilateral DBS of GPI. The comparison with adult post-mortem reports showed no significant differences and confirms the safety of DBS implantation in the pediatric population too.

From above or from below? That is the question. Comparison of the supraorbital approach with the endonasal approach. A cadaveric study.

BACKGROUND: Nowadays the endoscopic endonasal approach is increasingly being used to remove craniopharyngiomas, tuberculum sellae meningiomas and other presellar and parasellar lesions and its value in anterior skull base surgery is undisputed. Herein, we assess the relative advantages, disadvantages and feasibility of the keyhole eyebrow approach and the endonasal endoscopic approach in four cadaveric heads for the removal of presellar and parasellar lesions. METHODS: We used four cadaveric heads for 12 surgical dissections. The specimens were embalmed with two different techniques. Two bilateral supraorbital endoscopic assisted approaches and one transnasal expanded endonasal approach were performed for each head. We evaluated the feasibility, maneuverability and safety of each approach. We measured the operating room obtained with each approach and the distance from the main structures we reached. RESULTS: The technical feasibility of the endoscopic endonasal transphenoidal approach and the supraorbital eyebrow approach was reproduced in all four cadaveric heads. The transnasal approach gave us a good operating field medial to the two optic nerves and the two carotid arteries anteriorly until the frontal sinus and, posteriorly, the basilar artery, the emergence of the superior cerebellar arteries and posterior cerebral arteries. After performing the supraorbital approach, we viewed a wider field of the anterior skull base and we were able to reach the ipsilateral carotid artery, the optico-carotid recess, the pituitary stalk, the lamina terminalis until the contra lateral optic nerve and carotid artery, keeping a wider angle of maneuverability. CONCLUSIONS: Although the endoscopic transnasal approach has developed in leaps and bounds in the last decade, other transcranial approaches maintain their value. The supraorbital endoscopic approach is a minimally invasive approach and seems to be optimal for those lesions wider than 2 cm in the lateral extension and for all the paramedian lesions.

Problems of reconstructive cranioplasty after traumatic brain injury in children.

Cranial repair after traumatic brain injury in children is still burdened by unsolved problems and controversial issues, mainly due to the high rate of resorption of autologous bone as well as the absence of valid alternative material to replace the autologous bone. Indeed, inert biomaterials are associated to satisfactory results in the short period but bear the continuous risk of complications related to the lack of osteointegration capacity. Biomimetic materials claiming osteoconductive properties that could balance their mechanical limits seem to allow good cranial bone reconstruction. However, these results should be confirmed in the long term and in larger series. Further complicating factors that may affect cranial reconstruction after head injury should be identified in the possible associated alterations of CSF dynamics and in difficulties to manage the traumatic skin lesion and the surgical wound, which also might impact on the cranioplasty outcome. All the abovementioned considerations should be taken into account when dealing with the cranial reconstruction after decompressive craniectomy in children.

Facial Nerve Tumors in Children: Two Clinical Cases and a Review of the Literature.

We provide an extensive review of clinical features, diagnosis, and treatment of primitive facial nerve tumors in children, and report 2 recent personal observations. We conducted a comprehensive literature search through PubMed, Medline, and ScienceDirect and collected information on patients' age, symptoms, tumor types and sites, diagnostic procedures, surgical approaches, and outcomes. Overall, we reviewed 26 pediatric cases from 20 papers. About 69.2% of children presented with some degree of facial palsy. Other symptoms included hearing loss, dizziness, and tinnitus. 84.6% of tumors were schwannomas, followed by meningiomas, epithelioid hemangioendothelioma, and germ cell tumors. The geniculate ganglion was the most commonly affected segment of the facial nerve. A total of 92.3% of children received surgery as complete or partial tumor resection. Facial nerve function improved in 26.9% of children. No tumor recurrence was reported. Facial nerve tumors are extremely rare in children but should be considered in the differential diagnosis of facial palsy, even in newborns. Audiometric and radiologic examinations are necessary; radiologic imaging allows to determine tumor localization, and the correct surgical approach surgery is suggested in almost all cases.

SMARCE1-related meningiomas: A clear example of cancer predisposing syndrome.

We report the case of a 16-year-old girl presenting with spinal clear-cell multiple meningiomas (CCMs). In view of this presentation, we sequenced a bioinformatic panel of genes associated with susceptibility to meningioma, identifying a germline heterozygous variant in SMARCE1. Somatic DNA investigations in the CCM demonstrated the deletion of the wild-type allele (loss of heterozygosity, LOH), supporting the causative role of this variant. Family segregation study detected the SMARCE1 variant in the asymptomatic father and in the asymptomatic sister who, nevertheless, presents 2 spinal lesions. Germline heterozygous loss-of-function (LoF) variants in SMARCE1, encoding a protein of the chromatin-remodeling complex SWI/SNF, have been described in few familial cases of susceptibility to meningioma, in particular the CCM subtype. Our case confirms the role of NGS in investigating predisposing genes for meningiomas (multiple or recurrent), with specific regard to SMARCE1 in case of pediatric CCM. In addition to the age of onset, the presence of familial clustering or the coexistence of multiple synchronous meningiomas also supports the role of a genetic predisposition that deserves a molecular assessment. Additionally, given the incomplete penetrance, it is of great importance to follow a specific screening or follow-up program for symptomatic and asymptomatic carriers of pathogenic variants in SMARCE1.

Pediatric neurosurgery AC-after COVID-19: What has really changed? A review of the literature.

The COVID-19 outbreak has dramatically changed the organization of Pediatric Neurosurgery all over the world. The departments involved developed similar plans to maintain emergency surgeries without reducing clinical activities. The Association of Pediatric Neurosurgeons wrote different memoranda to detail the surgical procedures not to be postponed with special attention given to high-risk pathology for COVID-19 contamination, like trans-naso-sphenoidal surgery. On this basis, we have conducted a complete literature review focusing on many topics: hospital organization, patients and parents screening, surgical indication criteria, outpatient clinic and teleconsultation, telematic conference and meeting, fellowship and training, and virtual multidisciplinary meeting.

Section of the filum terminale: is it worthwhile in Chiari type I malformation?

A section of the filum terminale (SFT) is used for the surgical treatment of isolated tethered cord or that resulting from neurulation disorders. More recently, it has been proposed for the management of the occult tethered cord syndrome (OTCS), though it is still under debate. Even more controversial appears to be the use of SFT in patients with Chiari type I malformation (CIM), which is based on the possible presence of OTCS. This review shows that: (1) there are issues both in favor and against the occurrence of OTCS, (2) there is no significant correlation between CIM and tethered cord, the old "caudal traction theory" being not supported by clinical or experimental evidences. On these grounds, a relationship between CIM and OTCS is hard to be demonstrated, (3) a subgroup of patients with CIM suffering from OTCS may exist and benefit from SFT.

History of Chiari type I malformation.

Chiari type I malformation (CIM) was first described in the late 19th Century. However, it still raises a great interest among the scientific Community because of the increasing number of diagnosed cases, the still unclear pathogenesis and natural history and the different options in the surgical management. The present review aims at analyzing the centenary history of CIM, starting from the first description done by Hans Chiari to the more recent classification, in order to introduce such a complex disease and to show the way followed for its assessment over the time.

Hydrocephalus and Chiari type I malformation.

INTRODUCTION: [corrected] Hydrocephalus has been related to Chiari type I malformation (CIM) for a long time. The pathogenesis of this association is complex and still debated. DISCUSSION: A supratentorial hypertensive hydrocephalus may cause CIM, exerting pressure from above. Another pathogenetic hypothesis is based on the clinical and radiological data from patients affected by complex craniosynostosis, in which this association is more commonly observed as the consequence of a "cephalo-cranial disproportion" ultimately leading to a secondary hydrocephalus. In some cases, the concomitant presence of a stenosis of the jugular foramina would determine a condition of upward venous hypertension, resulting in the development of CIM and an associated hydrocephalus due to cerebellar parenchyma turgor. CONCLUSIONS: The radiological association of ventricular enlargement and hindbrain herniation would be the result of heterogeneous pathogenetic mechanisms which would then require specific therapeutic approaches. In this context, the endoscopic third ventricle-cisternostomy is gaining an increasing interest because of its more physiologic correction of the altered CSF dynamics and its minor interference on the developmental processes responsible for the association of hydrocephalus and CIM.

Complete section of the left vagus nerve does not preclude the efficacy of vagus nerve stimulation: illustrative case.

BACKGROUND: Vagus nerve stimulation (VNS) represents a valid therapeutic option for patients with medically intractable seizures who are not candidates for epilepsy surgery. Even when complete section of the nerve occurs, stimulation applied cranially to the involved nerve segment does not preclude the efficacy of VNS. Complete vagus nerve section with neuroma causing definitive left vocal cord palsy has never been previously reported in the literature. OBSERVATIONS: Eight years after VNS implant, the patient experienced worsening of seizures; the interrogation of the generator revealed high impedance requiring surgical revision. On surgical exploration, complete left vagus nerve section and a neuroma were found. Vocal cord atrophy was found at immediate postoperative laryngeal inspection as a confirmation of a longstanding lesion. Both of these events might have been caused by direct nerve injury during VNS surgery, and they presented in a delayed fashion. LESSONS: VNS surgery may be complicated by direct damage to the left vagus nerve, resulting in permanent neurological deficits. A complete section of the nerve also enables an efficacious stimulation if applied cranially to the involved segment. Laryngeal examination should be routinely performed before each VNS surgery to rule out preexisting vocal cord dysfunction.

Postoperative Epileptic Seizures in Children: Is the Brain Incision a Risk Factor?

BACKGROUND: Postoperative seizures (PSs) after neurosurgical operations are common but little is known about the role of surgical brain incision on their genesis. This topic has not been addressed so far. OBJECTIVE: To verify if the corticotomy affects the risk of PSs and postoperative epilepsy (PE) in children. METHODS: One hundred forty-three consecutive pediatric cases operated on for supratentorial lesions at the same institution in the last 15 yr have been retrospectively reviewed by dividing them into group A, 68 children who required brain corticotomy mainly for hemispheric tumors, and group B, 75 children treated through extracortical approaches mainly for suprasellar and optic tumors. Patients with possible "epileptic" biases, like preoperative seizures, were excluded. RESULTS: No significant differences have been found between group A and B as far as incidence of PSs (11.7% vs 14.5%) and PE (4.5% vs 6.5%), timing, and type of seizures are concerned after a mean 6.8 yr follow-up. The size of corticotomy in group A (<3 cm2 vs >3 cm2) had no impact on epileptogenesis as well as the other variables considered in both groups (age, sex, extent of lesion resection). CONCLUSION: This study shows that the surgical cortical "trauma" would not represent a risk factor for PSs and PE. According to the present analysis and the literature, other causes seem to be involved (namely, electrolytic imbalance and brain gliosis). This information is important for preoperative surgical planning and postoperative management. A validation by both adult series and prospective studies is needed.

Endoscopic Management of Pediatric Complex Hydrocephalus.

OBJECTIVE: To define the role of neuroendoscopy as an adjuvant technique for the management of pediatric complex hydrocephalus. METHODS: We performed a retrospective analysis of a series of pediatric patients who had undergone surgery for complex hydrocephalus from January 2002 to March 2017 at 2 pediatric neurosurgery units in Rome, Italy and Mansoura, Egypt. The endoscopic procedures performed included cyst or membrane fenestration, septum pellucidotomy, foraminoplasty, and aqueductoplasty with or without a stent. In selected cases, endoscopic third ventriculostomy was performed as a combined procedure. The mean follow-up period was 5 years. RESULTS: A total of 68 patients (26 females, 42 males), with a mean age of 2 years (range, 0-18), underwent 109 endoscopic procedures. Of the 68 patients, 39 (57%) were affected by multiloculated hydrocephalus, 17 by isolated lateral ventricle (24.6%), 3 by an excluded lateral ventricle horn (4.3%), 7 by an isolated fourth ventricle (10.1%), and 1 each by an isolated third ventricle and a cavum septi pellucidi cyst. The overall postoperative complications rate was 28% (shunt infection, 16.1%; shunt malfunction, 8.8%; subdural collection, 2.9%). At the latest clinical follow-up visit, 65% of the children had only 1 shunt, 25% (n = 17) had a double ventricular catheter, and 10% (n = 7) were shunt free. CONCLUSIONS: We have confirmed that neuroendoscopy has a main role in the long-term management of complex hydrocephalus, significantly contributing to the reduction of the number of shunts and the shunt revision rate. Neuronavigation should be performed in all cases in which the ideal trajectory should be established.