Integrated understanding of hydrocephalus — a practical approach for a complex disease

Most of childhood hydrocephalus are originating during infancy. It is considered to be a complex disease since it is developed on the basis of heterogeneous pathophysiological mechanisms and different pathological conditions as well as during different age groups. Hence, it is of relevant importance to have a practical concept in mind, how to categorize hydrocephalus to surgically better approach this disease. The current review should offer further basis of discussion on a disease still most frequently seen in Pediatric Neurosurgery. Current literature on pathophysiology and classification of pediatric hydrocephalus has been reviewed to integrate the different published concepts of hydrocephalus for pediatric neurosurgeons. The current understanding of infant and childhood hydrocephalus pathophysiology is summarized. A simplified concept based on seven factors of CSF dynamics is elaborated and discussed in the context of recent discussions. The seven factors such as pulsatility, CSF production, major CSF pathways, minor CSF pathways, CSF absorption, venous outflow, and respiration may have different relevance and may also overlap for the individual hydrocephalic condition. The surgical options available for pediatric neurosurgeons to approach hydrocephalus must be adapted to the individual condition. The heterogeneity of hydrocephalus causes mostly developing during infancy warrant a simplified overview and understanding for an everyday approach. The proposed guide may be a basis for further discussion and may serve for a more or less simple categorization to better approach hydrocephalus as a pathophysiological complex disease

Response to Letter regarding ”Motor and functional outcome of selective dorsal rhizotomy in children with spastic diplegia at 12 and 24 months of follow-up”

Studies show that psychiatric symptoms in adults and children are sometimes associated with serum neural autoantibodies. The significance of serum neural autoantibodies associated with psychiatric symptoms in children remains often unclear, but might be relevant for the extent and occurrence of psychiatric disease manifestation in later life, as well as therapy and outcome. For this narrative review, we sought articles listed in PubMed and published between 1988 and 2020 addressing the maternal-fetal transfer of neural autoantibodies and psychiatric disorders associated with serum neural autoantibodies. We identified six major subgroups of psychiatric disorders in children that are associated with serum neural autoantibodies: patients with attentional deficit hyperactivity disorder, autism spectrum disorder, obsessive compulsive disorder, Gilles de la Tourette syndrome, psychosis and catatonia. Furthermore, we summarized study findings from maternal-fetal transfer of Contactin-associated protein-like 2, N-methyl-d-aspartate receptor and fetal brain autoantibodies associated with behavioral effects in animals and humans. We hypothesize that the maternal transfer of serum neuronal autoantibodies during or after birth could result (1) in the ignition of an autoimmune-mediated inflammation having neurodevelopmental consequences for their children (autoimmune-priming-attack hypothesis) and (2) has a potential impact on the later manifestation of psychiatric disorders. Through this narrative review, we propose a diagnostic pathway for the clinical diagnosis of a potentially autoimmune origin of psychiatric symptoms in children while considering recent guidelines

Neuroendoscopic surgery in neonates — indication and results over a 10-year practice

Purpose: Neuroendoscopic procedures for treatment of term and preterm newborn infants, such as endoscopic lavage for posthemorrhagic hydrocephalus, are gaining popularity despite sparse data. This single-institution report compiles all neuroendoscopic surgical procedures performed in neonates during a 10-year period. Methods: Charts and electronic records were reviewed of all consecutive newborns who underwent a neuroendoscopic procedure before reaching a postmenstrual age of 44 weeks between 09/2010 and 09/2020. Available documentation was reviewed regarding the performed neuroendoscopic procedure, course of disease, complications, and all re-operations throughout the first year of life. Results: During the 10-year study period, 116 infants (median gestational age at birth: 29 (1)/7 weeks) underwent a total of 153 neuroendoscopic procedures (median postmenstrual age at surgery: 35 (0)/7 weeks). The most common indication at the time of the neuroendoscopic procedures (n = 153) was intraventricular hemorrhage (IVH, n = 119), intraventricular infection (n = 15), congenital malformation (n = 8), isolated 4th ventricle (n = 7), multiloculated hydrocephalus (n = 3), and tumor (n = 1). Thirty-eight of 116 children (32.8%) underwent 43 operative revisions after 153 neuroendoscopic procedure (28.1%). Observed complications requiring surgical revision were secondary infection (n = 11), CSF fistula (n = 9), shunt dysfunction (n = 8), failure of ETV (n = 6), among others. 72 children (62%) of 116 children required permanent CSF diversion via a shunt. The respective shunt rates per diagnosis were 47 of 80 (58.8%) for previously untreated IVH, 11 of 13 (84.6%) for intraventricular infection. Shunt survival rate for the first year of life was 74% for the whole cohort. Conclusion: The experience with this large cohort of neonates demonstrates the feasibility of neuroendoscopic technique for the treatment of posthemorrhagic or postinfectious hydrocephalus. Rate and type of complications after neuroendoscopic procedures were within the expected range. Assessing the potential long-term benefits of neuroendoscopic techniques has to await results of ongoing studies

Extended experience in parieto-occipital expansion surgery by meander technique—clinical and radiological evaluation

Introduction: Brachycephaly and anterior and posterior plagiocephaly appear as an isolated entity or manifest in syndromic conditions. In severe cases, possible treatment options currently comprise either cranioplasty or osteogenetic distraction. The aim of this paper is to retrospectively review the perioperative course of a series of children treated by posterior meander expansion technique at our institution with focus on the course of postoperative intracranial volume and eventual tonsillar descent evolution. Methods: Forty-two children received a posterior cranial vault remodeling by means of a posterior meander technique during a 7-year period. Hospital records were reviewed, and pre- and postoperative MRIs were analyzed for intracranial volume, cephalic and asymmetry index, and tonsillar position over time. Results: Median age at surgery was 11.5 months (range 17 days-10 years). Nineteen children had a symmetrical cranial deformity, twenty-three an asymmetrical synostosis. Half of the cohort showed a syndromic condition. Transfusions were administered in the majority (92.2%) of the cases. A significant postoperative increase of intracranial volume was present from 1188.9 ± 370.4 cm(3) to 1324.8 ± 352.9 cm(3) (p < 0.001). The asymmetry index showed a significant improvement postoperatively: 0.86 ± 0.06 versus 0.91 ± 0.05 (p < 0.001), while the cephalic index showed a non-statistical change (0.91 ± 0.11 versus 0.88 ± 0.08). Tonsillar herniation, bilateral or homolateral, showed no significant changes at early control, while a nonsignificant amelioration of tonsillar descent was seen among children older than 12 months at late imaging follow-up. Conclusion: Among the osteoplastic techniques, the posterior meander technique offers several advantages, such as early mobilization of the child, less bony defects, absence of implants, and a small complication rate. However, further comparative studies among different surgical techniques are needed

Mapping of long-term cognitive and motor deficits in pediatric cerebellar brain tumor survivors into a cerebellar white matter atlas

Purpose: Diaschisis of cerebrocerebellar loops contributes to cognitive and motor deficits in pediatric cerebellar brain tumor survivors. We used a cerebellar white matter atlas and hypothesized that lesion symptom mapping may reveal the critical lesions of cerebellar tracts. Methods: We examined 31 long-term survivors of pediatric posterior fossa tumors (13 pilocytic astrocytoma, 18 medulloblastoma). Patients underwent neuronal imaging, examination for ataxia, fine motor and cognitive function, planning abilities, and executive function. Individual consolidated cerebellar lesions were drawn manually onto patients' individual MRI and normalized into Montreal Neurologic Institute (MNI) space for further analysis with voxel-based lesion symptom mapping. Results: Lesion symptom mapping linked deficits of motor function to the superior cerebellar peduncle (SCP), deep cerebellar nuclei (interposed nucleus (IN), fastigial nucleus (FN), ventromedial dentate nucleus (DN)), and inferior vermis (VIIIa, VIIIb, IX, X). Statistical maps of deficits of intelligence and executive function mapped with minor variations to the same cerebellar structures. Conclusion: We identified lesions to the SCP next to deep cerebellar nuclei as critical for limiting both motor and cognitive function in pediatric cerebellar tumor survivors. Future strategies safeguarding motor and cognitive function will have to identify patients preoperatively at risk for damage to these critical structures and adapt multimodal therapeutic options accordingly

Biparietal meander expansion technique for sagittal suture synostosis in patients older than 1 year of age—technical note

Objective: Sagittal suture synostosis (SSS) is the most common form of craniosynostosis. For older patients, the strategy for surgical correction needs to consider diminished growth dynamics of the skull and an active reconstruction cranioplasty aims to sustain stability for the active child. We describe our technique of biparietal meander expansion (BME) technique for SSS for patients older than 1 year and retrospectively reviewed the perioperative course as well as the subjective experience of patients and caregivers during follow-up. Methods: The BME technique incorporates bilateral serpentine craniotomies and fixation of the consecutively expanded bone tongues with crossing sutures for patients with SSS older than 12 months of age at surgery. We reviewed patients undergoing this surgical technique for correction of SSS and collected data about the clinical course and performed a patients reported outcome measure (PROM) for patients or caregivers to evaluate subjective experience and outcome after surgical treatment. Results: BME was performed in 31 patients (8 females; median age: 43 months; range 13-388). The mean length of operation was 172.7 ± 43 minutes (range 115-294). Patients experienced no immediate complications or neurological morbidity after surgery. Considering a total of 21 completed PROM questionnaires, the head shape after surgery was evaluated as either "better" (57%) or "much better" (43%) compared to preoperatively. Eighty-one percent of patients or caregivers answered that the patient experiences no limitation in daily activities. Although 42.8% perceived the hospital as strenuous, 90.5% would choose to undergo this treatment again. Conclusion: BME is a feasible technique for older SSS patients resulting in immediate stability of the remodelled calvarium with a more normal head shape. The survey among caregivers or patients revealed a favourable subjectively experienced outcome after this type of surgical treatment of SSS in the more complex context of an older patient cohort

First experience with augmented reality neuronavigation in endoscopic assisted midline skull base pathologies in children

Introduction: Endoscopic skull base approaches are broadly used in modern neurosurgery. The support of neuronavigation can help to effectively target the lesion avoiding complications. In children, endoscopic-assisted skull base surgery in combination with navigation systems becomes even more important because of the morphological variability and rare diseases affecting the sellar and parasellar regions. This paper aims to analyze our first experience on augmented reality navigation in endoscopic skull base surgery in a pediatric case series. Patients and methods: A retrospective review identified seventeen endoscopic-assisted endonasal or transoral procedures performed in an interdisciplinary setting in a period between October 2011 and May 2020. In all the cases, the surgical target was a lesion in the sellar or parasellar region. Clinical conditions, MRI appearance, intraoperative conditions, postoperative MRI, possible complications, and outcomes were analyzed. Results: The mean age of our patients was 14.5 ± 2.4 years. The diagnosis varied, but craniopharyngiomas (31.2%) were mostly represented. AR navigation was experienced to be very helpful for effectively targeting the lesion and defining the intraoperative extension of the pathology. In 65% of the oncologic cases, a radical removal was proven in postoperative MRI. The mean follow-up was 89 +/- 79 months. There were no deaths in our series. No long-term complications were registered; two cerebrospinal fluid (CSF) fistulas and a secondary abscess required further surgery. Conclusion: The implementation of augmented reality to endoscopic-assisted neuronavigated procedures within the skull base was feasible and did provide relevant information directly in the endoscopic field of view and was experienced to be useful in the pediatric cases, where anatomical variability and rarity of the pathologies make surgery more challenging

Next generation of ventricular catheters for hydrocephalus based on parametric design

Background The flow pattern of the cerebrospinal fluid is probably the most important factor related to obstruction of ventricular catheters during the normal treatment of hydro cephalus. To better comprehend the flow pattern, we have carried out a parametric study via numerical models of ven tricular catheters. In previous studies, the flow was studied under steady and, recently, in pulsatile boundary conditions by means of computational fluid dynamics (CFD) in three dimensional catheter models. Objective This study aimed to bring in prototype models of catheter CFD flow solutions as well to introduce the theory behind parametric development of ventricular catheters. Methods A preceding study allowed deriving basic principles which lead to designs with improved flow patterns of ventric ular catheters. The parameters chosen were the number of drainage segments, the distances between them, the number and diameter of the holes on each segment, as well as their relative angular position. Results CFD results of previously unreleased models of ven tricular catheter flow solutions are presented in this study. Parametric development guided new designs with better flow distribution while lowering the shear stress of the catheters holes. High-resolution 3D printed catheter solutions of three models and basic benchmark testing are introduced as well. Conclusions The next generation of catheter with homoge neous flow patterns based on parametric designs may repre sent a step forward for the treatment of hydrocephalus, by possibly broadening their lifespan

Efficacy of endoscopic third ventriculostomy in old aged patients with normal pressure hydrocephalus

Normal pressure hydrocephalus (NPH) is a chronic disorder caused by interrupted CSF absorption or flow. Generally, shunt placement is first option for NPH treatment. Due to complications of ventriculo-peritoneal (VP) shunt placement, endoscopic third ventriculostomy (ETV) can be considered as an alternative treatment option. Here we report the efficacy of ETV especially in old aged patients with normal pressure hydrocephalus. Total 21 old aged patients with communicating hydrocephalus with opening pressure, measured via lumbar puncture, less than 20cm H2O underwent ETV. 15 patients had primary/idiopathic NPH and 6 patients had secondary NPH. All patients were studied with a MRI to observe the flow void at aqueduct and the fourth ventricle outflow. And all of them underwent ETV. In a group with peak velocity was higher than 5cm/s, nine patients (75%) were evaluated was ‘favorable’ and three of them (25%) was scored ‘poor’. In another group with peak velocity less than 5cm/s, three of them were scored ‘poor’ and two of them were scored ‘stable’. None of them was evaluated as ‘favorable’. We also evaluated the outcomes according to etiology: 12 patients (80% of the patients with primary NPH) were evaluated with ‘favorable’ after ETV treatment. Two patients (13.3%) were as ‘stable’. And one patient was as ‘poor’ evaluated. Five patients (83.3%) among patients with secondary NPH were as ‘poor’ evaluated and one of them was stable and no patient was as ‘favorable’ evaluated. 4 patients, which was as ‘poor’ evaluated in the group with the secondary NPH, underwent additional VP shunt implantation. Overall, the outcomes of the group with the idiopathic NPH after ETV treatment were more favorable than of the group with the secondary NPH. Our study suggest that ETV can be effective for selected elderly patients with primary/idiopathic NPH, when they satisfy criteria including positive aqueduct flow void on T2 Sagittal MRI and the aqueductal peak velocity, which is greater than 5cm/s on cine MRI