Severe Hydrocephalus Research Articles

Palliative surgery in a patient diagnosed with trisomy 18 and rare neurological comorbidities

Introduction: The authors describe a case of a patient with a prenatal diagnosis of Trisomy 18 with atypical manifestation, rare anomalies of the central nervous system and subsequent surgical treatment. Case presentation: The infant was diagnosed with holoprosencephalia, schizencephalia, corpus callosum agenesis and severe hydrocephalus. No significant heart, gastrointestinal or kidney defects were found. Being in otherwise good condition, the patient was qualified for a ventriculi-peritoneal shunt surgery due to rapidly increasing head circumference. Following the uneventful operation patient’s parents reported a general improvement in their child condition. Conclusions: Bearing in mind the possibility of complications and the child’s poor prognosis, the reasons for surgical intervention were evaluated, as well as the possible outcomes of withholding invasive treatment. Discussion over the benefits of palliative surgery and the risk of ‘palliative harm’ illustrate how selected children benefit from an interventionist approach.

A case of rapid deterioration in a subacute period after endoscopic third ventriculostomy

Background: Although generally a safe procedure, serious postoperative complications after endoscopic third ventriculostomy (ETV) for obstructive hydrocephaly have been rarely reported, such as delayed obstruction of the stoma at the third ventricle floor. Case description: A 20-year-old male was referred to our department because of severe headache and diplopia. A pineal tumour and obstructive hydrocephaly were detected in preoperative imaging. After tumour biopsy and ETV, the reduction of ventricle size and improvement of headaches were immediately observed. On the seventh day, however, he developed a rapidly progressing consciousness disturbance due to severe hydrocephalus leading to urgent secondary ETV. The original ventriculostomy stoma at the third ventricle floor was completely occluded by scar adhesion. The patient recovered well as previously and received additional treatment. Conclusion: Although very rare, occlusion of the ventriculostomy stoma can postoperatively occur in the subacute period. Patients undergoing ETV for obstructive hydrocephalus due to a pineal tumour should be carefully monitored to avoid serious consequences.

Evidence for the involvement of caspases in establishing proper cerebrospinal fluid hydrodynamics

A large number of cells undergo apoptosis via caspase activation during and after neural tube closure (NTC) in mammals. Apoptosis is executed by either intrinsic or extrinsic apoptotic pathways, and inhibition of each pathway causes developmental defects around NTC stages, which hampers the physiological roles of apoptosis and caspases after NTC. We generated transgenic mice in which a broad spectrum of caspases could be suppressed in a spatiotemporal manner by pan-caspase inhibitor protein p35 originating from baculovirus. Mice with nervous system-specific expression of p35 (Nestin-Cre (NCre);p35V mice) exhibited postnatal lethality within 1 month after birth. They were born at the expected Mendelian ratio, but demonstrated severe postnatal growth retardation and hydrocephalus. The flow of cerebrospinal fluid (CSF) between the third and fourth ventricles was disturbed, whereas neither stenosis nor abnormality in ciliary morphology was observed in the pathway of CSF flow. Hydrocephalus and growth retardation of NCre;p35V mice were not rescued by the deletion of RIPK3, an essential factor for necroptosis which occurs in the absence of caspase-8 activation during development. The CSF of NCre;p35V mice contained a larger amount of secreted proteins than that of the controls. These findings suggest that the establishment of proper CSF dynamics requires caspase activity during brain development after NTC.

Treatment Outcome of Hydrocephalus Associated with Vestibular Schwannoma.

Background and PurposeManaging hydrocephalus in patients with vestibular schwannoma (VS) is controversial. We evaluated the clinical factors associated with hydrocephalus.MethodsBetween 2000 and 2019, 562 patients with VS were treated at our institute. We applied endoscopic third ventriculostomy (ETV), external ventricular drainage (EVD), and ventriculoperitoneal (VP) shunts to patients with hydrocephalus. The relationships of patient, tumor, and surgical variables with the hydrocephalus outcome were assessed.ResultsPreoperative hydrocephalus (Evans ratio ≥0.3) was present in 128 patients. Six patients who received a preresectional VP shunt were excluded after analyzing the hydrocephalus outcome. Seven of the remaining 122 patients had severe hydrocephalus (Evans ratio ≥0.4). Primary tumor resection, VP shunting, ETV, and EVD were performed in 60, 6, 57, and 5 patients, respectively. The hydrocephalus treatment failure rate was highest in the EVD group. Persistent hydrocephalus was present in five (8%) and seven (12%) patients in the primary resection and ETV groups, respectively. Multivariate analysis revealed that severe hydrocephalus, the cystic tumor, and the extent of resection (subtotal resection or partial resection) were associated with hydrocephalus treatment failure.ConclusionsLarger ventricles and a higher cystic portion are predictive of persistent hydrocephalus. We recommend attempting near-total tumor resection in patients with VS.

Erken Çocukluk Döneminde Orta ve Geç Prematüre Bebeklerin Somatik Büyüme Özelliklerinin Erken Prematüre Bebeklerle Karşılaştırılması

Objective: The aim of this study was to compare the physical growth features of preterms infants at early childhood and to evaluate the perinatal and postnatal risk factors that affect growth. Method: Somatic growth features including weight, height, and target height, of moderate-late and early preterm infants followed-up in our unit were evaluated at a mean age of 42 months. The effects of intrauterine growth characteristics (IUGC) on current growth as well as the effects of perinatal and early neonatal problems were investigated. Results: A total of 232 moderate-late preterm infants (Group I) (mean gestational age [GA]: 34.9±1.2 weeks) and 112 early preterms (Group II) (mean GA: 29.9±1.6 weeks) were evaluated. The ratio of retardation in height was 2.6 % and 6.3% in Group I and Group II, respectively. The rate of failure in achieving the target height was higher in Group II (3.9% vs 8%). Growth differences in terms of height and weight were not significantly different between the two groups (p>0.05). Severe intraventricular hemorrhage (IVH), hydrocephalus and invasive mechanical ventilation requirements negatively affected the growth in height and reaching the target height (p<0.05). Conclusion: The moderate-late and early preterm infants had similar growth features in terms of weight, height, and target height when evaluated at 42 months of age

Characteristics of cerebral hemodynamics in patients with chronic brain ischemia

Background. Literature sources report conflicting results regarding the severity of clinical symptoms of hydrocephalus (HC) and their association with cerebral arterial blood flow (CABF); most studies do not show a direct relationship, while some suggest a link between clinical severity and progressive decrease in CABF. The study of hemodynamic changes in the brain of patients with chronic brain ischemia (CBI), elucidation of their relationship with cognitive impairments helps to improve diagnostic approaches and optimize the prognosis of the disease. The purpose of this study is to investigate the state of cerebral hemodynamics and to establish its relationship with changes in cognitive functions in patients with CBI and HC. Materials and Methods. A comprehensive examination of 110 patients with CBI and HC was performed. The localization of the HC and results of Montreal Cognitive Test (MoCA scale) were taken into account. Computed tomography of the brain was performed with subsequent determination of morphometric parameters and indices. The state of cerebral blood flow and structural changes of blood vessels were studied using transcranial color-coded duplex ultrasonography (TCCS) of intracranial vessels and extracranial divisions. Microsoft Excel 2011 and Statistica were used for statistical processing of the results. Results. It was found that there was a significantly larger diameter of both common carotid arteries (CCA), thickness of complex intima\media (CIM), maximum systolic velocity (Vs), velocity at the end of the diastolic cycle (Vd), as well as peripheral resistance (IR) in left CCA (p <0,05) in patients with CBI and HC compared with patients with CBI without HC. Significantly larger diameter of left internal carotid artery (ICA), Vs, Vd and IR was revealed; Vs and Vd in the right ICA; Vd in the extracranial devision of vertebral artery (VA) in patients with HC. A significant relationship was found between the following blood flow parameters and the values of the MoCA scale: Vs MCA/MoCA (r = 0,45, p <0,05), Vs ACA/MoCA (r = 0,38, p <0.05), ICA diameter/MoCA (r = -0,51, p <0,05). It was found that the diameter of CCA and the thickness of CIM were significantly larger in patients with CBI with internal HC in comparison with patients with CBI with external HC (p <0,01). Vs and Vd in patients with internal hydrocephalus were significantly lower, and IR was significantly higher compared with patients with external hydrocephalus (p <0,01). The diameter of ICA and IR in patients with CBI with internal HC was significantly higher, and Vs was significantly lower compared with patients with CBI and external HC (p <0,01). Significantly different values were found in Vs and IR meanings in middle cerebral artery (MCA) and Vs in anterior cerebral artery (ACA) in patients with CBI with internal HC compared with patients with CBI with external HC.Conclusions. Functional parameters of blood flow and structural changes of cerebral arteries in patients with CBI with concomitant hydrocephalus differed significantly from those in patients without hydrocephalus and depended on the type and severity of hydrocephalus. ICA occlusion, ICA stenosis >50%, and intracranial venous stasis were significantly more common in patients with severe and moderate HC compared with those with mild HC. A weak relationship was found between the structure of carotid arteries and blood flow in intracranial arteries in patients with CBI and HC. There was a significant relationship between blood flow indices and the values of the MoCA scale, which indicated the effect of changes in the vessels of the anterior circular basin on the cognitive functions and the lack of such connection with blood flow indices in vertebrobasilar basin.

In Reply to the Letter to the Editor Regarding “Reversible Severe Brainstem Herniation and Obstructive Hydrocephalus from Cystoperitoneal Shunt Overdrainage”

Suprasellar arachnoid cysts are a rare but important pediatric neurosurgical pathology with unknown ideal management. They have been previously managed with techniques including open craniotomy with microsurgical fenestration, cystoperitoneal shunting, endoscopic ventriculocystostomy, and endoscopic ventriculocystocisternostomy (VCC), without a consistent consensus on the best surgical approach. We present an overview of the literature on surgical management of suprasellar arachnoid cysts.A literature search following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was conducted for all articles evaluating treatment modalities for suprasellar arachnoid cysts, using PubMed, OVID, and Web of Science.Twenty-five articles on management of suprasellar arachnoid cysts in children were identified. Few published studies exist that examine different types of surgical management across a single institution. The majority of studies reported best clinical outcomes in patients treated with endoscopic approaches when compared with microsurgical fenestration or cystoperitoneal shunting, reporting lower rates of infection, shunt dependence, and need for revision in addition to better resolution of clinical symptoms. Furthermore, most studies argue that VCC is superior to ventriculocystostomy, offering better long-term improvement of clinical symptoms and lower rates of failure.This study examines the current literature on suprasellar arachnoid cyst surgical management to conclude that an endoscopic approach in comparison with other approaches has the best outcomes. Of the endoscopic options available, VCC provides patients with the best long-term resolution of symptoms and the lowest need for revision. These findings should be further investigated with larger multicenter studies to further compare different surgical techniques and outcomes.

Pediatric hydrocephalus outcomes in Lusaka, Zambia.

Hydrocephalus is a global disease that disproportionally impacts low- and middle-income countries. Limited data are available from sub-Saharan Africa. This study aims to be the first to describe pediatric hydrocephalus epidemiology and outcomes in Lusaka, Zambia. This retrospective cohort study included patients < 18 years of age who underwent surgical treatment for hydrocephalus at Beit-CURE Hospital and the University Teaching Hospital in Lusaka, Zambia, from August 2017 to May 2019. Surgeries included ventriculoperitoneal shunt insertions, revisions, and endoscopic third ventriculostomies (ETVs) with or without choroid plexus cauterization (CPC). A descriptive analysis of patient demographics, clinical presentation, and etiologies was summarized, followed by a multivariable analysis of mortality and 90-day complications. A total of 378 patients met the inclusion criteria. The median age at first surgery was 5.5 (IQR 3.1, 12.7) months, and 51% of patients were female (n = 193). The most common presenting symptom was irritability (65%, n = 247), followed by oculomotor abnormalities (54%, n = 204). Postinfectious hydrocephalus was the predominant etiology (65%, n = 226/347), and 9% had a myelomeningocele (n = 32/347). It was the first hydrocephalus surgery for 87% (n = 309) and, of that group, 15% underwent ETV/CPC (n = 45). Severe hydrocephalus was common, with 42% of head circumferences more than 6 cm above the 97th percentile (n = 111). The median follow-up duration was 33 (IQR 4, 117) days. The complication rate was 20% (n = 76), with infection being most common (n = 29). Overall, 7% of the patients died (n = 26). Postoperative complication was significantly associated with mortality (χ2 = 81.2, p < 0.001) with infections and CSF leaks showing the strongest association (χ2 = 14.6 and 15.2, respectively, p < 0.001). On adjusted multivariable analysis, shunt revisions were more likely to have a complication than ETV/CPC or primary shunt insertions (OR 2.45 [95% CI 1.26-4.76], p = 0.008), and the presence of any postoperative complication was the only significant predictor of mortality (OR 42.9 [95% CI 12.3-149.1], p < 0.001). Pediatric postinfectious hydrocephalus is the most common etiology of hydrocephalus in Lusaka, Zambia, which is similar to other countries in sub-Saharan Africa. Most children present late with neglected hydrocephalus. Shunt revision procedures are more prone to complication than ETV/CPC or primary shunt insertion, and postoperative complications represent a significant predictor of mortality in this population.

The role of external ventricular drainage for the management of posterior cranial fossa tumours: a systematic review.

Posterior cranial fossa tumours frequently develop hydrocephalus as first presentation in up to 80% of paediatric patients and 21.4% of adults, although it resolves after tumour removal in 70-90% and 96%, respectively. New onset hydrocephalus is reported in about 2.1% of adult and 10-40% of paediatric patients after posterior fossa surgery. There is no consensus concerning prophylactic external ventricular drainage (EVD) placement that is frequently used before posterior fossa lesion removal, as well in those cases without clear evidence of hydrocephalus. The aim of the study was to define the most correct management for patients who undergo posterior fossa tumour surgery, thus identifying cohorts of patients who are at risk of persistent hydrocephalus prior to surgery. A systematic review of literature has been performed, following PRISMA guidelines. Most of the studies reported CSF shunt only in the presence of hydrocephalus, whereas only a few authors suggested its prophylactic use in the absence of signs of ventricular dilatation. Predictive factors for postoperative hydrocephalus has been identified, including young age (< 3years), severe symptomatic hydrocephalus at presentation, EVD placement before surgery, FOHR index > 0.46 and Evans index > 0.4, pseudomeningocele, CSF leak and infection. The use of pre-resection CSF shunt in case of signs and symptoms of hydrocephalus is mandatory, although it resolves in the majority of cases. As reported by several studies included in the present review, we suggest CSF shunt also in case of asymptomatic hydrocephalus, whereas it is not indicated without evidence of ventricular dilatation.

Jarcho-Levin syndrome: A case report

Introduction: Jarcho-Levin syndrome is a genetic skeletal disorder characterized by short-trunk dwarfism with vertebral and rib anomalies. The association with other organs such as the spinal, brain, and the urinary system has been reported. The paper reports a rare case of Jarcho-Levin syndrome associated with neural tube defects and other congenital malformations.Case presentation: The patient came with neglected myelomeningocele and severe hydrocephalus. Type 3 growing skull fracture complicated the management of this syndrome. This paper describes the management of JLS associated with neural tube defects as well as the severe hydrocephalus and the growing skull fracture.Conclusion: The paper reports a rare case of Jarcho-Levin syndrome associated with neural tube defects and other congenital malformations. The treatment should be carefully tailored. Keywords: Growing skull fracture, hydrocephalus, Jarcho-Levin syndrome, myelomeningocele, spondylocostal dysostosis

A novel nonsense mutation in the L1CAM gene responsible for X-linked congenital hydrocephalus.

Congenital hydrocephalus is a descriptive diagnosis of symptoms, that are present for numerous reasons, including chromosomal disorders, genetic mutations, intrauterine infection and hemorrhage, amongst other factors. Mutation of L1CAM gene is the most frequent cause of congenital hydrocephalus, contributing to approximately 30% of X-linked congenital hydrocephalus. In the present study, we used whole-exome sequencing and Sanger sequencing to investigate an aborted male fetus present with severe congenital hydrocephalus at 24 weeks of gestation, whose mother had a history of two previous voluntary terminations of pregnancies as a result of hydrocephalus. Magnetic resonance imaging, an autopsy and electron microscopy were performed and the phenotypic changes were described. Whole-exome sequencing in the fetus, as well as variant segregation analysis, revealed a novel maternally derived hemizygous nonsense mutation (c.2865G>A; p. Y955*) in exon 21 of the L1CAM gene (NM_000425.4). Severe hydrocephalus was observed along with marked dilatation of lateral ventricles. An electron micrograph of the surface of lateral ventricle walls revealed a lack of ependymal cilia. The present study suggests that L1CAM mutation screening should be considered for a male fetus with isolated hydrocephalus, especially with a family history, which could facilitate prenatal diagnosis in a subsequent pregnancy.

Neocortical tissue recovery in severe congenital obstructive hydrocephalus after intraventricular administration of bone marrow-derived mesenchymal stem cells

BackgroundIn obstructive congenital hydrocephalus, cerebrospinal fluid accumulation is associated with high intracranial pressure and the presence of periventricular edema, ischemia/hypoxia, damage of the white matter, and glial reactions in the neocortex. The viability and short time effects of a therapy based on bone marrow-derived mesenchymal stem cells (BM-MSC) have been evaluated in such pathological conditions in the hyh mouse model.MethodsBM-MSC obtained from mice expressing fluorescent mRFP1 protein were injected into the lateral ventricle of hydrocephalic hyh mice at the moment they present a very severe form of the disease. The effect of transplantation in the neocortex was compared with hydrocephalic hyh mice injected with the vehicle and non-hydrocephalic littermates. Neural cell populations and the possibility of transdifferentiation were analyzed. The possibility of a tissue recovering was investigated using 1H High-Resolution Magic Angle Spinning Nuclear Magnetic Resonance (1H HR-MAS NMR) spectroscopy, thus allowing the detection of metabolites/osmolytes related with hydrocephalus severity and outcome in the neocortex. An in vitro assay to simulate the periventricular astrocyte reaction conditions was performed using BM-MSC under high TNFα level condition. The secretome in the culture medium was analyzed in this assay.ResultsFour days after transplantation, BM-MSC were found undifferentiated and scattered into the astrocyte reaction present in the damaged neocortex white matter. Tissue rejection to the integrated BM-MSC was not detected 4 days after transplantation. Hyh mice transplanted with BM-MSC showed a reduction in the apoptosis in the periventricular neocortex walls, suggesting a neuroprotector effect of the BM-MSC in these conditions. A decrease in the levels of metabolites/osmolytes in the neocortex, such as taurine and neuroexcytotoxic glutamate, also indicated a tissue recovering. Under high TNFα level condition in vitro, BM-MSC showed an upregulation of cytokine and protein secretion that may explain homing, immunomodulation, and vascular permeability, and therefore the tissue recovering.ConclusionsBM-MSC treatment in severe congenital hydrocephalus is viable and leads to the recovery of the severe neurodegenerative conditions in the neocortex. NMR spectroscopy allows to follow-up the effects of stem cell therapy in hydrocephalus.

Effects of aging on hydrocephalus after intraventricular hemorrhage

BackgroundHydrocephalus is a common and major complication that affects outcome after intraventricular hemorrhage (IVH). While aging impacts the occurrence of hydrocephalus in patients with IVH this and the underlying mechanisms have received little attention. The present investigation, therefore, studied the impact of aging on hydrocephalus after IVH in a rat model.MethodsYoung and aged (3 and 18 months old, respectively) male Fischer 344 rats had an intraventricular injection of 200 μl autologous blood or saline. Ventricular volume was estimated using magnetic resonance imaging (MRI), while ventricular wall damage, heme oxygenase-1 (HO-1) and epiplexus cell activation were quantified by histological staining and Western blot. Additionally, the impact of intraventricular iron injection was examined in young and aged rats.ResultsIntraventricular injection of autologous blood induced hydrocephalus in both young and aged rats but ventricular volumes were larger in aged rats compared to young rats from day 3 to day 14 followed IVH. In addition, ventricular wall damage and periventricular HO-1 upregulation were greater in aged versus young rats on day 1 after IVH. Aged rats also had more choroid plexus epiplexus cells on day 14 after IVH. Additionally, organized hematomas were observed in 23% (3/13) of aged rats but not in young rats after IVH. Organized hematomas in aged rats showed larger T2* lesions on MRI compared to rats with non-organized hematomas. Similar to the effects of IVH, intraventricular injection of iron resulted in more epiplexus cells activation and more severe hydrocephalus in aged compared to young rats.ConclusionsIVH causes more severe hydrocephalus in aged compared to young rats. Enhanced ventricular wall damage, epiplexus cell activation and iron overload may contribute to this aggravated hydrocephalus development in aged animals.

Luckenschadel Associated with Chiari Type II Malformation: An Autopsy Case Report

Background Luckenschadel is commonly associated with Chiari II malformation and myelomeningocele. The aim of this case report was to revisit this entity. Case report: Antenatal imaging performed at 32 weeks gestation showed severe hydrocephalus with brain parenchymal thinning, cerebellar hypoplasia and lumbar myelomeningocele, suggestive of Chiari type II malformation. Stillbirth occurred before the pregnancy termination. X-rays showed a characteristic honeycomb appearance of the skull. The female 34-week-old stillborn was severely macerated and presented with thin and fenestrated skull, ruptured lumber myelomeningocele and diastematomyelia. Hydrocephalus could not be confirmed because of cerebral maceration. Histological examination did not reveal any significant visceral alteration. Conclusion: This case report highlights two key points. Luckenschadel should be kept in mind when Chiari II malformation is diagnosed. Autopsy with radiographic assessment is very useful in revealing this congenital defect which may escape prenatal detection.

Does pre-resection endoscopic third ventriculostomy prevent the need for post-resection CSF diversion after pediatric posterior fossa tumor excision? A historical cohort study and review of the literature.

Children with posterior fossa tumors (PFTs) may present with hydrocephalus. Persistent (or new) hydrocephalus is common after PFT resection. Endoscopic third ventriculostomy (ETV) is sometimes performed prior to resection to 1) temporize hydrocephalus prior to resection and 2) prophylactically treat post-resection hydrocephalus. The objective of this study was to establish, in a historical cohort study of pediatric patients who underwent primary craniotomy for PFT resection, whether or not pre-resection ETV prevents the need for post-resection CSF diversion to manage hydrocephalus. The authors interrogated their prospectively maintained surgical neuro-oncology database to find all primary PFT resections from a single tertiary pediatric neurosurgery unit. These data were reviewed and supplemented with data from case notes and radiological review. The modified Canadian Preoperative Prediction Rule for Hydrocephalus (mCPPRH) score was retrospectively calculated for all patients. The primary outcome was the need for any form of postoperative CSF diversion within 6 months of PFT resection (including ventriculoperitoneal shunting, ETV, external ventricular drainage [EVD], and lumbar drainage [LD]). This was considered an ETV failure in the ETV group. The secondary outcomes were time to CSF diversion, shunt dependence at 6 months, and complications of ETV. Statistical analysis was done in RStudio, with significance defined as p < 0.05. A total of 95 patients were included in the study. There were 28 patients in the ETV group and 67 in the non-ETV group. Patients in the ETV group were younger (median age 5 vs 7 years, p = 0.04) and had more severe preoperative hydrocephalus (mean frontal-occipital horn ratio 0.45 vs 0.41 in the non-ETV group, p = 0.003) and higher mCPPRH scores (mean 4.42 vs 2.66, p < 0.001). The groups were similar in terms of sex and tumor histology. The overall rate of post-resection CSF diversion of any kind (shunt, repeat ETV, LD, or EVD) in the entire cohort was 25.26%. Post-resection CSF diversion was needed in 32% of patients in the ETV group and in 22% of the patients in the non-ETV group (p > 0.05). Shunt dependence at 6 months was seen in 21% of the ETV group and 16% of the non-ETV group (p > 0.05). The median time to ETV failure was 9 days. ETV failure correlated with patients with ependymoma (p = 0.02). Children who had ETV failure had higher mCPPRH scores than the ETV success group (5.67 vs 3.84, p = 0.04). Pre-resection ETV did not reliably prevent the need for post-resection CSF diversion. ETV was more likely to fail in children with ependymoma and those with higher mCPPRH scores. Based on the findings of this study, the authors will change the practice at their institution; pre-resection ETV will now be performed based on a newly defined protocol.

Advanced abdominal pregnancy: The challenges of management

Advanced abdominal pregnancy is a rare form of ectopic pregnancy with a high morbidity and mortality rate for both mother and fetus. It is usually seen in low resource setting where antenatal care attendance is poor resulting in late presentation and often with complications. While diagnosis can present a puzzle, a high index of suspicion is important. The management is challenging especially when undiagnosed. A case of an unbooked abdominal pregnancy that presented at 31 weeks 2 days' gestational age which resulted in a live female newborn with severe hydrocephalus at a tertiary hospital in North-central Nigeria is presented.

Endoscopic third ventriculostomy in posterior fossa tumor

Posterior fossa (PF) tumors are among the most frequent primary neoplasms in children. Proximity to the fourth ventricle, and therefore cerebrospinal fluid (CSF) pathways, predisposes children with PF tumors to the development of obstructive hydrocephalus. Some patients with PF tumors require a permanent CSF diversion procedure prior to or after tumor removal. However, management of hydrocephalus in children with resectable PF tumors is still controversial. In order to minimize the use of permanent shunts and to avoid associated complications, some investigators have advocated routine preoperative application of endoscopic third ventriculostomy (ETV) only in selected patients. We showed a clinical video case who experienced a preoperatively ETV before the surgical resection of their posterior fossa tumor. The patient is a 5 year-old boy presented with progressive headaches, vomits and somnolence. Young children with severe preoperative hydrocephalus and a midline tumor should be considered at risk when preoperative treatment decisions are made. *de Oliveira RS and Ballestero MFM contributed equally to this manuscript

Automatic volumetry of cerebrospinal fluid and brain volume in severe paediatric hydrocephalus, implementation and clinical course after intervention.

In childhood hydrocephalus, both the amount of cerebrospinal fluid and the brain volume are relevant for the prognosis of the development and for therapy monitoring. Since classical planar measurements of ventricular size are subject to strong limitations, imprecise and neglect brain volume, 3D volumetry is most desirable. We used and evaluated the robust segmentation algorithms of the freely available FSL-toolbox in paediatric hydrocephalus patients before and after specific therapy. Retrospectively 76 pre- and postoperative high-resolution T2-weighted MRI sequences (true FISP, 1 mm isovoxel) were analyzed in 38 patients with paediatric hydrocephalus (mean 4.4 ± 5.1 years) who underwent surgical treatment (ventriculo-peritoneal (VP) shunt n = 22, endoscopic third ventriculostomy (ETV) n = 16). After preprocessing, the 3D-datasets were skull stripped to estimate the inner skull surface. Following, a 2 class segmentation into different tissue types (brain matter and CSF) was performed. The volumes of CSF and brain were calculated. The method could be implemented in an automated fashion in all 76 MRIs. In the VP shunt cohort, the amount of CSF (p < 0.001) decreased. Consecutively brain volume increased significantly (p < 0.001). Following ETV, CSF volume (p = 0.019) decreased significantly (p = 0.012) although the reduction was less pronounced than after shunt implantation. Brain volume expanded (p = 0.02). A reliable automated segmentation of CSF and brain could be performed with the implemented algorithm. The method was able to track changes after therapy and detected significant differences in CSF and brain volumes after shunting and after ETV.

Life without a brain: Neuroradiological and behavioral evidence of neuroplasticity necessary to sustain brain function in the face of severe hydrocephalus

A two-year old rat, R222, survived a life-time of extreme hydrocephaly affecting the size and organization of its brain. Much of the cortex was severely thinned and replaced by cerebrospinal fluid, yet R222 had normal motor function, could hear, see, smell, and respond to tactile stimulation. The hippocampus was malformed and compressed into the lower hindbrain together with the hypothalamus midbrain and pons, yet R222 showed normal spatial memory as compared to age-matched controls. BOLD MRI was used to study the reorganization of R222’s brain function showing global activation to visual, olfactory and tactile stimulation, particularly in the brainstem/cerebellum. The results are discussed in the context of neuroadaptation in the face of severe hydrocephaly and subsequent tissue loss, with an emphasis on what is the “bare minimum” for survival.

Impaired neural differentiation and glymphatic CSF flow in the Ccdc39 rat model of neonatal hydrocephalus: genetic interaction with L1cam.

ABSTRACTNeonatal hydrocephalus affects about one child per 1000 births and is a major congenital brain abnormality. We previously discovered a gene mutation within the coiled-coil domain-containing 39 (Ccdc39) gene, which causes the progressive hydrocephalus (prh) phenotype in mice due to lack of ependymal-cilia-mediated cerebrospinal fluid (CSF) flow. In this study, we used CRISPR/Cas9 to introduce the Ccdc39 gene mutation into rats, which are more suitable for imaging and surgical experiments. The Ccdc39prh/prh mutants exhibited mild ventriculomegaly at postnatal day (P)5 that progressed into severe hydrocephalus by P11 (P<0.001). After P11, macrophage and neutrophil invasion along with subarachnoid hemorrhage were observed in mutant brains showing reduced neurofilament density, hypomyelination and increased cell death signals compared with wild-type brains. Significantly more macrophages entered the brain parenchyma at P5 before hemorrhaging was noted and increased expression of a pro-inflammatory factor (monocyte chemoattractant protein-1) was found in the cortical neural and endothelial cells in the mutant brains at P11. Glymphatic-mediated CSF circulation was progressively impaired along the middle cerebral artery from P11 as mutants developed severe hydrocephalus (P<0.001). In addition, Ccdc39prh/prh mutants with L1 cell adhesion molecule (L1cam) gene mutation, which causes X-linked human congenital hydrocephalus, showed an accelerated early hydrocephalus phenotype (P<0.05-0.01). Our findings in Ccdc39prh/prh mutant rats demonstrate a possible causal role of neuroinflammation in neonatal hydrocephalus development, which involves impaired cortical development and glymphatic CSF flow. Improved understanding of inflammatory responses and the glymphatic system in neonatal hydrocephalus could lead to new therapeutic strategies for this condition.This article has an associated First Person interview with the joint first authors of the paper.