Search

We demonstrate a case report of triventricular hydrocephalus due to an aqueductal web and stenosis which presented itself clinically solely with bilateral hand tremors in an adolescent male. The patient underwent Endoscopic third ventriculostomy (ETV) and the subsequent improvement in cerebrospinal fluid (CSF) flow resulted in complete resolution of his tremor. We propose a mechanism involving compression of the rubrospinal tract (or stretching of the frontal premotor area) and advise cranial imaging in cases of hand tremor to exclude this as a potential cause. Neurosurgical review and potential CSF diversion if triventriclar hydrocephalus is established should be considered as positive clinical outcome can be achieved. [ABSTRACT FROM AUTHOR]

Background: The application of endoscopic third ventriculostomy (ETV) for the treatment of obstructive hydrocephalus in shunt malfunction represents a paradigm shift, as it allows hydrocephalus to be transformed from a chronic condition treated with an artificial device to a curable disease. Methods: We present a 54-year-old male with a diagnosis of idiopathic Sylvian aqueduct stenosis treated with shunt. The patient presented to our institution with symptoms of shunt malfunction and an increase in ventricular size on imaging, which was his third episode throughout his life. Through a right precoronal approach, with prior informed consent from the patient, we performed foraminoplasty, endoscopic third ventriculostomy, and finally removal of the shunt system. Conclusion: ETV shows promise as a viable treatment option for shunt malfunction in noncommunicating obstructive hydrocephalic patients. Its potential to avoid VPS-related complications, preserve physiological CSF circulation, and provide an alternative drainage pathway warrants further investigation. [ABSTRACT FROM AUTHOR]

Long-standing overt ventriculomegaly in adults (LOVA) is a heterogenous group of conditions with differing presentations. Few studies have evaluated success rates of available surgical treatments, or ascertained the natural history. There is a need to assess the efficacy of both endoscopic third ventriculostomy (ETV) and ventriculoperitoneal shunt (VPS) as first-line treatments. We conducted a retrospective, single-centre study of adults with LOVA at a tertiary neurosurgery centre in England, UK, aiming to identify presentation, management strategy, and outcome following treatment. A total of 127 patients were included (mean age 48.1 years, 61/127 male). Most patients were symptomatic (73.2%, n = 93/127, median symptom duration 10 months). The most common symptoms were gait ataxia, headache, and cognitive decline (52.8%, 50.4%, and 33.9%, respectively). Fourteen patients had papilloedema. Ninety-one patients (71.7%) underwent surgery (84 ETV, 7 VPS). Over a median follow-up of 33.0 months (interquartile range [IQR] 19.0–65.7), 82.4% had a clinical improvement after surgery, and 81.3% had radiological improvement. Clinical improvement rates were similar between ETV and VP shunt groups (82.1% vs 85.7%, p = 0.812). Surgical complication rates were significantly lower in the ETV group than the VP shunt group (4.8% vs 42.9%, p < 0.001). Of the patients treated surgically, 20 (22.0%) underwent further surgery, with 14 patients improving. This study demonstrates the efficacy of ETV as a first-line treatment for LOVA. [ABSTRACT FROM AUTHOR]

Background: The study carried out to assess long-term outcomes of endoscopic third ventriculostomy (ETV) on less than two years of hydrocephalous children and to determine prognostic factors of ETV success in this population. Methods: our study was a retrospective cohort study carried out on 40 hydrocephalus children who underwent ETV surgery. Clinical data retrieved from patient’s medical records, and we did an active follow-up. We used Kaplan-Meier and life-table approaches to assess 6 months success rate of ETV. We also used multiple cox regression to determine prognostic factors associated with success. Moreover, the ROC curve analysis was used to assess how ETV success score (ETVSS) can predict the possibility of ETV failure. Results: The mean age of patients at surgery time was 7.8 (±7.8) months, and 60% were male. The mean failure time of ETV was 4.5 months, and the most common etiology was aqueduct (27.0%). According to our finding age at surgery time (hazard ratio [HR]=2.2, 95% CI=1.1, 3.4) and birth age (HR=4.4, 95% CI 1.1, 17.2) were the main factors associated with ETV failure. We also observed, statistically lower HR for patients with aqueduct etiology (HR=0.1, 95% CI=0.01, 0.9). Moreover, the area under the ROC curve was estimated at 76.1, illustrating partial validity to predict ETV failure in the study population. Conclusion: According to our findings, ETV could suggest treating Hydrocephaly in young kids with particular attention on over 6-month patients and those who were mature at birth. However, more studies are required to confirm our findings.

• The triad of gait disturbance, incontinence, and dementia in idiopathic normal pressure hydrocephalus (iNPH) can also be observed in hydrocephalus occlusus (HO). • There is no similar scale in HO research to assess neurological symptoms. • Clinical signs of HO are analyzed with the new iNPH Scale. • The presented iNPH Scale evaluates neurological symptoms and surgical outcome in HO. Comprehensively describe and compare (pre⁄postoperatively) the clinical symptomatology in adult non-communicated hydrocephalus. Associated hydrocephalus signs were analyzed with the idiopathic Normal Pressure Hydrocephalus Scale (iNPH Scale). A standardized clinical scale for non-communicated hydrocephalus is currently not in use. Ten patients with hydrocephalus occlusus (HO) were analyzed. Hydrocephalus signs were examined with the iNPH Scale in gait, neuropsychology, continence, and balance before and three months after treatment with shunt operation or third endoscopic ventriculostomy. Patients significantly improved in iNPH total score (25.8%) and gait score (35.4%) three months after neurosurgical intervention. Domain scores in neuropsychology, continence, and balance reached statistical trends (p ≤ 0.066). Most clinical symptoms and signs at baseline improved after surgery (dizziness, lapse of concentration, gait instability, and headache). Patients with non-communicated HO also showed classical hydrocephalus symptoms as communicated in iNPH patients. The iNPH Scale allows a structured neurological assessment over the disease's progress and surgical intervention. Further studies with a larger patient samples are necessary to support our results. [ABSTRACT FROM AUTHOR]

Aqueduct stenosis is a recognized cause of obstructive hydrocephalus in children and can be treated effectively with endoscopic third ventriculostomy. Preoperative magnetic resonance imaging is often diagnostic of the cause of aqueduct stenosis. We describe 2 pediatric cases with obstructive hydrocephalus secondary to a working diagnosis of idiopathic aqueduct stenosis. Following successful endoscopic third ventriculostomy, repeat magnetic resonance brain imaging revealed tectal plate glioma as the primary cause of obstruction. We believe these 2 reported cases demonstrate a previously unreported phenomenon whereby concealed tectal gliomas presenting with hydrocephalus are only unmasked following relief of hydrocephalus and decompression and normalization of the ventricular system. We aim to raise awareness about this unusual phenomenon and recommend routine postoperative interval imaging following endoscopic third ventriculostomy to avoid missing underlying pathology masquerading as aqueduct stenosis. [ABSTRACT FROM AUTHOR]

We present the case of a patient with severe obstructive sleep apnoea (OSA) and hypoventilation syndrome who had hydrocephalus and acquired aqueduct stenosis. A link between these conditions in our patient is postulated. We discuss the mechanisms through which this might have occurred and the potential problems which might arise in applying non-invasive ventilation to a patient with hydrocephalus.

Purpose: Age and etiology play a crucial role in success of endoscopic third ventriculostomy (ETV) as a treatment of obstructive hydrocephalus. Outcome is worse in infants, and controversies still exist whether ETV is superior to shunt placement. We retrospectively analyzed 70 patients below 2 years from 4 different centers treated with ETV and assessed success. Methods: Children < 2 years who received an ETV within 1994–2018 were included. Patients were classified according to age and etiology; < 3, 4–12, and 13–24 months, etiologically; aqueductal stenosis, post-hemorrhagic-hydrocephalus (PHH), tumor-related, fourth ventricle outflow obstruction, with Chiari-type II and following CSF infection. We investigated statistically the predictors for ETV success through computing Kaplan-Meier estimates using patient's follow-up time and time to ETV failure. Results: We collected 70 patients. ETV success rate was 41.4%. The highest rate was in tumor-related hydrocephalus and fourth ventricle outlet obstruction (62.5%, 60%) and the lowest rate was in Chiari-type II and following infection (16.7%, 0%). The below 3 months age group showed relatively lower success rate (33.3%) in comparison to older groups which showed similar results (46.4%, 46.6%). Statistically, a previous VP shunt was a predictor for failure (p value < 0.05). Conclusion: Factors suggesting a high possibility of failure were age < 3 months and etiology such as Chiari-type II or following infection. Altered CSF dynamics in patients with PHH and under-developed arachnoid villi may play a role in ETV failure. We do not recommend ETV as first line in children < 3 months of age or in case of Chiari II or following infection. [ABSTRACT FROM AUTHOR]

Wiedemann–Steiner syndrome (WWS) is a rare disorder characterized by hypotonia, postnatal growth restriction, striking facial dysmorphism, and hirsutism. It is caused by heterozygous pathogenic variants in KMT2A. This gene has an established role in histone methylation, which explains the overlap of WWS with syndromes caused by genes involved in chromatin remodeling. We describe an infant with a novel single base pair deletion in KMT2A with features consistent with WWS, as well as additional features of stenosis of aqueduct of Sylvius and broad toes. The usefulness of Face2Gene as a tool for identification of dysmorphology syndromes is discussed, as in this patient, it suggested WWS as the top candidate disorder. To the best of our knowledge, this is the first patient of WWS reported from India, with a novel genotype and expanded phenotype. [ABSTRACT FROM AUTHOR]

Background: Recently, most cases of hydrocephalus are related to obstruction. Accurate localization of the site of obstruction is crucial in determination of the treatment strategy.Purpose: To describe the phase-contrast and 3D-DRIVE findings in cases of obstructive hydrocephalus in paediatric patients and to determine their functional and anatomical correlates.Material and methods: Brain MRIs of 25 patients (2 months to 11 years) with obstructive hydrocephalus were retrospectively reviewed. Phase-contrast and 3D-DRIVE were performed to assess cerebrospinal (CSF) pathways through the aqueduct of Sylvius and subarachnoid spaces. In addition to flow velocity measurement at the aqueduct of Sylvius, functional and anatomical correlation was analysed at the level of aqueduct of Sylvius, infracerebellar CSF space and at the third ventriculostomy using Spearman’s rank test.Results: Aqueduct of Sylvius was the most common site of obstruction (19 patients) either secondary to focal, multifocal or tubular stenosis, adhesions, or secondary to extrinsic compression. Functional and anatomical correlation was analysed in 58 regions revealing strong correlation (ro = 0.8, p < .001). Functional anatomical mismatch was found in nine regions. Flow velocity measurements revealed diminished flow in most of the cases with obstruction at the aqueduct and normal velocity in cases with obstruction proximal to aqueductal level, while accelerated flow was seen in cases with infra-aqeuductal obstruction.Conclusion: Phase-contrast and 3D-DRIVE sequences are essential sequences in the diagnosis of hydrocephalus enabling perfect localization of the site of obstruction. Both sequences should be interpreted in conjunction to avoid false results. Velocity measurements through the aqueduct can help understand CSF hydrodynamics. [ABSTRACT FROM AUTHOR]

The SMARCC1 gene has been involved in congenital ventriculomegaly with aqueduct stenosis but only a few patients have been reported so far, with no antenatal cases, and it is currently not annotated as a morbid gene in OMIM nor in the Human Phenotype Ontology. Most of the reported variants are loss of function (LoF) and are often inherited from unaffected parents. SMARCC1 encodes a subunit of the mSWI/SNF complex and affects the chromatin structure and expression of several genes. Here, we report the two first antenatal cases of SMARCC1 LoF variants detected by Whole Genome Sequencing (WGS). Ventriculomegaly is the common feature in those fetuses. Both identified variants are inherited from a healthy parent, which supports the reported incomplete penetrance of this gene. This makes the identification of this condition in WGS as well as the genetic counseling challenging. [ABSTRACT FROM AUTHOR]

The purpose of this study is to evaluate the changing epidemiology of paediatric hydrocephalus over the past three decades in a single institution. All children treated for newly diagnosed hydrocephalus during the 1985–1990 (group A) and the 2000–2005 periods (group B) were enrolled and classified according to the associated cause of hydrocephalus. A significant 8.8% decrease of the incidence of hydrocephalus was noticed between the two time periods, resulting from the reduction of hydrocephalus associated to myelomeningocele, aqueduct stenosis ( p = 0.04), CNS infection ( p = 0.03), cranio-cerebral malformation and head injuries; post-haemorrhagic hydrocephalus remained stable, while the tumour-associated one increased ( p < 0.0001). No consistent differences in terms of rate of adjunctive surgery (30.3% versus 23.9%) and number revision procedures (200 versus 104) were recorded. The present study confirmed data from the literature about the declined incidence of paediatric hydrocephalus, which mainly results from the decrease of congenital malformations. In spite of the recent advances in neuroendoscopy and in the shunting valve design, the impact of hydrocephalus in the paediatric neurosurgical practice remains high. [ABSTRACT FROM AUTHOR]

This research uses a novel coupling of poroelastic theory and computational fluid dynamics to investigate acute hydrocephalus resulting from stenosis of the cerebral aqueduct. By coupling poroelastic theory with a multidimensional simulation of the cerebral aqueduct we are able to investigate, for the first time, the impact of physically relevant stenosis patterns on ventricular enlargement, accounting for the nonintuitive long time history responses of the ventricular system. Preliminary findings demonstrate clearly the importance that the fluidic-poroelastic coupling plays: ventricular enlargement is significantly smaller with local stenosis patterns and almost all of the observable pressure drop occurs across the stenosis. Short timescale effects [O(heartbeat)] are explored and their contribution to the long timescales interrogated. [ABSTRACT FROM AUTHOR]

Abstract: Delayed pneumocephalus is a rare but well-reported complication of cerebrospinal fluid diversion procedures. In most cases the air enters the intracranial cavity via a skull base defect. We report a case of hydrocephalus secondary to aqueduct stenosis. The patient developed pneumocephalus 2 months after successful placement of a ventriculoperitoneal shunt. We describe an attempt at endoscopic diagnosis and repair of the fistula. This was unsuccessful, presumably because the defect was too small to localize even with the use of intrathecal fluorescein. We subsequently performed a conventional craniotomy and anterior fossa repair with placement of an antisiphon device. We suggest that in certain cases, when patients present with long-standing hydrocephalus, it may be advisable to insert either a high-pressure valve or antisiphon device as a primary measure. [Copyright &y& Elsevier]

A tectal glioma presenting with late-onset aqueduct stenosis and obstructive hydrocephalus is usually categorized as a benign glioma. Apparent clinical or radiological progression justifies biopsy of the tumour. In this case, an unusual tumour shows aggressive and invasive growth after surgical intervention and chemoradiotherapy. [ABSTRACT FROM AUTHOR]

Discusses neurosurgical concepts of idiopathic normal pressure hydrocephalus (NPH). Endoscopic third ventriculostomy; Aqueduct stenosis; Clinical features; Heterogeneity of NPH; Lack of gold standard test; Similarity of NPH to other dementias.

Stenosis of the cerebral aqueduct seems to be a key event for the development of congenital hydrocephalus. The causes of such a stenosis are not well known. Overholser et al. in 1954 (Anat Rec 120:917–933) proposed the hypothesis that a dysfunction of the subcommissural organ (SCO) leads to aqueductal stenosis and congenital hydrocephalus. The SCO is a brain gland, located at the entrance of the cerebral aqueduct, that secretes glycoproteins into the cerebrospinal fluid that, upon release, assemble into a fibrous structure known as Reissner's fiber (RF). By the permanent addition of new molecules to its rostral end, RF grows and extends along the aqueduct, fourth ventricle, and central canal of the spinal cord. The immunological blockage of the SCO-RF complex has been used to test Overholser's hypothesis. The following was the sequence of events occurring in pregnant rats that had been immunized with RF glycoproteins: the mother produced anti-RF antibodies and transferred them to the fetus through the placenta and to the pup through the milk, and the antibodies reached the brain of the fetus and pup and blocked the SCO-RF complex. This resulted in a permanent absence of RF that was followed by stenosis of the cerebral aqueduct, and then by the appearance of hydrocephalus. The latter was patent until the end of the 6-month observation period. The chronic hydrocephalic state appeared, in turn, to induce new alterations of the SCO. It is concluded that a selective immunological knock out of the SCO-RF complex leads to hydrocephalus. [ABSTRACT FROM AUTHOR]

As the craniospinal space is a pressure loaded system it is difficult to conceptualize and understand the flow dynamics through the ventricular system. Aqueduct stenosis compromises flow, increasing the pressure required to move cerebrospinal fluid (CSF) through the ventricles. Under normal circumstances, less than one pascal (1 Pa) of pressure is required to move a physiological flow of CSF through the aqueduct. This is too small to measure using clinical pressure transducers. A computational fluid dynamics (CFD) program, CFX, has been used to model two forms of aqueduct stenosis: simple narrowing and forking of the aqueduct. This study shows that with mild stenoses, the increase in pressure required to drive flow becomes significant (86-125 Pa), which may result in an increased transmantle pressure difference but not necessarily an increased intraventricular pressure. Severe stenoses will result in both. Wall shear stresses increase concomitantly and may contribute to local damage of the aqueduct wall and further gliosis with narrowing. [ABSTRACT FROM AUTHOR]

Two cases are reported in which acute paraplegia developed subsequent to decompressive intracranial operations in patients with raised intracranial pressure and spinal cord tumours. Possible causative factors are discussed. [ABSTRACT FROM AUTHOR]

Fifteen infants with hydrocephalus ranging in age from 32 to 43 weeks from conception were studied. The auditory brainstem response (ABR) was measured 48 h prior to the placement of a CSF shunt and within 5 days following shunt insertion. Results of this study showed a general improvement in the ABR following placement of the shunt. No consistent patterns were observed that allowed a clear explanation of the cause and effect of the abnormal ABR. However, the changes seen in the ABR are caused by increased CSF pressure, which may compress the transmission fibers, and generators of the ABR producing a type of neuropraxis. Early shunting appears to have a better outcome on the ABR than later shunting. [ABSTRACT FROM AUTHOR]

Various ways in which blockage of cerebrospinal fluid pathways can lead to increasing intracranial pressure and enlargement of the ventricles are discussed. The necessity of understanding the valvular morphology involved is stressed as fundamental to the search for optimal treatment methods. [ABSTRACT FROM AUTHOR]

We present the case of a patient with severe obstructive sleep apnoea (OSA) and hypoventilation syndrome who had hydrocephalus and acquired aqueduct stenosis. A link between these conditions in our patient is postulated. We discuss the mechanisms through which this might have occurred and the potential problems which might arise in applying non-invasive ventilation to a patient with hydrocephalus., Learning Points: Patients with severe obstructive sleep apnoea (OSA) and hypoventilation require AVAPS-AE ventilation to deal with obstructive and central events which may occur.Arterial blood gas analysis and MRI of the brain are indicated in the assessment of patients with severe OSA.Hydrocephalus may develop in patients with severe OSA if there is a structural brain abnormality but may resolve with OSA treatment., Competing Interests: Conflicts of Interests: The Authors declare that there are no competing interests., (© EFIM 2021.)