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Background: Prenatal diagnoses of cystic malformations of the posterior fossa mainly encompass arachnoid cysts, Blake's pouch cysts and Dandy-Walker syndrome. To date, vermian cysts have not been reported prenatally. Objectives: To report a series of fetuses with a vermian cyst. Materials and methods: This was a single-center retrospective study conducted from 2012 to 2021. We included all fetuses presenting with a vermian cyst and excluded all other types of posterior fossa cyst. The cyst was visible at prenatal ultrasound (US) and/or magnetic resonance imaging (MRI). Postnatal imaging and/or clinical outcome data were available. Results: Sixteen fetuses fulfilled the inclusion criteria with a strong female predominance (n=13). US and MRI were performed at a mean gestational age of 29+5 and 33+1 weeks, respectively. In all patients, the cyst was in the vermian horizontal fissure. The mean longest dimension was about 10 mm. The vermis and other posterior fossa structures were otherwise normal. At postnatal imaging, 13 children underwent brain imaging including 11 MRIs with complete regression (n=9), stability (n=1) and increase in size (n=3) of the cyst. Psychomotor development was normal in 14 children. One child (with an inner ear malformation) showed a slight delay in walking and language acquisition. Slight walking ataxia was present in another child. Conclusion: We report 16 fetuses with posterior fossa cysts located within the vermis at the level of the horizontal fissure, diagnosed at US and/or MRI and carrying an overall excellent neurological prognosis. [ABSTRACT FROM AUTHOR]

Introduction: One of the most important unanswered questions in pediatric hydrocephalus is determining whether treatment with endoscopic third ventriculostomy (ETV) versus shunt results in improved health status and quality of life (QOL). To answer this, the International Infant Hydrocephalus Study (IIHS) was started in 2005 as a prospective, multicenter study to compare ETV and shunt in infants (< 24 months old) with symptomatic triventricular hydrocephalus from aqueductal stenosis. Herein, we present the 5-year primary outcome results.Methods: IIHS utilized a prospective comprehensive cohort design, in which patients received ETV or shunt, based on either randomization or parental preference. For this analysis, we pooled the randomized arm and the parental preference arm, analyzing them together. At 5 years of age, children were assessed with the Health Utilities Index Mark 2 (HUI-2) (primary outcome) and the Hydrocephalus Outcome Questionnaire (HOQ), a measure of QOL. Results were compared in an analysis of covariance, adjusting for baseline variables including age at surgery and baseline development status.Results: From a total of 158 patients who met eligibility criteria, complete 5-year outcomes were available on 78 (19 treated initially with shunt, 61 treated initially with ETV), assessed at a mean age of 62.1 months (SD 6.3). The mean 5-year HUI-2 utility score was 0.90 (SD 0.19) for ETV and 0.94 (SD 0.10) for shunt (p = 0.21). The mean 5-year HOQ overall score was 0.81 (SD 0.15) for ETV and 0.85 (SD 0.12) for shunt (p = 0.42). Similarly, there were no significant differences noted between 5-year HOQ subscores (cognitive, social-emotional, physical) or developmental measures at 1, 2, and 3 years.Conclusions: This is the first prospective direct comparison of long-term outcomes of ETV and shunt for infant hydrocephalus. These results suggest that overall health status and quality of life in this cohort of infants treated for aqueductal stenosis are high, with no significant difference between those treated initially with ETV or shunt.Trial registration: NCT00652470 [ABSTRACT FROM AUTHOR]

Purpose: Dandy-Walker malformation is a rare condition due to imperforation of the Blake’s pouch during intrauterine brain development, usually leading to early severe hydrocephalus. The association with holocord syringomyelia is rare, and from the Gardner’s first report in 1957, only 23 cases have been described, mostly from autopsy series and pre-MRI period. Besides a worsening of clinical picture, its occurrence generates some concern about the best surgical treatment that varies widely among the literature reports.Methods: An 11-year-old girl with Dandy-Walker malformation presented with a holocord syrinx due to the herniation of the lower pole of the posterior fossa cyst through the foramen magnum.Results: After an unsuccessful shunt revision, she underwent a cystoperitoneal shunt with regression of the syrinx and of neurological symptoms at the 12-month follow-up.Conclusions: Previous literature about pathogenesis, treatment, and follow-up is discussed and summarized. [ABSTRACT FROM AUTHOR]

Objective: To assess the outcome and efficacy of cochlear implantation in children with genetic syndromes.Method: Study design: case–control study.Setting: A cochlear implantation tertiary referral center.Patients: All pediatric cochlear implantation recipients with Waardenburg syndrome, Usher syndrome, Dandy–Walker syndrome, or albinism. A control group was appropriately matched to the syndromic group with regard to age at implantation and duration of device use.Intervention: Cochlear implantation.Main outcome measures: Subjects’ auditory abilities, speech intelligibility, and pure tone thresholds were compared between the syndromic and non-syndromic group.Results: A total of 25 subjects (13 syndromic and 12 non-syndromic) participated in the study. Neither auditory ability nor speech intelligibility scores differed significantly by group. The final PTA of both the groups showed normal-to-mild hearing loss: 26 dB HL in the syndromic group and 23 dB HL for the control group.Conclusions: Cochlear implant recipients with genetic syndromes achieved similar levels auditory perception and speech intelligibility as their peers with a genetic syndrome. The presence of any of the genetic syndromes described herein should not be a contraindication to cochlear implant provision, as it would have a positive impact on the patients’ sensory perception and lifestyle. [ABSTRACT FROM AUTHOR]

Background: Idiopathic normal pressure hydrocephalus (INPH) has no reliable biomarker to assist in the selection of patients who could benefit from ventriculo-peritoneal (VP) shunt insertion. The neurodegenerative markers T-tau and Aβ1-42 have been found to successfully differentiate between Alzheimer's disease (AD) and INPH and therefore are candidate biomarkers for prognosis and shunt response in INPH. The aim of this study was to test the predictive value of cerebrospinal fluid (CSF) T-tau and Aβ1-42 for shunt responsiveness. In particular, we pay attention to the subset of INPH patients with raised T-tau, who are often expected to be poor surgical candidates. Methods: Single-centre retrospective analysis of probable INPH patients with CSF samples collected from 2006 to 2016. Index test: CSF levels of T-tau and Aβ1-42. Reference standard: postoperative outcome. ROC analysis assessed the predictive value. Results: A total of 144 CSF samples from INPH patients were analysed. Lumbar T-tau was a good predictor of post-operative mobility (AUROC 0.80). The majority of patients with a co-existing neurodegenerative disease responded well, including those with high T-tau levels. Conclusion: INPH patients tended to exhibit low levels of CSF T-tau, and this can be a good predictor outcome. However levels are highly variable between individuals. Raised T-tau and being shunt-responsive are not mutually exclusive, and such patients ought not necessarily be excluded from having a VP shunt. A combined panel of markers may be a more specific method for aiding selection of patients for VP shunt insertion. This is the most comprehensive presentation of CSF samples from INPH patients to date, thus providing further reference values to the current literature. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this review is to comprehensively review the foramina of Luschka in regard to their discovery, embryology, anatomy, and surgical relevance. Methods: This subject review of the foramina of Luschka was composed after conducting a thorough review of the available literature on this topic using PubMed and other primary sources. Results: The foramina of Luschka are paired apertures located in the lateral recesses of the fourth ventricle, within the posterior cranial fossa. The foramina of Luschka are of importance clinically as their blockage can disrupt the flow of cerebrospinal fluid leading to the development of hydrocephalus. These apertures were first described by the German anatomist Hubert von Luschka in the nineteenth century. These foramina are thought to emerge at around the 26th week of development. The apertures project into the cerebellopontine angle at the lateral border of the pontomedullary sulcus. Conclusion: The foramina of Luschka may be surgically manipulated for the excision of tumors involving the fourth ventricle and surrounding structures and accessed for cochlear nucleus stimulation. [ABSTRACT FROM AUTHOR]

Introduction: Dandy-Walker malformation (DWM) is a congenital brain anomaly characterized by dysgenesis of the cerebellar vermis and the presence of a posterior fossa cyst. The association of syringomyelia with DWM is extremely rare. Case report: A 10-year-old patient who was diagnosed with DWM in infancy presented with progressive scoliosis and fecal incontinence. He had been treated with cystoventriculoperitoneal shunting with a Y-connection during infancy, which was followed by a revision 6 years later. During the revision surgery, intraventricular bleeding occurred and was managed conservatively. Imaging studies for the current visit revealed syringomyelia along the cervicothoracic spinal cord and a membranous structure around the cervicomedullary junction. Phase-contrast cine magnetic resonance imaging (MRI) revealed disturbed cerebrospinal fluid (CSF) flow across the membrane. We excised the arachnoid web that was tethering the brainstem and blocking CSF flow. Postoperatively, the patient experienced symptom relief, and the follow-up imaging study demonstrated a dramatic decrease in the size of the syringomyelia. Discussion: We suggest that syrinx formation in this patient was possibly caused by disturbed CSF flow and tethering of the brainstem. Conclusion: We experienced an unusual case of DWM with syringomyelia which was caused by an arachnoid web blocking CSF flow and tethering the brainstem. The arachnoid web seems to be formed by previous bleeding which occurred at the time of shunt revision. After excision of the arachnoid web, the patient showed good outcome. [ABSTRACT FROM AUTHOR]

Purpose: Endoscopic third ventriculostomy/choroid plexus cauterization (ETV/CPC) has gained popularity in its treatment of infantile hydrocephalus over the past decade. In this manuscript, we perform a systematic review and meta-analysis to determine the efficacy and safety of ETV/CPC, and to compare the procedural outcomes between North American and sub-Saharan African cohorts. Methods: Systematic review was performed using four electronic databases and bibliographies of relevant articles, with no language or date restrictions. Cohort studies of participants undergoing ETV/CPC that reported outcome were included using MOOSE guidelines. The outcome was time to repeat CSF diversion or death. Forest plots were created for pooled mean and its 95 % CI of outcome and morbidity. Results: Of 78 citations, 11 retrospective reviews (with 524 total participants) were eligible. Efficacy was achieved in 63 % participants at follow-up periods between 6 months and 8 years. Adverse events and mortality was reported in 3.7 and 0.4 % of participants, respectively. Publication bias was detected with respect to efficacy and morbidity of the procedure. A large discrepancy in success was identified between ETV/CPC in six studies from sub-Saharan Africa (71 %), compared to three studies from North America (49 %). Conclusions: The reported success of ETV/CPC for infantile hydrocephalus is higher in sub-Saharan Africa than developed nations. Large long-term prospective multi-center observational studies addressing patient-important outcomes are required to further evaluate the efficacy and safety of this re-emerging procedure. [ABSTRACT FROM AUTHOR]

Objectives: The relationships between shunt infection and predictive factors have not been previously investigated using Artificial Neural Network (ANN) model. The aim of this study was to develop an ANN model to predict shunt infection in a group of children with shunted hydrocephalus. Materials and methods: Among more than 800 ventriculoperitoneal shunt procedures which had been performed between April 2000 and April 2011, 68 patients with shunt infection and 80 controls that fulfilled a set of meticulous inclusion/exclusion criteria were consecutively enrolled. Univariate analysis was performed for a long list of risk factors, and those with p value < 0.2 were used to create ANN and logistic regression (LR) models. Results: Five variables including birth weight, age at the first shunting, shunt revision, prematurity, and myelomeningocele were significantly associated with shunt infection via univariate analysis, and two other variables (intraventricular hemorrhage and coincided infections) had a p value of less than 0.2. Using these seven input variables, ANN and LR models predicted shunt infection with an accuracy of 83.1 % (AUC; 91.98 %, 95 % CI) and 55.7 % (AUC; 76.5, 95 % CI), respectively. The contribution of the factors in the predictive performance of ANN in descending order was history of shunt revision, low birth weight (under 2000 g), history of prematurity, the age at the first shunt procedure, history of intraventricular hemorrhage, history of myelomeningocele, and coinfection. Conclusion: The findings show that artificial neural networks can predict shunt infection with a high level of accuracy in children with shunted hydrocephalus. Also, the contribution of different risk factors in the prediction of shunt infection can be determined using the trained network. [ABSTRACT FROM AUTHOR]

Background: The middle meningeal artery (MMA) is often sacrificed during neurosurgical procedures in the region of the pterion. This maneuver, herein, is hypothesized to be a potential reason for the development of postoperative hydrocephalus by injuring the vascular supply to the arachnoid granulations near the vertex of the skull, and thus disrupting their ability to allow for transfer of CSF from the subarachnoid space to the venous system. Materials and methods: To test this theory, the middle meningeal artery was isolated at the skull base and injected with India ink. Next, the superior sagittal sinus was opened and the arachnoid granulations inspected. Results: All specimens demonstrated ink within the arachnoid granulations indicating that their blood supply is completely or at least partially via the middle meningeal artery. This finding with an illustrative case of surgical cautery of the middle meningeal artery at the skull base with subsequent development of hydrocephalus supports our hypothesis of potential role of iatrogenic MMA injury causing hydrocephalus. Conclusions: Our cadaveric study shows that the blood supply of the arachnoid granulations of the superior sagittal sinus is via the middle meningeal artery. Additional cases of postoperative hydrocephalus following middle meningeal artery sacrifice are needed to support our hypothesis. [ABSTRACT FROM AUTHOR]

The human cerebellum has a protracted development that makes it vulnerable to a broad spectrum of developmental disorders including malformations and disruptions. Starting from 19 to 20 weeks of gestation, prenatal magnetic resonance imaging (MRI) can reliably study the developing cerebellum. Pre- and postnatal neuroimaging plays a key role in the diagnostic work-up of congenital cerebellar abnormalities. Diagnostic criteria for cerebellar malformations and disruptions are based mostly on neuroimaging findings. The diagnosis of a Dandy-Walker malformation is based on the presence of hypoplasia, elevation, and counterclockwise upward rotation of the cerebellar vermis and cystic dilatation of the fourth ventricle, which extends posteriorly filling out the posterior fossa. For the diagnosis of Joubert syndrome, the presence of the molar tooth sign (thickened, elongated, and horizontally orientated superior cerebellar peduncles and an abnormally deep interpeduncular fossa) is needed. The diagnostic criteria of rhombencephalosynapsis include a complete or partial absence of the cerebellar vermis and continuity of the cerebellar hemispheres across the midline. Unilateral cerebellar hypoplasia is defined by the complete aplasia or hypoplasia of one cerebellar hemisphere. Familiarity with these diagnostic criteria as well as the broad spectrum of additional neuroimaging findings is important for a correct pre- and postnatal diagnosis. A correct diagnosis is essential for management, prognosis, and counseling of the affected children and their family. [ABSTRACT FROM AUTHOR]

Background: Reliable prognostic tools to estimate the case fatality rate (CFR) and the development of chronic hydrocephalus (CHC) in aneurysmal subarachnoid hemorrhage (SAH) are not well defined. This study aims to investigate the practicability and reliability of Fisher, Graeb, and LeRoux scores for SAH patient prognosis. Methods: A total of 206 patients with aneurysmal SAH were retrospectively analyzed in prediction of CFR and CHC. Clinical data was evaluated and grading was performed using Fisher, Graeb, and LeRoux scores. Univariate and multivariate analyses were performed to identify relevant predictive parameters. Results: CFR was 17.0 % and was associated with higher age, higher Hunt & Hess (H&H) grade, lower Glasgow Coma Scale (GCS) at admission, as well as a higher Fisher, Graeb, and LeRoux score ( p < 0.001). There were 19.9 % that developed CHC requiring permanent cerebrospinal fluid diversion. Low initial GCS ( p = 0.003), high H&H ( p < 0.001), intracerebral hematoma ( p = 0.003), high Fisher ( p = 0.047), Graeb and LeRoux scores ( p < 0.001) were associated with a higher rate of ventricular-peritoneal shunting (VPS) in surviving patients. In multivariate analyses, Graeb score (odds ratio (OR) 1.183 [1.027, 1.363], p = 0.020), LeRoux score (OR 1.120 [1.013-1.239, p = 0.027), and H&H (OR 2.715 [1.496, 4.927], p = 0.001) remained independent prognostic factors for VPS. Conclusions: Graeb or LeRoux scores improve the prediction of shunt dependency and in parts of CFR in aneurysmal SAH patients therefore confirming the relevance of the extent and distribution of intraventricular blood for the clinical course in SAH. [ABSTRACT FROM AUTHOR]

Background: Hydrocephalus is common in patients with open neural tube defects (NTDs), but association with closed NTDs has rarely been reported. We describe three patients with untreated closed NTDs who required surgery for hydrocephalus. Case reports: Case 1: A full-term newborn presented with a skin-covered midline lumbar spine lipoma. Magnetic resonance imaging (MRI) scan demonstrated a lipomyelomeningocele. An ultrasound at birth showed mild ventriculomegaly but cerebrospinal fluid diversion was not offered because his fontanelle was soft. He was lost to follow-up and then presented at age 6 months with signs and symptoms of hydrocephalus. An endoscopic third ventriculostomy and choroid plexus coagulation were performed, and the lipomyelomeningocele was subsequently repaired electively. Case 2: A full-term neonate presented with a skin-covered midline lumbar spine lesion consistent with a meningocele on MRI scan. He was additionally noted to have macrocephaly, a full fontanelle, and ventriculomegaly on MRI scan. At age 2 days, an endoscopic third ventriculostomy and choroid plexus coagulation were performed. A ventriculoperitoneal shunt was required 1 month later. Three months after birth, an elective spinal cord untethering procedure was performed. Case 3: A neonate presented with a midline lumbar skin tag. An MRI scan demonstrated a lipomyelomeningocele, and surgery was planned electively. On preoperative evaluation at age 3 months, the patient had a tense fontanelle. The surgery was postponed, and a ventriculoperitoneal shunt was placed after imaging studies revealed hydrocephalus. Conclusion: Patients with closed NTDs should be assessed for clinical signs and symptoms of hydrocephalus because of this rare but possible association. [ABSTRACT FROM AUTHOR]

Object: The goal of this study is to evaluate whether an 'ideal shunt' exists. Methods: This is a retrospective analysis based on original papers in the field of Hydrocephalus and Shunts. Patients of all age groups, who had hydrocephalus, and underwent some form of CSF diversion were included. The study has been divided into four stages: from 1900 to 1949, 1950 to 1974, 1975 to 1999, and from 2000 to 2010. Results: In stage 1 ( historical era): Saphenous vein grafts, rubber conduits, and other materials were used in CSF diversions. In 1949, the first implantable shunt tube was developed by Nulsen. In stage 2 ( experimental stage): the Holter valve was developed. Newer innovations were developed in relation to the ventriculo-atrial shunt, which was the preferred CSF diversion. In stage 3 ( developmental stage), a large number of different design shunt systems were developed, with the aim of reducing complications. The ventriculo-peritoneal shunt had become the preferred CSF diversion. Also, the programmable valve was born. In stage 4 ( era of programmable valve, there is a preference for the use of programmable shunt systems. However, shunt failure rate at 1 year being around 25 to 40 %, and shunt survival at 1 and 2 years are 50-70 and 47-53 % in most series. Conclusion: Every shunt is an ideal shunt provided the choice of the shunt used should be made by the matching performance of the shunt system to the altered profile of CSF dynamics of a given patient. The most important factor being the opening pressure. [ABSTRACT FROM AUTHOR]

Objective: Multiloculated hydrocephalus may occur as a consequence of intraventricular hemorrhage or infection and is characterized by enlargement of multiple noncommunicating intraventricular and/or periventricular cysts. In this study, we report the outcomes of open and endoscopic fenestration for multiloculated hydrocephalus at our institution. Methods: Records of children who underwent endoscopic or open fenestration at St. Louis Children's Hospital from 1999 to 2011 were analyzed. The cause of MLH, operative parameters, length of hospital stay, and subsequent shunt intervention rate were recorded. Results: Twenty-five subjects were identified for study. Twelve subjects underwent open craniotomy and 13 underwent endoscopic fenestration. Endoscopic fenestration was associated with decreased blood loss, operative time, and length of stay ( p = 0.003, 0.002, 0.02, respectively). Subjects undergoing craniotomy had an average of 5.1 ± 4.5 subsequent shunt-related interventions versus 3.1 ± 4.0 in the endoscopy group ( p = 0.25). The craniotomy group's median subsequent shunt revision rate was 0.74 interventions per year versus 0.50 interventions per year in the endoscopy group ( p = 0.51). Fifty percent of subjects in the open fenestration group required additional fenestration surgery compared to 38.5 % in the endoscopic group ( p = 0.70). Conclusion: Both open and endoscopic fenestration appeared effective at improving shunt management. The endoscopic technique may offer advantages in operative time, blood loss, and length of hospital stay. These data suggest that endoscopic fenestration may be used as the initial approach for treatment of multiloculated hydrocephalus, with craniotomy and open fenestration used for more severe or refractory cases. [ABSTRACT FROM AUTHOR]

Hydrocephalus is a very common disease in developing countries. Congenital aqueductal obstruction and post-inflammatory hydrocephalus come on the top of the list of causes of hydrocephalus. Till the recent introduction of cranial endoscopy and despite their frequent complications, shunts were considered as the mainstream treatment for this disease. Endoscopic third ventriculostomy (ETV), especially for obstructive hydrocephalus, introduced a new era of treatment that is free of lifetime shunt dependency. This study was done to assess the efficacy of ETV for treating post-inflammatory hydrocephalus in a unique group of patients thus preventing-if possible-the lifetime shunt dependency and suffering. ETV was tried as a first-line therapy in 35 children (23 males and 12 females) with hydrocephalus proved to be secondary to intracranial infection. Mean age was 9.2 months (4-15). Twenty-four patients (68.6 %) were below the age of 6 months while 11 patients (31.4 %) were above 6 months. Twenty-five patients (71.4 %) had a head circumference of 3 cm and 10 patients (28.6 %) had a 5 cm or more increase in the head circumference above the 95th percentile. All the patients included were followed postoperatively with regular clinical, computerized tomography (CT), and magnetic resonance imaging (MRI) examinations as well as cerebrospinal fluid (CSF) analysis and culture. The overall success of ETV was 55.9 % (19/34). Nine (81.9 %) out of the 11 patients that were endoscopically documented to have aqueductal obstruction showed improvement. While out of the 23 patients with patent aqueduct, only 10 patients (43.4 %) had improved. Procedure-related complications were not encountered. CSF leakage from the surgical wound occurred in three patients and mild CSF infection occurred in one patient. ETV is a simple, safe, and effective method in treating not only obstructive hydrocephalus due to non-inflammatory etiology, but also post-inflammatory hydrocephalus especially when the aqueduct is obstructed. An overall 50 % improvement in our study and even more in other series encourage the trial of getting rid of the lifetime shunt complications and suffering. [ABSTRACT FROM AUTHOR]

PHACE syndrome is a neurocutaneous syndrome characterized by the association of large cutaneous hemangiomas and the cardiac and cerebral vascular anomalies. We report a 6-year-old female with PHACE syndrome presented with left facial hemangiomas, cystic lesion in the cerebral posterior fossa, coarctation of the aorta, aplasia of the left vertebral artery and stenosis of the left internal carotid artery. Surgical repair of the aorta with left heart bypass under beating heart was scheduled. We monitored regional cerebral oxygen saturation (rSO) with infrared spectroscopy in order to detect cerebral hypoperfusion. A decrease of rSO ipsilateral to the cerebrovascular anomalies occurred during anastomosis of the aorta, which was treated by reducing the flow rate of left heart bypass and by increasing the inhalational oxygen concentration. As children with PHACE syndrome are frequently accompanied with cerebrovascular anomalies and at a risk of cerebral hypoperfusion, prevention of cerebral hypoperfusion is crucially important during general anesthesia. [ABSTRACT FROM AUTHOR]

Introduction: Paediatric low-grade astrocytomas of the fourth ventricle are rare tumours, generally revealed by hydrocephalus. However, some patients present with a history of severe anorexia. It might be a harbinger, which if recognized, could lead to earlier diagnosis. We decided to examine our database in order to evaluate the incidence and signification of anorexia in this context. Methods: Retrospective monocentric study of cases of low-grade astrocytomas of the fourth ventricle operated between 1991 and 2012 in our paediatric neurosurgery department. We particularly observed the clinical presentation and long-term clinical, oncological and radiological evolution. Non-parametrical tests were used (Mann-Whitney, Fisher). Results: We reviewed 34 cases, 31 pilocytic astrocytomas and 3 diffuse astrocytomas, 16 boys and 18 girls, (M/F ratio 0.89). Mean age at diagnosis was 8 years old. Seven presented with notable anorexia, the average BMI in this group was ≤2 standard deviation (SD); with clinical signs evolving for 11.5 months. Twenty-seven children had no anorexia; average BMI in this group was +1 SD, with clinical evolution for 6 months on an average of p < 0.05. We found no significant difference regarding hydrocephalus or tumour location. In all children with anorexia, body mass index improved markedly in the postoperative follow-up, which lasted, on average, for 6 years. Conclusion: Anorexia with stunted body weight curve is a non-exceptional presentation in children with low-grade astrocytomas of the fourth ventricle. Unexplained or atypical anorexia with negative etiologic assessment should prompt cerebral imaging. Clinical improvement after surgical resection, could suggest a possible interaction between tumour tissue and appetite-suppressing peptide secretion. [ABSTRACT FROM AUTHOR]

Purpose: The differential vulnerability of white matter (WM) to acute and chronic infantile hydrocephalus and the related effects of early and late reservoir treatment are unknown, but diffusion tensor imaging (DTI) could provide this information. Thus, we characterized WM integrity using DTI in a clinically relevant model. Methods: Obstructive hydrocephalus was induced in 2-week-old felines by intracisternal kaolin injection. Ventricular reservoirs were placed 1 (early) or 2 (late) weeks post-kaolin and tapped frequently based solely on neurological deficit. Hydrocephalic and age-matched control animals were sacrificed 12 weeks postreservoir. WM integrity was evaluated in the optic system, corpus callosum, and internal capsule prereservoir and every 3 weeks using DTI. Analyses were grouped as acute (<6 weeks) or chronic (≥6 weeks). Results: In the corpus callosum during acute stages, fractional anisotropy (FA) decreased significantly with early and late reservoir placement ( p = 0.0008 and 0.0008, respectively), and diffusivity increased significantly in early (axial, radial, and mean diffusivity, p = 0.0026, 0.0012, and 0.0002, respectively) and late (radial and mean diffusivity, p = 0.01 and 0.0038, respectively) groups. Chronically, the corpus callosum was thinned and not detectable by DTI. FA was significantly lower in the optic chiasm and tracts ( p = 0.0496 and 0.0052, respectively) with late but not early reservoir placement. In the internal capsule, FA in both reservoir groups increased significantly with age ( p < 0.05) but diffusivity remained unchanged. Conclusions: All hydrocephalic animals treated with intermittent ventricular reservoir tapping demonstrated progressive ventriculomegaly. Both reservoir groups demonstrated WM integrity loss, with the CC the most vulnerable and the optic system the most resilient. [ABSTRACT FROM AUTHOR]

Introduction: Dandy-Walker Syndrome (DWS) is a constellation of congenital anomalies of the central nervous system consisting of cerebellar vermis hypoplasia or agenesis, cystic dilatation of the fourth ventricle, enlarged posterior fossa, and a high tentorium. Hydrocephalus is a common occurrence in DWS and its treatment varies between shunting-ventriculoperitoneal or cystoperitoneal or both, endoscopic cyst fenestration, and third ventriculostomy. Chronic cerebral herniation is a known complication of treatment in DWS; however, we present an unusual case of central brain herniation. Case report: A 2 year old boy with shunted DWS presented with status epilepticus. Initial CT brain scan showed no increase in ventricle or cyst size; however, tapping the shunt reservoir did not yield any CSF prompting a shunt revision. Postoperatively, he was very slow to wake and subsequently experienced an episode of fixed pupils and extensor posturing. MRI brain demonstrated severe herniation of both thalami through the tentorium. Conclusion: This child had chronic central brain herniation (CCBH) secondary to the shunting of his cyst. To our knowledge, this is the first case of CCBH following treatment of DW cyst [ABSTRACT FROM AUTHOR]

Introduction: The possibility of an association between Dandy-Walker malformation and occipital meningocele is well-known. However, just an overall number of about 40 cases have been previously reported. Giant occipital meningocele has been described only in three newborns. Incidence, pathology, clinical presentation, and proper management of this association are still poorly defined. Report of the case: An 8-year-old boy with Dandy-Walker malformation and giant (25 cm in diameter) occipital meningocele is presented. This boy was born without any apparent occipital mass and harbored no other significant malformations including hydrocephalus. On admission, he was neurologically intact and the giant occipital mass presented partially calcified cyst walls. Treatment consisted of the excision of the occipital malformation, cranioplasty, and cysto-peritoneal shunt. Outcome was excellent. Conclusions: To the best of our knowledge, among the few reported patients with Dandy-Walker malformation associated to occipital meningocele, this is the oldest one and the one with the largest occipital meningocele; he is unique with calcified walls of the occipital meningocele and the only one who survived the repair of the giant malformation. In Dandy-Walker malformation, occipital meningocele may develop and grow regardless of hydrocephalus. Giant size may be reached and the cyst may become calcified. Surgical repair may warrant favorable outcome. [ABSTRACT FROM AUTHOR]

Chronic herniation syndromes other than tonsillar herniation are not well-recognized. Transtentorial uncal herniation in its chronic form has been reported in only few case reports (Horowitz et al., J Neuroimaging 12:78–79, ; Naidich et al., Radiology 158:431–434, ; Ng and Valiante, J Clin Neurosci 16:944, ; Ng and Valiante, J Clin Neurosci 16:984, ). We hereby illustrate a case with this rare finding, including MR imaging, and analyze this phenomenon. A 15-year-old girl had a resection of a cerebellar pilocytic astrocytoma at 5 years of age. Two months later, she underwent a cystoperitoneal shunt for persistent headache and pseudomeningocele, secondary to an enlarging cyst at the tumor bed. Subsequently, her shunt was upgraded to a more conservative valve following clinical evidence of over drainage. This was also associated with imaging compatible with bilateral uncal herniation. After the procedure, she had relief of symptoms. We conclude that a negative pressure gradient in the posterior fossa, relative to the supratentorial compartment, is the etiology of the chronic uncal herniation in our patient. Comparing the case reports in the literature with our case, we postulate that chronic uncal herniation is a complication of shunting of a posterior fossa fluid cavity in children, many of them with Dandy–Walker syndrome and/or other cerebellar cystic formations. The treatment priority at presentation should be to rule out shunt malfunction. In the event of association with clinical over-drainage syndrome, there may be a role for changing the shunt system into a more conservative drainage combination. [ABSTRACT FROM AUTHOR]

PHACE and PHACES are acronyms for a syndrome of variable expression comprising posterior cranial fossa malformations, facial hemangiomas, arterial anomalies, aortic coarctation and other cardiac disorders, ocular abnormalities and stenotic arterial disease. We review five girls and three boys aged 1 month-14 years with disorders from this spectrum. Six had large facial hemangiomas but recent reports suggest that small hemangiomas may occur; hence our inclusion of two possible cases. We also focus on the recently recognized feature of progressive intracranial arterial occlusions, present in four of our patients, later than previously recognized, from 4 to 14 years of age. We suggest that many elements of this disorder could reflect an abnormality of cell proliferation and apoptosis. [ABSTRACT FROM AUTHOR]

Half of patients with the Dandy-Walker malformation (DWM) have normal intellectual development. We aimed to identify feature on MRI associated with good intellectual prognosis. We reviewed 20 patients with DWM diagnosed on MRI, mean age 14.6±9.9 years. We assessed their intellectual development and related it to the MRI features. We found two groups with a statistically different intellectual outcome. All 14 patients with normal intellectual development had a normal lobulation of the vermis, without supratentorial anomalies. Of the six patients with mental retardation, three had an abnormal vermis, together with dysgenesis of the corpus callosum. In the other three, there were normal vermian anatomy with associated anomalies. Normal lobulation of the vermis, in the absence of any supratentorial anomaly, appears to be a good prognostic factor in DWM. Preservation of cerebrocerebellar pathways and neonatal plasticity could explain the normal intellectual development. These findings might be useful in prenatal diagnosis. [ABSTRACT FROM AUTHOR]

We present the cranial MRI findings in a 4-month-old girl with an atretic parietal cephalocele associated with multiple cerebral and ocular anomalies including lobar holoprosencephaly, a Dandy-Walker malformation, agenesis of the corpus callosum, grey-matter heterotopia, extra-axial cysts in various locations, bilateral microphthalmia and a retroocular cyst. [ABSTRACT FROM AUTHOR]

As neuro-imaging techniques have improved, variations of the Dandy-Walker malformation are being reported. In order to succeed in the therapy and avoid or minimize the complications, the fluid dynamics have to be evaluated by contrast CT studies. We are reporting three different types of this malformation and discuss the therapeutic possibilities. [ABSTRACT FROM AUTHOR]

Congenital cerebellar ataxia comprises a heterogeneous group of birth defects involving brain stem and cerebellum, characterised by ataxia, spasticity, choreoathetoid movements and mental retardation. Three children with congenital cerebellar malformations are described. The presenting features were benign enlargement of head, myoclonic jerks, episodes of hypernoia, nystagmus, ataxia, motor delay and mental retardation. Computerised tomography revealed Dandy-Walker cyst in one case and in two cases cerebellar vermis agenesis was associated with a variable degree of brain stem involvement. Retinal dysplasia and post-axial polydactyly were associated abnormalities in one case as reported in Joubert's syndrome, an autosomal recessive type of familial cerebellar vermis dysgenesis. Genetic factors in the pathogenesis of congenital cerebellar ataxia are reviewed. The relevance of computerised tomography, electroretinography and prenatal diagnosis by fetal ultrasound is discussed. Genetic counselling in congenital cerebellar ataxia presenting in the first year of life is important because of the high risk of recurrence. [ABSTRACT FROM AUTHOR]

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Morphological and developmental changes of the ventricular system are analyzed in three major experimental models of congenital hydrocephalus in the rat: 6-aminonicotinamide (6-AN)-induced and LEW/Jms and HTX mutant hydrocephalus. The clinically comparable forms of hydrocephalus and problems occurring during each period of intrauterine hydrocephalus are then discussed. Comparative morphological study revealed that 6-AN-induced hydrocephalus was comparable to the Dandy-Walker syndrome and that the critical period regarding this syndrome in fetal life was at the time of 'legal termination'. The LEW/Jms and HTX mutant models were identical with regard to the form of progressive hydrocephalus in the postnatal period, but the condition underlying the hydrocephalus during the fetal period differed. The LEW/Jms model was comparable to primary congenital aqueductal stenosis (aqueductal agenesis), and the hydrocephalic state appeared in the period of 'intrauterine preservation' before pulmonary maturation was completed. On the other hand, the HTX fetuses demonstrated secondary change of the aqueduct in the perinatal period, although the model was considered to be of congenital communicating hydrocephalus. Cerebrospinal fluid (CSF) dynamics studied in the fetuses with 6-AN-induced hydrocephalus disclosed considerable pathophysiology comparable to 'hydromyelic hydrocephalus.' The historical trends of animal experimental models of congenital hydrocephalus are reviewed and comparable clinical problems suggested by those models discussed further. [ABSTRACT FROM AUTHOR]

Introduction: Few innovations in the method of tunneling shunt tubing for cerebrospinal fluid (CSF) shunt diversion have been made since this treatment of hydrocephalus was first developed. Therefore, this feasibility study was performed with the hope of identifying an improved technique that could potentially carry fewer complications. Materials and methods: On 10 cadaver sides and when placed in the supine position, small skin incisions were made at the clavicle and ipsilateral subcostal region, and magnets were used to pass standard shunt tubing between the two incisions. Results: Nickel-plated magnets were less effective in pulling the shunt tubing below the skin compared with ceramic magnets. Of these, magnets with pull strengths of 150-200 lbs were the most effective in dragging the subcutaneous tubing between the two incisions. No obvious damage to the skin from the overlying magnet was seen in any specimen. Conclusions: Few options exist for tunneling distal shunt tubing for CSF shunt procedures. Future patient studies are needed to determine if the technique described herein is superior to current methods, particularly when examining patient groups that are at a greater risk for injury during tunneling shunt catheters. [ABSTRACT FROM AUTHOR]

A case of Dandy-Walker syndrome was diagnosed by ultrasound during the 30th week of pregnancy, observed again more clearly at 33 weeks and confirmed by CT after birth. [ABSTRACT FROM AUTHOR]

The rare finding of strong periventricular enhancement was observed by means of intraoperative CT cisternography using lotrolan in a one-month-old boy with Dandy-Walker syndrome associated with occipital cephalocele. Chronological findings of intraoperative CT scanning are demonstrated and etiological considerations are discussed. [ABSTRACT FROM AUTHOR]

This is a report of a 18 year-old woman with two consecutive fetuses suffering from a Dandy-Walker malformation (DWM). The first diagnosis of Dandy-Walker syndrome was at 24 weeks gestation. According to the parent's request, this pregnancy was terminated. Seven months after that event a new case of Dandy-walker malformation was diagnosed in the same woman. She was at 21 weeks of gestation. [ABSTRACT FROM AUTHOR]

Ventriculoperitoneal or cyst-peritoneal shunts are the usual treatment for children with Dandy-Walker syndrome. A combined ventricular and cyst shunt is often necessary and, in some cases, multiple revisions have to be done. Two patients with this syndrome were treated with posterior fossa exploration and cyst fenestration after several shunt revisions. The procedure was well tolerated with good results in both cases. The patients' data and preliminary results are presented here. [ABSTRACT FROM AUTHOR]