Etiology Of Hydrocephalus Research Articles

Traitement de l’hydrocéphalie de l’enfant par ventriculocisternostomie endoscopique au Sénégal

IntroductionSince its advent, endoscopic third ventriculostomy (ETV) has been an effective alternative to shunt placement for the treatment of hydrocephalus. The aim of this study was to report the results of our experience with this technique in children in Senegal. Materials and methodsThis was a retrospective study of 70 cases of children aged between 5 months to 15 years who were treated by ETV in the Neurosurgery Department of Fann Hospital in Dakar, between January 2010 to December 2012. The results were evaluated based on the clinical criteria of Drake and the Canadian Pediatric Neurosurgery Study Group. The mean follow-up duration was 24 months (9–32 months). ResultsThe mean age at diagnosis was 29 months. A male predominance (sex-ratio 1/3) was observed. We also noted a macrocephaly in 64.4 % of cases, psychomotor retardation in 40 % and decreased vision in 31.4 %. Headache and vomiting were found in 42.8 % and 61.4 % respectively. The main etiology was a stenosis of the mesencephalon aqueduct (30 %), followed by a Dandy-Walker malformation (25.7 %). Significant intraoperative bleeding was found in 2.8 % of patients. The most common postoperative complication was CSF leakage (18.6 %), followed by infections (14.2 %). The success rate according to the clinical criteria of Drake was 71.4 %. This success rate was influenced by the age of patients and the hydrocephalus etiology. No deaths occurred. ConclusionThe endoscopic third ventriculostomy is a simple, safe and effective technique. Its advantages in terms of quality of life and morbidity compared with bypass valves makes it the technique of choice, particularly in developing countries.

Using a 2-variable method in radionuclide shuntography to predict shunt patency.

Radionuclide shuntography interpretation is uncertain when the tracer fails to enter the ventricles but quickly drains distally or when the tracer enters the ventricles but takes longer than 15 minutes to drain distally. The purpose of this study was to aid in the clinical interpretation of a variety of shuntography results and to determine the applicability of shuntography in different patient populations. The results of 259 shuntograms were reviewed. Chi-square analysis was performed to evaluate the relationship between clinical variables and shuntography results. Two-by-two binary classification analyses were performed to determine the sensitivity, specificity, positive predictive value, and negative predictive value for 4 different combinatorial types of shuntography results based on 2 variables: ventricular tracer entry and distal tracer drainage. Median patient age was 19 years, and 51% of patients were male. The most common presentation in patients undergoing shuntography was headache (169/254, 66.5%) with radiographically stable ventricle size. Of 227 patients with available imaging data, 163 (71.8%) presented with the same ventricle size as shown on a previous asymptomatic scan, 43 (18.9%) had larger ventricles, and 21 (9.2%) had smaller ventricles. Within 30 days of shuntography, 74 of 259 patients (28.6%) underwent surgical shunt exploration: 65 were found to have an obstructed shunt and 9 were found to have a patent shunt. Of those patients not undergoing surgery, the median length of benign clinical follow-up was 1051 days. Clinical variables were not significantly associated with shuntography results, including valve type (p = 0.180), ventricle size (p = 0.556), age (p = 0.549), distal drainage site (p = 0.098), and hydrocephalus etiology (p = 0.937). Shuntography results of patients with myelomeningocele were not dissociable from those of the group as a whole. Sensitivity to diagnose shunt failure was lowest (37.5%) but specificity was highest (97.2%) when the definition of a "normal" shuntogram included any tracer movement into the distal site within 45 minutes. Conversely, sensitivity was highest (87.5%) and specificity was lowest (51.4%) when the definition was limited exclusively to tracer entry into the ventricles and distal drainage within 15 minutes. Even with a stringent definition of a "normal" shuntogram, sensitivity and specificity were relatively low for a diagnostic test. Clinical variables such as valve type, ventricle size, patient age, distal drainage site, and etiology of hydrocephalus were not associated with shuntography results.

Pediatric endoscopic third ventriculostomy: a population-based study.

Endoscopic third ventriculostomy (ETV) is an alternative to ventriculoperitoneal shunting for hydrocephalus treatment. Choice of treatment options raises questions about which patients are likely to benefit from ETV. The authors performed a population-based analysis using an administrative claims database, examining current practice and outcomes for pediatric patients in the US. The authors queried the MarketScan (Truven Health Analytics) database for Current Procedural Terminology codes corresponding to ETV and ventriculoperitoneal shunting from 2003 to 2011; they included patients 19 years or younger and extracted data from initial and subsequent hospitalizations. Hydrocephalus etiology was classified with ICD-9-CM coding. ETV failure was defined as any subsequent ETV or shunt procedure. Five hundred one patients underwent ETV. Of these, 46% were female. The mean age was 8.7 ± 6.4 years (± SD). The mean follow-up was 1.9 ± 1.8 years. Etiology of hydrocephalus was primarily tumor (41.7%) and congenital/aqueductal stenosis (24.4%). ETV was successful in 354 patients (71%). The mean time to failure was 109.9 ± 233 days. Of the 147 patients with ETV failure, 35 (24%) underwent repeat ETV and 112 (76%) had shunt placement. Patients in age groups 0 to < 6 months and 6 months to < 1 year had a significantly higher rate of ETV failure than those 10-19 years (HR 2.9, p = 0.05; and HR 2.3, p = 0.001, respectively). History of prior shunt was associated with higher risk of failure (HR 2.5, p < 0.001). There were no significant associations between hydrocephalus etiology and risk of failure. A second wave of failures occurred at 2.5-3.5 years postoperative in tumor and congenital/aqueductal stenosis patients; this was not observed in other etiology groups. This study represents a cross-section of nationwide ETV practice over 9 years. ETV success was more likely among children 1 year and older and those with no history of prior shunt.

Endoscopic third ventriculostomy and choroid plexus cauterization in infants with hydrocephalus: a retrospective Hydrocephalus Clinical Research Network study

The use of endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) has been advocated as an alternative to CSF shunting in infants with hydrocephalus. There are limited reports of this procedure in the North American population, however. The authors provide a retrospective review of the experience with combined ETV + CPC within the North American Hydrocephalus Clinical Research Network (HCRN). All children (< 2 years old) who underwent an ETV + CPC at one of 7 HCRN centers before November 2012 were included. Data were collected retrospectively through review of hospital records and the HCRN registry. Comparisons were made to a contemporaneous cohort of 758 children who received their first shunt at < 2 years of age within the HCRN. Thirty-six patients with ETV + CPC were included (13 with previous shunt). The etiologies of hydrocephalus were as follows: intraventricular hemorrhage of prematurity (9 patients), aqueductal stenosis (8), myelomeningocele (4), and other (15). There were no major intraoperative or early postoperative complications. There were 2 postoperative CSF infections. There were 2 deaths unrelated to hydrocephalus and 1 death from seizure. In 18 patients ETV + CPC failed at a median time of 30 days after surgery (range 4-484 days). The actuarial 3-, 6-, and 12-month success for ETV + CPC was 58%, 52%, and 52%. Time to treatment failure was slightly worse for the 36 patients with ETV + CPC compared with the 758 infants treated with shunts (p = 0.012). Near-complete CPC (≥ 90%) was achieved in 11 cases (31%) overall, but in 50% (10 of 20 cases) in 2012 versus 6% (1 of 16 cases) before 2012 (p = 0.009). Failure was higher in children with < 90% CPC (HR 4.39, 95% CI 0.999-19.2, p = 0.0501). The early North American multicenter experience with ETV + CPC in infants demonstrates that the procedure has reasonable safety in selected cases. The degree of CPC achieved might be associated with a surgeon's learning curve and appears to affect success, suggesting that surgeon training might improve results.

Obliteration of the choroid plexus after endoscopic coagulation

Endoscopic choroid plexus coagulation (CPC) with or without endoscopic third ventriculostomy (ETV) has been shown to be effective for selected patients with hydrocephalus. However, whether the effect of the coagulation is temporary and the choroid plexus regenerates or can be obliterated has remained largely unknown. The authors evaluate the effectiveness of CPC and report 3 cases of obliteration demonstrated by direct endoscopic observation. The authors retrospectively analyzed the surgical results of patients with hydrocephalus primarily treated by CPC with or without ETV. Charts were reviewed for demographic data, clinical presentations, surgical therapies, and clinical outcomes. Eighteen patients with hydrocephalus were surgically treated using endoscopic CPC between July 2002 and July 2012. In 12 patients, ETV was concurrently performed. The etiology of hydrocephalus was posthemorrhagic in 5 patients, myelomeningocele in 3, postmeningitis in 2, congenital aqueductal stenosis in 1, hydranencephaly in 1, porencephaly in 1, and idiopathic in 5. The mean age at surgery was 8 months (range 0.3-24 months). The mean follow-up was 64 months. In 9 cases (50%), control of hydrocephalus was successful and the patients did not require further surgeries. In 9 patients (50%), treatment failed. Of these, 3 patients underwent repeat ETV 2, 3, and 38 months after the initial surgery. Endoscopic observation of the previous coagulation site revealed no regeneration of the choroid plexus in 2 patients, who underwent repeat ETV 2 and 3 months after CPC. In 1 patient who underwent repeat ETV 38 months after CPC, no regeneration of the choroid plexus, except for that in the proximity of the foramen of Monro, was observed. Endoscopic CPC with or without ETV can be a safe and effective treatment alternative to shunt placement in infantile hydrocephalus. Obliteration of the choroid plexus can persist in the relatively long term following CPC, which may contribute to the long-term control of hydrocephalus in successful cases.

The correlation analysis of hydrocephalus shunt postoperative infection factors and prognosis

Objective To analyze the hydrocephalus shunt postoperative infection factors and prognosis.Methods The clinical data of 403 patients with hydrocephalus shunts,including 64 cases of postoperative infection,were retrospectively analyzed.And the sex ratio,age level,differences in etiology and infection,and their relationship with prognosis were analyzed by logistic regression.Results There were 64 infection cases (15.9％),including 47 cases of meningitis (73.5％),10 cases of peritonitis (15.6 ％),and 7 cases of postoperative infection (10.9％).The results of single factor Logistic regression analysis showed that gender,idio-hydrocephaly,apoplexy were not significantly correlated with hydrocephalus shunt postoperative infection(P ＞0.05),while age,congenital hydrocephalus,intracranial neoplasms,infection time were correlated with hydrocephalus shunt postoperative infection (P ＜ 0.05).Age was negatively correlated with GOS (r =-0.478,P ＜ 0.05).Conclusion Age,hydrocephalus etiology and infection time were the risk factors for infection.Age is a factor affect the prognosis. Key words: Hydrocephalus ; Ventriculoperitoneal shunt; Risk factors ; Prognosis

Covert orienting in three etiologies of congenital hydrocephalus: the effect of midbrain and posterior fossa dysmorphology.

Covert orienting is related to the integrity of the midbrain, but the specificity of the relation is unclear. We compared covert orienting in three etiologies of congenital hydrocephalus (aqueductal stenosis [AS], Dandy-Walker malformation [DWM], and spina bifida myelomeningocele [SBM]--with and without tectal beaking) to explore the effects of midbrain and posterior fossa malformations. We hypothesized a stepwise order of group performance reflecting the degree of midbrain tectum dysmorphology. Performance on an exogenously cued covert orienting task was compared using repeated measures analysis of covariance, controlling for age. Individuals with SBM and tectal beaking demonstrated the greatest disengagement cost in the vertical plane, whereas individuals with AS performed as well as a typically developing (TD) group. Individuals with SBM but no tectal beaking and individuals with DWM showed greater disengagement costs in the vertical plane relative to the TD group, but better performance relative to the group with SBM and tectal beaking. Individuals with AS, DWM, and SBM and tectal beaking demonstrated poorer inhibition of return than TD individuals. Impairments in attentional disengagement in SBM are not attributable to the general effects of hydrocephalus, but are instead associated with specific midbrain anomalies that are part of the Chiari II malformation.

Abdominal cerebrospinal fluid pseudocyst: a comparative analysis between children and adults

Abdominal cerebrospinal fluid (CSF) pseudocyst is a rare but important complication in patients with ventriculoperitoneal shunt (VPS). In addition to presenting our experience, we performed a comparative analysis between children and adults with this entity. To the author's knowledge, there are no studies in which this condition has been compared. The PubMed database was searched for all relevant reports published from 1954 to 2012. The differences were statistically compared, especially regarding clinical investigations, etiology of the hydrocephalus, shunt revision, CSF infection, treatment, and recurrence. Chi-square test or Fisher's exact test was used to find associations among the variables. Compiled from literature, we found 393 cases of abdominal pseudocyst: 295 children, including our cases, and 55 adults, with age not informed in 43 cases. In children, 33 % of the patients have a positive culture on presentation, with higher incidence in children younger than 10 years. In contrast, only 15 % among adults were positive CSF culture. In total, 287 abdominal pseudocyst cases who underwent shunt revision have been reported; 78.4 % of children and 62.2 % of adults. The main occurrence of this complication according to the etiology of hydrocephalus in children was different from adults. The recurrence of pseudocyst occurred in 19.8 and 24.2 % of children and adults, respectively. The differences between children and adults might represent distinct trends on the etiology and treatment of this entity. Hence, additional well-designed cohort studies will be necessary to strengthen our findings.

Blindness : An Uncommon Presentation of Colloid Cysts = العمى : عرض إستثنائي لتكيسات غروانية

Colloid cysts may obstruct the foramina of Monro and lead to acute hydrocephalus.1,2 In patients with colloid cysts, intraventricular pressure can increase suddenly due to acute obstructive hydrocephalus.3,4 A sudden and dramatic rise in ventricular pressure may lead to cerebral herniation, infarction and may result in death.4 These images show a patient who was admitted to Ataturk Training and Research Hospital, Ankara, Turkey, with complaints of visual loss. Acute bilateral blindness is an emergent condition that may signal a life-threatening disease. A 46-year-old man with no previous neurological complaints was admitted to our hospital with symptoms of headache, loss of consciousness and the progressive loss of vision. A physical examination revealed less than 20/20 central visual acuity. Moderate hydrocephalus and bilateral periventricular oedema were detected by a computed tomography (CT) scan performed in the Emergency Department [Figure 1]. Magnetic resonance (MR) imaging and diffusion-weighted imaging (DWI) were recommended for the patient, as the aetiology of hydrocephalus could not be determined exactly by the initial CT scan. On MR imaging, a colloid cyst and acute hydrocephalus were detected. In addition, diffusion-weighted imaging (DWI) revealed areas with restricted diffusion on the bilateral occipital lobes. As a result of these findings, ventriculoperitoneal shunting was performed five hours after the patient was admitted. MR images were obtained after the procedure, which detected regression in the sizes of the lateral ventricles and in the periventricular cerebrospinal fluid reabsorption [Figure 1]. In addition, there were infarctions in the bilateral occipital lobes and no recovery of vision. The posterior circulation was normal and there were early subacute infarctions in the occipital lobes on the MR images taken one week after the shunt therapy. There was no improvement in the loss of vision of the patient at the second week follow-up. Figure 1 A–K: Preoperative computed tomography (CT) and postoperative magnetic resonance (MR) images of the patient. A, B & C: Preoperative sequential axial CT images showing hydrocephalus and periventricular oedema. D, E, F & G: Postoperative axial ...

Effectiveness of the Bactiseal Universal Shunt for reducing shunt infection in a sub-Saharan African context: a retrospective cohort study in 160 Ugandan children

Antibiotic-impregnated shunts have yet to find widespread use in the developing world, largely due to cost. Given potential differences in the microbial spectrum, their effectiveness in preventing shunt infection for populations in low-income countries may differ and has not been demonstrated. This study is the first to compare the efficacy of a Bactiseal shunt system with a non-antibiotic-impregnated system in a developing country. The Bactiseal Universal Shunt (BUS) was placed in 80 consecutive Ugandan children who required a shunt. In this retrospective cohort study, the outcome for that group was compared with the outcome for the immediately preceding 80 consecutive children in whom a Chhabra shunt had been placed. The primary end points were shunt failure, shunt infection, and death. Shunt survival was analyzed using the Kaplan-Meier method. Significance of differences between groups was tested using the log-rank test, chi-square analysis, Fisher's exact test, and t-test. There was no difference between groups in regard to age, sex, or etiology of hydrocephalus. Mean follow-up for cases of nonfailure was 7.6 months (median 7.8 months, interquartile range 6.5-9.5 months). There was no significant difference between groups for any end point. The BUS group had fewer infections (4 vs 11), but the difference was not significant (p = 0.086, log-rank test). Gram-positive cocci were the most common culturable pathogens in the Chhabra group, while the only positive culture in the BUS group was a gram-negative rod. These results provide equipoise for a randomized controlled trial in the same population and this has been initiated. It is possible that the observed trends may become significant in a larger study. The more complex task will involve determining not only the efficacy, but also the cost-effectiveness of using antibiotic-impregnated shunt components in limited-resource settings.

An Association between External Hydrocephalus in Infants and Reversible Collapse of the Venous Sinuses

The etiology of external hydrocephalus is usually ascribed to either a delay in maturation or obstruction of the arachnoid granulations, but the arachnoid granulations are absent in neonates. Venous outflow stenoses, similar to those seen in idiopathic intracranial hypertension (IIH), have been described in external hydrocephalus. A reversible collapse of the sinuses is known to operate in IIH, but collapsible sinuses have not been previously described in infants with external hydrocephalus. Three infants with external hydrocephalus had magnetic resonance venography at differing time points during their illness. The venous sinuses varied in size depending on the cerebrospinal fluid pressure similar to IIH in adults. External hydrocephalus may be analogous to IIH in adults.

Decreased head circumference in shunt-treated compared with healthy children

In this study, the authors' goal was to compare head circumference in hydrocephalic children during the first 4 years of ventriculoperitoneal (VP) shunt treatment with data on healthy children and to investigate predictors of skull growth in children with a VP shunt. Children from western Sweden treated for hydrocephalus with an initial VP shunt insertion performed between 2001 and 2006 who were younger than 12 months of age at the time of surgery were included. Children with major brain malformations, craniofacial syndromes, large cysts, and tumors were excluded. Head circumference, weight, and height at 9 defined ages up to 4 years were obtained and compared with data from a reference population of 3650 healthy children using the standard deviation score (SDS). Predictors (length, weight, etiology of hydrocephalus, valve type, number of revisions, valve setting, number of adjustments, and time of first surgery) for head circumference SDS and changes in head circumference SDS from shunt insertion at 1 year to last measurement were analyzed using bivariate and multiple linear regression analysis. Fifty children were included. The mean SDSs for head circumference in shunt-treated compared with healthy children were 1.95 ± 2.50 at shunt insertion (p < 0.001, n = 44), 0.38 ± 1.97 at 1 year (p = 0.27, n = 33), -0.96 ± 2.05 at 2 years (p = 0.046, n = 21), -1.39 ± 2.25 at 3 years (p = 0.026, n = 16), and 0.63 ± 3.34 at 4 years (p = 0.73, n = 4). Significant predictors for low head circumference SDS at 1 year of age were low weight (p = 0.002) and short height (p = 0.022) and at last measurement low weight (p < 0.0001), short height (p = 0.002), and 1-4 shunt revisions (p = 0.034). A significant predictor for change in head circumference SDS from shunt insertion to 1 year of age was the number of shunt valve revisions (p = 0.04) and at last measurement an etiology of intraventricular hemorrhage (p = 0.0058). Shunt-treated children have smaller head circumferences at 2 and 3 years of age than healthy children. Low weight, short height, etiology of intraventricular hemorrhage, and frequent shunt valve revisions are predictors for decreased head circumference. Prospective, randomized studies comparing skull growth using fixed and adjustable pressure-regulated shunt valves and flow-regulated valves are needed.

Indications for Pediatric External Ventricular Drain Placement and Risk Factors for Conversion to a Ventriculoperitoneal Shunt

Background/Aims: The external ventricular drain (EVD) is commonly used for the treatment of hydrocephalus and intracranial pressure (ICP) monitoring. In this study, we retrospectively reviewed indications for EVD placement over the last 20 years in pediatric patients at our institution. Additionally, we evaluated the rate that an EVD needed to be converted to a ventriculoperitoneal shunt (VPS) and ascertained how hydrocephalus etiology impacted long-term drainage requirements. Methods: Patients that received an EVD between 1990 and 2010 at our institution were identified using billing codes. Demographics, etiology of hydrocephalus, length of stay (LOS) and EVD duration were recorded. Only patients receiving their first EVD were included; those with any prior ventricular catheter (including prior shunts) were excluded from this study. Results: A total of 180 patients underwent first-time placement of an EVD over the 20-year period. Of these, 78.9% were placed on the right side and 81.7% were performed through a frontal burr hole site. The average age of all patients at their initial procedure was 9.3 years (median 9.8; range 0-18), hospital LOS was 22.5 days (median 16; range 1-174) and EVD duration was 7.8 days (median 7; range 1-37). Trauma (43.3%) and neoplasms (32.2%) were the most common indications. Approximately, one quarter of the patients were unable to become EVD independent and, therefore, required a VPS. Only patients receiving an EVD for traumatic brain injury were significantly less likely to be converted to a shunt than other causes of hydrocephalus (relative risk, RR = 0.18, 95% confidence interval, CI = 0.07-0.46). Conversely, patients with neoplasms (RR = 3.56, 95% CI = 1.74-7.31), obstructive hydrocephalus (RR = 5.48, 95% CI = 1.37-22.0) and intraventricular hemorrhage (RR = 9.86, 95% CI = 1.05-92.3) were more likely to need a shunt. Conclusion: Traumatic brain injuries and neoplasms represent the most common indications for EVD placement in the pediatric population. While patients with neoplasm were much more likely to need conversion to a VPS for long-term cerebrospinal fluid diversion, the majority of EVDs in patients with head injuries were successfully weaned.

The Success of Endoscopic Third Ventriculostomy in Children: Analysis of Prognostic Factors

Objective: The treatment of hydrocephalus in children with endoscopic third ventriculostomy (ETV) has particular features and is associated with different success rates (SR). The aim of this study was to identify putative factors that could influence the outcome of ETV in children. Methods: Clinical data of 114 consecutive patients under 18 years of age who underwent 116 consecutive ETVs from January 2000 to January 2010 were reviewed. Data were analyzed with regards to clinical and radiological SR. The actual long-term SR was compared to that predicted by the ETV Success Score (ETVSS) model. Results: The study group included 49 males (43%) and 65 females (57%) with a mean age of 6.17 ± 1.02 years (ranging from 11 days to 18 years) at surgery. Concerning the etiology of hydrocephalus, tumors and aqueductal stenosis (AS) were the most frequently observed, with each occurring in 33 cases (29%), followed by malformations in 24 (21%), cystic lesions in 6 (5%) and other etiologies in 18 patients (16%). The overall SR at the first ETV attempt was 80% (91/114), compared to 74.8% (variance 14.35, 95% CI 69.37-78.22) predicted by the ETVSS. Regarding age, SR was 58% in patients under 6 months of age, 65% in children between 6 months and 1 year, and 86% in children older than 1 year. SR for AS and hydrocephalus associated with posterior fossa tumors were 88 and 90%, respectively. Unsatisfactory results were related to previous intraventricular hemorrhage and infection. The overall complication rate in this series was 13%. Conclusion: ETV is safe and effective in children. In this series, the age of the patient and etiology of hydrocephalus were related to SR. Also, the ETVSS was accurate to predict outcome. In a long-term follow-up, surgical experience was statistically significant in reducing complications.

Communicating Hydrocephalus in Metachromatic Leukodystrophy

This child was developmentally normal until approximately 1 year of age, then regressed. When first evaluated at 2 years of age, she had developed spastic quadriparesis with decreased vision and verbal communication. A magnetic resonance imaging revealed bilateral diffuse signal abnormalities involving the splenium of the corpus callosum and the deep and periventricular white matter of the bilateral frontal and parietallobes,withrelativesparingofthesubcorticalwhitematter(Figs1 and 2). Her mother reported a similar clinical course for the patient’s brother that resulted in his death at about 3 years of age without a diagnosis. Extensive metabolic testing was undertaken and arylsulfatase-A deficiency was found. Analysis of the arylsulfatase-A gene revealed a previously described disease causing homozygous mutation [c.G364A (gene 513, EX2), p.G122s)]. The girl’s mother was confirmed to be an obligate carrier, but the father was not available for testing. The patient subsequently experienced severe neurological regression. Spinal magnetic resonance imaging was undertaken due to additional development of severe scoliosis. It revealed moderate to severe thoracolumbar levoscoliosis and an incidental finding of marked lateral ventricle enlargement with complete effacement of subarachnoid spaces around the temporal lobes. No spinal canal stenosis or spinal cord abnormalities were noted. A computed tomography scan of the brain was performed to confirm the partially imaged intracranial findings. It showed severe enlargement of the lateral, third, and fourth ventricles that was out of portion tothe degree of volume loss with dilatation of the anterior third ventricle (Fig 3). Other than intermittent fussiness and vomiting, there were no other clinical findings attributable to development of hydrocephalus. There were no clinical or radiologic findings to suggest tumor, infection, or hemorrhage as the etiology of hydrocephalus. A ventriculoperitoneal shunt was considered but not placed because of the child’s extremely poor prognosis after risks, benefits, and alternatives were discussed with the family.

Seizure Risk Factors in Shunted Hydrocephalic Patients

Objective: There are controversies about seizure risk factors in shunted hydrocephalic patients which can be due to having merged two different types of seizure (preshunt and postshunt seizures) in previous studies. Also, it is not known whether a considerable change in ventricular size after shunting can be a risk factor of postshunt seizures. Methods: 150 hydrocephalic patients who underwent shunting from 2006 to 2011 in the Children's Hospital Medical Center in Tehran, Iran, were visited at least 1 year after shunting to assess risk factors of preshunt and postshunt seizures. Ventricular size was assessed by using a radiologic index of bifrontal ratio (BFR). Results: Preshunt seizures were higher in patients with postinfectious hydrocephalus and intraventricular hemorrhage. Early shunting, history of shunt complications and shunt infection, and a high number of shunt revisions were significant risk factors for developing postshunt seizures. The change in BFR after shunting was not a significant risk factor of postshunt seizures. Conclusion: The difference between risk factors of preshunt seizures and postshunt seizures shows that they are two different types of seizures with different natures. Preshunt seizures are hard to prevent as they are related to the underlying disease of the brain or the etiology of hydrocephalus. However, postshunt seizures are related to the shunt and might be decreased by preventing shunt complications.

Endoscopic third ventriculostomy may have long term efficacy in low birth weight preterm newborns

AbstractEndoscopic third ventriculostomy (ETV) is an established treatment for hydrocephalus and is an alternative method avoiding shunts and its complications. There is still controversy regarding the higher risk of failure in younger infants. NARF was a male preterm newborn of 33 weeks old, with Moebius syndrome and 1,800 grams at birth. Detailed neonatal evaluation revealed hydrocephalus with bilateral ventricular index of 0.6. It was then decided, in spite of prematurity and low birth weight to perform ETV with the age of 25 days, with 1,850 grams. After discharge, follow-up was lost due to family moving to another city. By chance, after seven years child returned to our medical facility and a follow-up magnetic resonance revealed pervious ETV with high flow, demonstrating functionality of ventriculostomy. Patient's age and etiology of hydrocephalus are the most important factors influencing the success rate of ETV. Recently, the age between 6 months and 1 year old became a cutoff below which failure rates are reported to be particularly high and there are dichotomized experience. The results in low birth weight and preterm infants is not widely known and can be poor. Our case illustrates a successful ETV in a both preterm and low birth weight newborn and a satisfactory long term result, reinforcing the security and efficacy of ETV even in newborns.

A pilot study using residual newborn dried blood spots to assess the potential role of cytomegalovirus and Toxoplasma gondii in the etiology of congenital hydrocephalus

Congenital hydrocephalus is a condition characterized by accumulation of cerebrospinal fluid in the ventricles of the brain. Prenatal infections are risk factors for some birth defects. This pilot study investigated whether residual dried blood spots (DBS) could be used to assess infections as risk factors for birth defects by examining the associations between prenatal infection with Toxoplasma gondii (T. gondii) or cytomegalovirus (CMV) with congenital hydrocephalus. Case-infants with hydrocephalus (N=410) were identified among live-born infants using birth defects surveillance systems in California, North Carolina, and Texas. Control-infants without birth defects were randomly selected from the same geographic areas and time periods as case-infants (N=448). We tested residual DBS from case- and control-infants for T. gondii immunoglobulin M and CMV DNA. When possible, we calculated crude odds ratios (cORs) and confidence intervals (CIs). Evidence for prenatal T. gondii infection was more common among case-infants (1.2%) than control-infants (0%; p=0.11), and evidence for prenatal CMV infection was higher among case-infants (1.5%) than control-infants (0.7%; cOR: 2.3; 95% CI: 0.48, 13.99). Prenatal infections with T. gondii and CMV occurred more often among infants with congenital hydrocephalus than control-infants, although differences were not statistically significant. This pilot study highlighted some challenges in using DBS to examine associations between certain infections and birth defects, particularly related to reduced sensitivity and specimen storage conditions. Further study with increased numbers of specimens and higher quality specimens should be considered to understand better the contribution of these infections to the occurrence of congenital hydrocephalus.

Complications and subsequent removal of retained shunt hardware after endoscopic third ventriculostomy: case series

This case series highlights multiple complications and subsequent removal of retained shunt hardware in pediatric patients after successful endoscopic third ventriculostomy (ETV). Removal or retention of existing shunt hardware following ETV represents an important dilemma. Prior studies have reported infections and organ perforation related to nonfunctioning shunts but none in the context of successful ETV. Data obtained in 3 children with hydrocephalus treated at the authors' institution were retrospectively reviewed after the patients experienced complications due to retained shunt hardware following ETV. Etiologies of hydrocephalus included tectal glioma and intraventricular hemorrhage. All 3 patients had a history of multiple shunt revisions and underwent urgent ETV in the setting of a shunt malfunction. In each case, the entire shunt system was left in situ, but it became the source of subsequent complications. Two of the 3 patients presented with the shunt infected by gram-negative bacilli 10 days and 4.5 months postoperatively, respectively. The remaining patient experienced wound dehiscence over the shunt valve 4.5 months after ETV. In all patients, the complications were managed successfully by removing the shunt hardware. None of the patients required repeat shunt insertion from the time of removal throughout the follow-up period (mean 24 months, range 9-36 months). During the study period, a total of 6 patients with indwelling shunt hardware underwent ETV with the expectation of being shunt independent. Among these 6 patients, 3 experienced no complications from the retained hardware whereas 3 patients (50%) ultimately experienced adverse consequences related to retained hardware. This case series illustrates complications involving retained shunt hardware after successful ETV. These examples support consideration of shunt removal at the time of ETV in the appropriate context.

Lysophospholipid GPCRs in medical therapeutics and disease mechanisms

Lysophospholipids that include lysophosphatidic acid (LPA) and sphingosine 1‐phosphate (S1P) influence a spectacular range of cellular processes that affect virtually every vertebrate cell type. Their effects are mediated by a family of cell‐surface GPCRs designated by their receptor names LPA1–6 and S1P1–5, and the gene names LPAR1–6, S1PR1–5. These receptors couple to all major G proteins and consequently activate a diverse range of downstream pathways. New ways of measuring the interaction of a lipid with its cognate receptor through the use of back‐scattering interferometry (BSI) will be presented. In addition, medically relevant consequences of modulating lysophospholipid receptors for both therapeutic benefit – as evidenced by the actions of an S1P receptor modulator called fingolimod (Gilenya), the first oral medicine for treating multiple sclerosis – and mechanistic understanding of diseases – as illustrated through studies on LPA receptors in the etiology of hydrocephalus – will be discussed.