Cases Of Congenital Hydrocephalus Research Articles

Epidemiology of hydrocephalus in Brazil

ObjectiveDescribe the epidemiological profile and social-economic burden that hydrocephalus patients represent to the national public health system, using data available at the online database of the Brazilian Health Ministry (DataSUS). MethodsThis is a populational study based on descriptive statistics of all clinical and surgical appointments included in the DataSUS database. Data included herein were collected between 2015 and 2021 and subdivided into three main groups, related to hydrocephalus incidence and mortality, hospitalizations, and financial costs. ResultsIn the study period, 3993 new cases of congenital hydrocephalus were diagnosed, with 6051 deaths overall. The mortality rate in the country was 1.5/100000 live births and the prevalence was 0.374/100000 inhabitants. The number of hospitalizations resulting from treatment procedures and complications of hydrocephalus was 137,880 and there was a reduction of up to 27.2% during the SARS-CoV-2 pandemics concerning previous years. Total costs for hydrocephalus management in the country amounted to 140,610,585.51 dollars. ConclusionsHydrocephalus has a significant impact on public health budgets and pediatric mortality rates; however, it is probably underestimated, due to the paucity of demographic data and epidemiological studies in Latin America and, specifically, in Brazil. The dataSUS also has several limitations in accessing certain data related to hydrocephalus, making it difficult to have a more assertive understanding of the disease in Brazil. The results of this study provide important guidance for future research projects in clinical and experimental hydrocephalus and also the creation of public policies for better governance and care of hydrocephalus patients.

Lymphedema in Hydrocephalus: A Case Study

Background: Lymphedema is a combination of pathologic condition characterized by excessive local accumulation of interstitial fluid which is rich in proteins [1-3]. It may be an isolated phenomenon or associated with a multitude of other disabling local sequelae or even life-threatening systemic syndromes. In its purest form, the central disturbance is a low output failure of the lymphvascular system, that is, overall lymphatic transport is reduced. Lymphedema can be either primary or secondary [4]. Hydrocephalus is a build-up of fluid in the brain. The excess fluid puts pressure on the brain, which can damage it. Congenital hydrocephalus is when a baby is born with excess fluid in their brain which will cause permanent brain damage such as: speech problems and vision problems [5] Study Design: The case study was used to highlight the role of physical therapy interventions in the management of primary lower extremity lymphedema in patient with congenital hydrocephalus patient. Case Description: 37 years-old Saudi female, case of congenital hydrocephalus, deaf, mute, blind with shunting. Patient with severe bilateral lower limb primary lymphedema, which altered her level of function. Referred to physical therapy department by vascular clinic. PT Management and Outcome: The patient received daily sessions of complete decongestive therapy (CDT) for two weeks included: multi-layer bandaging (compression therapy), manual lymphatic drainage, skin / nail care education and decongestive exercises. At the end of second week, the patient’s circumference measurements improved significantly, and her level of independency improved. Discussion: The case study showed that the physical therapy interventions are safe, effective, in reducing lymphedema and overall quality of life (QOL) with hydrocephalus patients.

Analysis of early neonatal case fatality rate among newborns with congenital hydrocephalus, a 2000-2014 multi-country registry-based study.

Congenital hydrocephalus (CH) comprises a heterogeneous group of birth anomalies with a wide-ranging prevalence across geographic regions and registry type. The aim of the present study was to analyze the early neonatal case fatality rate (CFR) and total birth prevalence of newborns diagnosed with CH. Data were provided by 25 registries from four continents participating in the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR) on births ascertained between 2000 and 2014. Two CH rates were calculated using a Poisson distribution: early neonatal CFR (death within 7 days) per 100 liveborn CH cases (CFR) and total birth prevalence rate (BPR) per 10,000 births (including live births and stillbirths) (BPR). Heterogeneity between registries was calculated using a meta-analysis approach with random effects. Temporal trends in CFR and BPR within registries were evaluated through Poisson regression modeling. A total of 13,112 CH cases among 19,293,280 total births were analyzed. The early neonatal CFR was 5.9 per 100 liveborn cases, 95% confidence interval (CI): 5.4-6.8. The CFR among syndromic cases was 2.7 times (95% CI: 2.2-3.3) higher than among non-syndromic cases (10.4% [95% CI: 9.3-11.7] and 4.4% [95% CI: 3.7-5.2], respectively). The total BPR was 6.8 per 10,000 births (95% CI: 6.7-6.9). Stratified by elective termination of pregnancy for fetal anomalies (ETOPFA), region and system, higher CFR were observed alongside higher BPR rates. The early neonatal CFR and total BPR did not show temporal variation, with the exception of a CFR decrease in one registry. Findings of early neonatal CFR and total BPR were highly heterogeneous among registries participating in ICBDSR. Most registries with higher CFR also had higher BPR. Differences were attributable to type of registry (hospital-based vs. population-based), ETOPFA (allowed yes or no) and geographical regions. These findings contribute to the understanding of regional differences of CH occurrence and early neonatal deaths.

Effect of Ventriculoperitoneal Shunt on the Recovery of Brain Function in Children with Hydrocephalus

Objective: To analyze the effect of ventriculoperitoneal shunt on the recovery of brain function in children with hydrocephalus. Methods: The clinical data of 40 children with hydrocephalus were retrospectively analyzed. Ventriculoperitoneal shunt was performed with 9003 shunt tube and P.S. Shunt tube, B.C.E. shunt tube. Electroencephalogram (EEG), and brain CT/MRI were performed before and after surgery, and postoperative follow-up was carried out to observe the therapeutic effect. Results: In this study, there were seven cases of intracranial injury, seven cases of congenital hydrocephalus, 11 cases of ventricular end obstruction, three cases of abdominal end obstruction, nine cases complicated with bacterial infection, and 3 cases of shunt entering the scrotum. The prognosis of all the children was good, and there were no significant changes in eight cases. Conclusion: Ventriculoperitoneal shunt is effective in the treatment of children with hydrocephalus.

Folic acid supplementation and risk for congenital hydrocephalus in China.

We examined whether folic acid (FA) supplementation prevented congenital hydrocephalus (CH) in more than 200 000 births in China. A large population-based cohort study. All births at 20 complete gestational weeks, including live births, stillbirths and pregnancy terminations, and all structural birth defects regardless of gestational week were recorded. The prevalence of births with CH was classified by maternal characteristics and FA supplementation. CH was diagnosed in accordance with code 742.3 of the International Classification of Diseases, Ninth Revision, Clinical Modification, while non-neural tube defect (NTD) CH refers to CH without anencephaly (740), spina bifida (741) or encephalocele (742·0). A total of 247 831 pregnant women who delivered with known outcomes were included. A total of 206 cases of CH (0·83 per 1000 births) and 170 cases of non-NTD CH (0·69 per 1000 births) were recorded in the study. The prevalence of CH and non-NTD CH was higher in women in the no supplementation group than those in the FA supplementation group (0·92 and 0·72 v. 0·75 and 0·65 per 1000 births, respectively). FA supplementation during the periconceptional period significantly prevented CH (OR = 0·29, 95 % CI 0·12, 0·69) and non-NTD CH (OR = 0·34, 95 % CI 0·12, 0·97) in northern China, especially in a high-compliance group (≥ 80 %). Periconceptional FA supplementation did not significantly prevent CH overall in the current study. However, in the north of China with common maternal folate insufficiency, there was some evidence.

Exome sequencing implicates genetic disruption of prenatal neuro-gliogenesis in sporadic congenital hydrocephalus.

Congenital hydrocephalus (CH), characterized by enlarged brain ventricles, is considered a disease of excessive cerebrospinal fluid (CSF) accumulation and thereby treated with neurosurgical CSF diversion with high morbidity and failure rates. The poor neurodevelopmental outcomes and persistence of ventriculomegaly in some post-surgical patients highlight our limited knowledge of disease mechanisms. Through whole-exome sequencing of 381 patients (232 trios) with sporadic, neurosurgically treated CH, we found that damaging de novo mutations account for >17% of cases, with five different genes exhibiting a significant de novo mutation burden. In all, rare, damaging mutations with large effect contributed to ~22% of sporadic CH cases. Multiple CH genes are key regulators of neural stem cell biology and converge in human transcriptional networks and cell types pertinent for fetal neuro-gliogenesis. These data implicate genetic disruption of early brain development, not impaired CSF dynamics, as the primary pathomechanism of a significant number of patients with sporadic CH.

Epidemiology of anencephaly in a suburban Nigerian agrarian community in the rain forest: An 11-year retrospective study of a single hospital experience

BackgroundAnencephaly is a common but lethal form of neural tube defect which occurs as a result of failure of closure of the rostral neuropore. There is no data on this lethal congenital anomaly in our community. ObjectiveThe aim of this review is to document the prevalence of anencephaly in our community. MethodsA retrospective review of the labour ward register over eleven-year period from 2007 to 2017 was carried out. The total number of deliveries was evaluated for congenital anomalies of the central nervous system. The case notes of the mothers who gave birth to children with anencephaly were further reviewed for their epidemiological profile, antenatal care and use of folic acid, fetal profile and placental weight. The data obtained was analyzed and using inferential statistics conclusions were derived. ResultsThere were 5 cases of anencephaly out of the 3, 789 deliveries over the eleven year period resulting in 1.3 per 1000 deliveries. There was a case of congenital hydrocephalus but there was no documented case of encephalocele and myelomeningocele. The Male: Female ratio was 2:3. The mothers’ age range was 18 years to 33 years and all affected mothers were not registered for the antenatal care and have no documentation of folic acid supplementation during the pregnancy. ConclusionThis retrospective review showed that the prevalence of anencephaly in our agrarian community agrees with the lower end of the incidence spectrum obtainable in the available literature. There is, therefore, need for a well-designed prospective study to corroborate these findings.

Needle in a haystack: migration of ventriculoperitoneal shunt into scrotum of infant

BackgroundVentriculoperitoneal shunt malpositions are known complications which tend to increase the morbidity and recovery time of an elegant procedure. Distal malpositions into subcutaneous space due to improper closure or positioning can lead to shunt failure. Migration to the scrotum due to a patent processus vaginalis is, however, reported only in a handful of cases and, thus, needs to be highlighted and reported.ResultsWe report a case of congenital hydrocephalus that underwent shunting at the age of 3 months and reported to be 9 months later with the shunt tube being felt in the scrotum. Repositioning along with herniotomy was performed.ConclusionsA rare occurrence, scrotal migration, needs to recognized and reported. Potential preventive measures include truncation stiches and laproscopic assisted insertion.

Prevalence and trend of isolated and complicated congenital hydrocephalus and preventive effect of folic acid in northern China, 2005-2015.

Congenital hydrocephalus (CH) was a major birth defect of the central nervous system besides neural tube defects (NTDs). Few studies have focused on both the prevalence and trend of isolated and complicated CH in China. Data were drawn from a population-based birth defects surveillance program in five rural counties in northern China from 2005 to 2015. All livebirths and pregnancy terminations at any gestational age affected with CH were recorded. The prevalence and trend of isolated and complicated CH were examined. During the 11-year period, a total of 176,223 births and 357 CH cases were recorded, resulting in a prevalence rate of 20.3 CH cases per 10,000 births. Of the CH cases, 146 were isolated CH, resulting in a prevalence rate of 8.3 per 10,000 births. The pre-perinatal prevalence (<28 gestational weeks) was higher than the perinatal prevalence for both isolated and total CH. The prevalence rates of total and isolated CH showed a similar downward trend during the 11-year period. This downward trend was statistically significant after 2009 (p < 0.05), when a massive folic acid supplementation program was introduced. Although it decreased over time, the prevalence of CH remains high in this population which has a high prevalence of neural tube defects.

Trends in the prevalence of congenital hydrocephalus in 14 cities in Liaoning province, China from 2006 to 2015 in a population-based birth defect registry from the Liaoning Women and Children's Health Hospital.

The aim of this study was to assess the prevalence and trends of congenital hydrocephalus (CH) using a large population-based sample with cases retrieved from the Liaoning Birth Defects Registry, which included 14 cities, over a 10-year period. CH prevalence, percent change, average change, and contribution rates of each city were calculated. Statistical analysis was performed using a Poisson regression model. There was a total of 3008 CH cases among 3,248,954 live births during the observational period (9.26 cases/10,000 live births). On average, the prevalence of CH had significantly decreased by 11.84% each year (P < 0.01). The three leading cities with the highest prevalence of CH were Chaoyang (13.73/10,000), Huludao (13.18/10,000), and Fuxin (12.71/10,000), while Yingkou (6.06/10,000), Dalian (6.27/10,000), and Anshan (6.56/10,000) had the lowest prevalence. Notably, significantly decreasing trends were observed in 10 (71.4%) of these 14 cities. In addition, the cities of Fushun, Shenyang, and Dalian had contributed to more than one-third of the decreasing trend in Liaoning province. In conclusion, this study provides evidence of the decreasing prevalence of CH over a 10-year period (2006–2015) in Liaoning province. Preventative efforts should be strengthened to further reduce the risk of CH in these high prevalence areas.

Changes in prevalence and perinatal outcomes of congenital hydrocephalus among Chinese newborns: a retrospective analysis based on the hospital-based birth defects surveillance system

BackgroundLittle is known about the epidemiology of congenital hydrocephalus (CH) in China. This study aimed to depict recent changes in CH prevalence and perinatal outcomes of the affected newborns.MethodsData were obtained from the Chinese Birth Defects Monitoring Network (CBDMN), which collects demographic information on all newborns above 28 weeks of gestation, and clinical information on neonates with congenital anomalies. CH cases delivered during 2005–2012 were analyzed. Poisson regression was used to calculate prevalence ratios (PR) and 95% confidence intervals, and linear chi-square test was used to examine time trend of CH prevalence.ResultsFive thousand two hundred forty-five isolated and 1245 associated CH cases were identified among 10,574,061 newborns, yielding the prevalence of 4.96, 1.18 and 6.14 per 10,000 births for the isolated, associated and overall hydrocephalus, respectively. The annual prevalence of CH presented a decreasing trend (from 7.52 to 5.98 per 10,000 births, P < 0.001). Higher prevalence was found in both younger (<20 years, PR: 1.81, 95% CI: 1.56–2.10) and older (≥35 years, PR: 1.48, 95% CI: 1.36–1.61) maternal-age groups in comparison with the maternal-age group of 20 to 24 years. Higher prevalence was also found in infants born to mothers resided in rural areas, male infants, and multiple births. Of non-aborted infants with congenital hydrocephalus, 38.11% were born with low birth weight, 37.53% were preterm birth, and 20.69% died within 7 days after birth.ConclusionsOur findings present a relatively high prevalence and poor perinatal outcomes of CH in China, which can serve as a baseline for future study.

The impact of fetal middle cerebral artery Doppler on the outcome of congenital hydrocephalus

Objectives: The objective of this study is to investigate the impact of abnormal middle cerebral artery (MCA) Doppler on the perinatal mortality in fetuses with congenital hydrocephalus (CH).Methods: A prospective study of all fetuses with CH who delivered at our hospital over a period of 7 years. Data were obtained from the ultrasound, Labor room and intensive neonatal care unit (NICU) database. The Perinatal mortality rates were evaluated in relation to the following measures, associated congenital anomalies, cortical mantle thickness (CMT), and MCA Doppler abnormalities (absent or reversed diastole). The main outcome measure was perinatal mortality rate in relation to MCA Doppler changes.Results: A total of 85 cases of CH were diagnosed and managed. The birth prevalence of CH was 2.44 per 1000 live births. On one hand, the perinatal mortality rate was higher in those fetuses with non-isolated hydrocephalus, (37.25% (19/51) versus (35.29% (12/34, p = 0.854 and in those cases with CMT <10 mm, 38.78% (19/49) versus 33.33% (12/36) in those with CMT >10 mm, p = 0.607. On the other hand, the perinatal mortality rate was significantly higher in those fetuses with abnormal MCA Doppler, (100% (13/13) versus 25% (18/72), OR = 78.0, 95% CI (5.52–44085124.60), p < 0.001.Conclusions: Abnormal fetal MCA Doppler (absent or reversed diastole) appears to be a poor prognostic indicator with significantly high perinatal mortality in fetuses with CH.

Time trends in the prevalence and epidemiological characteristics of neural tube defects in Liaoning Province, China, 2006-2015: A population-based study

To evaluate the time trends in the prevalence of neural tube defects and all their subtypes as well as to identify the epidemiological characteristics of these malformations documented in the Liaoning Province of northeast China from 2006 to 2015. This was a population-based observational study using data from 3,248,954 live births as well as from 6217 cases of neural tube defects, 1,600 cases of anencephaly, 2,029 cases of spina bifida, 404 cases of encephalocele, and 3,008 cases of congenital hydrocephalus from 14 cities in Liaoning Province from 2006 to 2015. All analyses were conducted using SPSS software. During the observational period, the prevalence of neural tube defects, anencephaly, spina bifida, encephalocele, and congenital hydrocephalus was 19.1, 4.9, 6.2, 1.2, and 9.3 per 10,000 live births, respectively. Significantly decreasing trends were observed in the prevalence of all these malformations except for encephalocele. Notably, relatively higher prevalence rates were found in isolated compared with non-isolated malformations, with significant differences in selected characteristics (e.g., prognosis status, gestational age, and birth weight) between isolated and non-isolated cases of these malformations. The prevalence of neural tube defects showed a downward trend in Liaoning Province from 2006 to 2015. However, more attention should be focused on non-isolated cases in the future because of the severe clinical manifestations. Future prevention efforts should be strengthened to reduce the risk of these malformations, especially the non-isolated subtype, in areas with high prevalence.

Risk Factors for Congenital Hydrocephalus

To determine potential risk factors for congenital hydrocephalus (CH) in the current era, investigators retrospectively identified cases of CH in Mississippi during a 10-year period. By reviewing diagnostic codes from the University of Mississippi, the investigators identified 3471 patients, of whom 596 had confirmed diagnoses of CH and agreed to participate in the study. The university is uniquely positioned to provide comprehensive data because …

Syrinx-fourth ventricle and lateral ventricle-peritoneal shunts for congenital hydrocephalus with syringobulbia and syringomyelia: Case report

A case of congenital hydrocephalus with syringobulbia and syringomyelia was reported. Although his neurological condition had deteriorated because of shunt malfunction, progressing syringobulbia, and syringomyelia, he recovered because of the direct communication from the syrinx in the medulla to the fourth ventricle, the shunt from the syrinx in the cervical spinal cord to the fourth ventricle, and the lateral ventriculoperitoneal shunt.

Hydrocephalus complicating lymphocytic choriomeningitis virus infection

Lymphocytic choriomeningitis virus (LCMV) disease is a human zoonotic infection acquired by contact with infected mice, hamsters or their excreta.1 The reported spectrum of LCMV-associated central nervous system (CNS) disease ranges from mild aseptic meningitis to fatal meningoencephalitis. Hydrocephalus is a rare CNS complication of viral infections but there have been a few reports of LCMV causing acquired and congenital hydrocephalus.2–5 We believe this complication may not be so rare and report two cases of LCMV-associated hydrocephalus identified within a 12-month period. The first case is a child with transient hydrocephalus complicating LCMV meningitis and the second case is a full term infant born with congenital hydrocephalus and severe chorioretinitis. To our knowledge this is the first case of congenital hydrocephalus caused by LCMV reported in the United States.

Prognostic significance of the electrically elicited blink reflex in neonates.

The electrically elicited blink reflex was examined in ten normal neonates, 11 postasphyxial neonates, and 3 congenital hydrocephalus cases. The blink reflex was elicited in all cases. In normal neonates, the latencies and amplitudes were 10.9 +/- 0.7 msec and 159 +/- 62 microV at R1, 34.3 +/- 1.4 msec and 123 +/- 30 microV at R2, and 40.7 +/- 2.3 msec and 84 +/- 25 microV at R'2 respectively. Ischemic-hypoxic brain damage during the neonatal period mainly influenced the late components of the blink reflex. The blink reflex of the postasphyxial neonates showed significantly prolonged latencies of R2 and R'2. The amplitudes were increased in cases with a fair prognosis and decreased in cases with a poor prognosis. A case of congenital hydrocephalus with mental retardation also showed the prolonged latencies of R2 and R'2 in neonatal period. The blink reflex in neonates appears to be useful in predicting the outcome in cases of neonatal asphyxia and congenital hydrocephalus.

An experimental model of communicating hydrocephalus in C57 black mouse.

The authors report a new experimental model for the study of hydrocephalus. Hydrocephalus was produced in C57 black mouse by transplanting human glioma cultured cells. 20% of the animals developed hydrocephalus while the incidence of spontaneous hydrocephalus is only 1%. The transplanted cells disappeared within 72 hours. The mouse developed progressive hydrocephalus. There was no evidence of blockage of CSF pathways. The only abnormal microscopic finding was a scattered collection of lymphoid cells in some of the animals, which may be a hypersensitivity reaction to the cells. A similarly hypersensitive reaction to the foreign maternal protein which may enter the foetal circulation is suggested as the cause of hydrocephalus in some of the cases of congenital hydrocephalus.

Global rostral midbrain dysfunction secondary to shunt malfunction in hydrocephalus.

Two cases of congenital hydrocephalus with shunt malfunction are described. Both patients presented with clinical syndromes suggestive of global rostral mesencephalic dysfunction, including the sylvian aqueduct syndrome, a parkinsonian-like state, and quadriparesis. Each had computed tomographic findings that showed marked dilatation of the lateral and 3rd ventricles, and both improved with revision of their shunts. The anatomical substrates of the clinical findings are reviewed. A discussion of the probable mechanism proposes that dilatation of the 3rd ventricle and rostral aqueduct sufficiently explains the entire syndrome. (Neurosurgery, 7: 322-325, 1980).

Diagnostic criteria and assessment of the condition in surgically arrested hydrocephalus.

Many investigators differ in opinion regarding the clinical concept and accurate criteria for the definition of surgically arrested hydrocephalus. We have studied the diagnostic criteria and various conditions of arrested hydrocephalus through the investigation of a series of 67 patients treated by shunting operation for progressive hydrocephalus. Of these, shunts were removed in 14 cases and these patients had no need for further treatment. We made investigations using neurological, morphological, neuroradiological and psychological methods, CSF dynamic studies including radioisotope cisternography and intraventricular CSF pressure measurements, and biochemical study of fatty acid in CSF. On neurological examination, squint, poor vision and motor weakness were noted in the arrested stage in a few cases. Six patients were educable but slightly retarded, while two were imbeciles. Seven patients (50 %) presented no obvious physical handicaps, however, five were slightly handicapped. Intelligence developed and the degree of physical disability gradually diminished with increasing age. Physical handicaps and residual neurological signs were correlated with the etiology of hydrocephalus. After the shunting operation head circumference remained unchanged for a period and then gradually increased within normal developmental range. Thickness of the cerebral mantle increased from 8 to 30 mm in six cases of congenital hydrocephalus. Usually, cases where thickness of the cerebral mantle were less than 14 mm within 3 months after birth, the subject had a tendency to show poor prognosis. In the arrested stage, the CSF pulse waves consisted of multiphasic components, mean intraventricular CSF pressure was found to be constant by long-term monitoring, and various RI-cisternographic patterns were observed. Three cases showed transient ventricular reflux with early clearance and convexity flow. However, in another 11 cases changes in RI-cisternographic patterns were inconsistent. Examination of fatty acid metabolism in CSF revealed that the total value of fatty acid was closest to normal range after the operation. Arrested hydrocephalus proved to exist in varying degrees as the causes of hydrocephalus vary. Studies from various points of view provided valuable data as to the analysis of arrested hydrocephalus. Diagnosis of arrested hydrocephalus cannot be made on the basis of only simple examination, but should be made by collective judgement from the results of various examinations.