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A nationwide survey in 2000 investigated the causative and associated central nervous system (CNS) lesions of congenital hydrocephalus in Japan. The etiology and associated diseases in 393 patients with congenital hydrocephalus were analyzed and compared between 193 patients with prenatally diagnosed (fetal) hydrocephalus and 181 with postnatally d...
Context in source publication
Context 1
... Redmond, Wash., U.S.A.) and SPSS (version 8.0 for Windows; SPSS, Chicago, Ill., U.S.A.) with significance accepted at the 5% level. Tables 1 and 2 show the results of etiological classification of the 355 (90.3%) patients with primary hydrocephalus and the 28 (7.1%) patients with secondary hydrocephalus, respectively. The remaining 10 patients (2.5%) had unknown etiology. ...Similar publications
Rationale:
Holoprosencephaly (HPE) is a severe congenital brain malformation resulting from failed or incomplete forebrain division in early pregnancy.
Patient concerns:
In this study, we reported a 9-month old infant girl with mild microcephaly, semilobor HPE, and arachnoid cyst.
Diagnoses:
Potential genetic defects were screened directly usi...
Citations
... In a previous study, CH was found to be present with other congenital anomalies in as many as 44% of cases 6 CH was also shown to be associated with other CNS anomalies in previous studies. 24,25 Our results show increased odds of CH in children with other congenital anomalies, supporting these previous findings. It can also be attributed to syndromic hydrocephalus, which may be associated with intra-or extracranial anomalies. ...
OBJECTIVE
Hydrocephalus is the most common brain disorder in children and is more common in low- and middle-income countries. Research output on hydrocephalus remains sparse and of lower quality in low- and middle-income countries compared with high-income countries. Most studies addressing hydrocephalus epidemiology are retrospective registry studies entailing their inherent limitations and biases. This study aimed to investigate child-related, parental, and socioeconomic risk factors of congenital hydrocephalus (CH) in a lower-middle-income country.
METHODS
An investigator-administered questionnaire was used to query parents of patients with CH and controls who visited the authors’ institution from 2017 until 2021. Patients with secondary hydrocephalus and children older than 2 years of age at diagnosis were excluded. Uni- and multivariable logistic regression was performed to identify the factors affecting CH development.
RESULTS
Seven hundred forty-one respondents (312 cases and 429 controls) were included in this study. The authors showed that maternal diseases during pregnancy (OR 3.12, 95% CI 1.96–5.03), a lack of periconceptional folic acid intake (OR 1.92, 95% CI 1.32–2.81), being a housewife (OR 2.66, 95% CI 1.51–4.87), paternal illiteracy (OR 1.65, 95% CI 1.02–2.69), parental consanguinity (OR 3.67, 95% CI 2.40–5.69), a history of other CNS conditions in the family (OR 2.93, 95% CI 1.24–7.34), conceiving a child via assisted fertilization techniques (OR 3.93, 95% CI 1.57–10.52), and the presence of other congenital anomalies (OR 2.57, 95% CI 1.38–4.87) were associated with an independent higher odds of a child having CH. Conversely, maternal hypertension (OR 0.22, 95% CI 0.09–0.48), older maternal age at delivery (OR 0.93, 95% CI 0.89–0.97), and having more abortions (OR 0.80, 95% CI 0.67–0.95) were negatively correlated with CH.
CONCLUSIONS
Multiple parental, socioeconomic, and child-related factors were associated with higher odds for developing CH. These results can be utilized to guide parental counseling and management, and direct social education and prevention programs.
... A large survey of the aetiology of congenital hydrocephalus indicated that agenesis of the corpus callosum was a relatively common cause of foetal and to a lesser extent infantile hydrocephalus [74]. In a large literature review encompassing 705 cases of agenesis of the corpus callosum (ACC) hydrocephalus was present in some 23%, often associated with distinct syndromic states (Aicardi, Acrocallosal, Andermann and Shapiro syndromes) [75]. ...
... 13 La incidencia real de la hidrocefalia en el mundo es difícil de determinar por las diferencias en su definición etiológica y los diferentes criterios de inclusión y exclusión, así como su prevalencia en diferentes partes del planeta. 4,[86][87][88][89][90] Investigaciones recientes en la población sueca establecen un decrecimiento de la incidencia de hidrocefalia, 0.82 x 1 000 nacidos vivos en 1989-1998 versus 0.66 x 1 000 nacidos vivos entre 1999-2002, principalmente resultado de la disminución de los mielomeningoceles por la profilaxis con ácido fólico. Por otra parte, otros estudios reportan que no existen cambios significativos en la misma con reportes de hidrocefalia congénita en rangos de 1 x 1 000 nacidos vivos en diferentes países. ...
... Subsecuentemente, el rango de hidrocefalia asociado a la etiología congénita disminuye, en cambio el asociado a las causas tumorales aumenta; esta es la primera causa de hidrocefalia en el grupo I DVP de nuestro estudio, lo cual está en relación con estos estudios internacionales. 6,86,97,98 Es de señalar la alta incidencia de hidrocefalia en los países en desarrollo debido a la frecuencia muy alta de enfermedades congénitas y malformaciones, así como formas perinatales adquiridas en relación con cuidados perinatales deficientes. 4, 90, 99 ...
Resumen
La hidrocefalia es la entidad médica más frecuente que enfrentan los neurocirujanos pediátricos; constituye 40% de sus intervenciones quirúrgicas. Con el objetivo de diseñar un algoritmo que contribuya a decidir la mejor opción terapéutica en el paciente pediátrico con hidrocefalia tratada por derivación ventrículoperitoneal (DVP) y por ventriculocisternostomía endoscópica (VCE), se realizó una investigación de desarrollo, teniendo como punto de partida un estudio observacional, explicativo y prospectivo. El universo fue de 538 pacientes. La muestra quedó constituida por 188 pacientes, la cual se subdividió acorde con el patrón imagenológico y tratamiento aplicado en: grupo I con hidrocefalia comunicante y no comunicante, tratado por DVP y el grupo II con hidrocefalia no comunicante triventricular tratado por VCE con 112 y 76 pacientes, respectivamente, el grupo I DVP presentó fallo de la técnica en 63% de los tratados, con índice de sepsis de 22% y las complicaciones ocurrieron en 75% de los niños. El grupo II VCE presentó fallo en 38,1% de los pacientes, existieron complicaciones en 10,5% y el índice de sepsis fue de 3,5%. Ambas técnicas quirúrgicas son válidas en el tratamiento de la hidrocefalia, estando limitada la VCE a la hidrocefalia no comunicante triventricular. El puntaje de éxito de la VCE (PEVCE) fue predictivo en los resultados de esta y el algoritmo para la hidrocefalia pediátrica tratada por DVP y VCE permite seleccionar a los pacientes con indicación para cada técnica, basada en las características clínicas, estudios de imagen y el PEVCE.
... Northern China, which has historically had a high prevalence of NTDs (Li et al. 2006), as high as 31.5/10,000 in the study population in 2014 and it has been declining over time (Liu et al. 2016a). A study found that three fourths of CH cases were complicated with NTDs (Moritake et al. 2007). Therefore, it is reasonable to speculate that a high prevalence of CH may exist in populations with a high prevalence of NTDs and supplementation with folic acid may help decrease the prevalence of CH at the population level. ...
... Northern China, which has historically had a high prevalence of NTDs (Li et al. 2006), as high as 31.5/10,000 in the study population in 2014 and it has been declining over time (Liu et al. 2016a). A study found that three fourths of CH cases were complicated with NTDs (Moritake et al. 2007). Therefore, it is reasonable to speculate that a high prevalence of CH may exist in populations with a high prevalence of NTDs and supplementation with folic acid may help decrease the prevalence of CH at the population level. ...
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.
... A large survey of the aetiology of congenital hydrocephalus indicated that agenesis of the corpus callosum was a relatively common cause of foetal and to a lesser extent infantile hydrocephalus [74]. In a large literature review encompassing 705 cases of agenesis of the corpus callosum (ACC) hydrocephalus was present in some 23%, often associated with distinct syndromic states (Aicardi, Acrocallosal, Andermann and Shapiro syndromes) [75]. ...
The term Hydrocephalus relates to the presence of an excessive amount of cerebrospinal fluid (CSF), which may cause an increase in intracranial pressure with or without associated abnormal enlargement of the cerebral ventricles. Hydrocephalus is not a single pathological disease entity rather it is secondary to a variety of pathological processes or insults that cause an imbalance between the production and absorption of CSF.
... Cysts and cephaloceles are known causes of hydrocephalus, 29,30 but the pathogenesis of these malformations is poorly understood. Simple cysts have been attributed to accidental entrapment of cerebrospinal fluid within a split layer of arachnoid. ...
Few systematic assessments of developmental forms of hydrocephalus exist. We reviewed magnetic resonance images (MRIs) and clinical records of patients with infancy-onset hydrocephalus. Among 411 infants, 236 had hydrocephalus with no recognizable extrinsic cause. These children were assigned to 1 of 5 subtypes and compared on the basis of clinical characteristics and developmental and surgical outcomes. At an average age of 5.3 years, 72% of children were walking independently and 87% could eat by mouth; in addition, 18% had epilepsy. Distinct patterns of associated malformations and syndromes were observed within each subtype. On average, children with aqueductal obstruction, cysts, and encephaloceles had worse clinical outcomes than those with other forms of developmental hydrocephalus. Overall, 53% of surgically treated patients experienced at least 1 shunt failure, but hydrocephalus associated with posterior fossa crowding required fewer shunt revisions. We conclude that each subtype of developmental hydrocephalus is associated with distinct clinical characteristics, syndromology, and outcomes, suggesting differences in underlying mechanisms.
© The Author(s) 2015.
... Intracranial arachnoid cysts are a well-recognized cause of hydrocephalus [62,63]. Small, simple cysts have been attributed to an entrapment of CSF within the meninges [64], which could conceivably be a developmental accident. ...
Hydrocephalus is a common but complex condition caused by physical or functional obstruction of CSF flow that leads to progressive ventricular dilatation. Though hydrocephalus was recently estimated to affect 1.1 in 1,000 infants, there have been few systematic assessments of the causes of hydrocephalus in this age group, which makes it a challenging condition to approach as a scientist or as a clinician. Here, we review contemporary literature on the epidemiology, classification and pathogenesis of infantile hydrocephalus. We describe the major environmental and genetic causes of hydrocephalus, with the goal of providing a framework to assess infants with hydrocephalus and guide future research.
... The real incidence of paediatric hydrocephalus is hard to assess because of the divergences in the definition of the aetiology and the exclusion/inclusion criteria and in the prevalence in the different geographic areas [4,8,18,20,23,26,28]. Therefore, only a little number of studies addressing this topic is available in the literature when the changes of epidemiology are concerned. ...
... Subsequently, the rate of hydrocephalus associated to congenital aetiologies dropped, while that associated to tumours significantly increased (see Table 1). These results fit those from similar series in the literature [11,18,30] and corroborate data from the population-based studies. According to the aforementioned Swedish studies [25,26], indeed, the prevalence of hydrocephalus associated to myelomeningocele fell from 0.33/1,000 live births in 1989-1998 period to 0.18/1,000 live birth in the 1999-2002 period as result of the improved prenatal diagnosis and assistance. ...
The purpose of this study is to evaluate the changing epidemiology of paediatric hydrocephalus over the past three decades in a single institution.
All children treated for newly diagnosed hydrocephalus during the 1985-1990 (group A) and the 2000-2005 periods (group B) were enrolled and classified according to the associated cause of hydrocephalus.
A significant 8.8% decrease of the incidence of hydrocephalus was noticed between the two time periods, resulting from the reduction of hydrocephalus associated to myelomeningocele, aqueduct stenosis (p = 0.04), CNS infection (p = 0.03), cranio-cerebral malformation and head injuries; post-haemorrhagic hydrocephalus remained stable, while the tumour-associated one increased (p < 0.0001). No consistent differences in terms of rate of adjunctive surgery (30.3% versus 23.9%) and number revision procedures (200 versus 104) were recorded.
The present study confirmed data from the literature about the declined incidence of paediatric hydrocephalus, which mainly results from the decrease of congenital malformations. In spite of the recent advances in neuroendoscopy and in the shunting valve design, the impact of hydrocephalus in the paediatric neurosurgical practice remains high.
The writings about large hydrocephalic infant skulls in Egyptian medical literature can be found as early as 500 AD. A more scientific description is, however, credited to Hippocrates who coined the term “hydrocephalus” derived from the Greek word “hydro” meaning water and “kefale” meaning skull and described it as liquefaction of the brain with fluid accumulation surrounding the brain. The definition of hydrocephalus has since undergone several modifications. Simplistically, it was defined as the presence of dilated ventricles with an increased volume of intracranial cerebrospinal fluid (CSF). Hydrocephalus us the commonest neurosurgical condition in infants and children, and the CSF diversion procedures are the mainstay of management. Grossly hydrocephalic children in emergency present challenges due to associated congenital anomalies, macrocephaly, difficult airway, and a full stomach. This chapter provides a comprehensive review of CSF hydrodynamics, CSF diversion procedures, and anesthetic concerns in children with hydrocephalus.
