Nationwide Survey of the Etiology and Associated Conditions of Prenatally and Postnatally Diagnosed Congenital Hydrocephalus in Japan

Moritake, Kouzo; Nagai, Hidemasa; Miyazaki, Takeshi; Nagasako, Noriko; Yamasaki, Mami; Tamakoshi, Akiko

doi:10.2176/nmc.47.448

Cited by 17 publications

(8 citation statements)

References 15 publications

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“…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 CSF within a split layer of arachnoid 31 .…”

Section: Discussionmentioning

confidence: 99%

Two Hundred Thirty-Six Children With Developmental Hydrocephalus: Causes and Clinical Consequences

et al. 2015

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Few systematic assessments of developmental forms of hydrocephalus exist. We reviewed 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 one of five subtypes and compared on the basis of clinical characteristics, developmental and surgical outcomes. At an average age of 5.3 years, 72% of children were walking independently and 87% could eat by mouth. 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. 53% of surgically-treated patients experienced at least one 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.

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Section: Discussionmentioning

confidence: 99%

Two Hundred Thirty-Six Children With Developmental Hydrocephalus: Causes and Clinical Consequences

et al. 2015

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“…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.…”

Section: Hydrocephalus Accompanied By Other Physical Features (Table 2)mentioning

confidence: 99%

Infantile hydrocephalus: A review of epidemiology, classification and causes

Tully¹,

Dobyns²

2014

European Journal of Medical Genetics

316

246

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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.

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“…In this study, 75% of patients suffering from congenital hydrocephalus did not have any associated central nervous abnormalities. A nationwide survey for aetiology of congenital hydrocephalus in Japan also showed that more than 90% of cases with congenital hydrocephalus were not associated with any other central nervous system pathologies [7]. …”

Section: Discussionmentioning

confidence: 99%

CSF Shunt Complications in Infants – An Experience from Pakistan

Bakhsh

2011

Pediatr Neurosurg

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Aims and Objective: The main aim of the study was to find out the postoperative complications and long-term outcome of cerebrospinal fluid-shunted patients in an infantile age group with a poor socioeconomic background. Material and Methods: A prospective study of 100 cases of infantile hydrocephalus was conducted at Pakistan Institute of Medical Sciences, Islamabad, Pakistan. All selected patients suffering from congenital and post-meningitic hydrocephalus were operated on for ventriculoperitoneal shunt. However, cases of hydrocephalus secondary to myelomeningocele were not included. Patients were followed postoperatively for 2 years. Prior consent for surgery was taken from all parents and the study was approved by the hospital’s academic and ethical council. Results: The majority of patients were brought quite late to the medical facilities because of social taboos and socioeconomic conditions. Early signs and symptoms of hydrocephalus were either ignored or misunderstood. Shunt infection and shunt failure rates remained at 14 and 10%, respectively. 50% of shunt blocks occurred in the first postoperative year and another 50% within 2 years. The highest percentage (40–45%) of death occurred in patients suffering from congenital hydrocephalus, in the first postoperative year. Conclusions: This study concluded that the outcome and prognosis of shunted infantile hydrocephalus in developing countries is suboptimal due to different socioeconomic conditions. However, under such circumstances, careful selection of patients, timely intervention and adherence to meticulous aseptic surgical techniques can alter the prognosis of cerebrospinal fluid-shunted infants.

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Nationwide Survey of the Etiology and Associated Conditions of Prenatally and Postnatally Diagnosed Congenital Hydrocephalus in Japan

Cited by 17 publications

References 15 publications

Two Hundred Thirty-Six Children With Developmental Hydrocephalus: Causes and Clinical Consequences

Two Hundred Thirty-Six Children With Developmental Hydrocephalus: Causes and Clinical Consequences

Infantile hydrocephalus: A review of epidemiology, classification and causes

CSF Shunt Complications in Infants – An Experience from Pakistan

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