2015
DOI: 10.1177/0883073815592222
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Two Hundred Thirty-Six Children With Developmental Hydrocephalus: Causes and Clinical Consequences

Abstract: 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 pat… Show more

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Cited by 34 publications
(22 citation statements)
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“…Various etiologies of fetal ventriculomegaly have been demonstrated, including acquired causes such as intraventricular hemorrhage, infection, or neoplasms. 4,5 When these have been excluded, radiologists must carefully assess MR images to identify additional brain abnormalities that may suggest a developmental/malformative cause of the hydrocephalus. 9 DMJ dysplasia is a rare malformation first described in 6 children born from 3 consanguineous Egyptian families, with facial dysmorphisms, severe cognitive impairment, axial hypotonia, spastic quadriparesis, vasomotor instability, unexplained fever, and seizures.…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…Various etiologies of fetal ventriculomegaly have been demonstrated, including acquired causes such as intraventricular hemorrhage, infection, or neoplasms. 4,5 When these have been excluded, radiologists must carefully assess MR images to identify additional brain abnormalities that may suggest a developmental/malformative cause of the hydrocephalus. 9 DMJ dysplasia is a rare malformation first described in 6 children born from 3 consanguineous Egyptian families, with facial dysmorphisms, severe cognitive impairment, axial hypotonia, spastic quadriparesis, vasomotor instability, unexplained fever, and seizures.…”
Section: Discussionmentioning
confidence: 99%
“…Several causes of developmental hydrocephalus have been described in fetuses and children, mostly associated with myelomeningocele, aqueductal obstruction, posterior fossa crowding, and cysts or cephaloceles. [4][5][6] To the best of our knowledge, no imaging features of DMJ dysplasia have been described in fetuses with developmental ventriculomegaly. Here, we have reported the second-trimester prenatal MR imaging of 13 fetuses with moderateto-severe obstructive ventriculomegaly presenting unequivocal features of DMJ dysplasia, and we correlated the imaging with available pathology and/or postnatal data.…”
mentioning
confidence: 98%
“…The risk factors observed in our study were decided after reviewing the literature. [3][4][5][6][7] Low socioeconomic status was defined as per the Kuppuswamy scale. 13 Management of both mother and child was tailored according to the gestational age and associated medical conditions.…”
Section: Exclusion Criteriamentioning
confidence: 99%
“…The etiology of hydrocephalus is multifactorial which includes presence of intracranial malformations (neural tube defects, aqueductal stenosis, etc), genetic mutations, hemorrhage, infections, and tumors. [3][4][5] Several risk factors such as low birth weight, prematurity, gestational diabetes mellitus, and ethnicity have been implicated in its etiopathogenesis. 6,7 Ultrasonography is the gold standard for diagnosing congenital hydrocephalus.…”
mentioning
confidence: 99%
“…In communicating (non-obstructive) hydrocephalus, the four interconnected cavities, or ventricles, expand with increasing fluid pressure, and secondary cerebral abnormalities ensue (McAllister, 2012). Congenital or infantile onset hydrocephalus is highly heterogeneous in etiology, with a prevalence of 1 in 1,000 births (Jeng, Gupta, Wrensch, Zhao, & Wu, 2011; Tully et al, 2016). Genetic etiologies of congenital hydrocephalus are rarely identified and include mutations in L1CAM , AP1S2 , and POMT1 (Tully & Dobyns, 2014).…”
Section: | Introductionmentioning
confidence: 99%