Benign communicating hydrocephalus in children

Neuroradiology (Impact Factor: 2.49). 04/1981; 21(2):93-6. DOI: 10.1007/BF00342987
Source: PubMed


A review was performed of the computed tomograms (CTs) of 500 children which had been reported as showing widening of the supratentorial subarachnoid spaces with normal cerebral substance. On the basis of this a radiological diagnosis of cerebral atrophy had been made in all but five, who were said to have megalencephaly. From these, the children with large or abnormally enlarging heads, but normal or only slightly enlarged ventricles, were selected; there were 40 such cases (8%). The clinical condition either improved or remained stable over a period of 2 years; in the majority the scan abnormality regressed (22.5%) or remained static (67.5%). In three cases there was slight progression of the CT changes before stabilisation, but only one case developed classical communicating hydrocephalus necessitating a shunt procedure. This condition is a generally benign and mild form of communicating hydrocephalus, for which an aetiological factor was apparent in about two-thirds of the cases studied.

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    • "In most reported cases, there is no obvious cause of the external hydrocephalus, and it is therefore classified as idiopathic. However, it has been reported after numerous situations and conditions such as prematurity and intraventricular hemorrhage [78, 87, 101, 115, 160], meningitis [77, 87], metabolic disorders [17], steroid therapy [66], chemotherapy [54], neurosurgery [80], and trauma [77, 87]. "
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    ABSTRACT: Benign external hydrocephalus in infants, characterized by macrocephaly and typical neuroimaging findings, is considered as a self-limiting condition and is therefore rarely treated. This review concerns all aspects of this condition: etiology, neuroimaging, symptoms and clinical findings, treatment, and outcome, with emphasis on management. The review is based on a systematic search in the Pubmed and Web of Science databases. The search covered various forms of hydrocephalus, extracerebral fluid, and macrocephaly. Studies reporting small children with idiopathic external hydrocephalus were included, mostly focusing on the studies reporting a long-term outcome. A total of 147 studies are included, the majority however with a limited methodological quality. Several theories regarding pathophysiology and various symptoms, signs, and clinical findings underscore the heterogeneity of the condition. Neuroimaging is important in the differentiation between external hydrocephalus and similar conditions. A transient delay of psychomotor development is commonly seen during childhood. A long-term outcome is scarcely reported, and the results are varying. Although most children with external hydrocephalus seem to do well both initially and in the long term, a substantial number of patients show temporary or permanent psychomotor delay. To verify that this truly is a benign condition, we suggest that future research on external hydrocephalus should focus on the long-term effects of surgical treatment as opposed to conservative management.
    Neurosurgical Review 06/2011; 34(4):417-32. DOI:10.1007/s10143-011-0327-4 · 2.18 Impact Factor
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    ABSTRACT: A 5 1/2-year-old boy with congenital myopathy associated with communicating hydrocephalus is described. Motor milestones were delayed with generalized nonprogressive muscular weakness and moderately elevated serum CK. Muscle biopsy revealed multifocal areas of decreased oxidative enzyme activity which were characterized ultrastructurally by myofibrillar disorganization and Z-band streaming with decrease or absence of mitochondria in this area. The head circumference measured more than 98 percentile at 14 months. The CT scan disclosed moderately dilated ventricles and enlarged cortical sulci. Radioisotope cisternograms showed early ventricular reflux and persistence of radioactivity especially in the parasaggital region even after 72 hours. Some common pathogenetic mechanism may have been involved in the development of both the myopathy and the hydrocephalus.
    Brain and Development 02/1982; 4(6):455-62. DOI:10.1016/S0387-7604(82)80073-9 · 1.88 Impact Factor
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    ABSTRACT: This study compares the sonographic and CT findings in a group of infants with macrocrania and correlates those findings with neurologic outcome to determine the diagnostic accuracy and prognostic value of sonography. Sonographic findings in 255 infants with macrocrania are described. Of the 195 term infants examined, 130 had normal sonograms, 11 (5.6%) had significant abnormalities, and 54 had increased intra- and/or extraaxial fluid spaces. Of the 60 former preterm infants, 33 had normal sonograms, four (6.7%) had significant abnormalities, and 23 had increased fluid spaces or small resolving germinal matrix hemorrhages. The patients with significant abnormalities usually had head circumferences greater than the 95th percentile and had neurologic abnormalities. There was good correlation between sonography and CT in 30 of the 36 patients evaluated by both. In six there was mild discrepancy in the volume of the extraaxial fluid. No significant abnormality was missed by sonography. CT did not contribute any additional information. Neurologic follow-up was available for 202 patients. Nineteen percent of the term infants and 24% of the former preterm infants were abnormal on neurologic follow-up. Most patients with normal sonograms were normal on follow-up. Twelve of the term and four of the preterm infants with normal sonograms were developmentally delayed on follow-up. Increased CSF in the ventricles and/or extraaxial spaces was a common abnormality, but it usually is associated with a normal neurologic outcome and represents "benign macrocrania." We conclude that an infant with an enlarged or enlarging head should have a neurologic examination and head circumference measurement. If the patient has a head circumference greater than the 95th percentile, particularly if there are abnormal neurologic findings, further evaluation is indicated. Sonography is the initial procedure recommended since it accurately evaluates ventricular size, extraaxial fluid, and congenital malformations. If sonography is normal or shows mildly increased fluid spaces, then follow-up head circumference measurement and clinical evaluation will probably suffice. CT is indicated if there is a significant abnormality on sonography that requires further clarification.
    American Journal of Roentgenology 07/1988; 150(6):1359-65. DOI:10.2214/ajr.150.6.1359 · 2.73 Impact Factor
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