Unusual clinical manifestations of disturbed CSF dynamics in hydrocephalic children |
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Authors: | Rachid Azeddine Bech Marianne Juhler |
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Affiliation: | (1) University Clinic of Neurosurgery, NK2092, Rigshospitalet, Blegdamsvej 9, 2100 Copenhagen, Denmark, DK |
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Abstract: | Objects: Clinical symptoms and signs of increased intracranial pressure (ICP) may be nonspecific and unreliable, or even entirely
absent, in hydrocephalic infants and children. Even with a radiological examination, it is often difficult to distinguish
between ”arrested hydrocephalus” and slowly progressive hydrocephalus requiring treatment. Methods: We present two cases with unusual and initially misleading clinical manifestations of increased ICP. In both cases, the disturbed
cerebrospinal fluid (CSF) dynamics, i.e. raised ICP, were not recognised until demonstrated by a long-term ICP monitoring.
In a 5-month-old boy with normal head circumference and normal psychomotor development, the sudden onset of episodes of torticollis
and screaming were the only symptoms. No pathology underlying the developing hydrocephalus and the raised ICP could be established,
but the boy’s condition improved after a shunt operation. In the other case, symptoms and signs consisted primarily in a slowly
progressive dilatation of the facial veins with onset at the age of 2–3 years. As the girl also presented a congenital subvalvular
aortic stenosis, the venous congestion was initially thought to reflect a vena cava superior syndrome. Further radiological
examinations, however, revealed an extensive sinus thrombosis underlying the raised ICP. The girl underwent shunt insertion,
and the venous congestion was reduced. Conclusions: The cases illustrate that though clinical and radiological findings may be very doubtful, or unusual for increased ICP, direct
diagnostic long-term ICP monitoring should always be contemplated. Only ICP monitoring can reveal with certainty whether disturbed
CSF dynamics are involved, requiring a CSF diverting operation to treat and stabilise the condition.
Received: 6 December 1999 |
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Keywords: | Infants Hydrocephalus Increased ICP Clinical findings ICP monitoring Shunts |
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