Unusual clinical manifestations of disturbed CSF dynamics in hydrocephalicchildren

Objects:Clinical symptoms and signs of increased intracranial pressure (ICP ) may be nonspecific and unreliable, or even entirely absent, in hydrocepha lic infants and children. Even with a radiological examination. it is often difficult to distinguish between "arrested hydrocephalus" and slowly progr essive hydrocephalus requiring treatment. Methods: We present two cases wit h unusual and initially misleading clinical manifestations of increased ICP . In both cases, the disturbed cerebrospinal fluid (CSF) dynamics, i.e. rai sed ICP, were not recognised until demonstrated by a long-term ICP monitori ng. In a 5-month-old boy with normal head circumference and normal psychomo tor development, the sudden onset of episodes of torticollis and screaming were the only symptoms. No pathology underlying the developing hydrocephalu s 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 p rimarily 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 subvalvula r aortic stenosis, the venous congestion was initially thought to reflect a vena cava superior syndrome. Further radiological examinations, however, r evealed an extensive sinus thrombosis underlying the raised ICP. The girl u nderwent shunt insertion, and the venous congestion was reduced. Conclusion s: 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 reve al with certainty whether disturbed CSF dynamics are involved, requiring a CSF diverting operation to treat and stabilise the condition.