Unusual clinical manifestations of disturbed CSF dynamics in hydrocephalic children

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.

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.

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.