Management of Slit Ventricle Syndrome in Children

Initial Management at Presentation

  • ICP monitoring: ICP monitoring is a good starting point in evaluating a child with small ventricles, headaches, and no evidence of a shunt malfunction, as it can differentiate high from low ICPs. In one series more than half of children monitored had intracranial hypotension (16).
  • Shunt revision or ETV: High ICP will require shunt revision surgery (often difficult with small ventricles) (7), the addition of another shunt type (lumboperitoneal or cisterna magna), or shunt externalization with attempts to increase ventricular size and perform an ETV with the goal of eventual shunt removal.
  • Decompressive craniectomy: Management of refractory elevated ICP with a decompressive craniectomy has been successful in several small series (18, 19).
  • Revision of valve: Low ICPs require hydration, medication, adjustment of a programmable shunt, or the addition of an antisiphon component to the distal shunt. An abdominal binder is an option for refractory low-pressure symptoms (22).

Adjunctive Therapies

  • Headache management: Headache management is indicated and possible consultation with a neurologist when intracranial hypotension, hypertension, and/or shunt malfunction have been ruled out.
  • Steroids: Short-term steroids have been reported to be useful in transiently improving symptomatic complaints and delaying the need for surgery (25).

Follow-up

  • Routine hydrocephalus follow-up: Follow-up is essential in the clinic for imaging of shunt and ventricles, especially if the shunt has been removed and an ETV has been performed.