The Slit Ventricle Syndrome

It is important to distinguish between the radiological "label" of slit ventricles, a not uncommonly seen appearance on post-shunt CT scans where the ventricles are barely recognisable but the patient is free of symptoms, and the less frequently encountered clinical symptom complex that may accompany slit-like ventricles. It has been estimated that only 11% of patients with radiologically confirmed slit ventricles demonstrated the clinical syndrome. The clinical syndrome is usually one of episodic headache, which may be positional, vomiting, occasionally with vague gastrointestinal symptoms and the reservoir, if present, may be slow to refill. The symptoms will frequently have a cyclical pattern, episodes lasting from between a few hours to 2 or 3 days, the individual being quite well in between "attacks".

It is postulated that the condition results from a loss of ventricular wall compliance. The small-volume ventricles intermittently collapse around the catheter, temporarily blocking it. Intracranial pressure has to build up in order to distend the non-compliant ventricles; during this period symptoms will be present. Once the ventricle begins to expand, the catheter can again begin to function and symptoms subside.

The syndrome may be accompanied by either low or high intracranial pressure and differentiating these is frequently difficult on clinical grounds alone. A period of ICP monitoring may be a useful aid in the diagnosis and may guide subsequent treatment [10].

If ICP is low then the therapeutic options include upgrading the valve or insertion of an anti-siphon device. Such maneuvres may be associated with re-expansion of the previously collapsed ventricle.

In the presence of raised ICP, clearly it is essential to establish that the shunt is patent. If this is so and symptoms persist, then subtemporal decompression may afford relief. The removal of bone, usually ipsilateral, to the shunt removes some of the constraint upon ventricular dilation, improving compliance and permitting focal expansion of the ventricular cavity. This has been reported to improve symptoms and reduce the number of subsequent shunt-related problems [15].

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