Hypertensive slit ventricle syndrome: pseudotumor cerebri with a malfunctioning shunt?

slit ventricles

J Neurosurg 119:1503–1510, 2013

Symptomatic shunt malfunction without ventricular enlargement is known as slit ventricle syndrome (SVS). Patients presenting with this syndrome are not a homogeneous group. Of the 5 different types classified by Rekate, Type 1 is caused by CSF overdrainage and is associated with low pressures; Types 2 and 3 are associated with shunt blockage and elevated CSF pressures; Type 4 is cephalocranial disproportion that increases brain parenchymal pressure but not CSF pressure; and Type 5 is headache unrelated to shunt function. The low and normal CSF pressure types are relatively well understood, but the high-pressure forms are more problematic. In the high-pressure forms of SVS it is said that the lack of ventricular dilation is related to a reduction in brain compliance analogous to idiopathic intracranial hypertension or pseudotumor cerebri. Despite this, there is little evidence in the literature to support this conjecture.

With this in mind, 3 cases of SVS associated with elevated CSF pressure are presented. The MR venogram findings and hemodynamics of these 3 cases are shown to be identical to those of pseudotumor cerebri. A literature review indicates that an underlying venous impairment may be functioning in the patients who re-present with small ventricles following shunt malfunction.

Prevention of ventricular catheter obstruction and slit ventricle syndrome by the prophylactic use of the Integra antisiphon device in shunt therapy for pediatric hypertensive hydrocephalus: a 25-year follow-up study

J Neurosurg Pediatrics 5:4–16, 2010.(DOI: 10.3171/2008.7.17690)

This 25-year follow-up study was performed on 120 children with hypertensive hydrocephalus to evalu- ate the influence of the early prophylactic implantation of the Integra antisiphon device (ASD, Integra Neurosciences Ltd.) on the rate of proximal shunt obstructions and the frequency of symptomatic slit ventricle syndrome (SVS). The adaptability of the ASD to growth, proper positioning of the ASD as a necessity for its successful performance, and the 3 phases of SVS development are discussed.

Method. Since 1978, the ASD has consistently been implanted either at the time of primary shunt insertion (66 neonates, mean follow-up 11 years) or during revisions of preexisting shunts (54 children, mean follow-up 11.8 years). The complication rate among the 54 children before ASD implantation (mean follow-up 8.3 years) was com- pared with that among all 120 patients once an ASD had been inserted. Shunt complications were documented as ventricular catheter, distal catheter, and infectious complications.

Results. The study revealed a significant long-term reduction in ventricular catheter obstructions and hospitaliza- tions due to intermittent intracranial hypertension symptoms (symptomatic SVS) after both primary and secondary ASD implantation. Data in the study suggest that the high rate of ventricular catheter obstruction in pediatric shunt therapy is caused by hydrostatic suction induced by differential-pressure valve shunts during mobilization of the patient and that the development of a SVS can be traced back to this constant suction, which causes chronic CSF overdrainage and ventricular noncompliance. Recurrent ventricular catheter obstruction and SVS can be prevented by prophylactic supplementation of every shunt system with an ASD.

Conclusions. To inhibit chronic hydrostatic suction, to prevent overdrainage and proximal shunt obstruction, and to avoid SVS and thus improve the patient’s quality of life, the prophylactic implantation of an ASD in every pediatric hydrocephalus shunt is recommended.