Lumbar CSF Shunting Preferentially Drains the Cerebral Subarachnoid over the Ventricular Spaces: Implications for the Treatment of Slit Ventricle Syndrome

Abstract

Based on a proposed pathophysiology of slit ventricle syndrome (SVS), we have hypothesized that lumboperitoneal shunting exerts effects in SVS patients by increasing the buffering capacity for raised intracranial pressure (ICP) via an increase in cerebrospinal fluid drainage from the cerebral subarachnoid space (SAS). We describe 3 SVS patients with patent lumbar subarachnoid drainage but under-functioning ventriculoperitoneal shunts (VPS) who presented with ventriculomegaly (not SVS), and persistence of shunt malfunction like symptoms. Revision of the VPS resulted in complete resolution of symptoms despite a finding of low pressure in the ventricular space. This supports the hypothesis that lumboperitoneal shunting preferentially drains the SAS over the intraventricular space and in these cases allows the ‘SVS’ ventricles to enlarge by creating a pressure gradient from ventricles to SAS through the cortical mantle.

The Slit Ventricle Syndrome: Advances Based on Technology and Understanding

“…There is a major difference between the management scheme proposed by Le et al [17] , which is philosophically refl ected in the article by Khorasani et al [20] and the one that we have used. Le et al [17] used the LP shunt as an adjunct to ventricular shunting; we obtain an additional study before placing an LP shunt.…”

“…At the 2001 Annual Meeting of the Joint Section on Pediatric Neurological Surgery, the senior author of this paper [20] (D.M.F.) and I, without prior knowledge of each other's work, made back-to-back presentations on the use of LP shunts for the management of SVS.…”

Successful treatment of post-shunt craniocerebral disproportion by coupling gradual external cranial vault distraction with continuous intracranial pressure monitoring

“…3,6,8,15,17 Thus, we argue, the compromised cranial compliance inherent in patients with CCD can be clearly captured by the presence of plateau waves on ICP monitoring.…”

Valve Pressure Upgrade May Produce Progressive Deterioration of Vision in Children with Slit Ventricle Syndrome

“…In patients with PC it has been suggested that in order to avoid brain injury due to the small size of the ventricles, CSF drainage should be conducted by an LP shunt rather than a VP shunt [5,7,8] . However, some authors have advocated the VP shunt as LP shunts may lead to possible herniation complications, Chiari malformation development, particularly in children, and also because LP shunts have a higher failure rate after long-term follow-up [4,19] .…”

“…In particular in a child with SVS, which develops after VP shunting for the treatment of pseudotumor cerebri (PC), the LP shunt is most commonly employed [5,7,8] . However, due to the possible problem of herniation and frequent malfunction with LP shunts in children, it has recently been suggested by some authors that a valve upgrade and third ventriculostomy may also be an efficacious alternative mode of therapy [9][10][11] .…”