Changes in Intracranial Pressure after Calvarial Expansion Surgery in Children with Slit Ventricle Syndrome

Abstract

The effect of calvarial expansion on symptom relief and intracranial pressure (ICP) in three children with slit ventricle syndrome (SVS) and intracranial hypertension despite a functioning ventricular shunt is reported. These children presented with a clinical picture of SVS, accompanied by slit-like ventricles on cranial computer tomography scan and intracranial hypertension. Calvarial expansion was performed by mans of an anterior approach in one case and a posterior approach (modified tiara plastic) in the other two cases. After calvarial expansion, symptoms of intracranial hypertension were abolished in one case and markedly reduced in two cases (observation period 25–36 months). Comparison of ICP before and after surgery was performed by means of new software (Sensometrics™ Pressure Analyser, version 1.2) that revealed a significant reduction in the number of abnormal ICP elevations after surgery. The results were not accompanied by changes in the size of the cerebral ventricles. This study demonstrates that in children with SVS and intracranial hypertension despite a functioning shunt, calvarial expansion may reduce ICP and produce long-lasting symptom relief. In these cases, we suggest that intracranial hypertension was caused by compromised intracranial volume.

The Problematic Slit Ventricle Syndrome

“…The key to good outcomes appears to be establishing the clinical pattern by a good history and examination (in particular evidence of synostosis) and instituting therapy accordingly [3, 4, 6, 16, 17, 18]. …”

Cranial vault expansion by distraction osteogenesis

“…20,35 Classically these procedures were done in one stage and the reported complications include, but are not limited to, infection, unsatisfactory cosmetic outcome, seizures, and permanent adverse impact on neurological function. 18,35 Distraction osteogenesis is the surgical induction of bone growth by applying distractive force across an osteotomy site, and this technique is becoming more common for expanding the cranium and for advancing the midface. 2,14,15,22,29,31,33,38,40,42,49 Distraction osteogenesis is a historical outgrowth of various methods of fracture reduction and retention that date back almost 2000 years.…”

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

“…He also suggested appropriate modes of therapy such as medication, shunt removal or revision, valve upgrading, LP shunting, and cranial expansion surgery according to his classification. But there still exists controversy over which mode is the most efficacious since most studies to date have not objectively compared the various methods and only presented successful results of each mode independently [5,[8][9][10][11][19][20][21] . It would seem appropriate to perform cranial expansion surgery, including decompression surgery, in cases where after brain development restriction develops as a result of secondary craniosynostosis after shunting [19,20] .…”