Craniocerebral disproportion: a topical review and proposal toward a new definition, diagnosis, and treatment protocol.

INTRODUCTION: At some point in their lives, patients previously shunted for hydrocephalus may experience chronic, debilitating headaches, despite the fact that their shunts are functioning properly. Previously published reports have suggested that a subset of these patients may be suffering from an iatrogenic craniocerebral disproportion (CCD) and, therefore, may benefit from procedures that expand the available intracranial space. A unified definition of this disorder, however, is lacking. DISCUSSION: Here, the authors chart the history (including historical terminology) of CCD, review its incidence, describe its signs, symptoms, and associated radiologic findings, and expound upon its pathophysiology. Next, a concise clinical definition of CCD based on the temporal correlation of headaches with the appearance of plateau waves on intracranial pressure (ICP) monitoring is proposed. The authors conclude with a discussion of the various therapeutic strategies employed previously to treat this disorder and present their individualized treatment strategy based upon the simultaneous utilization of ICP monitors and gradual external cranial vault expansion.

Steady-state indicators of the intracranial pressure dynamic system using geodesic distance of the ICP pulse waveform.

Normal functioning of the brain depends on the homeostasis (∼ steady state) of its various physiological sub-systems, one of which is the intracranial pressure (ICP) dynamic system. The ICP dynamic system of an injured brain is susceptible to various acute changes that should ideally be detected by ICP monitoring even for comatose patients. However, the status quo of ICP monitoring solely targets mean ICP. We aimed to demonstrate a novel approach to detect acute deviation from steady state of an ICP dynamic system in an absence of significant mean ICP changes. We hypothesized that steady state of ICP dynamic systems is reflected as ICP pulses of similar mean ICP levels resembling each other for a given subject. A general framework was used to derive such a steady-state indicator that can accommodate different metrics of inter-pulse distance and different statistics of the distance histograms. In addition to conventional Euclidean distance and Pearson correlation, geodesic distance between pulses was introduced as a novel metric. These different ways of calculating steady-state indicators under the proposed framework were evaluated on three types of continuous ICP recordings: (1) those between two consecutive brain imaging studies that demonstrated acute ventricular enlargement for slit ventricle syndrome (SVS) patients undergoing a trial of shunt externalization and clamping (SVS+); (2) those between consecutive brain imaging studies from the SVS patients under the same trial but without ventricular enlargement (SVS-); (3) overnight recordings from normal pressure hydrocephalus (NPH) patients. It was observed that only the standard deviation of geodesic distance correctly differentiated between SVS+ and SVS- and between SVS+ and NPH while avoiding discriminating between SVS- and NPH. It was also found that 45% SVS+ cases had a multimodal geodesic distance histogram while none of SVS- and 3.8% of NPH cases had such a multimodal histogram. Pulses with a large number of distant pulses for the five multimodal-histogram SVS+ cases fell in short time windows indicating that acute ventricular changes may have occurred in these confined time windows during which no significant changes of mean ICP were observed. In contrast, the pulses with a large number of distant pulses for the two multimodal-histogram NPH cases did not cluster temporally. In conclusion, the geodesic inter-pulse distance is a promising metric to quantify distance intrinsic to the underneath geometric structure of ICP signals and hence is a more suitable way to derive a steady-state indicator of an ICP dynamic system.

Analysis of therapeutic choices for slit ventricle syndrome after cyst-peritoneal shunting for temporal arachnoid cysts in children.

OBJECT: The goal in this study was to investigate early diagnostic evidence, optimal therapeutic strategies, and prophylactic methods for slit ventricle syndrome (SVS) in patients with temporal lobe arachnoid cysts who received cyst-peritoneal (CP) shunts. METHODS: Six cases of SVS in patients with temporal lobe arachnoid cysts who received CP shunts were treated by the senior authors in 2 institutions between January 2005 and January 2009. The radiological data, treatment process, and therapeutic results were reviewed retrospectively. RESULTS: There were 4 boys and 2 girls, whose mean age at presentation was 4 years and 1 month. The main clinical symptoms were severe headache, nausea, vomiting, and decreasing eyesight. Radiological studies showed normal or slightly smaller than normal ventricles, and arachnoid cysts that had shrunk dramatically. The most effective treatment for SVS was to replace the original shunts with devices that had a programmable valve, or if this was unsuccessful, to replace the original shunts with ventriculoperitoneal (VP) shunts. All symptoms in these patients disappeared, and the mean follow-up duration was 24 months. CONCLUSIONS: The SVS presents more often in patients with hydrocephalus who undergo VP shunting. However, it is also a serious complication in patients with arachnoid cysts who receive a CP shunt. The SVS is not a single condition; rather, different pathophysiological conditions can underlie this complicated syndrome. Because there are no striking morphological changes on radiological studies of the SVS in the patients with CP shunts, it is not easy for this syndrome to be diagnosed in time and treated rationally. Misdiagnosis and delayed treatment usually occur. The use of programmable shunts or VP shunts to replace the original shunt is an optimal therapeutic choice. The use of low-pressure shunts to treat arachnoid cysts should be abandoned unless dictated by specific indications.

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.

OBJECT: This 25-year follow-up study was performed on 120 children with hypertensive hydrocephalus to evaluate 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 compared 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 hospitalizations 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.