After the introduction of shunt treatment for the management of childhood hydrocephalus, a wide variety of complications related to this treatment modality have been recognized. The entity of slit ventricle syndrome (alternatively, symptomatic ventricular coaptation) is one of them, is frequently encountered in the pediatric population and its symptom complex resembles that of shunt failure. We conducted research on PubMed^®, MEDLINE^®, and Web of Science^®, using the keywords: “slit ventricles,” “slit ventricle syndrome,” “SVS” and “ventricular coaptation.” The aim of our review was to trace the advances made through the past decades, concerning our knowledge about the clinical characteristics, pathophysiology, and treatment options of this entity. The discrepancy among researchers about the offending etiology and the optimum treatment algorithm of this entity, as well as the necessity of an updated concept regarding shunt over drainage is analyzed. The multiple treatment modalities proposed and pathophysiologic mechanisms implicated for the treatment of slit ventricle syndrome illustrate the complexity of this entity. Consequently, the issue requires more detailed evaluation. In this review, we comment on all the main facets related to shunt over drainage and the resultant slit ventricle syndrome.

The current management strategy of hydrocephalus mainly involves the insertion of a ventriculoperitoneal shunt and is inherently related with a complication widely known as shunt over-drainage. Albeit this is a well-recognized complication, the true incidence and severity of this phenomenon remains undefined and most probably underdiagnosed, necessitating a more comprehensive pathophysiologic and therapeutic consideration. The slit ventricle syndrome is intimately related with the entity of shunt over-drainage, although who’s the definition of the former is implicated by a lack of universally accepted inclusion criteria. Another point of controversy is related with the absence of widely accepted criteria that would be able to discriminate the existing differentiations between these two entities. This is reflected in the fact that there are many proposed, relevant, treatment protocols. The background for all this data is based on the uncertainty and ambiguity regarding the pathophysiological mechanisms that are implicated. Current efforts are centered on the implementation of precautionary measures, as well as on treatment of both of these entities. Currently, there are enough evidence that support the concept that prevention of siphoning via the use of gravitational valves or antisiphon devices is the most efficacious means contained in our current therapeutic armamentarium. We attempt to present an overview of this complex entity, emphasizing on the hydrodynamics of the cerebrospinal fluid circulation in conditions harboring a ventriculoperitoneal shunt, the effect of the siphoning effect and the role of programmable valves and anti-siphon devices in our effort to eliminate this phenomenon. Based on an extensive literature review and on expert opinion, we concluded that the insertion of an anti-siphon device (gravitational shunt valves) could reliably address the issue of over-drainage, when a patient assumes a vertical position. Besides that, there are ongoing prospective studies centered on the safety and efficacy of adjustable gravitational valves, whose results are of ultimate importance. It is of paramount importance to be recognized that, due to the complexity of the pathophysiology of shunted hydrocephalus, lifelong follow-up of patients with ventriculoperitoneal shunts is necessary.

Cerebellar mutism syndrome (CMS), also known as posterior fossa syndrome, is an entity that entails a constellation of signs and symptoms which are recorded in a limited number of pediatric patients who have been operated on mainly for tumors involving the posterior cranial fossa, and more precisely, the region of the vermis. Medulloblastoma seems to constitute the most commonly recognized pathological substrate, associated with this entity. The most prevalent constituents of this syndrome are noted to be a, often transient, although protracted, language impairment, emotional lability, along with cerebellar and brainstem dysfunction. Apart from that, a definite proportion of involved individuals are affected by irreversible neurological defects and long-lasting neurocognitive impairment. A bulk of literature and evidence based on clinical trials exist, which reflect the continuous effort of the scientific community to highlight all perspectives of this complex phenomenon. There are several circumstances that intervene in our effort to delineate the divergent parameters that constitute the spectrum of this syndrome. In summary, this is implicated by the fact that inconsistent nomenclature, poorly defined diagnostic criteria, and uncertainty regarding risk factors and etiology are all constituents of a non-well-investigated syndrome. Currently, a preliminary consensus exists about the identification of a group of diagnostic prerequisites that are managed as sine qua non, in our aim to document the diagnosis of CMS. These include language impairment and emotional lability, as proposed by the international Board of the Posterior Fossa Society in their consensus statement. It is common concept that midline tumor location, diagnosis of medulloblastoma, younger age at diagnosis, and preoperatively established language impairment should be accepted as the most determinant predisposing conditions for the establishment of this syndrome. A well-recognized pathophysiological explanation of CMS includes disruption of the cerebellar outflow tracts, the cerebellar nuclei, and their efferent projections through the superior cerebellar peduncle. Despite the relative advancement that is recorded regarding the diagnostic section of this disease, no corresponding encouraging results are reported, regarding the available treatment options. On the contrary, it is mainly targeted toward the symptomatic relief of the affected individuals. The basic tenet of our review is centered on the presentation of a report that is dedicated to the definition of CMS etiology, diagnosis, risk factors, clinical presentation, and clinical management. Apart from that, an effort is made that attempts to elucidate the paramount priorities of the scientific forum, which are directed toward the expansion our knowledge in the era of diagnostics, prevention, and therapeutic options for patients suffering from CM, or who are at risk for development of this syndrome.

An isolated or trapped fourth ventricle is a relatively rare, although serious, adverse effect of hemorrhagic, infectious, or inflammatory processes that involve the central nervous system. This entity usually occurs after successful shunting of the lateral ventricles and may become clinically evident with the development of delayed clinical deterioration. This decline of the neurological status of the patient is evident after an initial period of improvement of the relevant symptoms. Surgical treatment options include cerebrospinal fluid shunting procedures, along with open surgical and endoscopic approaches. Complications related to its management are common and are related with obstruction of the fourth ventricular catheter, along with cranial nerve or brainstem dysfunction. We used the keywords: “isolated fourth ventricle,” and “trapped fourth ventricle,” in PubMed^® and Web of Science^®. Treatment of the trapped fourth ventricle remains a surgical challenge, although the neurosurgical treatment armamentarium has broadened. However, prompt recognition of the clinical and neurological findings that accompany any individual patient, in conjunction with the relevant imaging findings, is mandatory to organize our treatment plan on an individual basis. The current experience suggests that any individual intervention plan should be mainly based on the underlying pathological substrate of hydrocephalus. This could help us to preserve the patient’s life, on an emergent basis, as well as to ensure an uneventful neurological outcome, maintaining at least the preexisting level of neurological function.