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      Slit ventricle syndrome: Historical considerations, diagnosis, pathophysiology, and treatment review

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          Abstract

          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.

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          The scientific history of hydrocephalus and its treatment.

          S Kunze, S. B. Hashemi, A Aschoff … (1999)
          Hydrocephalus cases were regularly described by Hippocrates, Galen, and early and medieval Arabian physicians, who believed that this disease was caused by an extracerebral accumulation of water. Operative procedures used in ancient times are neither proven by skull findings today nor clearly reported in the literature. Evacuation of superficial intracranial fluid in hydrocephalic children was first described in detail in the tenth century by Abulkassim Al Zahrawi. In 1744, LeCat published findings on a ventricular puncture. Effective therapy required aseptic surgery as well as pathophysiological knowledge--both unavailable before the late nineteenth century. In 1881, a few years after the landmark study of Key and Retzius, Wernicke inaugurated sterile ventricular puncture and external CSF drainage. These were followed in 1891 by serial lumbar punctures (Quincke) and, in 1893, by the first permanent ventriculo-subarachnoid-subgaleal shunt (Mikulicz), which was simultaneously a ventriculostomy and a drainage into an extrathecal low pressure compartment. Between 1898 and 1925, lumboperitoneal, and ventriculoperitoneal, -venous, -pleural, and -ureteral shunts were invented, but these had a high failure rate due to insufficient implant materials in most cases. Ventriculostomy without implants (Anton 1908), with implants, and plexus coagulation initially had a very high operative mortality and were seldom successful in the long term, but gradually improved over the next decades. In 1949, Nulsen and Spitz implanted a shunt successfully into the caval vein with a ball valve. Between 1955 and 1960, four independent groups invented distal slit, proximal slit, and diaphragm valves almost simultaneously. Around 1960, the combined invention of artificial valves and silicone led to a worldwide therapeutic breakthrough. After the first generation of simple differential pressure valves, which are unable to drain physiologically in all body positions, a second generation of adjustable, autoregulating, antisiphon, and gravitational valves was developed, but their use is limited due to economical restrictions and still unsolved technical problems. At the moment, at least 127 different designs are available, with historical models and prototypes bringing the number to 190 valves, but most of these are only clones. In the 1990s, there has been a renaissance of endoscopic ventriculostomy, which is widely accepted as the method of first choice in adult patients with aquired or late-onset, occlusive hydrocephalus; in other cases the preference remains controversial. Both new methods, the second generation of valves as well as ventriculostomy, show massive deficits in evaluation. There is only one randomized study and no long-term evaluation.
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            Shunts vs endoscopic third ventriculostomy in infants: are there different types and/or rates of complications? A review.

            L Massimi, C C de Miranda Rocco, G Tamburrini (2006)
            The decision-making process when we compare endoscopic third ventriculostomy (ETV) with shunts as surgical options for the treatment of hydrocephalus in infants is conditioned by the incidence of specific and shared complications of the two surgical procedures. Our literature review shows that the advantages of ETV in terms of complications are almost all related to two factors: (a) the avoidance of a foreign body implantation and (b) the establishment of a 'physiological' cerebrospinal fluid (CSF) circulation. Both these kinds of achievements are particularly important in infants because of the relative high rate of some intraoperative (i.e. abdominal) and late (secondary craniosynostosis, slit-ventricle syndrome) shunt complications in this specific subset of patients. On the other side, the main factor which is claimed against ETV is the relatively high risk of immediate mortality and neurological complications. Clinical manifestations of neurological structure damage seem to be more frequent in infants, probably due to the more relevant effect of parenchymal and vascular damage in this age group; however, both the immediate mortality and neurological damage risk of ETV procedures should be weighted against the long-term mortality and the late neurological damage which is not infrequently described as a consequence of shunt malfunction and proximal shunt revision procedures. Infections are possible in both ETV and extrathecal CSF procedures, especially in infants. However, the incidence of infective complications is significantly lower in case of ETV (1-5% vs 1-20%). Moreover, different from shunting procedures, infections in children with third ventriculostomy have a more benign course, being generally controlled by antibiotic treatment alone.
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              The natural history of hydrocephalus. Detailed analysis of 182 unoperated cases.

              Jerome M Laurence, Brad S. Coates (1962)
              Article 0 comments Cited 22 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Brain Circ
                Journal ID (iso-abbrev): Brain Circ
                Journal ID (publisher-id): BC
                Title: Brain Circulation
                Publisher: Wolters Kluwer - Medknow (India )
                ISSN (Print): 2394-8108
                ISSN (Electronic): 2455-4626
                Publication date Collection: Jul-Sep 2021
                Publication date (Electronic): 27 August 2021
                Volume: 7
                Issue: 3
                Pages: 167-177
                Affiliations
                [1] Department of Neurosurgery, Pediatric Hospital of Athens, “Agia Sophia,”, Athens, Greece
                [1 ] Department of Neurosurgery, 251 Greek Air Force Hospital, Athens, Greece
                Author notes
                Address for correspondence: Dr. Dimitrios Panagopoulos, Thivon and Papadiamantopoulou St, Goudi, 11527, Athens, Attica, Greece. E-mail: dimpanayop@ 123456gmail.com
                Article
                Publisher ID: BC-7-167
                DOI: 10.4103/bc.bc_29_21
                PMC ID: 8459697
                PubMed ID: 34667900
                SO-VID: 75748fec-7ae3-4c8f-b31e-9500f1ba07e5
                Copyright © Copyright: © 2021 Brain Circulation
                License:

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                Date received : 03 April 2021
                Date revision received : 08 May 2021
                Date accepted : 21 May 2021
                Categories
                Subject: Review Article

                Keywords: anti-siphon device,programmable valve,slit ventricle,upgrade of opening pressure
                Data availability:
                Keywords: anti-siphon device, programmable valve, slit ventricle, upgrade of opening pressure

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