Article
It's not always the shunt to blame: sterile peritoneal malabsorption of cerebrospinal fluid in children
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Authors
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Thomas Beez
- Klinik für Neurochirurgie, Medizinische Fakultät, Heinrich-Heine-Universität, Düsseldorf, Deutschland
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Zarela Krause Molle
- Klinik für Neurochirurgie, Medizinische Fakultät, Heinrich-Heine-Universität, Düsseldorf, Deutschland
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Hans-Jakob Steiger
- Universitätsklinikum Düsseldorf, Neurochirurgische Klinik, Düsseldorf, Deutschland
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Sevgi Sarikaya-Seiwert
- Klinik für Neurochirurgie, Medizinische Fakultät, Heinrich-Heine-Universität, Düsseldorf, Deutschland
| Published: | June 9, 2017 |
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Outline
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Objective: Ventriculoperitoneal shunt (VPS) malfunction due to peritoneal malabsorption of cerebrospinal fluid (CSF) is a rarely described and incompletely understood phenomenon in the absence of infection. We sought to analyze the incidence and risk factors of this complication in a large contemporary cohort of children with VPS.
Methods: Cases were defined as reoperations due to peritoneal CSF malabsorption in the absence of proven infection or occlusion of the shunt system itself. Patients were retrospectively identified from an institutional database comprising 181 pediatric VPS implantations performed between 2010 and 2016 and relevant medical charts were reviewed.
Results: Sterile CSF malabsorption occurred in 4 patients (2 male, 2 female) at a mean age of 12.5 months (range 1 to 24 months). An increase in abdominal girth and ventricular size were the presenting signs in all patients. Excessive intraabdominal accumulation of fluid could be detected on ultrasound. Regarding the etiology of hydrocephalus, three patients had previous brain tumor resections (two medulloblastomas, one choroid plexus carcinoma) and one patient had congenital aqueductal stenosis. Analysis of possible risk factors revealed previous chemotherapy in the three oncological patients (actinomycin D in one and methotrexate in two patients, respectively) and premature birth at 30 weeks of gestation in the child with congenital hydrocephalus. Of note, one oncological patient was subsequently diagnosed with veno-occlusive disease of the portal vein after additionally developing hepatomegaly and raised liver enzymes, presumably secondary to chemotherapy with actinomycin D. All patients underwent temporary externalization of the VPS and then conversion to a ventriculoatrial shunt (VAS) with adjustable Codman Hakim valves used in three cases and a Miethke paediGAV 4/19 valve in one patient. Ventricular size improved in all patients and ascites regressed.
Conclusion: VPS failure due to peritoneal malabsorption of CSF is a rare event and should be taken into account in shunted children presenting with concomitant CSF underdrainage and significant ascites. Risk factors suggested by our data include previous chemotherapy as well as prematurity. Conversion to a VAS appears to be an effective treatment. To further analyze this rare complication a multicentric registry would be desirable.
