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Choroid plexus coagulation in trisomy 9 mosaic–related hydrocephalus—a case report

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Abstract

In trisomy 9 mosaicism, plexus hypertrophy has been described as a phenotypical feature and cause of hydrocephalus. We report on a 15-month-old child with hydrocephalus and trisomy 9 mosaicism primarily diagnosed in amniocentesis. After implantation of a ventriculoperitoneal shunt and subsequent revision, he presented with an exhaustion of peritoneal absorption leading to massive ascites. The implantation of a peritoneal drainage offered the unique opportunity to monitor cerebrospinal fluid (CSF) production indirectly via abdominal CSF drainage. In an individual trial, we performed endoscopic choroid plexus cauterization to reduce cerebrospinal fluid production, which failed to reduce excessive CSF production. In a second procedure, a ventriculoatrial shunt was implanted and succeeded to treat persistent hydrocephalus.

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Authors and Affiliations

  1. Department of Neurosurgery, University Hospital Leipzig, Liebigstraße 20, 04103, Leipzig, Germany

    Johannes Kasper, Matthias Krause, Jürgen Meixensberger & Florian Wilhelmy

  2. Department of Children and Adolescence Health, University Hospital Leipzig, Liebigstraße 20, 04103, Leipzig, Germany

    Manuela Siekmeyer

  3. Department of Imaging and Radiation Therapy, Division of Pediatric Radiology, University Hospital Leipzig, Liebigstraße 20, 04103, Leipzig, Germany

    Daniel Gräfe

Authors
  1. Johannes Kasper
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  2. Matthias Krause
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  3. Manuela Siekmeyer
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  4. Daniel Gräfe
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  5. Jürgen Meixensberger
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  6. Florian Wilhelmy
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Correspondence to Florian Wilhelmy.

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On behalf of all authors I declare no conflict of interest.

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Kasper, J., Krause, M., Siekmeyer, M. et al. Choroid plexus coagulation in trisomy 9 mosaic–related hydrocephalus—a case report. Childs Nerv Syst 36, 1803–1805 (2020). https://doi.org/10.1007/s00381-020-04643-1

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  • DOI: https://doi.org/10.1007/s00381-020-04643-1

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