1. Complex Management of Hydrocephalus Secondary To Choroid Plexus Hyperplasia
- Author
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Jason A. Chen, Saksham Gupta, Ian Tafel, Benjamin R. Johnston, Joshua D. Bernstock, Jennifer Judge, David J Segar, Benjamin C. Warf, Osama Aglan, Ari D Kappel, Richard S. Dowd, Scellig S D Stone, Alaa S. Montaser, and Katie Pricola Fehnel
- Subjects
medicine.medical_specialty ,business.industry ,Endoscopic third ventriculostomy ,Hyperplasia ,medicine.disease ,Hydrocephalus ,Surgery ,03 medical and health sciences ,0302 clinical medicine ,medicine.anatomical_structure ,Cerebrospinal fluid ,030220 oncology & carcinogenesis ,Hydrocele ,medicine ,Abdomen ,Choroid plexus ,Neurology (clinical) ,Choroid ,business ,030217 neurology & neurosurgery - Abstract
Background Hyperplasia of the choroid plexus represents a rare cause of communicating hydrocephalus in children. Recent work has associated such disease with genetic abnormalities (such as perturbations in chromosome 9). Given such extensive cerebrospinal fluid (CSF) overproduction, patients with choroid plexus hyperplasia often fail CSF diversion and therefore require adjuvant interventions. Case Description We present the case of a male infant with a ventriculoperitoneal shunt and radiographic choroid hyperplasia who presented to our institution with a massive abdominal hydrocele caused by an inability to absorb the significant amount of CSF drainage into the abdomen. Conclusion The child was treated with an endoscopic third ventriculostomy and choroid plexus coagulation; however, he still required CSF diversion via a ventriculoatrial shunt. A genetic workup showed tetraploidy of chromosome 9. We discuss criteria for selection of treatment strategies, including endoscopic third ventriculostomy with choroid plexus coagulation and/or CSF diversion, that may prevent the need for re-operation in select patients with hydrocephalus due to choroid plexus hyperplasia.
- Published
- 2020