Diffuse choroid plexus hyperplasia: an under-diagnosed cause of hydrocephalus in children?

Abstract

Hydrocephalus is a common neurological disorder in children and the result of a variety of causes. However, with the advancement of imaging modalities, particularly MRI, previously reported rarer causes of hydrocephalus in children are now being more readily appreciated. We report an 11-year-old boy with diffuse villous hyperplasia of the choroid plexus. He had a ventriculo-peritoneal (VP) shunt in-situ and a prior diagnosis from infancy of congenital aqueduct stenosis as the cause of his hydrocephalus. His current presentation was with further shunt dysfunction. CT and MRI demonstrated enlarged choroid plexuses but did not confirm aqueduct stenosis. CSF overproduction was demonstrated from the externalized ventricular drain. The enlarged choroid plexuses were surgically resected and histology confirmed choroid plexus hyperplasia. Identification of choroid plexus hyperplasia is important since the neurosurgical management of hydrocephalus is not VP shunt insertion, but resection of the hyperplastic choroid plexus.

Hydrocephalus Due to Diffuse Villous Hyperplasia of the Choroid Plexus -Case Report-

“…[1][2][3]5,7,[9][10][11][12][13][14][15][16]18,19,21,22) In most patients, VP shunt surgery was performed but failed, resulting in marked ascites. This suggests difficulty in establishing the diagnosis of this disease in the initial stage.…”

Cerebrospinal fluid hypersecretion in pediatric hydrocephalus

“…Disturbances in homeostasis can lead to hydrocephalus that results from CSF hypersecretion secondary to choroid plexus hyperplasia (CPH) 4 or nonobstructive tumors of the choroid plexus, such as choroid plexus papilloma (CPP), 11 which are rare causes of pediatric hydrocephalus. In the literature, CPH is also referred to as diffuse villous hyperplasia of the choroid plexus or villous hypertrophy.…”

“…In the literature, CPH is also referred to as diffuse villous hyperplasia of the choroid plexus or villous hypertrophy. 4 The difference between hyperplasia and hypertrophy of the choroid plexus is not always stated explicitly; therefore, for the purposes of this review, cases of CPH, diffuse villous hyperplasia of the choroid plexus, and villous hypertrophy will be referred to as cases of CPH.…”

“…The diagnosis of hydrocephalus with CPP and CPH origin is critical, because the standard treatment is not a shunt procedure but, rather, resection of the tumor or excessive CPE. 4,27,45 The initial diagnosis of CPP or CPH has been difficult historically, especially before imaging techniques such as MRI. 27,55 In general, the diagnosis is made after shunt failure or the development of ascites, which prompts a revision or externalization of the shunt.…”

“…3,4,14,18,20,27,38,40,46,55,56,90,96,102,117,123,127 After surgical intervention (e.g., CPC or tumor removal), the rates of CSF production decreased, 4,20,35,46,51,90,96,117 and in some cases, there was no further need for a shunt. 27,51,56 In addition to CPP and CPH, overproduction of CSF contributing to hydrocephalus has also been implicated in idiopathic intracranial hypertension, 59 infectious hydrocephalus, 16 and intraventricular hemorrhage-associated hydrocephalus, 126 but secretion rates in the patients with these conditions have not been well documented.…”