Diffuse villous hyperplasia of choroid plexus

Abstract

Diffuse villous hyperplasia of choroid plexus (DVHCP) is a rare condition which is characterized by the presence of diffuse enlargement of the entire choroid plexus throughout the length of the choroidal fissure and overproduction of CSF. The diagnosis of diffuse villous hyperplasia of choroid plexus can be established by the MR demonstration of diffusely large, contrast enhanced choroid plexus in the cases of overproduction hydrocephalus. Although some authors recommend choroid plexus excision or coagulation, ventriculo-atrial shunt insertion is a simple and effective treatment modality in cases of diffuse villous hyperplasia of the choroid plexus. In this report we present a case of diffuse villous hyperplasia of the choroid plexus and a short review of the literature. To our knowledge, in the CT and MRI era only 5 cases of DVHCP cases have been reported.

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

“…15,19) Indeed, 3-year followup examination showed good control of hydrocephalus and absence of morphological changes in the choroid plexus in previous patients. 2,14) Similar results were obtained in our patient, although the follow-up period was only 6 months. We also evaluated other options (biopsy or resection), but selected VA shunt without biopsy and resection, considering the risks of shunt failure due to bleeding-related increase in the CSF protein level when a shunt becomes necessary, infection due to long-term external drainage, and electrolyte abnormalities due to the removal of a large volume of CSF from the body.…”

“…15) However, the enlarged choroid plexuses were symmetric and resembled DVHCP on imaging in some cases, 12) enabling diagnosis only after pathological examination. Most patients diagnosed with DVHCP [1][2][3]5,7,10,13,14,16,18,19,21,22) showed symmetric choroid plexus enlargement, but some exhibited irregular enlargement 21) or cyst formation. 6) In addition, no pathological findings were available in 3 patients including our patient, so the diagnosis of DVHCP was based on imaging findings.…”

“…11) In addition, there are risks of neurological complications, 22) and adverse effects of invasiveness, such as bleeding. 21) On the other hand, a method in which excessive CSF is returned to the systemic circulation using a VA shunt has been proposed, 14) which is noninvasive and straightforward, but whether the direct return of a large volume of CSF to the systemic circulation is appropriate remains questionable. The most important disadvantage is that definite histological diagnosis cannot be established.…”

“…In the majority of patients 2,3,5,7,[10][11][12][13][14][15][16]18,19,21,22) including ours, VP shunt surgery was performed first, but failed due to marked ascites. Subsequently, resection or endoscopic coagulation was performed in most patients to inhibit CSF production, followed by radical or shunt surgery.…”

“…6) In addition, no pathological findings were available in 3 patients including our patient, so the diagnosis of DVHCP was based on imaging findings. 3,14) Differential diagnosis is possible based on imaging findings, 14) and VA shunt surgery is the simplest and most appropriate choice, 22) considering the risks of complications of resection such as hemianopsia, nystagmus, and bleeding during the operation due to the rich blood flow in the choroid. 15,19) Indeed, 3-year followup examination showed good control of hydrocephalus and absence of morphological changes in the choroid plexus in previous patients.…”