Choroidal artery embolization in the management of cerebrospinal fluid overproduction: case report and review of the literature

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Diffuse villous hyperplasia of the choroid plexus (DVHCP) is a rare cause of communicating hydrocephalus. DVHCP may be diagnosed radiographically and through histological evaluation. It may be associated with genetic abnormalities, particularly involving chromosome 9. Due to CSF overproduction, patients with DVHCP often fail management with shunting alone and may require adjuvant interventions. The authors present the case of a child with partial trisomy 9p and delayed diagnosis of hydrocephalus with radiographic evidence of DVHCP who was successfully managed with ventriculoperitoneal shunt (VPS) placement, adjuvant bilateral endoscopic choroid plexus coagulation (CPC), and the novel application of anterior choroidal artery embolization. In addition, a systematic MEDLINE search was conducted using the keywords “diffuse villous hyperplasia,” “choroid plexus hypertrophy,” and “idiopathic cerebrospinal fluid overproduction.” Clinicopathological characteristics and outcomes of the present case were reviewed and compared to those in the literature.

A 14-month-old girl with partial trisomy 9p presented with macrocephaly and radiographic evidence of communicating hydrocephalus and DVHCP. Ventriculoperitoneal shunting resulted in distal failure due to inadequate CSF absorption, and ventriculoatrial shunt (VAS) placement was not possible due to multiple cardiac anomalies. Daily CSF production was reduced via endoscopic third ventriculostomy and bilateral CPC, followed by distal choroidal artery embolization, enabling VPS re-internalization. The embolization was complicated by radiographic evidence of an iatrogenic cerebral infarct, but this was clinically occult. Thirty-two additional cases of communicating hydrocephalus due to DVHCP are reported in the literature: 27 pediatric, 3 adult, and 2 postmortem. Genetic abnormalities were noted in 14, with 7 (50%) involving chromosome 9. Twelve patients underwent plexectomy (9 bilateral, 2 unilateral, 1 partial), and 10 underwent CPC (4 bilateral, 3 unilateral, and 3 unspecified), with or without shunting. Eight patients were successfully managed with shunting alone (6 VASs, 2 VPSs), and none underwent arterial embolization.

DVHCP is a rare cause of communicating hydrocephalus that may be associated with genetic abnormalities. A thorough review of the literature highlights diagnostic criteria and interventional options involved in managing this cause of CSF overproduction. The present case demonstrates that angiographic confirmation of prominent choroidal arteries may contribute to the diagnosis DVHCP. In addition, embolization of the distal choroidal arteries may be considered as a potential adjuvant treatment in patients for whom conventional treatments have failed or are not feasible.

ABBREVIATIONS AChA = anterior choroidal artery; CPC = choroid plexus coagulation; CPP = choroid plexus papilloma; DCA = diagnostic cerebral angiography; DVHCP = diffuse villous hyperplasia of the choroid plexus; ETV = endoscopic third ventriculostomy; EVD = external ventricular drain; ICA = internal carotid artery; MRA = MR angiography; VAS = ventriculoatrial shunt; VPS = ventriculoperitoneal shunt.

Article Information

Correspondence Amanda M. Saratsis: Northwestern University Feinberg School of Medicine, Chicago, IL. asaratsis@luriechildrens.org.

INCLUDE WHEN CITING Published online March 22, 2019; DOI: 10.3171/2019.1.PEDS18519.

Disclosures Dr. Alden reports being a consultant for Biomarin Pharmaceutical.

© AANS, except where prohibited by US copyright law.

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    PRISMA flowchart of citations identified and evaluated. Format provided by Moher D, Liberati A, Tetzlaff J, Altman DG, The PRISMA Group (2009). Preferred Reporting Items for Systematic Reviews and Meta-Analyses: The PRISMA Statement. PLoS Med 6(7): e1000097. doi:10.1371/journal.pmed1000097.

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    A–D: Preoperative T2-weighted axial MR brain images (A and B) demonstrating severe communicating hydrocephalus, and T1-weighted, postcontrast axial images (C and D) demonstrating prominent, enhancing choroid plexus in the lateral ventricles. E–H: Preembolization MRA of the brain. Axial time-of-flight source image with evidence of enlarged branches of the distal AChA to the choroid of the right lateral ventricle (arrow; E). Axial composite 3D reformatted image of MRA demonstrating an aplastic A1 segment of the left anterior cerebral artery and P1 segment of the left posterior cerebral artery with associated fetal configuration of the left posterior cerebral artery, considered a normal variation. Prominent right AChA is noted (arrow; F). Three-dimensional time-of-flight MRA images of the right and left ICAs, demonstrating enlarged AChAs bilaterally (arrows; G and H). Figure is available in color online only.

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    Diagnostic cerebral angiography via right femoral artery access and placement of a 4-Fr glide catheter. A and B: Anteroposterior and lateral views of a right ICA injection, demonstrating the enlarged right AChA (arrow). C and D: Anteroposterior and lateral views of left ICA injection, demonstrating the enlarged left AChA (arrow). E: Roadmap image demonstrating the microcatheter in the right AChA beyond the plexal point. F and G: Anteroposterior and lateral microcatheter angiography of the distal right AChA. H and I: Anteroposterior and lateral microcatheter angiography of the distal left AChA. Figure is available in color online only.

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    A–D: Diagnostic and interventional cerebral angiography. Lateral roadmap view (A), demonstrating the cast of Onyx in the distal right AChA (arrow). Lateral view, right ICA injection (B) demonstrating patency of the right AChA up to the point of occlusion at the plexal point (arrow). Lateral view, left ICA injection (C) demonstrating patency of the left AChA up to the point of occlusion at the plexal point (arrow). Unsubtracted magnified lateral angiographic view (D) from left ICA injection demonstrating the Onyx cast (arrowhead) and patency of the proximal left AChA (arrow). Anteroposterior (E) and lateral (F) radiographic views of the skull demonstrating the Onyx cast in both distal AChAs. Postembolization axial DynaCT image (G) demonstrating the proximal end of the Onyx cast in both AChAs, at the level of the plexal points (arrows). Figure is available in color online only.

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    MRI of the brain performed 2 days after embolization. A–D: Axial diffusion-weighted images demonstrating infarction of the choroid (arrow) and the right posteromedial thalamus (arrowhead). Susceptibility artifact secondary to the Onyx embolic agent noted bilaterally. E and F: Coronal T2-weighted images demonstrating the right thalamic ischemia (arrows). G: Axial gradient-echo T2-weighted image demonstrating the susceptibility artifact from the Onyx material in the choroidal branches (arrows). Figure is available in color online only.

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