Cerebral revascularization in cerebral proliferative angiopathy: a systematic review

Abstract

<jats:sec><jats:title>OBJECTIVE</jats:title><jats:p>Cerebral proliferative angiopathy (CPA) is considered a discrete vascular malformation of the brain separate from classical brain arteriovenous malformations (AVMs). It has unique angiographic characteristics and has been hypothesized to result from chronic cortical ischemia and perinidal oligemia. Treatment with cerebral revascularization has been proposed in an attempt to disrupt regional hypoperfusion and interrupt the angiogenesis that defines CPA. A systematic review of the literature pertaining to the role of cerebral revascularization may highlight a treatment paradigm for this rare disease.</jats:p></jats:sec><jats:sec><jats:title>METHODS</jats:title><jats:p>A systematic review was performed in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. MEDLINE and Embase were searched from inception for papers relating to CPA. Included articles were categorized according to methodology (case series or imaging study) and treatment modality (conservative, radiation, endovascular, or revascularization). A synthesis was compiled summarizing the current evidence regarding cerebral revascularization in CPA.</jats:p></jats:sec><jats:sec><jats:title>RESULTS</jats:title><jats:p>The initial search revealed 43 articles, of which 28 studies met the inclusion criteria. Nine studies were identified that described imaging findings, which suggested hemodynamic dysregulation and perinidal impairments in the cerebrovascular reserve could be identified compared to unaffected hemispheres and classical brain AVMs. Six studies including 7 patients undergoing indirect forms of cerebral revascularization were identified. Clinical and radiological outcomes following revascularization were favorable in all but one study.</jats:p></jats:sec><jats:sec><jats:title>CONCLUSIONS</jats:title><jats:p>A small body of radiological and clinical studies has emerged, suggesting that CPA is a response to perinidal oligemia. While the long-term clinical efficacy of revascularization remains unclear, early results suggest that this may be a novel treatment paradigm for patients with CPA.</jats:p></jats:sec>

Cerebral proliferative angiopathy depicted by four-dimensional computed tomographic angiography: A case report

“…4 D) in the lesion. Based on these imaging findings, unlike AVM and AVF, the abnormal vessels had no high-flow arteriovenous shunts, and the patient was finally diagnosed with CPA in accordance with the previously reported imaging findings [1] , [2] , [3] , [4] , [5] . His symptoms and imaging findings remained unchanged 1 year after the diagnosis.…”

“…Cerebral proliferative angiopathy (CPA) is a rare cerebrovascular disorder characterized by diffuse abnormal vessels with intermingled brain parenchyma fed by many arteries and draining into many veins without high-flow arteriovenous shunts, which is distinct from cerebral arteriovenous malformation (AVM) and arteriovenous fistula (AVF) [1] , [2] , [3] , [4] , [5] . CPA is usually confirmed by conventional digital subtraction angiography (DSA).…”

Removal of malformation in cerebral proliferative angiopathy: illustrative case

“…Also, it is currently considered that such ischemia and perinidal oligemia work as the main causes of the symptoms, and this theory works as the rationale for revascularization treatment. 1,2 Many CPA treatment modalities have been attempted, including embolization, radiosurgery, medication, and revascularization. 2,3 Revascularization such as encephalo-duro-arterio-synangiosis is currently considered a reasonable treatment option.…”

“…1,2 Many CPA treatment modalities have been attempted, including embolization, radiosurgery, medication, and revascularization. 2,3 Revascularization such as encephalo-duro-arterio-synangiosis is currently considered a reasonable treatment option. 2 Although the evidence is still lacking, revascularization seems to be relatively superior to other methods, and this finding is consistent with the suggested pathogenesis of the disease.…”

Central nervous system vascular malformations: A clinical review

“…CPA is defined as a diffuse network of abnormal vessels surrounded by normal brain parenchyma. One hypothesis for the etiology of these lesions is chronic hypoperfusion 24 . In a review of 1400 brain AVMs, only 3.4% were identified as CPA 25 .…”