Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2024 Oct;38(5):1047-1051.
doi: 10.1080/02688697.2021.2013437. Epub 2021 Dec 13.

Volume staged stereotactic radiosurgery and endovascular embolization in the treatment of cerebral proliferative angiopathy: lessons learned

Affiliations
Case Reports

Volume staged stereotactic radiosurgery and endovascular embolization in the treatment of cerebral proliferative angiopathy: lessons learned

Aaron Brake et al. Br J Neurosurg. 2024 Oct.

Abstract

Purpose of the ArticleCerebral proliferative angiopathy (CPA) is a rare and recently characterized vascular malformation that is often mistaken for a large, diffuse arteriovenous malformation (AVM). However, distinguishing the two entities is critical, as while the diseases may appear similar on imaging, they are completely different entities. The most distinguishing features of CPA compared to AVM are the presence of normal functioning brain within the 'nidus' of the abnormality and the proliferative nature of the nidus. While the management of AVM is considered well understood, the management of CPA is unclear. Typical treatment may include conservative management, targeted embolization, and/or surgical revascularization.Materials and MethodsHere, we present a patient who was initially diagnosed with a large, diffuse AVM in the posterior fossa. Initially managed conservatively, the development of progressive, debilitating neurologic symptoms prompted treatment. We pursued staged endovascular intervention and improved her initial outlook. Thereafter, volume-staged stereotactic radiosurgery (VS-SRS) was pursued to attempt to achieve a definitive treatment.Results and ConclusionsUltimately, while the treatment proved successful clinically and radiographically, the post-treatment course was exceptionally challenging. In retrospect, it is clear the working diagnosis was incorrect, and CPA was the true diagnosis. To our knowledge, this is the first known application of this treatment approach for CPA. However, the post-treatment course and final clinical outcome likely reflect the important differences between AVM and CPA. For these reasons, we are cautious to recommend the treatment course as prescribed in this case but hope to highlight important lessons learned in managing this rare condition.

Keywords: AVM; CPA; Vascular malformation; endovascular; stereotactic radiosurgery.

PubMed Disclaimer

Publication types

MeSH terms

LinkOut - more resources