title: Development of Abnormal Hemispheric Vascular Networks Mimicking Cerebral Proliferative Angiopathy in a Child Originally Diagnosed with Deep-Seated Arteriovenous Fistula.
abstract: Cerebral proliferative angiopathy (CPA), which is characterized by diffuse vascular abnormalities with intermingled normal brain parenchyma, is a rare clinical entity distinct from classical cerebral arteriovenous malformations. Its pathology at initial state and subsequent course of progression has totally been undetermined. We herein presented a case of a child who was initially diagnosed with deep-seated arteriovenous fistula (AVF), and ultimately developed symptomatic CPA-like vascular lesion over a long period of clinical follow-up. A 7-month-old boy was incidentally found to have an AVF in the right basal ganglia and conservatively followed up. Serial magnetic resonance angiograms revealed the gradual proliferation and enlargement of pial and medullary vessels surrounding the AVF. Seven years later, he had a transient ischemic attack followed by intraventricular hemorrhage. A catheter angiogram showed a diffuse large vascular malformation composed of 2 distinct structures, including AVF in the right basal ganglia and the surrounding proliferated pial and medullary arteries in the right hemisphere. Single-photon emission computed tomography with N-isopropyl[123I]-p-iodoamphetamine revealed apparent hemodynamic compromise on the right hemisphere. Targeted embolization of the pseudoaneurysm originating from the right A1 perforator was performed to prevent rebleeding without complications. The patient had no further cerebrovascular events. Perinidal hypoperfusion induced by a deep-seated AVF could be one of the underlying pathologies of progressive angiogenic activity. This is the first case showing the development of abnormal hemispheric vascular networks mimicking CPA, which offers insight into the pathogenesis of this new entity.
authors.affiliation: Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Sakata
authors.affiliation: Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan. Electronic address: fujimur@nsg.med.tohoku.ac.jp.
authors.forename: Miki
authors.initials: M
authors.lastname: Fujimura
authors.affiliation: Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Sato
authors.affiliation: Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan.
authors.forename: Kuniyasu
authors.initials: K
authors.lastname: Niizuma
authors.affiliation: Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan.
authors.forename: Hidenori
authors.initials: H
authors.lastname: Endo
authors.affiliation: Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan.
authors.forename: Teiji
authors.initials: T
authors.lastname: Tominaga
mesh_terms: D017541:Aneurysm, False / Q000209:etiology / Q000628:therapy; D001164:Arteriovenous Fistula / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology / Q000628:therapy; D001479:Basal Ganglia / Q000098:blood supply*; D002533:Cerebral Angiography / Q000379:methods; D002543:Cerebral Hemorrhage / Q000209:etiology; D002560:Cerebrovascular Circulation; D054022:Cerebrum / Q000098:blood supply*; D002648:Child; D018450:Disease Progression; D004621:Embolization, Therapeutic; D006439:Hemodynamics; D006801:Humans; D033162:Incidental Findings; D007223:Infant; D002532:Intracranial Aneurysm / Q000209:etiology / Q000628:therapy; D002538:Intracranial Arteriovenous Malformations / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology / Q000628:therapy; D002546:Ischemic Attack, Transient / Q000209:etiology; D018810:Magnetic Resonance Angiography; D008297:Male; D009389:Neovascularization, Pathologic; D055420:Perfusion Imaging / Q000379:methods; D011237:Predictive Value of Tests; D013997:Time Factors; D015899:Tomography, Emission-Computed, Single-Photon; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
keywords: Angiogenesis; arteriovenous malformation; cerebral proliferative angiopathy; intracerebral hemorrhage; pathogenesis