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Palavani LB, Mitre LP, Almeida MV, Neto LBA, Ferreira MY, Semione G, Farias CAG, Batista S, Soares LGB, Pereira FS, Filho LM, Bertani R, Lawton MT, Figueiredo EG. Presentation and management of cerebral proliferative angiopathy: a systematic review and meta-analysis on treatment outcomes. Neurosurg Rev 2024; 47:664. [PMID: 39312085 DOI: 10.1007/s10143-024-02878-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/28/2024] [Revised: 09/13/2024] [Accepted: 09/14/2024] [Indexed: 10/15/2024]
Abstract
Cerebral Proliferative Angiopathy (CPA) is a rare brain vascular malformation, similar to Arteriovenous Malformations (AVM) but lacking of early venous drainage. Presentation and treatment outcomes were investigated, examining for morbidity, mortality and complications. A meta-analysis was conducted according to PRISMA guidelines. PubMed, Embase and Web Of Science were searched with keywords such as "cerebral proliferative angiopathy" and "management". We pooled and meta-analyzed outcomes on documented CPA cases. 11,079 studies were pooled as a result of manual citation searching, 50 studies were included, adding up to 115 CPA cases. The majority of patients were females (1.38:1), with a mean age of presentation of 26.9 (19.4) years. Headache (46%) and seizures (34%) were the most common presenting symptoms. 37% of patients presented with focal neurologic deficit. Patients managed conservatively from the surgical standpoint (i.e. nonoperative management) did not undergo homogenous treatment strategies, and major complications were at 47% (95% CI: 17%, 76%), with a 1% mortality (95% CI: 0%, 6%). Surgical and embolization interventions presented the highest proportion of major complications, 66% (95% CI: 33%, 99%) and 73% (95% CI: 42%, 100%), respectively. The embolization subgroup led in mortality, with 3% (95% CI: 0%, 10%). No death was documented in patients undergoing surgery. CPA has a similar presentation to brain arteriovenous malformations, but its treatment outcomes are potentially worse. This difference is not attributable to heterogeneity in assigning patient treatment strategies. This highlights the need for more accurate diagnostic methods.
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Affiliation(s)
- Lucca B Palavani
- Faculty of Medicine, Max Planck University Center, Indaiatuba, São Paulo, Brazil
| | - Lucas Pari Mitre
- Faculty of Medicine, Faculdade de Ciências Médicas da Santa Casa de São Paulo, São Paulo, Brazil.
- Faculty of Medicine, Santa Casa de São Paulo School of Medical Sciences, Rua Dr Cesário Motta Júnior, N° 112, Santa Cecília, São Paulo, SP, Brazil.
| | - Miguel V Almeida
- Faculty of Medicine, State University of Ceará, Fortaleza, Ceará, Brazil
| | - Luis B A Neto
- Faculty of Medical Sciences, Universidade de Pernambuco, Recife, Brazil
| | | | - Gabriel Semione
- Department of Medicine, University of West of Santa Catarina, Joaçaba, SC, Brazil
| | | | - Sávio Batista
- Faculty of Medicine, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil
| | - Luís G B Soares
- Department of Neurosurgery, Federal University of São Paulo, São Paulo, Brazil
| | - Felipe S Pereira
- Department of Neurosurgery, Federal University of São Paulo, São Paulo, Brazil
| | | | - Raphael Bertani
- Department of Neurosurgery, São Paulo University, São Paulo, São Paulo, Brazil
| | - Michael T Lawton
- Department of Neurosurgery, Barrow Neurological Institute, Phoenix, AZ, USA
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Luo FR, Zhou Y, Wang Z, Liu QY. Cerebral proliferative angiopathy in pediatric age presenting as neurological disorders: A case report. World J Clin Cases 2023; 11:8071-8077. [DOI: 10.12998/wjcc.v11.i33.8071] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/09/2023] [Revised: 10/29/2023] [Accepted: 11/14/2023] [Indexed: 11/24/2023] Open
Abstract
BACKGROUND Cerebral proliferative angiopathy (CPA) is a rare subtype of arteriovenous malformation. It is extremely rare in pediatric patients and has serious implications for developing children. However, reports of these disorders worldwide are limited, and no uniform reference for diagnosis and treatment options exists. We report the case of a 6-year-old with CPA having predominantly neurological dysfunction and review the literature on pediatric CPA.
CASE SUMMARY We report the case of a pediatric patient with CPA analyzed using digital subtraction angiography (DSA) who presented initially with a neurological disorder as the main manifestation. This case is the basis for further discussion of the clinical presentation, pathogenesis, diagnosis, and treatment of CPA in children. After the cerebral DSA, the patient was treated conservatively with sedation, fluid replacement, and blood anticoagulation. She could not cooperate with the follow-up magnetic resonance imaging examination because of her young age, and her family declined further treatment because of the surgery’s high risk. She was followed up for 3 months; her symptoms did not worsen.
CONCLUSION This report of rare pediatric CPA can inform and advance clinical research on congenital cerebrovascular diseases.
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Affiliation(s)
- Fu-Ren Luo
- Department of Radiology, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province, China
| | - Ying Zhou
- Department of Radiology, Mianyang Central Hospital, Mianyang 621000, Sichuan Province, China
| | - Zhong Wang
- Department of Radiology, Mianyang Central Hospital, Mianyang 621000, Sichuan Province, China
| | - Qi-Yu Liu
- Department of Radiology, The Affiliated Hospital of Southwest Medical University, Luzhou 646000, Sichuan Province, China
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Gerstl JVE, Bernstock JD, Kappel AD, Torio EF, Du R. Recurrent Intraventricular Haemorrhage in Cerebral Proliferative Angiopathy - A Case Report and Review of the Literature. Neurohospitalist 2023; 13:345-350. [PMID: 37701260 PMCID: PMC10494813 DOI: 10.1177/19418744231180046] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/14/2023] Open
Abstract
Cerebral proliferative angiopathy (CPA) is an entity distinct from that of classical arteriovenous malformations. As such, few reports have considered the long-term follow-up of patients with hemorrhage in CPA. Accordingly, herein the authors present a case of recurrent hemorrhage in CPA with 32 years of follow-up and in so doing summarize the literature of hemorrhagic cases in CPA. A 19-year-old presented with focal awareness seizures and diagnostic work-up revealed a left hemispheric vascular lesion. The patient presented again with intracranial hemorrhage at ages 28, 43 and 51. Angioarchitectural workup revealed intermingled brain parenchyma between vascular spaces, absence of dominant feeders and a clear nidus consistent with CPA. The size and diffuse nature of the lesion deemed it inoperable. Given our case and review of the literature it is apparent that CPA has a high risk of re-hemorrhage in the rare event that hemorrhage does occur.
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Affiliation(s)
- Jakob V. E. Gerstl
- Department of Neurosurgery, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA
| | - Joshua D. Bernstock
- Department of Neurosurgery, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA
| | - Ari D. Kappel
- Department of Neurosurgery, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA
| | - Erickson F. Torio
- Department of Neurosurgery, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA
| | - Rose Du
- Department of Neurosurgery, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA
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Srivastava T, Gandhi A, Kumar R. Clinico-radiological features of cerebral proliferative angiopathy: A large case series from India. Neuroradiol J 2022; 35:742-750. [PMID: 35614873 PMCID: PMC9626836 DOI: 10.1177/19714009221096816] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
Abstract
BACKGROUND Based on angiographic features, a rare subgroup, different from classical brain AVMs, called cerebral proliferative angiopathy (CPA) was suggested by Lasjaunias et al. in 1989. There are only few reports of CPA described from Asia. Liu et al. has described 5 cases of CPA from China. The objective of study was to evaluate the clinico-radiological features of patients with CPA in our cohort and to find out the differences between our study and the study by Lasjaunias and Liu et al. METHODS We retrospectively analysed cases of CPA from March 2012 to April 2020. All radiological images of the patients with AVMs were studied and findings suggestive of CPA were identified. We found 10 patients with CPA out of 272 cases of AVM (10/272) and described the various demographic and clinico-radiological features. RESULTS The age of the patients with CPA in our series varied from 14 to 53 years and the mean age was 28.6 years. There was male preponderance (60%) with male to female ratio of 3:2. The most common presentation was seizure (70%). Intracranial haemorrhage was present in five patients. One patient had cerebral infarction. CONCLUSIONS There were substantial differences in clinico-radiological profile of patients with CPA in our cohort as compared to the study by Lasjaunias et al. and Liu et al. in form of male dominance, higher rate of ICH and seizure. In our knowledge, this is the second largest series of CPA worldwide and the first largest series in Asia.
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Affiliation(s)
| | - Ashok Gandhi
- Department of Neurosurgery, SMS Medical College and Hospital, Jaipur, India
| | - Rahul Kumar
- GS Neuroscience Clinic and Reasearch
Centre, Patna, India
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Hess RM, Zhang JF, Cappuzzo JM, Bregy A, Levy EI. Cerebral Proliferative Angiopathy Presenting as Subdural Hematoma: A Case Report and Systematic Literature Review. World Neurosurg 2022; 164:281-289. [PMID: 35636668 DOI: 10.1016/j.wneu.2022.05.096] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/03/2022] [Revised: 05/20/2022] [Accepted: 05/21/2022] [Indexed: 11/29/2022]
Abstract
Cerebral proliferative angiopathy (CPA) is a rare cerebrovascular pathology that presents with unique clinical features due to distinct histologic, angiographic, and pathophysiologic characteristics that separate it from classical arteriovenous malformation. The disorder is characterized by uncontrolled angiogenesis in which functional brain parenchyma is interspersed with abnormal vascular channels without a distinct nidus. Common presenting symptoms include headache, seizures, and stroke-like symptoms. Hemorrhagic presentations are rare in contrast to the typical presentations of classical arteriovenous malformation. Here, we report a young woman with a history of a suspected connective tissue disorder who presented to the emergency department with worsening headaches in a delayed fashion after experiencing minor head trauma and was found to have a left-sided subdural hematoma. Angiography confirmed a diagnosis of CPA after abnormal cortical vasculature was noted during the patient's craniotomy. A systematic review of CPA cases described in the literature was performed using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, with the findings discussed.
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Affiliation(s)
- Ryan M Hess
- Department of Neurosurgery, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York, USA; Department of Neurosurgery, Gates Vascular Institute at Kaleida Health, Buffalo, New York, USA
| | - Jeff F Zhang
- Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York, USA
| | - Justin M Cappuzzo
- Department of Neurosurgery, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York, USA; Department of Neurosurgery, Gates Vascular Institute at Kaleida Health, Buffalo, New York, USA
| | - Amade Bregy
- Department of Neurosurgery, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York, USA; Department of Neurosurgery, Gates Vascular Institute at Kaleida Health, Buffalo, New York, USA
| | - Elad I Levy
- Department of Neurosurgery, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York, USA; Department of Radiology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York, USA; Canon Stroke and Vascular Research Center, University at Buffalo, Buffalo, New York, USA; Department of Neurosurgery, Gates Vascular Institute at Kaleida Health, Buffalo, New York, USA; Jacobs Institute, Buffalo, New York, USA.
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Hemorrhagic cerebral proliferative angiopathy in two pediatric patients: case reports. Childs Nerv Syst 2022; 38:789-794. [PMID: 33956211 DOI: 10.1007/s00381-021-05181-0] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/30/2020] [Accepted: 04/19/2021] [Indexed: 10/21/2022]
Abstract
Cerebral proliferative angiopathy (CPA) is an infrequent vascular malformation. It is composed of a nidus, arterial feeders, and venous drainage. Some special features differentiate it from the common arteriovenous malformations (AVM). The nidus has normal cerebral tissue intermingled and occupies a large portion of the brain. There is a frequent transdural arterial supply. Arterial feeders and draining veins are of small caliber concerning the nidus size. Ischemic strokes are the most common clinical event. Intracranial bleeding when occurs has a worse prognosis due to its high recurrence rate. We have presented two cases of CPA in pediatric patients who required surgical resolution of their acute episode.
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Srivastava T, Gandhi A. A rare case of cerebral proliferative angiopathy with acute cerebral infarction and facial capillary malformation. Neuroradiol J 2021; 35:504-507. [PMID: 34550835 PMCID: PMC9437504 DOI: 10.1177/19714009211042884] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
Abstract
We are describing a case of a 14-year-old girl who developed acute cerebral infarction which was documented on diffusion-weighted magnetic resonance imaging (DWI). On detailed evaluation, diagnosis of cerebral proliferative angiopathy (CPA) was made. Incidentally, she had capillary malformation on the forehead contralateral to cerebral vascular malformation. To our knowledge, this is the first case of CPA in the literature where DWI abnormality was due to acute cerebral infarction.
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Affiliation(s)
| | - Ashok Gandhi
- Department of Neurosurgery, SMS Medical College, India
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Ye F, Richard SA, Zhang Y, Xu X, Wu X, He J, Wu M, Gao H. Cerebral proliferative angiopathy with hemorrhage and infarction as complications after conservative treatment: A case report. INTERDISCIPLINARY NEUROSURGERY 2021. [DOI: 10.1016/j.inat.2021.101184] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/31/2022] Open
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Xia Y, Yu XF, Ma ZJ, Sun ZW. Hemorrhagic transformation of ischemic cerebral proliferative angiopathy: A case report. World J Clin Cases 2021; 9:5730-5736. [PMID: 34307632 PMCID: PMC8281439 DOI: 10.12998/wjcc.v9.i20.5730] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/11/2021] [Revised: 04/06/2021] [Accepted: 05/06/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Cerebral proliferative angiopathy (CPA) is a rare vascular disease characterized by the presence of diffuse vascular proliferation, progressive vascular hyperflow and vasodilation of multiple vessels in the normal brain parenchyma. Unlike cerebral arteriovenous malformations, CPA has a mixed appearance between that of lesions with cell proliferation and endothelial proliferation. To date, the pathogenesis of CPA is unclear, in which changes induced by cortical ischemia in the elastic layer of the blood supply artery and smooth muscle cells may be involved.
CASE SUMMARY In this article, we retrospectively analyzed a case of hemorrhagic transformation of ischemic CPA diagnosed by digital subtraction angiography and reviewed the related literature for further exploration of its pathogenesis, diagnosis and treatment.
CONCLUSION The information in the present case report may facilitate further clinical research on this cerebrovascular disease.
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Affiliation(s)
- Yu Xia
- Department of Neurology, The First Affiliated Hospital of Anhui Medical University, Hefei 230022, Anhui Province, China
| | - Xian-Feng Yu
- Department of Neurology, The First Affiliated Hospital of Anhui Medical University, Hefei 230022, Anhui Province, China
| | - Zhen-Juan Ma
- Department of Neurology, The First Affiliated Hospital of Anhui Medical University, Hefei 230022, Anhui Province, China
| | - Zhong-Wu Sun
- Department of Neurology, The First Affiliated Hospital of Anhui Medical University, Hefei 230022, Anhui Province, China
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