R Gondar1, A El Rahal2, Z Kulcsár3, K Schaller2, S Momjian2. 1. Neurosurgery Division, Department of Clinical Neurosciences, Faculty of Medicine, Geneva University Medical Center, Geneva, Switzerland; Department of Spine Surgery and Neurosurgery, University of Fribourg, Hôpital Fribourgeois, Chemin des Pensionnats 2-6, 1700 Fribourg, Switzerland. Electronic address: rjag20@gmail.com. 2. Neurosurgery Division, Department of Clinical Neurosciences, Faculty of Medicine, Geneva University Medical Center, Geneva, Switzerland. 3. Interventional Neuroradiology Division, Department of Radiology and Medical Informatics, Faculty of Medicine, Geneva University Medical Center, Geneva, Switzerland; Department of Neuroradiology, University Hospital of Zurich, Frauenklinikstraße 10, 8091 Zürich, Switzerland.
Abstract
BACKGROUND: Intracranial arteriovenous malformations (AVMs) are rare lesions that can be congenital or acquired in early childhood, with fatal outcome in approximately 30% of cases. De novo formation during adulthood without established predisposing vascular pathology or previous brain insult is even less frequent. CASE DESCRIPTION: We present a case of de novo brain AVM in an alcoholic Child-B cirrhosis setting. Thirty previously reported cases presented de novo AVM in patients of all ages that had another previous brain pathology or insult, such as AVM resection. Seventeen of those cases occurred in adult patients, with only 2 showing no significant predisposing factor. The present pathophysiological review covers and completes Mullan's hemodynamic "two-hit" model, associating probable thrombotic predisposition to AVM with brain insult triggering a later stage based on angiogenic stimuli. CONCLUSIONS: This case report and literature review renews previously discussed hemodynamic theories and contributes to a fuller understanding of the pathogenesis and progression of AVM. We postulate a causal link between hepatopathy and de novo AVM, which should be strengthened and interpreted based on recent genetic data and future prospective studies.
BACKGROUND:Intracranial arteriovenous malformations (AVMs) are rare lesions that can be congenital or acquired in early childhood, with fatal outcome in approximately 30% of cases. De novo formation during adulthood without established predisposing vascular pathology or previous brain insult is even less frequent. CASE DESCRIPTION: We present a case of de novo brain AVM in an alcoholic Child-B cirrhosis setting. Thirty previously reported cases presented de novo AVM in patients of all ages that had another previous brain pathology or insult, such as AVM resection. Seventeen of those cases occurred in adult patients, with only 2 showing no significant predisposing factor. The present pathophysiological review covers and completes Mullan's hemodynamic "two-hit" model, associating probable thrombotic predisposition to AVM with brain insult triggering a later stage based on angiogenic stimuli. CONCLUSIONS: This case report and literature review renews previously discussed hemodynamic theories and contributes to a fuller understanding of the pathogenesis and progression of AVM. We postulate a causal link between hepatopathy and de novo AVM, which should be strengthened and interpreted based on recent genetic data and future prospective studies.