Spontaneous appearance of de novo intracranial arteriovenous malformation in hepatic cirrhosis

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.

Risk of Cerebral Aneurysm Rupture After Liver Transplantation: Development and Validation of a Hemorrhagic Stroke Scoring Model

“…Nonetheless, a sequence of initial thrombogenic, inflammatory, mechanical, or ischemic insults has been observed to lead to the pro-angiogenic state required for the development of these vascular malformations. 29 Also hepatopathy may play a pivotal role in this process, given its common etiological association with peripheral systemic AVMs. Chronic venous hypertension resulting from hepatopathy can induce tissue hypoxia, thereby contributing to angiogenesis.…”

Multiple De Novo Cerebral Arteriovenous Malformations in a Patient with Alcoholic Liver Cirrhosis

“…We present a very rare case of cAVM that was confirmed to be an acquired form associated with LC. Only two cases of de novo cAVM associated with LC have been reported in the literature [ 2 3 ]. However, unlike the previously reported cases, our patient showed multiple cAVMs, which are known to only occur in 0.3%–4% of all cases of cAVMs, except in cases of hereditary autosomal disease [ 4 ].…”

Spontaneous Bilateral Subdural Hematomas in a Patient With Cryptogenic Liver Cirrhosis

“…Diffuse cerebral atrophy is also commonly seen in cirrhotic patients, especially those with alcohol use, resulting in the higher vulnerability of bridging vessels to rupture, leading to SDH formation. Increased de-novo arteriovenous malformations (AVMs) in cirrhotic patients might be related to venous hypertension and pro-angiogenic changes resulting from hepatic dysfunction [ 14 ]. Rupture of these AVMs at the cortical surface may present with isolated acute SDH [ 6 , 15 ].…”