Spontaneous appearance of de novo intracranial arteriovenous malformation in hepatic cirrhosis.

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.

Subdural hematoma after endoscopic third ventriculostomy: Struggling against the Laplace law.

BACKGROUND: Endoscopic third ventriculostomy (ETV) is an ideal treatment for obstructive hydrocephalus. Although ETV is a safe procedure, several complications related to this procedure have been reported in the literature. We present a rare case of late onset symptomatic bilateral subdural hematoma after an uneventful ETV that proved particularly difficult to solve. CASE DESCRIPTION: A 61-year-old male patient presented in our neurosurgery department three months after ETV (aqueductal stenosis) with progressive headaches and anomic aphasia. The MRI revealed bilateral chronic subdural hematomas (chSDH). They were treated via a burr hole evacuation, subduroperitoneal and external subdural drains proving to be refractory to the first two strategies. Postoperatively, his headaches improved. At the last follow-up the patient's status remains improved and there is no radiological evidence of significant residual collections. CONCLUSION: This case confirms that chSDH formation is a rare possible complication following ETV even in the presence of a normal early postoperative image. Patients should be followed-up more closely for possible subdural collection formation. In the cases of very long-term hydrocephalus with a thin cerebral mantle, brain elastic properties are likely to be altered. As there is no possibility to close the internal shunt, the stoma, we advocate external subdural drainage to reinflate the brain, in the first intention or at least after an initial failed burr hole evacuation.