Cavernous malformation hemorrhage due to trans-mural pressure alterations after cerebrospinal fluid diversion: a case report

Abstract

Background: Cavernous malformations are rare cerebral pseudo-vascular lesions with annualized bleeding rates of 0.5-3% in most studies. Of the various explored risk factors for bleeding to date, only prior hemorrhage has shown significant correlation. Case presentation: In this case, we describe a 65-year old man with a peri-ventricular atrial cavernous malformation that hemorrhaged after CSF diversion via ventriculoperitoneal shunting. Serial imaging showed that bleeding continued until the shunt was revised with a programmable valve set at maximum resistance with the addition of a gravitational unit, thereby lowering the trans-mural pressure differential across the cavernous malformation. Conclusions: Given that other vascular lesions are subject to hemorrhage from alterations in trans-mural pressure dynamics, we hypothesize that cavernous malformations are similarly affected by trans-mural pressure gradients as they are composed of primitive vascular elements. This hypothesis is corroborated by the temporal correlation of interventions, imaging, and exam findings in the present case, and suggests a potentially important risk factor for hemorrhage in CM patients that affects prognostication and management.

Cerebral Cavernous Malformation Bleeding Following Cerebrospinal Fluid Diversion Surgery: A Case Report and Literature Review

“…It is known that the morbidity of re-bleeding is higher than the first bleeding event [ 5 ]. The relation between changes in ICP and bleeding risk is well established in other vascular malformations such as cerebral and aortic aneurysm and arteriovenous malformation [ 8 ]; however, only one case report exists that describes this phenomenon in CM [ 8 ]. Similarly, in our case, a patient with a previously bled left atrial CM with a presentation of hydrocephalus that was treated with programmable Anti-Siphon ventriculoperitoneal shunt in which CM-related haemorrhage was stopped after multiple trials of valve rate adjustments [ 8 ].…”

“…The only proven risk factors for CM bleeding include brainstem location and previous bleeding. Here, we present the second case of CM bleeding following CSF diversion [ 8 ] and discuss the effect of intracranial pressure (ICP) changes on the intramural cavernoma pressure changes relevant to CM bleeding.…”