Morbidity after Hemorrhage in Children with Untreated Brain Arteriovenous Malformation

Abstract

Background: Children with untreated brain arteriovenous malformations (bAVM) are at risk of encountering life-threatening hemorrhage very early in their lives. The primary aim of invasive treatment is to reduce unfavorable outcome associated with a bAVM rupture. A better understanding of the morbidity of bAVM hemorrhage might be helpful for weighing the risks of untreated bAVM and invasive treatment. Our aim was to assess the clinical outcome after bAVM rupture and identify features to predict severe hemorrhage in children. Methods: We identified all consecutive children admitted to our institution for bAVMs between July 2009 and December 2014. Clinical outcome after hemorrhagic presentation and subsequent hemorrhage was evaluated using the modified Rankin Scale (mRS) for children. The association of demographic characteristics and bAVM morphology with severe hemorrhage (mRS >3 or requiring emergency hematoma evacuation) was studied using univariate and multivariable regression analyses. A nomogram based on multivariable analysis was formulated to predict severe hemorrhage risk for individual patients. Results: A total of 134 patients were identified with a mean treatment-free follow-up period of 2.1 years. bAVM ruptured in 83 (62%) children: 82 had a hemorrhage at presentation and 6 of them experienced a recurrent hemorrhage during follow-up; 1 patient had other diagnostic symptoms but bled during follow-up. Among them, 49% (41/83) had a severe hemorrhage; emergency hematoma evacuation was required in 28% of them (23/83), and 24% (20/83) remained as disabled (mRS ≥3) at last follow-up. Forty-six percent (38/82) of children with hemorrhagic presentation were severely disabled (mRS >3). Forty-three percent (3/7) were severely disabled after subsequent hemorrhage. The annual rate of severe subsequent hemorrhage was 1% in the overall cohort and 3.3% in children with ruptured presentation. All the subsequent severe hemorrhage events occurred in children with severe hemorrhage history (7%, 3/41). Periventricular location, non-temporal lobe location, and long draining vein were predictors for severe hemorrhage in pediatric untreated bAVMs. A nomogram based on bAVM morphology was contracted to predict severe hemorrhage risk for individual patients, which was well calibrated and had a good discriminative ability (adjusted C-statistic, 0.72). Conclusions: Evaluating bAVM morbidity and morphology might be helpful for weighing the risks of untreated bAVM in pediatric patients.

Long-term outcomes and prognostic predictors of 111 pediatric hemorrhagic cerebral arteriovenous malformations after microsurgical resection: a single-center experience

“…The short-term neurological outcomes were evaluated 1 week after the surgery, and the long-term neurological outcomes were assessed at the last follow-up. The neurological status were assessed by modified Rankin Scale (mRS) system for children with agespecific modification as described previously [22]. The presence of new or persisting neurological deficits, or repeat hemorrhage were collected during the follow-up.…”

Contemporary management of brain arteriovenous malformations in mainland China: a web-based nationwide questionnaire survey

“…In fact, pediatric bAVMs were often treated more aggressively than adults [21]. Minimum trauma and maximum occlusion were the intervention principle for pediatric bAVMs [22]. Similar to giant bAVMs, multi-modality strategy was more preferred for pediatric bAVMs in this study.…”

Early Rebleeding after Brain Arteriovenous Malformation Rupture, Clinical Impact and Predictive Factors: A Monocentric Retrospective Cohort Study

“…Rupture is the leading cause of death and dependence in the natural history of brain arteriovenous malformations (BAVMs) and is an important reason for incurring social and healthcare costs related to the treatment of this disease [1,2,3]. Recurrent hemorrhage is a major concern in BAVMs management.…”