title: Treatment of multiple arteriovenous malformations in pediatric patients with hereditary hemorrhagic telangiectasia and spontaneous hemorrhage. Report of two cases.
abstract: Due to inheritance of an autosomal dominant genetic mutation, patients with hereditary hemorrhagic telangiectasia (HTT) have an increased risk of harboring a cerebral arteriovenous malformation (AVM). They are also significantly more likely to have multiple AVMs. The natural history of AVMs in patients with HHT as well as their rate of hemorrhage is not clearly defined. Furthermore, spontaneous regression of such lesions has been reported. Treatment of cerebral AVMs in patients with HHT presents a particular challenge, especially with detection of incidental lesions following screening of asymptomatic patients. The management of HHT in two pediatric patients in whom hemorrhaging from a cerebral AVM occurred but who also had other lesions is presented here. Both patients were treated with stereotactic radiosurgery.
authors.affiliation: Department of Neurosurgery, Royal Adelaide Hospital, Adelaide, South Australia. yuhk34@hotmail.com
authors.forename: Yu-Hung
authors.initials: YH
authors.lastname: Kuo
authors.forename: Stephen
authors.initials: S
authors.lastname: Santoreneos
authors.forename: Daniel
authors.initials: D
authors.lastname: Roos
authors.forename: Brian P
authors.initials: BP
authors.lastname: Brophy
mesh_terms: D002533:Cerebral Angiography; D002648:Child; D005260:Female; D006801:Humans; D002538:Intracranial Arteriovenous Malformations / Q000150:complications / Q000235:genetics / Q000601:surgery*; D020300:Intracranial Hemorrhages / Q000150:complications / Q000235:genetics / Q000601:surgery*; D019635:Neurosurgical Procedures; D011182:Postoperative Care; D016634:Radiosurgery; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications / Q000235:genetics / Q000601:surgery*; D014057:Tomography, X-Ray Computed