Neuropediatrics 2011; 42 - P069
DOI: 10.1055/s-0031-1274041

Multiple arteriovenous malformations of the brain: A case report

E Sterl 1, SL Zeder 1, HG Eder 2, GE Klein 3, E Sorantin 4, U Gruber-Sedlmayr 5, M Brunner-Krainz 5, B Plecko 5
  • 1Univ.- Klinik für Kinder- und Jugendchirurgie, Graz, Austria
  • 2Univ.- Klinik für Neurochirurgie, Graz, Austria
  • 3Univ.- Klinik für Radiologie, Klinische Abteilung für vaskuläre und interventionelle Radiologie, Graz, Austria
  • 4Univ.- Klinik für Radiologie, Klinische Abteilung für Kinderradiologie, Graz, Austria
  • 5Univ.- Klinik für Kinder- und Jugendheilkunde, Klinische Abteilung für Neuropädiatrie, Graz, Austria

Introduction: In the general population the incidence of arteriovenous malformations (AVM) is ˜1 per 100.000 per year. About 9% of patients with cerebral vascular lesions suffer from multiple AVMs. Multiple AVMs often become evident during the course of disease. Some patients with multiple AVMs can be assigned to Rendu-Osler-Weber or Parkes-Weber-Syndrom (RASA I).

Case report: A 13 y old male patient presented with a swirring noise over the left A. carotis and puls-synchronized tinnitus.

CE- angiography revealed a combined durale-piale AVM in the region of the right basal ganglia and brainstem. There were signs of increased intracranial pressure with enlarged lateral ventricles, compressed 4th ventricle and herniation of the cerebellar tonsills.

Within a period of 4 years the patient underwent 10 sessions of endovascular therapy (embolisation by coiling). After his 3rd embolisation, a cerebellar AVM became apparent. The 8th embolization was complicated by retroperitoneal bleeding and scrotal hematoma necessitating surgical intervention. Aside from transient hypasthesia of his right upper leg, he was free of neurological symptoms over the following 3 years. At the age of 17y he presented with acute left-sided hemiparesis due to an intracranial bleeding within the basal ganglia of the right hemisphere. He developed posthemorrhagic hydrocephalus needing extracranial drainage followed by a ventriculo-peritoneal shunt. Shunt revision was complicated by subdural and subarachnoidal bleeding and neurologic deterioration. In addition he developed a difficult to treat focal epilepsy. Molecular analysis of the RASA I gene was normal.

Conclusion: Despite repeated embolisation this patient with multiple AVMs developed spontaneous intracranial bleeding, posthemorrhagic hydrocephalus and severe neurologic sequelea. Beside this endovascular therapy neurosurgery and stereotactic radiosurgery (e.g. gamma knife) or a combination of these have to be considered to prevent hemorrhages in patients with multiple AVMs.