Purpose: In this article we explore the various aspects of the supratentorial Single-Hole AVFs (ST AVFs) in children, focusing on their clinical features, angio-architecture, treatment indications and the role and results of endovascular management.
Materials and methods: Among 1565 cases of brain AVMs seen at our neurovascular center, 620 cases were seen in the pediatric age group (<or= to 15 year-old) and 303/620 were non-Galenic brain AVMs. Among 52 brain AVFs found in children, we reviewed 41 pediatric patients with supratentorial single-hole cerebral AVFs (M/F=28/13) with a total of 63 AVFs. Mean age at presentation was 24 months. Four cases had their lesion diagnosed in utero without particular prognostic significance. 17 cases were multifocal with a total of 39 AVFs. The most common clinical presentation leading to diagnosis was cardiac sufficiency in (31.7%), epilepsy (24.4%), macrocrania (14.6%). Venous ectasia (87.8%) and pial venous stenosis (41.5%) were the most frequent angiographic features. There were 11 cases of Hemorrhagic Heriditary Telangiectasia (HHT1) (including two suspicious cases) among the 41 patients (26.8%) and among those 4 cases had single AVFs and 7 cases had multiple AVFs.
Results: Thirty-five children were treated in our institution: 34/35 by embolization alone, 1/35 by a combination of embolization and radiosurgery. 32/35 patients were treated with glue alone, 3/35 with coils (2/3 with glue also). A total of 57 separate lesions were treated. On clinical follow up (from 1985-2002, mean 4.2 years), 88.6% of patients proved to be either asymptomatic, improved from previous clinical symptoms or stabilized. 40% of patients had their lesion(s) already completely excluded. Post operative mortality was 5.6% (2/35), permanent neurological morbidity 3% (1/33). Twenty-six of these 33 patients (78.6%) were neurologically normal. 5/33 patients continue to have neurological symptoms or retardation. 2/33 patients died during follow up (1 ischemic stroke from Pulmonary AVF, 1 death despite partial embolisation).
Conclusion: Cerebral arteriovenous fistulae are a rare disease, but not infrequently seen in neonates and infants with AVMs. In one fourth of these patients HHT is suspected to be present. The AVFs are always superficial and fed by pial (cortical) arteries. They seldom reveal a hemorrhagic event. They are similar to those encountered in the posterior fossa or spinal cord. Endovascular treatment using NBCA was the treatment modality chosen resulting in a high rate of success and allowing children to grow up normally with no hemorrhages on follow up and no new symptom other than those already present on admission.