Idiopathic syrinx in the pediatric population: a combined center experience Clinical article

Object. The treatment of vein of Galen aneurysmal malformation (VGAM) is among the most challenging of all the neurovascular disorders. Methods. Between 1984 and 2005, 26 consecutive patients with VGAMs were treated by the authors' group, and their data were prospectively collected; 12 patients presented with congestive heart failure (CHF) and 10 had hydrocephalus. Five patients did not undergo endovascular treatment because of minimal symptoms or severe comorbidities. Twelve patients underwent embolization for refractory CHF requiring ventilation: 7 of these 12 patients had the procedure in the neonatal stage (5 survived), and 5 were treated in infancy or childhood following successful aggressive medical treatment (4 survived). The other 9 patients underwent endovascular surgery to treat a progressively enlarging head circumference; there was imaging evidence of ventricular enlargement and/or signs of developmental delay (6 underwent surgery in infancy and 3 in childhood; 1 patient died). Results. The survival rate in this series was 76.9% (20 of 26). Fourteen (66.7%) of 21 patients who underwent endovascular treatment had no developmental delay. An analysis of various factors demonstrated that comorbidities and embolization effect (procedure success and long-term effect) were prognostic factors of survival. In addition, the patient's age at embolization was significantly higher in those with developmental delay than in those without (559.86 +/- 535.43 days vs 94.83 +/- 95.77 clays, respectively; p = 0.028). Conclusions. The authors conclude that neonatal VGAMs can be treated successfully with a strategic approach integrating antenatal diagnosis, endovascular surgery, treatment at intensive care facilities, and the cooperative efforts of different specialties. In the authors' experience, patients in whom VGAM was diagnosed and managed in infancy or childhood had more than 90% long-term survival. (DOI: 10.3171/2010.9.PEDS0956)