2025 Volume 53 Issue 1 Pages 40-47
Background: Pediatric arteriovenous malformations (AVMs) often present with a small, diffuse nidus, making identification of the rupture point challenging during acute rupture. This diffuse nature increases the likelihood of residual AVMs during removal. Pediatric AVMs exhibit dynamic changes and tend to recur. The optimal treatment strategy and appropriate duration and methods of follow-up remain undefined.
Methods: In this study, we reviewed the clinical challenges and outcomes of seven cases of ruptured AVMs in patients aged ≤16 years treated at our institution between 2012 and 2024. AVMs in surgically accessible regions require elective surgical resection after endovascular embolization, if feasible, whereas those located deeper warrant Gamma Knife radiosurgery (GKS). A follow-up cerebral digital subtraction angiography (DSA) is necessitated during the early postoperative period, 1–2 years after surgery, and ideally, just before adulthood.
Results: As per the Spetzler–Martin grading system, six and one cases were graded Grade 2 and Grade 3, respectively. Based on angiographic findings, nidus characteristics were classified as compact in four cases and diffuse in three. The primary treatment modality was elective surgery, with the treatment involving preoperative embolization followed by removal in four cases, acute hematoma partial evacuation/external decompression followed by removal in two cases, and GKS in one case. Nidus removal was performed on average 29.8 days after the hemorrhage. The modified Rankin Scale scores at 90 days after hemorrhage were 0 in two cases, and 2 in five cases, indicating favorable outcomes. One of the six patients undergoing nidus removal displayed a hidden compartment on early postoperative DSA, while another experienced recurrence three years after complete surgical resection, which was confirmed angiographically. Both cases had diffuse nidus involvement and required additional GKS. Complete obliteration of the AVMs was confirmed in all seven cases through cerebral DSA during an average follow-up of 5.5 years.
Conclusion: Considering the pathological and clinical characteristics of pediatric AVMs, long-term careful follow-up is crucial even after curative surgery. DSA is deemed indispensable for a thorough follow-up and should be performed shortly after the surgery to check for residual nidus or vascular anomalies potentially obscured by the hematoma, then at 1–2 years postoperatively to evaluate for recurrence, and again when transitioning to adulthood to detect any late-onset recurrence or de novo AVM.
