Paediatric intracranial dural arteriovenous fistulas: clinical characteristics, treatment outcomes and prognosis
Xin Su, Yongjie Ma, Zihao Song, Ming Ye, Hongqi Zhang, Peng Zhang
doi: 10.1136/svn-2024-003122
Stroke & Vascular Neurology（SVN）最新上线文章“Paediatric intracranial dural arteriovenous fistulas: clinical characteristics, treatment outcomes and prognosis”，来自首都医科大学宣武医院神经外科张鸿祺教授、介入放射诊断治疗中心张鹏教授团队等。
与成人硬脑膜动静脉瘘（dural arteriovenous fistulas, DAVFs）相较，儿童DAVFs具有临床表现独特、治愈率低、预后不良等特点，但受限于样本量较小，其远期预后及随访资料尚不详。
作者团队记录并分析了所在机构2002年～2022年期间43例儿童DAVFs连续患者的临床资料。根据预后差异（Lasjaunias分类），将DAVFs分为婴儿型和非婴儿型（成人型和硬脑膜窦畸形（dural sinus malformation, DSM））。
研究结果显示，首次出现症状的平均年龄为8.4±6.0岁。29例男孩和14例女孩在从出生至18岁之间发病。10例≤1岁的患者中，5例出现无症状性心脏肥大；33例>1岁的患者中，5例出现无症状性心脏肥大（p=0.022）。42例（88.4%）患者接受了单纯血管内治疗，9.3%接受了放射外科手术、钻孔栓塞或手术。28例（65.1%）患者在治疗结束时DAVF闭塞。其中，26例仅接受了栓塞治疗，1 例栓塞联合手术，1 例接受钻孔栓塞治疗。患者总体并发症发生率为9.3%，均源于血管内治疗。根据Lasjaunias分类，成人型18 例，婴儿型17例，DSM8例。与非婴儿型DAVFs相比，婴儿型DAVFs治疗次数更多、治愈率更低、预后更差（p<0.001, 0.003 and 0.021, respectively）。平均随访时间为41.4±36.2个月（3～228个月）。8例（22.9%）患者死亡。
Figure 1. Kaplan-Meier curves demonstrating probability of survival rates for all patients as the follow-up time in months. Kaplan-Meier curves (log-rank test) illustrating probability of survival rates of paediatric DAVFs in non-infantile-type DAVFs were higher than infantile-type DAVFs.
Figure 2. A paediatric patient in age range of 10 presented with headache, bilateral impaired vision and papilledema. (A–C) The sigmoid sinus and transverse sinus were occluded (venous sinus thrombosis) on the right side and the superficial cerebral vein (A and B, black arrow heads) was mainly involved in venous drainage. On the left side, the sigmoid sinus and transverse sinus were patent (C). Note the marked venous congestion (B). (D–F) Right external carotid artery and vertebral artery angiograms revealed a right Cognard III/Borden III/adult type isolated transverse sinus (E and F, white arrow heads) dural arteriovenous fistula mainly supplied by right posterior branch and petrosal branch of the middle meningeal artery, right transosseous branches of the left occipital artery, the right posterior meningeal artery and posterior cerebral artery with cortical veins drainage. (G) Complete embolisation was achieved via the posterior meningeal artery and posterior cerebral artery using Glubran, the middle meningeal artery using Onyx. (H) No recurrence was observed 3 months after complete embolisation. There has been a slight improvement in vision compared with before, and modified Rankin Scale score was 1.
Figure 3. An infant presented with vomiting and seizure also diagnosed with congenital heart disease (ventricular septal defect). (A, B) Sagittal T1-weighted MRI (A) and axial T2-weighted MRI revealed a massive torcular herophili. (C–E) Common carotid artery and vertebral artery angiograms revealed a midline Cognard IIa+b/Borden II dural arteriovenous fistula with dural sinus malformation mainly supplied by bilateral occipital artery and dural branches of the left posterior inferior cerebellar artery. Note that both transverse sinuses have poor drainage, and the draining veins primarily involved the straight sinus-internal cerebral vein and the superior sagittal sinus-cortical veins. (F) Complete embolisation was achieved via the posterior inferior cerebellar artery using coils and Onyx. (G, H) There was no recurrence 21 months after complete embolization. He was asymptomatic, and no developmental delays had been found.
Figure 4. A paediatric patient in the age range of 10 presented with haemiplegia and seizure. (A–C) External and internal carotid artery angiograms revealed high-grade infantile type/multifocal (transverse sinus, anterior cranial fossa) dural arteriovenous fistulas mainly supplied by multiple dural and pial branches from the occipital artery, ophthalmic artery, meningohypophyseal trunk and middle cerebral artery, and so on. Due to bilateral transverse sinus obstruction, venous drainage primarily involved the straight sinus, the superior sagittal sinus and internal cerebral vein. (D, E) The blood flow in the arteriovenous fistula has significantly decreased after four treatment sessions, but new blood vessels continue to appear during each angiography examination and embolisation. The anatomic cure of the fistula was difficult to achieve, and the patient eventually decided to undergo regular follow-up. Unfortunately, the patient died 8 years after the last partial embolisation due to intracranial haemorrhage.
研究结论：目前大多数成人型DAVFs和DSMs可通过栓塞术有效治疗，疗效和预后良好。但婴儿型DAVFs的治疗仍存在挑战，预后通常较差。
更多内容详见文章原文：https://svn.bmj.com/content/early/2024/06/05/svn-2024-003122，或点击文末“阅读原文”。
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