Jagadeesan Bharathi D, Grande Andrew W, Guillaume Daniel J, Nascene David R, Tummala Ramachandra P
Department of Radiology.
Department of Neurosurgery, and.
J Neurosurg Pediatr. 2015 Jul;16(1):74-9. doi: 10.3171/2014.12.PEDS145. Epub 2015 Apr 24.
Dural sinus malformations (DSMs) are rare congenital malformations that can be midline or lateral in location. Midline DSMs have been reported to have a worse prognosis than lateral DSMs and have traditionally been more difficult to manage. The authors report 2 unusual manifestations of midline DSMs and their management with percutaneous transfontanelle embolization. The first patient (Case 1) presented at 21 days of life with a large midline DSM and multiple highflow dural and pial arteriovenous shunts. The child developed congestive cardiac failure and venous congestion with intracranial hemorrhage and seizures within a few weeks. The second patient (Case 2) presented with a large midline DSM found on prenatal imaging that was determined to be a purely venous malformation on postnatal evaluation. This large malformation resulted in consumptive coagulopathy and apneic episodes from brainstem compression. The patient in Case 1 was treated initially with endovascular embolization and eventually with curative percutaneous-transfontanelle embolization. The patient in Case 2 was treated with percutaneous transfontanelle embolization in combination with posterior fossa decompression and cranial expansion surgery.
硬脑膜窦畸形（DSM）是罕见的先天性畸形，可位于中线或外侧。据报道，中线DSM的预后比外侧DSM差，并且传统上更难处理。作者报告了中线DSM的2种不寻常表现及其经皮经囟门栓塞治疗方法。首例患者（病例1）出生21天时出现一个巨大的中线DSM以及多处高流量硬脑膜和软脑膜动静脉分流。该患儿在几周内出现充血性心力衰竭、静脉充血伴颅内出血和癫痫发作。第二例患者（病例2）在产前影像学检查中发现一个巨大的中线DSM，产后评估确定为单纯静脉畸形。这个大畸形导致消耗性凝血病和脑干受压引起的呼吸暂停发作。病例1的患者最初接受血管内栓塞治疗，最终接受根治性经皮经囟门栓塞治疗。病例2的患者接受了经皮经囟门栓塞治疗，并结合后颅窝减压和颅骨扩张手术。
