Department of Neurosurgery, Institute of Medicine, University of Tsukuba, Ibaraki, 305-8575, Japan.
Department of Pediatrics, Institute of Medicine, University of Tsukuba, Ibaraki, 305-8575, Japan.
Childs Nerv Syst. 2023 Aug;39(8):2245-2249. doi: 10.1007/s00381-023-05961-w. Epub 2023 Apr 21.
Dural sinus malformations (DSMs) are rare congenital vascular diseases characterized by a giant venous pouch with or without arteriovenous shunts. We present a neonatal case of DSM that was diagnosed prenatally and treated via endovascular intervention in the early postnatal period. The patient presented with a large DSM involving the torcular Herophilion prenatal magnetic resonance imaging (MRI). Enlargement of the head circumference and respiratory failure rapidly progressed after birth. On the 5th day after birth, the neonate underwent endovascular occlusion via the umbilical artery. The arteriovenous shunt was occluded, and the reflux from the enlarged venous pouch to the dural sinus was decreased. No additional procedure other than ventriculoperitoneal shunting was required. The neonate's development slowly caught up to normal parameters. Follow-up MRI demonstrated the successful development of the venous drainage system. DSMs are characterized by an abnormally dilated dural sinus, which can block the venous return and ultimately increase intracranial pressure and cerebral ischemia. Long-term follow-up indicates that an abnormally developed dural sinus can be reconstructed by appropriate and timely treatment.
硬脑膜窦畸形(DSMs)是一种罕见的先天性血管疾病,其特征为巨大的静脉囊,伴或不伴有动静脉分流。我们报告了一例新生儿 DSM 病例,该病例在产前被诊断,并在新生儿早期通过血管内介入治疗。该患者在产前磁共振成像(MRI)中显示存在累及 Torcular Herophili 的大型 DSM。出生后,头围迅速增大,呼吸衰竭进展迅速。在出生后第 5 天,新生儿通过脐动脉进行了血管内闭塞。动静脉分流被阻断,从扩大的静脉囊中反流到硬脑膜窦的血流量减少。除了脑室-腹腔分流术外,无需进行其他额外的治疗。新生儿的发育逐渐恢复正常。随访 MRI 显示静脉引流系统发育良好。DSMs 的特征是硬脑膜窦异常扩张,可导致静脉回流受阻,最终导致颅内压升高和脑缺血。长期随访表明,通过适当和及时的治疗,可以重建发育异常的硬脑膜窦。