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新生儿大脑大静脉动脉瘤样畸形的超声产前诊断与急诊介入放射治疗

Ultrasound Prenatal Diagnosis and Emergency Interventional Radiologic Therapy of Galen Aneurysmal Malformation in a Newborn.

作者信息

Bohiltea Roxana Elena, Turcan Natalia, Mihalea Cristian, Dorobat Bogdan, Cinteza Eliza Elena, Dan Adriana, Mihai Magdalena, Dimitriade Adela, Boros Cristian, Dumitru Mircea, Gobej Ionut, Munteanu Octavian, Cirstoiu Monica Mihaela

机构信息

"Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania ; University Emergency Hospital, Bucharest, Romania.

Department of Interventional Neuroradiology, NEURI, Hopital Bicetre, APHP, Paris Sud Universite´ - Paris, France ; Department of Neurosurgery, University of Medicine and Pharmacy "Victor Babes," Timisoara, Romania.

出版信息

Maedica (Bucur). 2016 Dec;11(4):334-340.

Abstract

Located under the cerebral hemispheres and draining the anterior and central regions of the brain into the sinus of the posterior cerebral fossa, the vein of Galen aneurysmal malformation is considered to be a rare cause of hydrocephaly. The presence of this condition in the neonatal period typically includes intractable heart failure and a poor prognosis. We report a case of aneurysm of the vein of Galen diagnosed prenatally at 28 weeks of gestation, with the delivery at term by caesarean section of a female infant. Sonographically, the vein of Galen appeared in the mid-sagittal plane, large, supratentorial, non-pulsatile; on color Doppler, the structure filled with bright color, reflecting a turbulent venous flow. A low grade of ventriculomegaly was present during the evolution of pregnancy; regarding the cardiovascular function, an intrauterine right cardiac insufficiency overlapped a tricuspid regurgitation and right atrial dilatation. A multidisciplinary committee decided a neonatal embolization of the aneurysm as an emergency requirement due to increased pulmonary hypertension developed in the next 24 hours after birth. After the embolization of the two main drainage vessels, the cardiac dysfunction persists. Two days later the evolution became unfavorable, leading to the necessity of the second embolization, which resulted in a 48 hours' coma and death, due to a cerebral hemorrhage secondary to thrombosis and fissure of the embolized aneurysm. The prognosis for the neonate with malformation of the Galen vein depends upon the severity of the cardiovascular status. Embolization represents actually the treatment of choice with the best results of these cases, but the mortality remains as high as 50 percent even in the most specialized centers of the world. As far as we know this is the only case of Galen aneurysmal malformation in Romania which beneficiated of embolization by interventional treatment in neonatal period.

摘要

大脑大静脉动脉瘤样畸形位于大脑半球下方,将脑前部和中央区域的血液引流至后颅窝窦,被认为是脑积水的罕见病因。新生儿期出现这种情况通常包括难治性心力衰竭和预后不良。我们报告一例在妊娠28周时产前诊断为大脑大静脉动脉瘤的病例,足月剖宫产分娩一名女婴。超声检查显示,大脑大静脉出现在正中矢状面,大,幕上,无搏动;彩色多普勒检查时,该结构充满亮色,提示静脉血流紊乱。孕期过程中存在轻度脑室扩大;关于心血管功能,宫内右心功能不全合并三尖瓣反流和右心房扩张。由于出生后24小时内出现肺动脉高压加重,多学科委员会决定将新生儿动脉瘤栓塞作为紧急治疗措施。栓塞两条主要引流血管后,心脏功能障碍持续存在。两天后病情恶化,导致必须进行第二次栓塞,栓塞动脉瘤血栓形成和破裂继发脑出血,导致48小时昏迷后死亡。大脑大静脉畸形新生儿的预后取决于心血管状况的严重程度。栓塞实际上是这些病例的首选治疗方法,效果最佳,但即使在世界上最专业的中心,死亡率仍高达50%。据我们所知,这是罗马尼亚唯一一例在新生儿期接受介入治疗栓塞的大脑大静脉动脉瘤样畸形病例。

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