Gverić-Ahmetasević Snjezana, Colić Ana, Gverić Tugomir, Gasparović Vesna Elvedi, Pavlisa Goran, Ozretić David
University of Zagreb, Zagreb University Hospital Center, Neonatal Intensive Care Unit, Department of Obstetrics and Gynecology, Zagreb, Croatia.
Coll Antropol. 2011 Jan;35 Suppl 1:303-7.
Cerebral sinovenous thrombosis in neonatal period may cause neurological impairment, epilepsy, and lead to stroke. It is caused primarily by coagulopathy of numerous reasons, occasionally perinatal asphyxia, traumatic delivery and hyperhomocysteinemia. Dandy-Walker malformation is characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle, and enlargement of the posterior fossa. Dandy-Walker malformation, variant, and mega cisterna magna represent a spectrum of developmental anomalies. Insults to developing cerebellar hemispheres and the fourth ventricle are believed to be the cause of malformation. Our patient was born from noncomplicated pregnancy, noncomplicated nontraumatic vaginal delivery at term, excellent Apgar scores, without peculiarities in clinical status. She was brest-fed by the 42nd hour of life when she had rightsided seizures during sleep that repeated for five times in next 24 hours. Brain Ultrasound (US) revealed clot in left lateral ventricle, slight dilatation of left ventricle, both sided periventricular echodensity, ischemia, slight enlargement of forth ventricle and a bit smaller cerebellum. There was no visible flow through left transverse, superior sagittal and straight sinus. Magnetic Resonance (MRI) confirmed the finding and showed thrombosis of left and right transverse venous sinuses and confluence of sinuses. Electroencephalogram (EEG) showed leftsided focal changes. The newborn was treated with phenobarbiton for 8 days and had no convulsions during that period. All coagulation parameters, homocistein, lipoproteins (a) and D-dimers were normal. There were no mutations on FV R506Q, PT 20210A, MTHFR 677C/T. No antiphospholipides were found. Heart US showed no structural anomalies. No other patology or risk factors were present at the time. Before discharge, US showed hydrocephalus. Flow in affected sinuses was visible with color Doppler. MRI showed recanalization of affected sinuses, also hydrocephalus and presentation of Dandy Walker On EEG there was borderline finding. Due to progression of hydrocephalus ventriculo-peritoneal shunt was placed. In age of 1 year EEG was slower for age but without focus. Neurological development was normal for age. The question is whether this child had intrauterine insult and inception of Dandy Walker with further postnatal progress of thrombosis and evolution to full picture of Dandy Walker with hydrocephalus OR thrombosis that led to development of hydrocephalus and Dandy Walker malformation in this child were accidental coexistance.
新生儿期脑静脉窦血栓形成可能导致神经功能损害、癫痫,并引发中风。其主要由多种原因导致的凝血功能障碍引起,偶尔由围产期窒息、难产和高同型半胱氨酸血症导致。Dandy-Walker畸形的特征是小脑蚓部发育不全或发育不良、第四脑室囊性扩张以及后颅窝扩大。Dandy-Walker畸形、变异型和巨大枕大池代表了一系列发育异常。发育中的小脑半球和第四脑室受到损伤被认为是畸形的原因。我们的患者足月顺产,孕期及分娩过程均无并发症,阿氏评分良好,临床状态无异常。出生后42小时开始母乳喂养,睡眠中出现右侧癫痫发作,在接下来的24小时内反复发作5次。脑部超声(US)显示左侧脑室内有血栓,左侧脑室轻度扩张,双侧脑室周围回声增强、缺血,第四脑室轻度扩大,小脑稍小。左侧横窦、上矢状窦和直窦未见血流信号。磁共振成像(MRI)证实了上述发现,并显示左侧和右侧横窦及窦汇血栓形成。脑电图(EEG)显示左侧局灶性改变。新生儿接受苯巴比妥治疗8天,在此期间无惊厥发作。所有凝血参数、同型半胱氨酸、脂蛋白(a)和D-二聚体均正常。FV R506Q、PT 20210A、MTHFR 677C/T均无突变。未发现抗磷脂抗体。心脏超声显示无结构异常。当时无其他病理情况或危险因素。出院前,超声显示脑积水。彩色多普勒显示受累窦内有血流信号。MRI显示受累窦再通,也有脑积水及Dandy-Walker畸形表现。脑电图检查有临界发现。由于脑积水进展,行脑室-腹腔分流术。1岁时脑电图检查结果较同龄人慢,但无病灶。神经发育与年龄相符。问题是这个孩子是否在宫内受到损伤并开始出现Dandy-Walker畸形,随后在出生后血栓形成进一步发展,最终演变成伴有脑积水的完整Dandy-Walker畸形,还是血栓形成导致了这个孩子脑积水和Dandy-Walker畸形的发生,只是偶然并存。