背景:Dandy-Walker畸形是一种罕见的先天性脑缺损,以第四脑室囊性扩张为特征,后颅窝扩大。病因仍然知之甚少,但被认为是多因素的,很少由颅内出血引起。我们描述了一例已知分娩后患有与蛛网膜下腔出血相关的Dandy-Walker畸形的男性新生儿,这是一个安静的罕见的介绍,只在之前的一些文献中讨论过。
方法:我们介绍了一例罕见的足月男婴经阴道分娩,在产前异常扫描中被诊断为Dandy-Walker畸形.出生时,婴儿发出微弱的哭声,紫癜,呼吸窘迫和癫痫发作。分娩后计算机断层扫描显示蛛网膜下腔出血。此外,在影像学检查中发现了脑积水,并采用脑室-腹腔分流术治疗,作为早期干预的结果,后颅窝囊肿和脑积水的明显改善。
结论:很少有文献研究表明胎儿早期颅内出血与Dandy-Walker畸形的发展之间存在关联,因为它会影响后颅窝组件的生长。然而,我们的病例强调了早期诊断为Dandy-Walker畸形的足月婴儿分娩后自发性蛛网膜下腔出血的异常表现.
结论:本报告强调了早期识别和实施与脑出血相关的脑积水的适当管理的重要性,以预防高颅内压和脑干疝的并发症,并获得最佳结果。
BACKGROUND: Dandy-Walker malformation is a rare congenital brain defect characterized by vermian agenesia with cystic dilatation of the fourth ventricle, and posterior fossa enlargement. The etiology is still poorly understood but is presupposed to be multifactorial, infrequently caused by intracranial hemorrhage. We describe a case of male newborn known to have Dandy-Walker malformation associated with subarachnoid bleeding after the delivery, which is a quiet rare presentation only discussed in a few literatures before.
METHODS: We present a rare case of a full-term male baby delivered vaginally, who was diagnosed with Dandy-Walker malformation during antenatal anomaly scan. At birth, the baby presented with a weak cry, cyanosis, respiratory distress and seizure. Post-delivery computed tomography scan revealed subarachnoid hemorrhage. In addition, a hydrocephalus was noted on the imaging and treated with ventriculoperitoneal shunt insertion with marked improvement of the posterior fossa cyst and the hydrocephalus as an outcome of early intervention.
CONCLUSIONS: Few literature studies showed an association between intracranial bleeding during early fetal life and the development of Dandy-Walker malformation as it affects the posterior fossa components growth. However, our case highlights on an unusual presentation of the spontaneous subarachnoid hemorrhage after the delivery in a full-term baby diagnosed with Dandy-Walker malformation earlier.
CONCLUSIONS: This report highlights the importance of early recognition and implementing appropriate management of the hydrocephalus that associated with intracerebral bleeding to prevent the complications of high intracranial pressure plus brainstem herniation and achieve the best possible outcome.