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首页> 外文期刊>The Canadian Journal of Neurological Sciences: le Journal Canadien des Sciences Neurologiques >Iniencephaly in an adult patient
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Iniencephaly in an adult patient

机译：成年患者的无脑

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A 24-year-old woman presented to the emergency department with a history of headache and malaise. She was known for a cervico-occipital meningoencephalocele repair and the placement of a ventriculoperitoneal (VP) shunt in infancy; she has otherwise been developing normally, has a normal intellect and is currently living independently. She had a short neck with low posterior hairline, and obvious spinal deformity (Figure 1). Neurologic examination was unremarkable with absence of long tract signs, no gait abnormality, no oculomotor or other cranial nerve deficits and no evidence of gross cerebellar dysfunction. Maternal and family history were negative. Her VP shunt had been revised six weeks earlier following an episode of malfunction. Investigation revealed an infection of the VP shunt with Propionibacterium acnes (P. acnes); the shunt hardware was therefore removed, an external ventricular drain was installed and she was placed on intravenous antibiotic therapy. After an appropriate course of antibiotics, magnetic resonance imaging (MRI) was done to assess the possibility of an endoscopic third ventriculostomy (ETV) (Figure 2). Judging the situation potentially favourable, we performed an ETV and the external ventricular drain was left in place. However, the patient could not be weaned from her ventricular drain, so a new VP shunt was installed after confirmation that her cerebrospinal fluid (CSF) was sterile. The postoperative course was uneventful, and she was discharged three days after surgery. At three months follow-up, she is currently doing very well.
机译：一名24岁的妇女因头痛和全身不适的历史来到急诊科。她因宫颈枕脑膜脑膨出的修复和婴儿期腹膜腹腔（VP）分流的放置而闻名。她本来可以正常发展，具有正常的智力并且目前独立生活。她的脖子短，后发际线低，明显有脊柱畸形（图1）。神经系统检查无异常，无长道征象，没有步态异常，没有动眼或其他颅神经功能障碍，也没有明显的小脑功能障碍的证据。母体和家族史均为阴性。发生故障后六周前，对她的副总裁分流器进行了修改。调查显示，痤疮丙酸杆菌（痤疮丙酸杆菌）感染了VP分流器。因此，移除了分流硬件，安装了外部心室引流管，并对其进行了静脉内抗生素治疗。经过适当的抗生素疗程后，进行了磁共振成像（MRI）以评估内镜第三脑室造口术（ETV）的可能性（图2）。判断情况可能有利，我们进行了ETV手术，并保留了外部心室引流管。但是，患者无法从心室引流中断奶，因此在确认其脑脊液（CSF）无菌后，安装了新的VP分流器。术后过程平稳，术后三天出院。经过三个月的随访，她目前情况良好。

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