Kiymaz Nejmi, Yilmaz Nebi, Demir Ismail, Keskin Siddik
Department of Neurosurgery, Yuzuncu Yil University, Medical School, Van, Turkey.
Pediatr Neurosurg. 2010;46(1):6-11. doi: 10.1159/000314051. Epub 2010 May 5.
An encephalocele is a herniation of the brain and the meninges through a skull defect protruding towards the exterior. The condition is not rare when compared to spinal dysraphisms, but the worldwide incidence is not precisely known. The cases involving occipital encephaloceles which we have diagnosed in our clinic and the surgical approaches for this rare condition are presented herein.
Thirty patients who were diagnosed with occipital encephaloceles and referred to our Neurosurgery Clinic at the Yuzuncu Yil University, Faculty of Medicine Research Hospital between 2000 and 2009 were enrolled in this study. The age of the patient, size of the sac, pathologies that accompanied the condition, and treatments applied were assessed.
In the present study, 30 patients (22 girls and 8 boys), whose ages varied between newborn and 14 months, were evaluated. The encephalocele sac was located in the occipital region in 27 patients (90%) and in the occipitocervical region in 3 patients (3%). Nine (30%) of the 30 patients died; 2 in the preoperative period, 2 in the postoperative early period (0-7 days) and 5 in the late postoperative period (first week to 3 months). With the exception of the 2 patients who died preoperatively, surgery was performed on all of the patients. The mortality rate in our study was 29%.
Our study demonstrated that factors which determine the prognosis of patients diagnosed with occipital encephaloceles include the size of the sac, the contents of the neural tissue, hydrocephaly, infections, and pathologies that accompany the condition. An occipital encephalocele is a congenital neurologic condition with an extremely high morbidity and mortality in spite of the treatments rendered pre- and postoperatively.
脑膨出是指脑和脑膜通过颅骨缺损向外部突出的一种疝出情况。与脊柱裂相比，这种病症并不罕见，但全球发病率尚无确切数据。本文介绍了我们诊所诊断出的枕部脑膨出病例以及针对这种罕见病症的手术方法。
本研究纳入了2000年至2009年间被诊断为枕部脑膨出并转诊至于祖尔恰伊勒大学医学院研究医院神经外科诊所的30例患者。对患者的年龄、囊袋大小、伴随病症以及所采用的治疗方法进行了评估。
在本研究中，对30例年龄在新生儿至14个月之间的患者（22例女孩和8例男孩）进行了评估。27例患者（90%）的脑膨出囊位于枕部区域，3例患者（3%）位于枕颈区域。30例患者中有9例（30%）死亡；2例于术前死亡，2例于术后早期（0 - 7天）死亡，5例于术后晚期（第一周至3个月）死亡。除2例术前死亡的患者外，所有患者均接受了手术。我们研究中的死亡率为29%。
我们的研究表明，决定枕部脑膨出患者预后的因素包括囊袋大小、神经组织内容物、脑积水、感染以及伴随病症。尽管进行了术前和术后治疗，但枕部脑膨出仍是一种先天性神经系统疾病，发病率和死亡率极高。
