Dumrongpisutikul Netsiri, Triampo Atip, Janthanimi Pun, Lerdlum Sukalaya
*Department of Radiology, Faculty of Medicine, Chulalongkorn University †Department of Radiology, King Chulalongkorn Memorial Hospital, Bangkok, Thailand.
J Craniofac Surg. 2017 Mar;28(2):454-458. doi: 10.1097/SCS.0000000000003357.
Frontoethmoidal encephalomeningocele (FEEM) is a congenital disorder characterized by herniation of brain and meninges through an anterior skull defect. The main pathological changes are found internally at the foramen cecum and externally at the frontonaso-orbital region. The aim of this study was to determine the incidence of developmental anomalies found in FEEM using multidetector computed tomography images of the facial bone.
A total of 78 patients who underwent multidetector computed tomography scan of the facial bone during the January 1, 2003 to June 31, 2012 study period were retrospectively reviewed. Demographic data, size of internal defect, and intracranial anomalies were recorded.
Associated brain and ophthalmic anomalies were identified in 53 patients (67.9%), and all of those had brain anomalies. The most common brain anomalies were absent/undetermined septum pellucidum (75.5%), ventricular dilatation (71.7%), abnormal frontal horn (67.9%), and dysgenesis of the corpus callosum (58.5%). Eight patients (10.3%) had ophthalmic anomalies. Patients with brain and ophthalmic anomalies tended to have a higher incidence of large-size internal defects.
The authors found a high prevalence of developmental anomalies in FEEM patients. Absent/undetermined septum pellucidum was the most commonly found anomaly in this study. Brain and ophthalmic anomalies tended to have a higher incidence of large-size internal bone defects. Consistent with the authors' acknowledged limitation, further studies using dedicated brain MRI and magnetic resonance angiography to evaluate parenchymal abnormalities and vascular anomalies may be beneficial for surgical planning, prognosis, and the identification of clinical correlations.
额筛部脑膨出（FEEM）是一种先天性疾病，其特征是脑和脑膜通过颅骨前部缺损疝出。主要病理变化在内部见于盲孔，在外部见于额鼻眶区域。本研究的目的是利用面部骨骼的多排螺旋计算机断层扫描图像确定FEEM中发现的发育异常的发生率。
回顾性分析2003年1月1日至2012年6月31日研究期间接受面部骨骼多排螺旋计算机断层扫描的78例患者。记录人口统计学数据、内部缺损大小和颅内异常情况。
53例患者（67.9%）发现有相关的脑和眼部异常，所有这些患者都有脑异常。最常见的脑异常是透明隔缺如/未确定（75.5%）、脑室扩张（71.7%）、额角异常（67.9%）和胼胝体发育不全（58.5%）。8例患者（10.3%）有眼部异常。有脑和眼部异常的患者往往内部大尺寸缺损的发生率更高。
作者发现FEEM患者中发育异常的患病率很高。透明隔缺如/未确定是本研究中最常见的异常。脑和眼部异常往往内部大尺寸骨缺损的发生率更高。与作者承认的局限性一致，使用专用脑磁共振成像和磁共振血管造影进一步研究以评估实质异常和血管异常可能有助于手术规划、预后以及确定临床相关性。
