Schick Bernhard, Prescher Andreas, Hofmann Erich, Steigerwald Christof, Draf Wolfgang
Department of Otolaryngology and Head and Neck Surgery, University of Homburg-Saar, Kirrberger Str, 66421 Homburg, Germany.
Eur Arch Otorhinolaryngol. 2003 Oct;260(9):518-21. doi: 10.1007/s00405-003-0620-0. Epub 2003 May 8.
Occult malformations of the skull base are rare anomalies, but can cause severe complications such as meningitis. Detailed skull base investigations for detecting cerebrospinal fluid fistulas or celes are often not initiated until after a history of recurrent meningitis. We present a child first seen at the age of 12 with recurrent episodes of bacterial meningitis since early childhood, requiring antibiotic prophylaxis for years. High-resolution computed tomography revealed a chronic sinusitis and a bony defect on the right olfactory groove, while magnetic resonance imaging and CT-cisternography indicated no cerebrospinal fluid fistula or cele at that time. Endonasal surgery for chronic sinusitis was performed with a confirmed bony defect on the right olfactory groove and an olfactory fibre without its sleeve-like dura prolongation running into an adjacent ethmoidal cell, necessitating that it be covered. In the absence of any antibiotics a new episode of meningitis occurred 5 years after surgery. CT-cisternography and magnetic resonance imaging were repeated, now indicating a transclival bony defect with a meningocele in its proximal part, most probably presenting a canalis basilaris medianus. Endonasal surgery confirmed this bony defect after adenoidectomy, and closure was accomplished. No further meningitis has been observed for 2 years. Congenital skull base defects may be difficult to detect, but sufficient surgical closure after their precise delineation is mandatory to prevent infectious endocranial complications. The presence of more than one developmental skull base defect should be considered during careful radiological skull base evaluation, which has to include the clivus in order not to overlook rare basilar malformations.
颅底隐匿性畸形是罕见的异常情况,但可导致如脑膜炎等严重并发症。对于检测脑脊液瘘或脑膨出的详细颅底检查通常直到有复发性脑膜炎病史后才开始。我们报告一名12岁首次就诊的儿童,自幼儿期起就有复发性细菌性脑膜炎发作,多年来需要抗生素预防。高分辨率计算机断层扫描显示慢性鼻窦炎和右侧嗅沟骨质缺损,而磁共振成像和CT脑池造影当时未显示脑脊液瘘或脑膨出。对慢性鼻窦炎进行了鼻内手术,术中证实右侧嗅沟骨质缺损,一条嗅神经纤维没有其袖状硬脑膜延伸进入相邻筛窦气房,需要对其进行覆盖。在未使用任何抗生素的情况下,术后5年发生了一次新的脑膜炎发作。再次进行CT脑池造影和磁共振成像,此时显示经斜坡骨质缺损,其近端有一个脑膜膨出,很可能是基底正中管。鼻内手术在腺样体切除术后证实了这一骨质缺损,并完成了修补。2年来未再观察到脑膜炎发作。先天性颅底缺损可能难以检测,但在精确描绘后进行充分的手术修补对于预防颅内感染并发症至关重要。在仔细的颅底影像学评估过程中应考虑存在不止一处发育性颅底缺损,评估必须包括斜坡,以免遗漏罕见的基底畸形。