Raman Sharma Rewati, Mahapatra Ashok K, Pawar Sanjay J, Thomas Chona, Al-Ismaily Mohamed
The National Neurosurgery Centre, Khoula Hospital, Mina-Al-Fahal, Postal Code-116, Sultanate of Oman.
J Clin Neurosci. 2002 Jan;9(1):89-92. doi: 10.1054/jocn.2001.0861.
Trans-sphenoidal encephaloceles are rare entities. Only about 20 such cases have been reported in the world literature to date. Among these, the trans-sellar variety is rarest (five cases). We present two cases of trans-sellar trans-sphenoidal encephaloceles seen over the last 12 years. The first case was a 5 month old male infant presenting with nasal obstruction and a mass in the mouth. The second case was a 14 year old boy presenting with chronic nasal obstruction since early childhood. In the first case, the mass was coming to the mouth through the split palate. In the second case, the mass could be seen in the posterior half of the nose by rhinoscopy. The computed tomography scans including coronal and sagittal reconstructions had revealed trans-sellar trans-sphenoid encephaloceles in both the patients. The magnetic resonance imaging in the second case showed the cavity and the structures of the floor of the third ventricle forming the main fundal contents. The magnetic resonance angiography revealed dipping of the ectatic anterior cerebral arteries in the neck of the encephalocele sac in this case. Extracranial trans-palatal approach and the repair of encephalocele was done in the first case with the palatal reconstruction at the same sitting. In the second patient, a trans-nasal approach with bilateral maxillotomies was performed to repair the encephalocele, and the skull base defect was repaired by a split rib graft, which was fixed with mini-plate and screws posteriorly at the clivus and anteriorly at the anterior edge of the bony defect. The cleft lip and hypertelorism also were corrected at the same sitting. Our first case had CSF leak on the 10th postoperative day and developed fulminant meningitis, despite appropriate antibiotics, and succumbed. The second patient had a good postoperative recovery and a satisfactory follow up for 2 years.
经蝶窦脑膨出是罕见的病症。迄今为止，世界文献中仅报道了约20例此类病例。其中，经鞍型最为罕见（5例）。我们呈现过去12年中见到的2例经鞍型经蝶窦脑膨出病例。第一例是一名5个月大的男婴，表现为鼻塞和口腔肿物。第二例是一名14岁男孩，自幼即出现慢性鼻塞。第一例中，肿物经腭裂进入口腔。第二例中，经鼻镜检查可在鼻腔后半部看到肿物。包括冠状位和矢状位重建的计算机断层扫描显示两名患者均为经鞍型经蝶窦脑膨出。第二例的磁共振成像显示第三脑室底部的腔隙和结构构成了主要的囊内容物。该例磁共振血管造影显示扩张的大脑前动脉在脑膨出囊颈部下垂。第一例采用颅外经腭入路并修复脑膨出，同时一期进行腭裂修复。第二例患者采用经鼻入路并双侧上颌骨切开术修复脑膨出，颅底缺损采用劈开肋骨移植修复，在斜坡后部和骨缺损前缘前部用微型钢板和螺钉固定。唇裂和眼距增宽也在同一期得到矫正。我们的第一例患者术后第10天出现脑脊液漏，尽管使用了适当的抗生素仍发生暴发性脑膜炎，最终死亡。第二例患者术后恢复良好，随访2年情况满意。
