Department of Pediatric Neurosurgery, Anna Meyer Children's Hospital, Florence, Italy
Neurosurgery. 2009 Dec;65(6):1140-5; discussion 1145-6. doi: 10.1227/01.NEU.0000351780.23357.F5.
Median perisellar congenital transsphenoidal encephalocele (CTE) is a rare entity associated with multiple endocrine, visual, and respiratory deficits. The most likely causative factor for these pathological alterations is distension of neural structures (hypothalamic-pituitary system, optic pathway), resulting in protrusion of the dural sac through a sphenoid bone defect into the pharynx. The continuity with the extracranial space can be associated with an increased risk of tearing of the sac, with consequent cerebrospinal fluid leakage and subsequent increase in the risk of infection. We retrospectively reviewed the surgical treatment of CTE in our hospital using either an extracranial transoral or transnasal approach.
We retrospectively reviewed our database. Between July 1994 and June 2005, CTE we identified 6 patients. Five of them were treated by a surgical intervention. The first patient was treated via a transcranial approach but had a relapse of the prolapse 11 years later. The relapse was treated with an extracranial transpalatal approach. Four patients were treated with an extracranial surgical approach: an extracranial transoral approach was performed in 2 cases, and an extracranial transnasal approach was used in the other 2 cases. Surgery was not performed in 1 patient because the parents refused to consent to the procedure.
Preoperative symptoms remained stable or improved in all of the patients after the surgical procedure and worsened in the patient who did not have a surgical intervention. Two patients experienced a palatal dehiscence. No mortality was recorded in this series of patients.
The surgical treatment of CTE is indicated to stop the progression or improve symptoms related to this disease entity. If approached correctly, the extracranial approach is a safe procedure with subsequent low morbidity.
鞍旁先天性经蝶窦脑膨出（CTE）较为罕见，常伴有多种内分泌、视觉和呼吸功能缺陷。这些病理改变最可能的致病因素是神经结构（下丘脑-垂体系统、视路）的扩张，导致硬脑膜囊通过蝶骨缺陷向咽腔突出。与颅外空间的连续性可能与囊破裂的风险增加有关，继而导致脑脊液漏，并随后增加感染的风险。我们回顾性分析了我院采用颅外经口或经鼻入路治疗 CTE 的情况。
我们回顾性地分析了我们的数据库。1994 年 7 月至 2005 年 6 月期间，我们共发现 6 例 CTE 患者。其中 5 例接受了手术治疗。第 1 例患者经颅入路治疗，但 11 年后再次出现膨出复发。复发后采用颅外经口入路治疗。4 例患者采用颅外手术治疗：2 例采用颅外经口入路，2 例采用颅外经鼻入路。1 例患者未行手术治疗，因为其父母拒绝同意手术。
所有患者术后术前症状均稳定或改善，未行手术的患者症状恶化。2 例患者出现腭部裂开。本系列患者无死亡病例。
手术治疗 CTE 可阻止疾病进展或改善相关症状。如果正确实施，颅外入路是一种安全的手术方法，随后的发病率较低。
