Chernikova N A, Satanin L A, Shelesko E V, Danilov G V, Agrba S B, Shkarubo A N, Mazerkina N A, Satanina M L
Burdenko Neurosurgical Center, Moscow, Russia.
Pirogov Russian National Research Medical University, Moscow, Russia.
Zh Vopr Neirokhir Im N N Burdenko. 2024;88(5):102-109. doi: 10.17116/neiro202488051102.
In rare cases, basal encephaloceles can include important brain structures (pituitary gland, chiasm, third ventricle walls, cerebral vessels).
We reviewed 43 articles (78 cases) devoted to basal encephaloceles involving functionally important structures. Analysis included transsphenoidal and sphenoethmoidal hernias.
Nasal breathing disorders were found in 76.9% of cases, decreased visual acuity - 53.8%, endocrine disorders - 44.9%, nasal CSF leakage - 14.1%, meningitis - 10.3%. Concomitant malformations occurred in 85.9%. Severe respiratory disorders (52.6%, <0.05) and multiple concomitant malformations (71.7%, <0.05) were more common among patients with sphenoethmoidal encephalocele. Among 78 patients, 73.1% underwent surgery while follow-up was preferable in 26.9% of cases. There was progression of endocrine disorders (9.5%), visual impairment (4.8%) and nasal breathing disorders (4.8%) in follow-up period. Surgical treatment also has many risks. Transcranial, transnasal, transoral and combined approaches are currently used. It was not possible to establish the most effective surgical approach due to heterogeneous clinical data. Postoperative complications occurred in 47.4% of cases (endocrine disorders - 33.3%, CSF leakage - 8.8%, visual impairment - 1.8%, infectious complications - 14%). Mortality rate was 3.5%.
Congenital basal encephalocele involving functionally important structures is an extremely rare developmental defect. Such patients often have concomitant anomalies. Comprehensive examination is necessary to estimate the structures included in hernial sac and concomitant diseases. Treatment strategy should be determined individually after thorough examination and comparison of all risks. Surgical treatment is associated with high risk of complications due to damage to the structures comprising encephalocele.
在罕见情况下，基底脑膨出可累及重要脑结构（垂体、视交叉、第三脑室壁、脑血管）。
我们回顾了43篇（78例）关于累及功能重要结构的基底脑膨出的文章。分析包括经蝶窦和蝶筛窦脑膨出。
76.9%的病例存在鼻呼吸障碍，53.8%视力下降，44.9%内分泌紊乱，14.1%鼻脑脊液漏，10.3%发生脑膜炎。85.9%伴有其他畸形。蝶筛窦脑膨出患者中严重呼吸障碍（52.6%，<0.05）和多种合并畸形（71.7%，<0.05）更为常见。78例患者中，73.1%接受了手术，26.9%的病例更适合进行随访。随访期间内分泌紊乱（9.5%）、视力损害（4.8%）和鼻呼吸障碍（4.8%）有进展。手术治疗也有许多风险。目前采用经颅、经鼻、经口及联合入路。由于临床数据异质性，无法确定最有效的手术入路。47.4%的病例发生术后并发症（内分泌紊乱 - 33.3%，脑脊液漏 - 8.8%，视力损害 - 1.8%，感染性并发症 - 14%）。死亡率为3.5%。
累及功能重要结构的先天性基底脑膨出是一种极其罕见的发育缺陷。此类患者常伴有其他异常。需要进行全面检查以评估疝囊内包含的结构及合并疾病。应在全面检查并比较所有风险后个体化确定治疗策略。由于对构成脑膨出的结构造成损伤，手术治疗并发症风险高。
