European Archives of Oto-Rhino-Laryngology ( IF 1.9 ) Pub Date : 2024-01-08 , DOI: 10.1007/s00405-023-08355-5 Zheng Jiang 1 , Deming Yang 2 , Mailudan Ainiwaer 1 , Qiong Li 3 , Wei Mo 1 , Feng Liu 1
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Purpose
Clinical presentations encompass respiratory, feeding issues, nasopharyngeal mass, meningitis, CSF leakage, craniofacial anomalies, and endocrine problems. Surgery is the primary treatment, transitioning from frontal craniotomy to endoscopic methods, offering improved outcomes. Yet, more studies are needed. A comprehensive review on trans-sellar trans-sphenoidal encephalocele (TSTSE) is missing. Our study aims to fill this gap, offering a comprehensive perspective for physicians.
Methods
This review adhered to the PRISMA guideline. Eligible studies focused on human subjects, specifically trans-sellar encephaloceles, and provided comprehensive treatment details. English language articles published up to April 11th, 2023, were considered. Two trained researchers conducted article screening using consistent criteria. Data extraction covered various aspects, including clinical presentation, surgical methods, and outcomes, with results presented descriptively in two tables. Due to the rarity of this congenital anomaly, meta-analysis and publication bias assessment were not feasible. Data extraction was independently conducted by two reviewers, with subsequent cross-verification.
Results
A total of 36 patients were identified from 14 studies, the most frequently observed clinical presentation was dyspnea (41.67%) and the most frequently observed accompanying anomaly was cleft lip/palate (55.56%). CT and MRI were adopted in nearly all the cases, and trans-nasal approach was the most often used surgical approach (57.14%) with the ‘soft material combination’ the most commonly used method for cranial base repairment (35.71%). A total of two deaths occurred and diabetes insipidus was the most common perioperative complication which occurred in six surgery patients (21.43%).
Conclusion
TSTSE predominantly affects males and presents with dyspnea, visual deficits, pituitary insufficiency, and cranial base-related symptoms. Early diagnosis is critical, with advanced imaging playing a key role. Endocrine assessment is vital for hormone management. Surgery offers symptom relief but entails risks, including reported fatalities and complications. The choice between surgery and conservative management requires careful deliberation. The trans-nasal approach is favored for its reduced trauma, yet further research is necessary to validate this preference.
中文翻译：
先天性经鞍区经蝶脑膨出：诊断、治疗和预后的系统评价
目的
临床表现包括呼吸、喂养问题、鼻咽肿块、脑膜炎、脑脊液漏、颅面异常和内分泌问题。手术是主要治疗方法，从额部开颅手术过渡到内窥镜方法，可改善预后。然而，还需要更多的研究。缺少对经鞍区经蝶脑膨出 (TSTSE) 的全面综述。我们的研究旨在填补这一空白，为医生提供全面的视角。
方法
本次审查遵循 PRISMA 指南。符合条件的研究主要针对人类受试者，特别是经鞍区脑膨出，并提供了全面的治疗细节。截至 2023 年 4 月 11 日发表的英文文章均予以考虑。两名训练有素的研究人员使用一致的标准进行文章筛选。数据提取涵盖各个方面，包括临床表现、手术方法和结果，结果描述性地呈现在两个表格中。由于这种先天性异常的罕见性，荟萃分析和发表偏倚评估是不可行的。数据提取由两名评审员独立进行，并随后进行交叉验证。
结果
14 项研究共鉴定出 36 名患者，最常见的临床表现是呼吸困难 (41.67%)，最常见的伴随异常是唇裂/腭裂 (55.56%)。几乎所有病例均采用CT和MRI，其中经鼻入路是最常用的手术方式（57.14%），“软材料组合”是最常用的颅底修复方法（35.71%）。共发生2例死亡，尿崩症是最常见的围手术期并发症，共发生6例（21.43%）。
结论
TSTSE 主要影响男性，表现为呼吸困难、视力障碍、垂体功能不全和颅底相关症状。早期诊断至关重要，其中先进的影像学发挥着关键作用。内分泌评估对于激素管理至关重要。手术可以缓解症状，但也会带来风险，包括报告的死亡和并发症。手术和保守治疗之间的选择需要仔细考虑。经鼻入路因其减少创伤而受到青睐，但需要进一步的研究来验证这种偏好。
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