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Craniopharyngeal canal and its spectrum of pathology

机译:颅咽管及其病理学范围

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BACKGROUND AND PURPOSE: The craniopharyngeal canal is a rare, well-corticated defect through the midline of the sphenoid bone from the sellar floor to the anterosuperior nasopharyngeal roof. We reviewed a series of craniopharyngeal canals to determine a system of classification that might better our understanding of this entity, highlight the range of associated pathologic conditions, and optimize patient treatment. MATERIALS AND METHODS: Available MR imaging, CT, and clinical data (from 1989-2013) of 29 patients (10 female, 15 male, 4 unknown; median age, 4 years; age range, 1 day-65 years) with craniopharyngeal canals were retrospectively examined. Qualitative assessment included orthotopic or ectopic adenohypophysis and the presence of a tumor and/or cephalocele. The midpoint anteroposterior diameter was measured. Clinical and imaging data were evaluated for pituitary dysfunction and accompanying anomalies. RESULTS: Craniopharyngeal canals were qualitatively separated into 3 types: incidental canals (type 1); canals with ectopic adenohypophysis (type 2); and canals containing cephaloceles (type 3A), tumors (type 3B), or both (type 3C), including pituitary adenoma, craniopharyngioma, dermoid, teratoma, and glioma. Quantitative evaluation showed a significant difference (P < .0001) in the anteroposterior diameters of type 1 canals (median, 0.8; range, 0.7-1.1 mm), type 2 canals (median, 3.9, range, 3.5-4.4 mm), and type 3 canals (median, 9.0; range, 5.9-31.0 mm) imparting small, medium, and large descriptors. Canals with cephaloceles all contained an ectopic adenohypophysis. The craniopharyngeal canals were associated with pituitary dysfunction (6/29) and congenital anomalies (8/29). CONCLUSIONS: Accurate diagnosis and classification of craniopharyngeal canals are valuable to characterize lesions requiring surgery, identify patients with potential pituitary dysfunction, and avoid iatrogenic hypopituitarism or CSF leak during surgical resection of nasopharyngeal masses.
机译:背景与目的:颅咽管是一种罕见的,皮质良好的缺损,其穿过蝶骨的中线,从蝶鞍底部到上颌鼻咽部顶部。我们回顾了一系列颅咽管,以确定一种分类系统,该分类系统可能会使我们更好地了解该实体,突出相关病理状况的范围,并优化患者治疗。材料与方法:29例颅咽管患者(10例女性,15例男性,4例未知;中位年龄为4岁;年龄范围为1天至65岁)的可用MR成像,CT和临床数据(从1989-2013年开始)进行回顾性检查。定性评估包括原位或异位腺垂体以及是否存在肿瘤和/或头突。测量中点前后直径。对垂体功能障碍和伴随异常的临床和影像学数据进行了评估。结果:鼻咽管被定性分为3种类型:副管(1型);鼻咽管(1型);鼻管(1型);鼻管(3型)。异位腺垂体的运河(2型);以及包含头突(3A型),肿瘤(3B型)或两者(3C型)的运河,包括垂体腺瘤,颅咽管瘤,皮样,畸胎瘤和神经胶质瘤。定量评估显示,第1类管(中位数,0.8;范围,0.7-1.1毫米),第2类管(中位数,3.9,范围,3.5-4.4毫米)的前后直径有显着差异(P <.0001),并且类型3的运河(中位9.0;范围5.9-31.0毫米),赋予了小,中和大的描述符。带头突的运河都含有异位腺垂体。颅咽管与垂体功能障碍(6/29)和先天性异常(8/29)有关。结论:准确诊断和分类颅咽管对于确定需要手术的病变,鉴定可能存在垂体功能障碍的患者以及避免在鼻咽部肿块手术切除过程中避免医源性垂体功能低下或脑脊液漏出具有重要价值。

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