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颅咽管及其病变谱。

Craniopharyngeal canal and its spectrum of pathology.

机构信息

From the Department of Radiology (T.A.A., K.L.S., H.R.H.), University of Utah, Salt Lake City, Utah.

出版信息

AJNR Am J Neuroradiol. 2014 Apr;35(4):772-7. doi: 10.3174/ajnr.A3745. Epub 2013 Nov 1.

Abstract

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.

摘要

背景与目的

颅咽管是一种罕见的、有良好皮质的中线缺陷,从蝶鞍底部延伸到前上鼻咽顶。我们回顾了一系列颅咽管病例,旨在建立一种分类系统,以更好地了解这一实体,突出相关病理状况的范围,并优化患者的治疗。

材料与方法

回顾性分析了 1989 年至 2013 年间 29 例(10 例女性,15 例男性,4 例不详;中位年龄 4 岁;年龄范围 1 天至 65 岁)颅咽管患者的磁共振成像(MR)、CT 和临床资料。定性评估包括正中或异位腺垂体和肿瘤及/或脑脊膜膨出的存在。测量中点前后直径。评估垂体功能障碍和伴随的异常的临床和影像学数据。

结果

颅咽管在定性上可分为 3 型:偶然管(1 型);有异位腺垂体的管(2 型);和包含脑脊膜膨出(3A 型)、肿瘤(3B 型)或两者(3C 型)的管,包括垂体腺瘤、颅咽管瘤、皮样囊肿、畸胎瘤和胶质瘤。定量评估显示,1 型管(中位数 0.8;范围 0.7-1.1mm)、2 型管(中位数 3.9,范围 3.5-4.4mm)和 3 型管(中位数 9.0,范围 5.9-31.0mm)的前后直径有显著差异(P<.0001),分别赋予小、中、大描述。有脑脊膜膨出的管均包含异位腺垂体。颅咽管与垂体功能障碍(6/29)和先天性异常(8/29)有关。

结论

准确的诊断和分类对需要手术的病变具有重要价值,可以识别有潜在垂体功能障碍的患者,并避免在切除鼻咽肿块时发生医源性垂体功能减退或 CSF 漏。

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