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涉及持续性颅咽管的疾病:病例系列

Disorders Involving a Persistent Craniopharyngeal Canal: A Case Series.

作者信息

Poonia Seerat K, Cazzador Diego, Kaufman Adam C, Kohanski Michael A, Kuan Edward C, Tong Charles C L, Carlson Roy D, Borsetto Daniele, Emanuelli Enzo, Palmer James N, Adappa Nithin D

机构信息

Department of Otorhinolaryngology - Head and Neck Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States.

Otorhinolaryngology Unit, Department of Neurosciences, University of Padua, Padua, Italy.

出版信息

J Neurol Surg B Skull Base. 2020 Oct;81(5):562-566. doi: 10.1055/s-0039-1692638. Epub 2019 Jun 21.

Abstract

A persistent craniopharyngeal canal (CPC) is a rare embryologic remnant that presents as a well-corticated defect of the midline sphenoid body extending from the sellar floor to the nasopharynx. Our case series aims to describe three unique presentations of this congenital anomaly and their subsequent management.  Retrospective review.  Tertiary academic medical center.  Patients who underwent endoscopic transnasal surgical repair of a CPC lesion.  Resolution of symptoms and surgical outcomes.  A total of three patients were identified. The clinical presentation varied, however, all cases prompted further imaging which demonstrated a persistent CPC and associated pathologic lesion. The presentation of a persistent CPC with nasal obstruction and subsequent iatrogenic cerebrospinal fluid leak as in Case 1 demonstrates the importance of imaging in this work-up. Cases 2 and 3 in the series were representative of the larger subset of patients in the literature who present with the defect incidentally but still warrant surgical management. Nonetheless, a standard approach to diagnosis with preoperative imaging and subsequent transnasal endoscopic repair of the skull base defect was undertaken.  The persistent CPC is a rare congenital anomaly associated with diverse pathology and careful review of preoperative radiology is critical to the management. When warranted, subsequent surgical repair and reconstruction is associated with excellent postoperative outcomes.

摘要

持续性颅咽管(CPC)是一种罕见的胚胎残余物,表现为蝶骨体中线的皮质良好的缺损,从鞍底延伸至鼻咽部。我们的病例系列旨在描述这种先天性异常的三种独特表现及其后续治疗。

  • 回顾性研究。

  • 三级学术医疗中心。

  • 接受内镜经鼻手术修复CPC病变的患者。

  • 症状缓解情况及手术结果。

共确定了3例患者。临床表现各不相同,然而,所有病例均促使进一步成像,显示存在持续性CPC及相关病理病变。如病例1所示,持续性CPC伴有鼻塞及随后的医源性脑脊液漏,这表明成像在该检查中的重要性。该系列中的病例2和病例3代表了文献中更大的一部分偶然出现该缺损但仍需手术治疗的患者。尽管如此,还是采用了术前成像诊断及随后经鼻内镜修复颅底缺损的标准方法。

持续性CPC是一种罕见的先天性异常,与多种病理情况相关,仔细审查术前放射学对治疗至关重要。如有必要,后续的手术修复和重建可带来良好的术后效果。

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J Comput Assist Tomogr. 2010 Jul;34(4):612-4. doi: 10.1097/RCT.0b013e3181dbe5d1.
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