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鼻脑膜脑膨出:16 例临床病理特征和诊断的回顾性研究。

Nasal meningoencephalocele: A retrospective study of clinicopathological features and diagnosis of 16 patients.

机构信息

Department of Pathology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China.

Department of Imaging Center, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China.

出版信息

Ann Diagn Pathol. 2020 Dec;49:151594. doi: 10.1016/j.anndiagpath.2020.151594. Epub 2020 Aug 13.

Abstract

OBJECTIVE

Nasal meningoencephalocele (encephalocele or cephalocele) is a rare condition with congenital, traumatic, or spontaneous origins. We investigated the clinicopathological characteristics of nasal encephaloceles to improve pathologists' and clinicians' understanding of this disease.

METHODS

Sixteen patients with nasal encephaloceles were enrolled in this retrospective study investigating the condition's clinical and morphological features.

RESULTS

Patients' average age was 37.8 (±20.8) years. The ratio of men to women was 2.2:1, patients' mean age was 47.4 (±11.8) years, and 10/16 patients had spontaneous encephaloceles. All patients with traumatic and spontaneous encephaloceles presented with cerebrospinal fluid leak. In 9/16 patients, the skull defect site occurred on the lateral wall of the sphenoid sinus. Both congenital patients experienced nasal obstruction. Histopathology, herniated tissues were brain and/or meningeal tissue, and the brain tissue was almost mature glial tissue.

CONCLUSION

Nasal meningoencephalocele is a rare condition that can be challenging to diagnose. In patients with recurrent clear nasal discharge or in children with a unilateral nasal mass, a high index of suspicion for encephalocele is essential. In this study, spontaneous cases were most common in adults, and the lateral wall of the sphenoid sinus was the most common location.

摘要

目的

鼻脑膜脑膨出(脑膨出或脑膜脑膨出)是一种罕见的疾病,其起源为先天性、外伤性或自发性。我们研究了鼻脑膨出的临床病理特征,以提高病理学家和临床医生对这种疾病的认识。

方法

本回顾性研究纳入了 16 例鼻脑膜脑膨出患者,旨在研究其临床和形态学特征。

结果

患者的平均年龄为 37.8(±20.8)岁。男女比例为 2.2:1,患者的平均年龄为 47.4(±11.8)岁,10/16 例为自发性脑膜脑膨出。所有外伤性和自发性脑膜脑膨出患者均出现脑脊液漏。在 16 例患者中,有 9 例颅骨缺损部位位于蝶窦外侧壁。2 例先天性脑膜脑膨出患者均表现为鼻塞。组织病理学检查显示,疝出的组织为脑和/或脑膜组织,脑组织几乎为成熟的胶质组织。

结论

鼻脑膜脑膨出是一种罕见的疾病,诊断具有一定难度。对于反复出现清亮鼻分泌物的患者,或单侧鼻腔肿块的儿童,应高度怀疑脑膜脑膨出。在本研究中,自发性脑膜脑膨出最常见于成年人,蝶窦外侧壁是最常见的发病部位。

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