Posterior cervical congenital dermal sinus tract: case report and review of literature.

IF 0.7 Q4 CLINICAL NEUROLOGY Spinal Cord Series and Cases Pub Date : 2023-08-02 DOI:10.1038/s41394-023-00575-5
Amir Vokshoor, Harseerat Jajj, Tiffany Grunwald, Steven Kolker, Jack Petros
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Abstract

Background and importance: Congenital dermal sinus tract (DST) is a rare spinal dysraphism characterized by a persistent tract lined by epithelial cells, beginning at the epidermis and terminating in deeper tissue layers. With 1% of all congenital DST cases found in the cervical region, only 4% of all cases are diagnosed after the age of 20.

Clinical presentation: In this case, a 65-year-old woman with a congenital DST at the cervical level presented with symptoms of neck and some arm pain, suboccipital headaches, and unique external characteristics. Neck Disability Index and visual analog scale were used to assess the patient's preoperative and postoperative pain, and quality of life. Patient underwent an operative intervention, where the DST was surgically removed followed by interlaminar decompression at C1-C2, excision of the epidural component, and biopsy followed by plastic surgical repair. Pathology analysis indicated a squamous epithelial-lined sinus tract interacting with the dura. Most notably, a meningothelial proliferation with associated psammomatous calcifications was identified, similar to a meningioma.

Conclusion: A review of literature was conducted to further discuss clinical and radiological presentation as well as to document the novel appearance of this congenital DST. As one of the oldest cases of DST, it demonstrated unusual pathological characteristics with a meningothelial proliferation, compatible with meningioma, reported at the epidural level.

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颈后先天性真皮窦道:1例报告及文献复习。
背景和重要性:先天性真皮窦道(DST)是一种罕见的脊柱异常,其特征是由上皮细胞排列的持续性通道,始于表皮,终止于更深的组织层。1%的先天性DST病例发现于宫颈区域,只有4%的病例在20岁以后被诊断出来。临床表现:本例患者为65岁女性,颈段先天性DST,表现为颈部和部分手臂疼痛,枕下头痛,并有独特的外部特征。采用颈部残疾指数和视觉模拟量表评估患者术前、术后疼痛及生活质量。患者接受手术干预,其中手术切除DST,然后在C1-C2椎板间减压,切除硬膜外成分,活检后进行整形手术修复。病理分析显示鳞状上皮排列的窦道与硬脑膜相互作用。最值得注意的是,发现脑膜上皮增生并伴有沙质钙化,类似脑膜瘤。结论:回顾文献,进一步讨论临床和影像学表现,并记录这种先天性DST的新外观。作为最古老的DST病例之一,它表现出不同寻常的病理特征,在硬膜外水平报道了脑膜上皮增生,与脑膜瘤相容。
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来源期刊
Spinal Cord Series and Cases
Spinal Cord Series and Cases Medicine-Neurology (clinical)
CiteScore
2.20
自引率
8.30%
发文量
92
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