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斯特奇-韦伯综合征:病例研究

Sturge Weber Syndrome: A Case Study.

作者信息

Neerupakam Mahesh, Reddy Podduturi Sanjay, Babu Beeraboina Anand, Krishna Guttikonda Vamsi

机构信息

Reader, Department of Oral Medicine and Radiology, Lenora Institute of Dental Sciences, Rajanagaram, Rajahmundry, Andhra Pradesh, India.

Professor, Department of Oral Medicine and Radiology, Lenora Institute of Dental Sciences, Rajanagaram, Rajahmundry, Andhra Pradesh, India.

出版信息

J Clin Diagn Res. 2017 May;11(5):ZD12-ZD14. doi: 10.7860/JCDR/2017/25593.9891. Epub 2017 May 1.

Abstract

The aim of this case review was to touch upon the various clinical presentations and diagnostic features of Sturge-Weber syndrome (SWS) as seen in the dental/medical practice. Sturge-Weber syndrome is a rare congenital disorder that belongs to a group of disorders collectively known as the phakomatoses. The characteristic pathological elements of the disease include leptomeninges angioma extending out to cerebral cortex with angiomatous lesions on the same side and unilateral facial nevus that affects trigeminal nerve division. The classic oral lesions involve haemangiomatous gingival lesion limited on the same side of upper or lower jaw. An 18-year-old female patient reported to the Department of Oral Medicine and Radiology with oral manifestations of SWS. The evaluation of the patient, radiological findings as well as the management is discussed in this case report. It is important for the dental physician to be aware of the non-oral manifestations of SWS in order to identify and manage it appropriately. The challenge here is to see the oral manifestation as part of the syndrome and not as an isolated oral condition. Early diagnosis and appropriate treatment plan is imperative to prevent development of complications.

摘要

本病例回顾的目的是探讨在牙科/医疗实践中所见到的斯特奇-韦伯综合征(SWS)的各种临床表现和诊断特征。斯特奇-韦伯综合征是一种罕见的先天性疾病,属于一组统称为 phakomatoses 的疾病。该疾病的特征性病理要素包括延伸至大脑皮质的软脑膜血管瘤,同侧伴有血管瘤病变,以及影响三叉神经分支的单侧面部痣。典型的口腔病变为局限于上颌或下颌同侧的血管瘤性牙龈病变。一名18岁女性患者因SWS的口腔表现就诊于口腔医学与放射科。本病例报告讨论了对该患者的评估、影像学检查结果以及治疗方法。牙科医生了解SWS的非口腔表现对于正确识别和处理该病很重要。这里的挑战在于将口腔表现视为综合征的一部分,而非孤立的口腔疾病。早期诊断和恰当的治疗方案对于预防并发症的发生至关重要。

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Sturge Weber Syndrome: A Case Study.斯特奇-韦伯综合征:病例研究
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Sturge-Weber syndrome type II treated with PDL 595 nm laser.用595纳米脉冲染料激光治疗II型斯-韦综合征。
Postepy Dermatol Alergol. 2015 Feb;32(1):63-6. doi: 10.5114/pdia.2014.40948. Epub 2015 Feb 3.
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Pediatr Neurol. 2002 May;26(5):387-90. doi: 10.1016/s0887-8994(01)00412-x.
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Sturge-Weber syndrome without facial nevus.无面部痣的斯特奇-韦伯综合征。
Pediatr Neurol. 2000 May;22(5):400-2. doi: 10.1016/s0887-8994(00)00127-2.
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Neurocutaneous syndromes.神经皮肤综合征
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