Bhansali Rahul S, Yeltiwar Ramreddy K, Agrawal Amit A
Department of Periodontics, Government Dental College and Hospital, Nagpur, Maharashtra, India.
J Periodontol. 2008 Mar;79(3):549-55. doi: 10.1902/jop.2008.060478.
Sturge-Weber syndrome (encephalotrigeminal angiomatosis) is a rather uncommon congenital condition that is characterized by a combination of venous angioma of leptomeninges over the cerebral cortex and ipsilateral angiomatous lesions of the face and sometimes of the skull, jaws, and oral soft tissues. It is commonly referred to as Sturge-Weber syndrome after Sturge and Weber who first described this affliction in 1879. This article presents a case of Sturge-Weber syndrome associated with severe gingival enlargement, its management, and follow-up results.
A 15-year-old male patient was referred to the Department of Periodontics, Government Dental College and Hospital, for severe gingival enlargement. A detailed dental and medical history, clinical examination, and investigations confirmed the diagnosis of Sturge-Weber syndrome. This report reveals a classic presentation of the syndrome with emphasis on its oral manifestations. Periodontal management included thorough scaling and root planing followed by periodontal flap surgery to treat the gingival enlargement. Histopathologic examination of the excisional biopsy specimen revealed features suggestive of fibrous gingival enlargement.
Reevaluation of the patient after 2 years showed remarkable (90%) reduction of the gingival enlargement in the maxillary arch and complete diminution (100%) in the mandibular arch. However, a slight recurrence was noted in the maxillary right quadrant.
Sturge-Weber syndrome is clinically important to the periodontist because of its associated gingival vascular features and their complicating manifestations. Periodic systemic and oral examinations are recommended to identify and prevent any complications from the cranial and oral lesions.
斯特奇-韦伯综合征（脑三叉神经血管瘤病）是一种相当罕见的先天性疾病，其特征是大脑皮质软脑膜静脉血管瘤与同侧面部有时还有颅骨、颌骨及口腔软组织的血管瘤性病变同时存在。1879年斯特奇和韦伯首次描述了这种疾病，此后通常将其称为斯特奇-韦伯综合征。本文介绍了一例伴有严重牙龈增生的斯特奇-韦伯综合征病例、其治疗方法及随访结果。
一名15岁男性患者因严重牙龈增生被转诊至政府牙科学院和医院牙周病科。详细的牙科和病史、临床检查及各项检查确诊为斯特奇-韦伯综合征。本报告揭示了该综合征的典型表现，并重点关注其口腔表现。牙周治疗包括彻底的龈上洁治和根面平整，随后进行牙周翻瓣手术以治疗牙龈增生。切除活检标本的组织病理学检查显示有纤维性牙龈增生的特征。
2年后对该患者进行复查发现，上颌牙弓牙龈增生显著减轻（90%），下颌牙弓完全消退（100%）。然而，上颌右象限有轻微复发。
斯特奇-韦伯综合征对牙周病医生具有重要临床意义，因其相关的牙龈血管特征及其复杂表现。建议定期进行全身和口腔检查，以识别和预防颅部及口腔病变的任何并发症。
