Saroj Gyanendra, Gangwar Anshul, Dhillon Jatinder Kaur
Associate Professor, Department of Pedodontics and Preventive Dentistry Maulana Azad Institute of Dental Sciences, New Delhi, India.
Professor, Department of Pedodontics and Preventive Dentistry, Institute of Dental Sciences, Bareilly, Uttar Pradesh, India.
Int J Clin Pediatr Dent. 2016 Jan-Mar;9(1):82-5. doi: 10.5005/jp-journals-10005-1339. Epub 2016 Apr 22.
Sturge-Weber syndrome (SWS) is a rare, nonhereditary developmental condition that is characterized by a hamartomatous vascular proliferation of the brain, resulting in multiple angiomas that occur on the same side due to arteriovenous malformations. It is believed to be caused by persistence of a vascular plexus around the cephalic portion of the neural tube and is present at birth in about 1 in every 50,000 babies. It is one of the phakomatoses which is often associated with port-wine stains (PWSs) of the face, glaucoma, seizures, mental retardation and ipsilateral leptomeningeal angioma. Many people with SWS probably never know they have it. Hypothyroidism is a condition that arises from inadequate release of thyroid-stimulating hormone to stimulate an otherwise normal thyroid gland. This condition is often associated with a deficient secretion of other pituitary hormone, and growth hormone deficiency occurs with an increased prevalence in SWS, presumably secondary to involvement of the hypothalamic-pituitary axis. Diagnosis is made by the presence of a facial PWS and evidence of leptomeningeal angioma either by skull X-ray or computed tomography scan that shows intracranial calcifications. Presently, there is no specific treatment for SWS, and the management of the clinical manifestations and complications is still far from adequate. Here, we report the case of hypothyroidism associated with SWS with oral and facial manifestations in an 11-year-old boy. How to cite this article: Saroj G, Gangwar A, Dhillon JK. Hypothyroidism and Sturge-Weber Syndrome associated with Bilateral Port-wine Nevus. Int J Clin Pediatr Dent 2016;9(1): 82-85.
斯特奇-韦伯综合征（SWS）是一种罕见的非遗传性发育疾病，其特征是脑部错构瘤性血管增生，由于动静脉畸形导致同侧出现多个血管瘤。据信它是由神经管头部周围血管丛持续存在引起的，每50000名婴儿中约有1名在出生时就患有此病。它是一种 phakomatoses，常与面部葡萄酒色斑（PWSs）、青光眼、癫痫、智力迟钝和同侧软脑膜血管瘤有关。许多患有SWS的人可能从未意识到自己患病。甲状腺功能减退症是一种由于甲状腺刺激激素释放不足而无法刺激原本正常的甲状腺所引起的病症。这种病症常与其他垂体激素分泌不足有关，生长激素缺乏症在SWS中的患病率增加，推测是继发于下丘脑-垂体轴受累。通过面部PWS的存在以及通过颅骨X线或计算机断层扫描显示颅内钙化来证明软脑膜血管瘤的存在进行诊断。目前，SWS尚无特效治疗方法，对其临床表现和并发症的处理仍远远不够。在此，我们报告一名11岁男孩患SWS并伴有口腔和面部表现的甲状腺功能减退症病例。如何引用本文：Saroj G, Gangwar A, Dhillon JK. 甲状腺功能减退症与双侧葡萄酒色痣相关的斯特奇-韦伯综合征。《国际临床儿科牙科学杂志》2016；9(1): 82 - 85。
