Department of Oral Diagnosis and Radiology, Faculty of Dentistry, Hacettepe University, Ankara, Turkey.
Dentomaxillofac Radiol. 2013;42(2):58444855. doi: 10.1259/dmfr/58444855. Epub 2012 Jan 12.
Proteus syndrome is a rare disorder with progressive asymmetrical and disproportionate overgrowth of various tissues of the body. The syndrome is characterized by a wide range of malformations, including craniofacial deformities. Extraoral examination revealed several of the classical craniofacial features of Proteus syndrome: pronounced hemifacial hypertrophy, macrodactyly and hyperostosis. Intraoral examination revealed a high arched palate and gingival hyperplasia. Other findings were unilateral enlargement of the tongue, alveolar growth and dilaceration of the roots of the teeth. There were severe degenerative changes and deformities in the left temporomandibular joint but the oversized condyle was asymptomatic; there was no pain, limitation and deviation at mouth opening. Treatment was not necessary owing to the asymptomatic situation but periodic follow-up with clinical and radiographic examination was considered. The aim of this article is to describe the radiographic manifestations of an asymptomatic condyle malformation and other craniofacial, oral and dental findings in a 33-year-old female patient with known Proteus syndrome.
Proteus 综合征是一种罕见的疾病,表现为身体各组织的进行性不对称和不成比例的过度生长。该综合征的特征是多种畸形,包括颅面畸形。口腔外检查显示出 Proteus 综合征的几种典型颅面特征:明显的半侧面部肥大、巨指(趾)和骨肥厚。口腔内检查显示高拱形腭和牙龈增生。其他发现包括单侧舌体增大、牙槽骨生长和牙齿根部扭曲。左侧颞下颌关节有严重的退行性改变和畸形,但过大的髁突无症状;开口时无疼痛、受限和偏斜。由于无症状,不需要治疗,但考虑进行定期的临床和影像学随访。本文旨在描述一名已知患有 Proteus 综合征的 33 岁女性患者的无症状髁突畸形和其他颅面、口腔及牙齿的影像学表现。