PHACES综合征是后窝畸形综合征表现的首字母缩写,血管瘤,动脉异常,主动脉缩窄/心脏缺损,眼睛异常和胸骨畸形。婴儿血管瘤是婴儿期最常见的肿瘤。区域性牙齿发育不良,通常被称为“鬼牙”,是牙釉质和牙本质的罕见局部发育畸形,严重程度不同,导致受影响牙齿的异常临床和影像学表现。本报告描述了一例罕见的2岁白种人男性,被诊断患有PHACES综合征,并伴有多区域牙齿发育不良。二十颗牙齿中有十颗发育不良。患者在医院环境下进行全身麻醉治疗。由于敏感,所有受影响的乳牙都被拔除,脓肿和极差的长期预后。往前走,一个长期的跨学科的方法将是必要的,以解决这个孩子的牙列,因为它的发展。
PHACES syndrome is an acronym for the syndromic presentation of Posterior fossa malformation, Hemangioma, Arterial anomalies, Coarctation of aorta/cardiac defects, Eye abnormalities and Sternal malformations. Infantile hemangiomas are the most common tumors of infancy. Regional
odontodysplasia, commonly referred to as \"ghost teeth\", is a rare localized developmental malformation of enamel and dentin with varying levels of severity that results in unusual clinical and radiographic appearances of affected teeth. This report describes a rare
case of a two-year-old Caucasian male diagnosed with PHACES syndrome also presenting with multi-regional
odontodysplasia. Ten of twenty teeth were dysplastic. The patient was treated under general anesthesia in a hospital setting. All affected primary teeth were extracted due to sensitivity, abscess and extremely poor long-term prognosis. Moving forward, a long-term interdisciplinary approach will be necessary to address this child\'s dentition as it develops.