Abstract：
Hallermann Streiff syndrome (HSS) is a rare congenital abnormality with about 200 case reports in the literature. Its etiology is unknown although it may be due to a sporadic mutation. Diagnosis is based on the association of craniofacial malformation, dental abnormalities, hypotrichosis, atrophy of the skin, proportionate nanism, congenital cataract and bilateral microphtalmos. Cranio-facial deformities are the main signs detected and the most easily recognizable. We report cranio-facial and oral signs from a systemic literature review, and illustrate our findings with two of our patients diagnosed with HSS. Common cranio-facial manifestations are craniofacial malformation with a « parrot beak » nose, micrognathia, aprominent skull, sutures closing anomaly, malocclusion, dental anomalies, eyebrows and eyelash lack and atrophy of the nose skin. Knowledge of these signs should allow for early diagnosis and adequate treatment and follow up.
摘要：
HallermannStreiff综合征（HSS）是一种罕见的先天性异常，文献报道约200例。尽管可能是由于偶发性突变，但其病因尚不清楚。诊断是基于颅面畸形的关联，牙齿异常,毛发减少症,皮肤萎缩，相称的纳米主义，先天性白内障和双侧小脑。颅面畸形是检测到的主要体征，也是最容易识别的。我们通过系统的文献综述报告颅面和口腔体征，并说明了我们对两名诊断为HSS的患者的发现。常见的颅面表现是颅面畸形，有“鹦鹉嘴”鼻子，小颌畸形,突出的头骨，缝线闭合异常，错牙合,牙齿异常,眉毛和睫毛缺乏和鼻子皮肤萎缩。对这些体征的了解应允许早期诊断，适当的治疗和随访。
