遗传综合征患者的蝶鞍形态:一项系统综述。
Sella turcica morphology in patients with genetic syndromes: A systematic review.
作者信息
Roomaney Imaan A, Chetty Manogari
机构信息
Oral Biology, Craniofacial and Dental Genetics, Faculty of Dentistry, University of Western Cape, Tygerberg, South Africa.
出版信息
Orthod Craniofac Res. 2021 May;24(2):194-205. doi: 10.1111/ocr.12426. Epub 2020 Sep 28.
The sella turcica is an important anatomical reference used in orthodontics for the evaluation of craniofacial growth. Studies have found variations in the sella turcica morphology in patients with syndromes affecting the craniofacial complex. This review aims to determine whether genetic syndromes involving the craniofacial complex are associated with abnormal radiographic sella turcica morphology and whether there is a pattern of malformation which is consistent within each syndrome. An electronic database search was conducted to identify relevant studies. We included primary studies describing the morphology of the sella turcica on lateral radiographs in human subjects with genetic syndromes involving the craniofacial complex. No restrictions were placed on language or timeframe. PROSPERO registration CRD42019148060. Thirty-eight studies were included in this review. A 'J'-shaped sella was found in patients with Hutchinson-Gilford-Progeria syndrome and other syndromes. A bulbous dorsum sellae was highly prevalent Cleidocranial dysplasia, and a bulbous dorsum sellae and uneven contours of the clivus was found in Cri du chat syndrome. A steep clivus was described in patients with Axenfeld-Rieger syndrome. An oblique anterior wall was the most frequent malformation found in Down's syndrome. Genetic syndromes affecting the craniofacial complex are associated with abnormal morphology of the radiographic sella turcica. Clinicians should be observant of abnormal sella turcica morphology which can be a sign of undiagnosed or subclinical syndromes. More high-quality studies are needed which use standardized and objective methods of determining the morphology of the sella turcica.
蝶鞍是正畸学中用于评估颅面生长的重要解剖学参考标志。研究发现,影响颅面复合体的综合征患者的蝶鞍形态存在差异。本综述旨在确定涉及颅面复合体的遗传综合征是否与蝶鞍的异常影像学形态相关,以及每种综合征内是否存在一致的畸形模式。通过电子数据库检索来识别相关研究。我们纳入了描述涉及颅面复合体的遗传综合征患者侧位X线片上蝶鞍形态的原始研究。对语言或时间范围未作限制。PROSPERO注册号为CRD42019148060。本综述纳入了38项研究。在哈钦森-吉尔福德-普罗geria综合征和其他综合征患者中发现了“J”形蝶鞍。球形蝶鞍背在锁骨颅骨发育不全中高度常见,在猫叫综合征中发现了球形蝶鞍背和斜坡轮廓不均匀。在Axenfeld-Rieger综合征患者中描述了陡峭的斜坡。倾斜的前壁是唐氏综合征中最常见的畸形。影响颅面复合体的遗传综合征与蝶鞍的异常影像学形态相关。临床医生应注意蝶鞍形态异常,这可能是未诊断或亚临床综合征的迹象。需要更多使用标准化和客观方法确定蝶鞍形态的高质量研究。