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Crouzon氏综合征又名颅面骨形成不全症,系由颅面骨缝过早愈合所致,自幼常呈尖头,双眼球突出,上颌发育不良,下颌相对向前突,呈反咬合,有时因颅内压增高或视神经受压而失明。本病多为常染色体显性遗传,但亦有1/3为散发病例报告。国内曾见个例报告,及一家系报告。本文对我院眼科近3～4年所遇6例进行了系统检查,家系调查及行部分染色体检查,现报告如下。
Crouzon’s syndrome, also known as craniofacial dysplasia, is caused by the premature healing of the craniofacial suture. Since childhood, Crouzon’s syndrome often has pointed, prominent eyes, maxillary dysplasia, mandibular relative forward protrusion, Sometimes due to increased intracranial pressure or optic nerve compression and blindness. The disease is mostly autosomal dominant, but also 1/3 for the distribution of case reports. There have been a few cases of domestic reports, and one department report. This article on our hospital ophthalmic nearly 3 to 4 years encountered six cases of systematic examination, pedigree investigation and line part of the chromosome examination, are as follows.