Gossage D, Perrin J M, Butler M G
Am J Med Genet. 1987 Apr;26(4):915-9. doi: 10.1002/ajmg.1320260420.
We recently examined a 26-month-old boy with abnormal face, blepharophimosis, hypertelorism, apparently low-set ears, micrognathia, arachnodactyly, talipes equinovarus, and joint contractures. Subsequently he manifested failure to thrive, respiratory infections, and developmental delay. These congenital anomalies and associated findings are consistent with a diagnosis of the Marden-Walker syndrome. He also had mild pyloric stenosis and duodenal bands, not previously reported in this syndrome. This syndrome appears to be an autosomal recessive trait in some families. A summary of findings of the 16 previous published patients is presented.
我们最近检查了一名26个月大的男孩,他面部异常,有睑裂狭小、眼距过宽、耳朵明显低位、小颌畸形、蜘蛛指、马蹄内翻足和关节挛缩。随后,他出现了生长发育迟缓、呼吸道感染和发育延迟。这些先天性异常及相关表现符合马登-沃克综合征的诊断。他还患有轻度幽门狭窄和十二指肠束带,该综合征此前未见有此报告。在一些家族中,此综合征似乎为常染色体隐性遗传性状。本文呈现了此前已发表的16例患者的研究结果总结。