Temtamy S A, Shoukry A S, Raafat M, Mihareb S
Birth Defects Orig Artic Ser. 1975;11(2):104-8.
Two first cousins, the offspring of consanguineous marriages, had features suggestive of Marden-Walker syndrome. Phenotypic similarities and differences for Schwartz-Jampel syndrome have been discussed. Main features of the Marden-Walker syndrome are failure to thrive, marked motor and mental retardation, and multiple malformations in the form of peculiar facies associated wilth poor muscle mass, mild congenital joint contractures, pigeon breast, kyphoscoliosis and arachnodactyly. Peculiar facies is due to blepharophimosis, congenital ptosis, hypoplastic mandible and low-set and malformed ears. Posterior median cleft of the palate as well as cardiac and renal anomalies were noted in the case reported by Marden and Walker. Our Case 2 had dextrocardia. The present report suggests autosomal recessive inheritance of this syndrome.
两名近亲结婚的第一代堂兄妹具有提示马登-沃克综合征的特征。已讨论了施瓦茨-扬佩尔综合征的表型异同。马登-沃克综合征的主要特征为生长发育迟缓、明显的运动和智力发育迟缓,以及多种畸形,表现为特殊面容,伴有肌肉量少、轻度先天性关节挛缩、鸡胸、脊柱侧凸和蜘蛛指。特殊面容是由于睑裂狭小、先天性上睑下垂、下颌发育不全以及耳朵低位和畸形所致。马登和沃克报告的病例中还发现了腭后正中裂以及心脏和肾脏异常。我们的病例2有右位心。本报告提示该综合征为常染色体隐性遗传。