Hadziselimovic F, Fliegel C H, Miny P
Department of Gastroenterology, Children's Hospital, University Clinics, Basel, Switzerland.
Clin Dysmorphol. 2001 Jan;10(1):33-6. doi: 10.1097/00019605-200101000-00007.
An identical pattern of malformations was found in two brothers both having microcephaly and severe developmental delay. Additionally, they had hypotelorism, epicanthic folds, and convergent strabismus. There was shortening of either the radius or the tibia and shortening of the first metacarpals. Persistently dorsally flexed fingers and toes were noted, all of which are unusually long. Both boys had a high-pitched voice and were unable to communicate verbally at the age of 4.5 years. They both developed short stature. One brother has anal atresia; the other had a pulmonary artery atresia, VSD, ASD, and an over-riding aorta. This apparently new syndrome is possibly an autosomal, or a X-linked recessive trait.
在两个患有小头畸形和严重发育迟缓的兄弟中发现了相同的畸形模式。此外,他们有眼距过窄、内眦赘皮和共同性斜视。桡骨或胫骨缩短,第一掌骨缩短。手指和脚趾持续背屈,且都异常长。两个男孩都有高音调嗓音,在4.5岁时无法言语交流。他们都身材矮小。一个兄弟患有肛门闭锁;另一个患有肺动脉闭锁、室间隔缺损、房间隔缺损和主动脉骑跨。这种明显的新综合征可能是常染色体或X连锁隐性性状。