Cenani Lenz syndactylism

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Cenani Lenz syndactylism (also known as Cenani's syndactyly) is a congenital malformation syndrome involving both upper and lower extremities. It is thought to be inherited as an autosomal recessive genetic trait.

Eponym

The syndrome is named after Turkish (Asim Cenani) and German (Widukind Lenz) medical geneticists. [WhoNamedIt|synd|2130] [cite journal | author=Cenani A, Lenz W | title= [Total syndactylia and total radioulnar synostosis in 2 brothers. A contribution on the genetics of syndactylia] | journal=Z Kinderheilkd | year=1967 | pages=181–90 | volume=101 | issue=3 | pmid=4298043]

Presentation

It is characterized by a nearly symmetrical presence of a spoon hand (classical type) or, more frequently, an oligodactylous hand. Individuals with this syndrome present the following symptoms: carpal, metacarpal and digital synostoses, disorganization of carpal bones, numeric reduction of digital rays and toe syndactyly. Additionally, other symptoms may include radio-ulnar synostosis, brachymesomelia, radius head dislocation, metatarsal synostoses and numeric reduction of rays.

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