Cenani Lenz syndactylism
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Overview
Cenani Lenz syndactylism (also known as Cenani's syndactyly) is a congenital malformation syndrome involving both, upper and lower extremity. It is thought to be inherited autosomal recessive. The syndrome is named after Turkish (Asim Cenani) and German (Widukind Lenz) medical geneticists.
It is characterised by a nearly symmetrical presence of a spoon hand (classical type) or, more frequently, an oligodactylous hand. All individuals with this syndrome had the following symptoms: carpal, metacarpal and digital synostoses, disorganisation of carpal bones, numeric reduction of digital rays and syndactyly of toes. Additionally some individuals have some of the following symptoms: radio-ulnar synostosis, brachymesomelia, radius head dislocation, metatarsal synostoses and numeric reduction of rays.
References
- Cenani A, Lenz W (1967). "[Total syndactylia and total radioulnar synostosis in 2 brothers. A contribution on the genetics of syndactylia]". Z Kinderheilkd. 101 (3): 181–90. PMID 4298043.
- Nezarati MM, McLeod DR (2002). "Cenani-Lenz syndrome: report of a new case and review of the literature". Clin Dysmorphol. 11 (3): 215–8. PMID 12072805.
- Harpf C, Pavelka M, Hussl H (2005). "A variant of Cenani-Lenz syndactyly (CLS): review of the literature and attempt of classification". Br J Plast Surg. 58 (2): 251–7. PMID 15710123.