Cenani Lenz syndactylism

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Cenani Lenz syndactylism Classification and external resources
OMIM	212780
DiseasesDB	32523

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.

[edit] Eponym

The syndrome is named after Turkish (Asim Cenani) and German (Widukind Lenz) medical geneticists.^[1][2]

[edit] 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.

[edit] References

• Nezarati MM, McLeod DR (2002). "Cenani-Lenz syndrome: report of a new case and review of the literature". Clin Dysmorphol 11 (3): 215-8. doi:10.1097/00019605-200207000-00014. 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. doi:10.1016/j.bjps.2004.10.024. PMID 15710123. 

v • d • e Congenital malformations and deformations of musculoskeletal system / musculoskeletal abnormality (Q65-Q79, 754-756) Congenital limb deformities Upper limbs Cleidocranial dysostosis - Madelung's deformity - Sprengel's deformity - Wallis Zieff Goldblatt syndrome Lower limbs hip: Dislocation of hip/Hip dysplasia - Upington disease knee: Genu valgum - Genu varum feet: Club foot - Flat feet - Pes cavus Upper and/or lower limbs dactyly: Polydactyly/Syndactyly (Webbed toes) - Arachnodactyly - Cenani Lenz syndactylism reduction deficits: Acheiropodia - Amelia - Ectrodactyly - Phocomelia multiple joints: Arthrogryposis - Larsen syndrome - Rapadilino syndrome Craniofacial abnormalities Macrocephaly - Platybasia Craniosynostosis: Scaphocephaly - Oxycephaly - Trigonocephaly Craniofacial dysostosis: Crouzon syndrome - Hypertelorism - Hallermann-Streiff syndrome - Treacher Collins syndrome Craniodiaphyseal dysplasia Dolichocephaly - Greig cephalopolysyndactyly syndrome - Plagiocephaly Other axial skeleton spine: spinal curvature (Scoliosis) - Klippel-Feil syndrome - Spondylolisthesis ribs: Cervical rib - Bifid rib sternum: Pectus excavatum - Pectus carinatum Osteochondrodysplasia development of cartilage, tubular bones and spine: Achondrogenesis/Hypochondrogenesis - Boomerang dysplasia - Thanatophoric dysplasia - Short rib-polydactyly syndrome - Chondrodysplasia punctata (Rhizomelic chondrodysplasia punctata, Conradi-Hünermann syndrome) - Achondroplasia (Hypochondroplasia) - Osteosclerosis (Raine syndrome) - Ellis-van Creveld syndrome - Otospondylomegaepiphyseal dysplasia - Spondyloepiphyseal dysplasia congenita collagen diseases: Osteogenesis imperfecta - Ehlers-Danlos syndrome combined/other: McCune-Albright syndrome - Osteopetrosis - Metaphyseal dysplasia - Recessive multiple epiphyseal dysplasia - Hereditary multiple exostoses - Osteopoikilosis - Chondrodystrophy - Osteodystrophy - Atelosteogenesis, type II - Diastrophic dysplasia - Camurati-Engelmann disease - Antley-Bixler syndrome Other abdominal wall (Congenital diaphragmatic hernia, Omphalocele, Gastroschisis, Prune belly syndrome) See also non-congenital conditions (M, 710-739)