Polydactyly

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	Polydactyly;
	Right hand with mid-ray duplication
ICD-10	Q69
ICD-9	755.0
OMIM	603598
DiseasesDB	24853
eMedicine	derm/692

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2] Kiran Singh, M.D. [3]

Overview

Polydactyly or polydactylism (from the Greek poly = "many" + daktylos = "finger"), also known as hyperdactyly, is a congenital physical anomaly consisting of supernumerary fingers or toes. When each hand or foot has six digits, it is sometimes called "sexdactyly", "hexadactyly", or "hexadactylism". Although the condition is usually not life-threatening or even particularly debilitating, most people in Western societies have the extra digits removed surgically.

The extra digit is usually a small piece of soft tissue; occasionally it may contain bone without joints; rarely it may be a complete, functioning digit. The extra digit is most common on the ulnar (little finger) side of the hand, less common on the radial (thumb) side, and very rarely within the middle three digits. The extra digit is most commonly an abnormal fork in an existing digit, or it may rarely originate at the wrist like a normal digit does.

Polydactyly can occur by itself, or more commonly, as one feature of a syndrome of congenital anomalies. When it occurs by itself, it is associated with autosomal dominant mutations in single genes, i.e. it is not a multifactorial trait. [4] But mutation in a variety of genes can give rise to polydactyly. Typically the mutated gene is involved in developmental patterning, and a syndrome of congenital anomalies results, of which polydactyly is one feature.

The condition has an incidence of 1 in every 500 live births^[1] although the frequency is higher in some groups (an example is the Amish in the United States, due to the founder effect).

Types of polydactyly

Polydactyly with cleft lip/palate and psychomotor retardation (Varadi-Papp Syndrome)
Polydactyly myopia syndrome
Polydactyly postaxial dental and vertebral
Polydactyly postaxial with median cleft of upper lip
Polydactyly postaxial
Polydactyly preaxial type 1
Polydactyly syndrome middle ray duplication
Polydactyly visceral anomalies cleft lip palate

Diagnosis

Physical Examination

Extremities

Rudimentary polydactyly. Adapted from Dermatology Atlas.^[2]
Rudimentary polydactyly. Adapted from Dermatology Atlas.^[2]
Rudimentary polydactyly. Adapted from Dermatology Atlas.^[2]
Rudimentary polydactyly. Adapted from Dermatology Atlas.^[2]
Rudimentary polydactyly. Adapted from Dermatology Atlas.^[2]
Rudimentary polydactyly. Adapted from Dermatology Atlas.^[2]
Rudimentary polydactyly. Adapted from Dermatology Atlas.^[2]
Rudimentary polydactyly. Adapted from Dermatology Atlas.^[2]
Rudimentary polydactyly. Adapted from Dermatology Atlas.^[2]
Rudimentary polydactyly. Adapted from Dermatology Atlas.^[2]
Rudimentary polydactyly. Adapted from Dermatology Atlas.^[2]

Examples

Ellis-Van Creveld syndrome
Left foot with postaxial polydactyly of 5th ray
Right hand with mid-ray duplication

References

↑ Polydactylism FAQ
↑ ^2.00 ^2.01 ^2.02 ^2.03 ^2.04 ^2.05 ^2.06 ^2.07 ^2.08 ^2.09 ^2.10 "Dermatology Atlas".

External links

Template:Wiktionarypar

Template:WikiDoc Sources

[drgreene-1] Polydactylism FAQ

[Dermatology_Atlas-2] 2.00 ^2.01 ^2.02 ^2.03 ^2.04 ^2.05 ^2.06 ^2.07 ^2.08 ^2.09 ^2.10 "Dermatology Atlas".

[1]

[2]

v t e Congenital malformations and deformations of musculoskeletal system (Q65-Q79, 754-756)
Limbs	hip: Dislocation of hip/Hip dysplasia - Upington disease feet (Club foot, Flat feet, Pes cavus) systemic dislocations Larsen syndrome head, face, spine and chest: skull, face and jaw (Dolichocephaly, Greig cephalopolysyndactyly syndrome, Plagiocephaly) - spine Scoliosis - chest (Pectus excavatum, Pectus carinatum) any combination head, face, jaw, upper limb, lower limb, pelvis, dactyly Antley-Bixler syndrome - Schmitt Gillenwater Kelly syndrome dactyly Polydactyly/Syndactyly (Webbed toes) - Cenani Lenz syndactylism reduction deficits (Acheiropodia, Amelia, Ectrodactyly, Phocomelia) upper limb (Cleidocranial dysostosis, Madelung's deformity, Sprengel's deformity, Wallis Zieff Goldblatt syndrome) knee (Genu valgum, Genu varum) other Arthrogryposis
Skull and facial bones	Carpenter syndrome - Craniodiaphyseal dysplasia - Craniosynostosis (Scaphocephaly) - Crouzon syndrome - Hypertelorism - Macrocephaly - Oxycephaly - Platybasia - Saethre-Chotzen syndrome - Treacher Collins syndrome - Trigonocephaly
Spine and bony thorax	Klippel-Feil syndrome - Spondylolisthesis - Cervical rib - Bifid rib
Osteochondrodysplasia	developement 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 congenita) - Ellis-van Creveld syndrome - Otospondylomegaepiphyseal dysplasia - Spondyloepiphyseal dysplasia congenita - Osteogenesis imperfecta - McCune-Albright syndrome - Osteopetrosis - Metaphyseal dysplasia - Recessive multiple epiphyseal dysplasia - Hereditary multiple exostoses - Osteopoikilosis - Chondrodystrophy - Osteodystrophy - Atelosteogenesis, type II - Diastrophic dysplasia
Other	abdominal wall (Congenital diaphragmatic hernia, Omphalocele, Gastroschisis, Prune belly syndrome) - Ehlers-Danlos syndrome
See also non-congenital conditions (M, 710-739)