Introduction
Polydactyly is the most common congenital anomaly of the forefoot.1,2,3,4,5,6,7
History of the Procedure
Treatment for polydactyly of the foot has changed little over time. Removing the extra digit by disarticulation is the standard treatment.3,8,9,10
Problem
Polydactyly most commonly refers to the presence of 6 toes on one foot, but more toes are possible. Polydactyly may be associated with syndactyly. It most frequently occurs as an isolated trait with autosomal dominant inheritance and variable penetrance. Other patterns of inheritance, sporadic occurrence, and association with syndromes are also possible.1 (See also the eMedicine article Syndactyly.)
Frequency
Incidence is 1.7 cases per 1000 live births. Incidence is higher in blacks (3.6-13.9 cases per 1000 live births) than in whites (0.3-1.3 cases per 1000 live births).
Etiology
Polydactyly may occur as an isolated trait or in conjunction with certain syndromes, and there is a positive family history in 30% of cases. The syndromes with which polydactyly has been associated include Ellis-van Creveld syndrome, trisomy 13, tibial hemimelia, and trisomy 21. Polydactyly is bilateral in 50% of cases and has a slight male predilection. (See also the eMedicine articles Fibular Hemimelia and Ellis-van Creveld Syndrome, as well as the article Recognizing the Clinical Features of Trisomy 13 Syndrome on Medscape.)
Pathophysiology
Postaxial polydactyly (lateral ray) is the most common polydactyly,6 occurring in 80% of cases, followed by preaxial polydactyly (medial) and then central polydactyly. The duplication may range from a well-formed articulated digit to a rudimentary digit. Abnormalities of the associated metatarsal commonly occur in polydactyly. A morphologic classification was described by Venn-Watson,1 based on the configuration of the metatarsal (see Image 1), as follows:
- Normal metatarsal with distal phalanx duplication
- Block metatarsal
- Y-shaped metatarsal
- T-shaped metatarsal
- Normal metatarsal shaft with wide head
- Duplicated ray
A first metatarsal bracket epiphysis (longitudinal epiphyseal bracket) may occur in preaxial polydactyly.
Presentation
The patient may present in infancy or at a later date when cosmesis or shoe fit becomes a concern.
Obtain a thorough family history, and exclude association with syndromes. Perform a thorough musculoskeletal examination to exclude any other congenital anomalies or signs that occur with known syndromes.
Indications
Surgery is indicated to improve cosmesis and to improve shoe fit. It is usually performed when the patient is aged approximately 1 year, so the effect on development and walking is minimal. Surgery should be delayed until skeletal development (ossification) has occurred within the affected rays so that accurate anatomic assessment is possible.
Relevant Anatomy
Contraindications
No absolute contraindications to surgery exist. However, parents may choose not to excise the duplicate digit for personal reasons.
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References
Venn-Watson EA. Problems in polydactyly of the foot. Orthop Clin North Am. Oct 1976;7(4):909-27. [Medline].
Beaty JH. Polydactyly. In: Operative Pediatric Orthopaedics. 2nd ed. Mosby-Year Book;1995:112-114.
Hart ES, Grottkau BE, Rebello GN, Albright MB. The newborn foot: diagnosis and management of common conditions. Orthop Nurs. Sep-Oct 2005;24(5):313-21; quiz 322-3. [Medline].
Herring JA. Polydactyly. In: Tachdjian's Pediatric Orthopaedics from the Texas Scottish Rite Hospital for Children. 3rd ed. WB Saunders Co;2001:1021-1024.
Jones KL. Smith's Recognizable Patterns of Human Malformation. 5th ed. WB Saunders Co;1997.
Lee HS, Park SS, Yoon JO, Kim JS, Youm YS. Classification of postaxial polydactyly of the foot. Foot Ankle Int. May 2006;27(5):356-62. [Medline].
Turra S, Gigante C, Bisinella G. Polydactyly of the foot. J Pediatr Orthop B. May 2007;16(3):216-20. [Medline].
Lim YJ, Teoh LC, Lee EH. Reconstruction of syndactyly and polysyndactyly of the toes with a dorsal pentagonal island flap: a technique that allows primary skin closure without the use of skin grafting. J Foot Ankle Surg. Mar-Apr 2007;46(2):86-92. [Medline].
Morley SE, Smith PJ. Polydactyly of the feet in children: suggestions for surgical management. Br J Plast Surg. Jan 2001;54(1):34-8. [Medline].
Mubarak SJ, O'Brien TJ, Davids JR. Metatarsal epiphyseal bracket: treatment by central physiolysis. J Pediatr Orthop. Jan-Feb 1993;13(1):5-8. [Medline].
Yucel A, Kuru I, Bozan ME, Acar M, Solak M. Radiographic evaluation and unusual bone formations in different genetic patterns in synpolydactyly. Skeletal Radiol. Aug 2005;34(8):468-76. [Medline].
Leeson MC, Wilcox PG, Weiner DS. Congenital duplication of the foot and toes. Foot Ankle. Jan-Feb 1985;5(4):191-7. [Medline].
Weaver KM, Henry GW, Reinker KA. Unilateral duplication of the great toe with anterolateral tibial bowing. J Pediatr Orthop. Jan-Feb 1996;16(1):73-7. [Medline].
Further Reading
Keywords
duplicate toe, extra toe, duplicate digit, extra digit, forefoot anomaly, syndactyly, Ellis-van Creveld syndrome, trisomy 13, tibial hemimelia, trisomy 21
Overview: Polydactyly of the Foot