Clinical Presentation, Surgical Treatment, and Outcome in Radial Polydactyly

Abstract

Radial polydactyly or ‘thumb duplication’ is the most common congenital upper limb anomaly (‘CULA’) affecting the thumb. The clinical presentation is highly diverse, ranging from an extra thumb floating on a skin bridge to complicated thumb triplications with triphalangeal, deviating, and hypoplastic components. Radial polydactyly can be classified into one of 7 osseous presentations using the Wassel classification, with type IV (45%), type II (20%), and type VII (15%) occurring most frequently. When faced with a radial polydactyly case, hand surgeons specialised in congenital anomalies must weigh the preoperative functional potential and degree of hypoplasia of both thumbs in order to decide whether to resect one thumb and reconstruct the other (‘resection and reconstruction’), excise a central part of both thumbs and unite the lateral tissues into one thumb (‘the Bilhaut procedure’), transfer the better-developed distal tissues of one thumb onto the better-developed proximal tissues of the other (‘on-top plasty’), or discard both severely hypoplastic thumbs and pollicise the index finger. Mere excision of the hypoplastic thumb is rarely indicated since it often requires subsequent revision surgery. Even after being treated by experienced surgeons, about 15% of patients with polydactyly will need additional procedures to correct residual and/or new problems such as deviation from the longitudinal axis and joint instability. Nevertheless, radial polydactyly patients usually achieve unimpaired everyday hand function postoperatively.

Zusammenfassung

Die radiale Polydaktylie, auch Doppeldaumen genannt, ist die häufigste angeborene den Daumen betreffende Fehlbildung der oberen Extremität. Das klinische Erscheinungsbild weist eine große Variationsbreite auf, von einem nur an einer dünnen Hautbrücke flottierenden Daumenanhängsel bis hin zum Dreifach-Daumen mit dreigliedrigen, deviierenden und hypoplastischen Anteilen. Die Wassel-Klassifikation, bei der die radiale Polydaktylie anhand der knöchernen Situation eingeteilt wird, unterscheidet 7 Typen, wobei der Typ IV mit 45%, der Typ II mit 20% und der Typ VII mit 15% am häufigsten vorkommen. Bei der Planung der chirurgischen Behandlung der radialen Polydaktylie gilt es für den mit angeborenen Fehlbildungen vertrauten Handchirurgen abzuwägen, ob einer der beiden Daumen und ggf. welcher ein größeres funktionelles Potenzial hat bzw. welcher weniger gut ausgebildet ist. In Betracht kommen die Resektion des einen und Rekonstruktion des anderen Daumens, die Resektion der einander zugewandten „zentralen“ Anteile beider Daumen mit anschließendem Zusammenfügen der beiden äußeren Anteile zu einem Daumen (Bilhaut-Operation), die Transposition des besser entwickelten distalen Anteils des einen auf den besser entwickelten proximalen Anteils des anderen Daumens (on-top-plasty) und die Entfernung beider ausgeprägt hypoplastischer Daumen mit anschließender Pollizisation des Zeigefingers. Das bloße „Abschneiden“ ist selten indiziert, da es oft spätere Revisionsoperationen nötig macht. Selbst in der Hand des Erfahrenen bedürfen 15% der Patienten mit einer radialen Polydaktylie weiterer Eingriffe zur Behandlung verbliebener und/oder neu aufgetretener Probleme, z. B. bei Achsabweichungen oder Gelenkinstabilitäten. Dennoch erlangen Patienten mit einer radialen Polydaktylie in der Regel eine für den Alltagsgebrauch uneingeschränkte Handfunktion.

Key words

thumb duplication - Bilhaut procedure - radial polydactyly

Schlüsselwörter

Doppeldaumen - Bilhaut-Operation - Radiale Polydaktylie

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