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Thumb Duplication: Concepts and Techniques Michael A CORE Metadata, citation and similar papers at core.ac.uk Provided by PubMed Central Review Article Clinics in Orthopedic Surgery 2012;4:1-17 • http://dx.doi.org/10.4055/cios.2012.4.1.1 Thumb Duplication: Concepts and Techniques Michael A. Tonkin, MD Department of Hand Surgery and Peripheral Nerve Surgery, University of Sydney, Royal North Shore Hospital, St. Leonards and Department of Hand Surgery, University of Sydney, Children’s Hospital at Westmead, Westmead, Australia Within the Oberg, Manske, Tonkin (OMT) classifi cation, thumb duplications are a failure of formation and/or differentiation affect- ing the radial-ulnar axis of the hand plate. The Wassel description of seven types of thumb duplication provides a good structure from which an approach to management is based. The aim of surgical reconstruction is to obtain a stable, mobile thumb of ad- equate size and appropriate shape. The most common form of reconstruction is removal of the lesser digit and reconstruction of the dominant digit. Surgical techniques address the problems of deviation, instability and lack of size. The disadvantages of the Bilhaut-Cloquet procedure, these being joint stiffness and a nail ridge, may be lesser concerns when reconstruction of one digit will not create a satisfactory thumb of adequate mobility, stability, alignment and size. Complicated problems of triphalangism, triplication, ulnar dimelia and the rare circumstance in which neither of the duplicated thumbs may be adequately reconstructed present specifi c challenges which demand alternative techniques. Keywords: Thumb duplication, Classifi cation, Assessment, Reconstruction Thumb duplication is classified within the International anomaly (Table 1).2) It recognises that formation and dif- Federation of Societies for Surgery of the Hand (IFSSH)/ ferentiation occur together, not as separate independent Swanson classifi cation of congenital anomalies of the hand processes, and directs us to the site of insult in the devel- and upper limb as a “duplication” (group 3).1) Included are oping limb bud. It also indicates whether the anomaly in- radial polydactyly, central polydactyly, ulnar polydactyly volves the whole of the upper limb or the hand alone. and proximal duplications such as ulnar dimelia. Although It is beyond the scope of this article to explore in de- groups 1 and 2, “failure of formation” and “failure of diff er- tail the genetic pathways and the disruptions within these entiation”, give some imprecise suggestion as to the cause which lead to thumb duplication. However, application of of a particular anomaly, other groups of this classifi cation, the principles upon which the OMT classifi cation is based “duplication”, “overgrowth”, “undergrowth” and “constric- provides some understanding of how anomalies of devel- tion ring syndrome” (and perhaps also that of “generalised opment and patterning occur. Thumb duplications are a skeletal anomalies”) are purely descriptive. Our increasing failure of formation and/or differentiation affecting the understanding of the molecular biology of limb growth radial/ulnar axis of the hand plate. Th e primary signal cen- and patterning demands a reappraisal of the classifi cation tre involved is the zone of polarising activity (ZPA) in the of congenital limb anomalies, one that refl ects this current posterior part of the developing limb bud. Sonic Hedge- knowledge. Th e Oberg, Manske, Tonkin (OMT) classifi ca- hog protein, which is expressed in the ZPA, plays a major tion implicates the axis of formation and differentiation role in determining radial-ulnar characteristics. Abnormal which is primarily involved in the development of the expression of other morphogens such as Hox genes, Bone Morphogenic protein and Gli-3 are involved in the devel- opment of thumb duplications. Received November 14, 2011; Accepted December 30, 2011 Th e Wassel description of seven types of thumb du- Correspondence to: Michael A. Tonkin, MD plication is that which is most familiar to congenital hand Department of Hand Surgery and Peripheral Nerve Surgery, Royal North surgeons (Fig. 1).3) It is based on the level of the skeleton at Shore Hospital, St. Leonards, NSW 2065, Australia which the duplication occurs and is simple. It provides no Tel: +61-2-9926-7067, Fax: +61-2-9926-7774 information as to which thumb is dominant, whether there E-mail: [email protected] Copyright © 2012 by Th e Korean Orthopaedic Association Th is is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Clinics in Orthopedic Surgery • pISSN 2005-291X eISSN 2005-4408 2 Tonkin. Th umb Duplication: Concepts and Techniques Clinics in Orthopedic Surgery • Vol. 4, No. 1, 2012 • www.ecios.org Table 1. The OMT Classifi cation of Hand and Upper Limb Anomalies is convergence and/or divergence at any particular joint level, the stability of joints, nor the presence of soft tissue Malformations anomalies. Triphalangism is clumsily placed separately in Failure of axis formation/differentiation: entire upper limb type VII, although it may accompany skeletal anomalies Proximal-distal outgrowth Brachymelia with brachydactyly which diff er from that classically depicted in Wassel’s type Symbrachydactyly VII. It may be preferable to describe the duplication ac- cording to its skeletal level (types I to VI) and add “accom- Transverse defi ciency panied by triphalangism in the radial and/or ulnar thumb”, Intersegmental defi ciency as appropriate. A basic fl aw in the Wassel classifi cation is Radial-ulnar (anteroposterior) axis Radial longitudinal defi ciency that this it is based on an assessment of the skeleton, the true nature of which may not be radiologically apparent in Ulnar longitudinal defi ciency the skeletally immature. For instance, a type I duplication Ulnar dimelia may be classified as a type II until ossification of a basal Radioulnar synostosis cartilaginous connection becomes apparent. It is oft en not Humero-radial synostosis possible to defi ne the status of physes and epiphyses. Nev- Dorsal-ventral axis Nail-patella syndrome ertheless, the Wassel terminology provides quite a good structure from which one may approach the management Failure of axis formation/differentiation: handplate of a thumb duplication. The more experienced surgeon Radial-ulnar (anteroposterior) axis Radial polydactyly will have an understanding of the anomalies that are likely Triphalangeal thumb to be associated with each type and which may require at- Ulnar polydactyly tention during reconstruction, just as an understanding of Dorsal-ventral axis Dorsal dimelia (palmar nail) “camptodactyly” infers the presence of specifi c anatomical anomalies which accompany that diagnosis. Hypoplastic/aplastic nail Failure of handplate formation/differentiation: unspecifi ed axis Soft tissue Syndactyly PREOPERATIVE ASSESSMENT Camptodactyly The aim of surgical reconstruction is to obtain a stable, Skeletal defi ciency Brachydactyly mobile thumb of adequate size and appropriate shape. Sta- Clinodactyly bility and size relate to strength, both for grip and pinch. Thumb mobility is largely dependant upon the integrity Kirner’s deformity of the carpometacarpal (CMC) joint, usually normal in Metacarpal and carpal synostoses Wassel types I to IV, but possibly with varying degrees of Complex Cleft hand maldevelopment or underdevelopment in types V and VI. Synpolydactyly Mobility at the metacarpophalangeal (MCP) and interpha- Apert hand langeal (IP) joints, although necessary for normal thumb function, is perhaps not as important. Th e range of normal Deformations MCP joint motion is variable, both in the amount of fl ex- Constriction ring sequence ion and the presence or absence of hyperextension. Loss of Arthrogryposis IP joint fl exion impairs tip pinch, but children compensate Trigger digits eff ectively without appreciating any loss of function. When the surgeon is confronted with a confl ict between stability Not otherwise specifi ed and mobility, it is reasonable to accept a decrease in distal Dysplasias joint motion to obtain joint stability, if the CMC joint is Hypertrophy intact. Finally, the shape of the thumb relates to aesthetics Macrodactyly and is important. These components of thumb function demand at- Upper limb tention when planning surgical reconstruction. It may be Upper limb and macrodactyly obvious to the casual eye that one thumb is the dominant Tumorous conditions thumb. However, there may be defi ciencies of the thumb Reprinted with permission from Elsevier Health Science Journals.2) to be retained. The parents are warned that surgery may OMT classifi cation: Oberg, Manske, Tonkin (OMT) classifi cation. 3 Tonkin. Th umb Duplication: Concepts and Techniques Clinics in Orthopedic Surgery • Vol. 4, No. 1, 2012 • www.ecios.org Fig. 1. The Wassel classifi cation of thumb duplication. Reprinted with permission from Springer-Verlag.3) Fig. 2. Appearance of the dominant thumb when the examiner’s hand conceals the radial duplicate. involve the reconstruction of the dominant thumb, not reconstructive surgeon. The principle is to complete the simply removal of the smaller digit, usually the radial. A reconstruction of the retained thumb at the time of the comparison with the normal thumb of the opposite hand removal of the smaller digit, attending to all anatomical will demonstrate
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