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Considerations on etiology, correction and treatment of camptodactyly
CONGENITAL
defect. The variable expression within each family indicates the role of additional factors and leads us to a hypothesis that TPT gene is a regulatory gene involved in hand development during embryogenesis.The prevalence of the TPT in this population is estimated to be at least 100 times higher than in the world population, which gives a very good opportunity to localise and identify the disease-gene. Using the strategy of linkage analysis, we have localised the TPT gene on the long arm of chromosome 7. To our knowledge, this is the first time that a human gene involved solely in the pathologic morphogenesis of the hand has been localised. Cloning of the TPT gene and its functional characterisation will help us to understand not only the underlying aetiology of congenital malformations of the hand, but also the processes that are involved in a normal limb development. Finding the locus for the TPT gene is the first step in the establishment of a new, pathogenetic classification of complex hand malformations, as a supplement to current morphological classifications.
Considerations on etiology, correction and treatment of camptodactyly B. Reichert, P. Brenner, A. Berger Plastic Surgery, Hannover Medical School, Hannoveu, Germany
Camptodactyly is described as an isolated deformity of the PIP-joint, mostly of the small finger, not preceded by trauma. Numerous causeshave been described to explain this deformity. In our opinion it seemsto be reasonable to assumean imbalance between flexing and extending forces at the PIP-joint. Atypical insertion of the lumbrical muscles may be found, but is very rare. We report on the operative findings, methods of correction and late results of 26 patients that were operated upon for camptodactyly between 1982 and 1993. 13 were males, 13 were
9
females. Mean age at the time of the first oneration was 13.5 years (min I year, max. 39 years). Our concept of treatment includes functional reconstructions as well as static procedures. We recommend operative treatment in young children, since results are superior, if patients come to surgery before the age of 14. Still favorable results with full active range of motion are rare, even when correction has been performed in early childhood. Today there is no concept on the treatment of camptodactyly that is generally accepted. We therefore are interested in an open discussion on our practice.
The shamrock flap for centralization of radial club hand D. M. Evans, D. R. Gateley, J. S. Lewis Department of Plastic Surgery, St Thomas’ and Guy’s Hospital, London, UK
Various incisions have been used to approach the wrist joint in order to correct radial club hand by centralization or radialization. With most existing approaches there is some tension in the skin on the radial side and redundant skin on the ulnar side which requires excision to leave a satisfactory contour. The ideal incision would provide additional skin on the radial side and take up the slack on the ulnar side, as well as giving wide accessto the wrist and surrounding structures. We describe the use of a bilobed flap which fulfils these requirements. Redundant skin on the ulnar side is transposed into the dorsal midline over the wrist to replace a similar flap which moves to the radial defect created by straightening the wrist. The wide exposure afforded by this shamrock-shaped flap provides wider accessto all aspects of the joint than any other incision, and leaves a smoothly contoured wrist without discarding any skin. The approach has been used for centralization of radial club hand in five caseswithout complication and with excellent contour of the wrist.
defect. The variable expression within each family indicates the role of additional factors and leads us to a hypothesis that TPT gene is a regulatory gene involved in hand development during embryogenesis.The prevalence of the TPT in this population is estimated to be at least 100 times higher than in the world population, which gives a very good opportunity to localise and identify the disease-gene. Using the strategy of linkage analysis, we have localised the TPT gene on the long arm of chromosome 7. To our knowledge, this is the first time that a human gene involved solely in the pathologic morphogenesis of the hand has been localised. Cloning of the TPT gene and its functional characterisation will help us to understand not only the underlying aetiology of congenital malformations of the hand, but also the processes that are involved in a normal limb development. Finding the locus for the TPT gene is the first step in the establishment of a new, pathogenetic classification of complex hand malformations, as a supplement to current morphological classifications.
Considerations on etiology, correction and treatment of camptodactyly B. Reichert, P. Brenner, A. Berger Plastic Surgery, Hannover Medical School, Hannoveu, Germany
Camptodactyly is described as an isolated deformity of the PIP-joint, mostly of the small finger, not preceded by trauma. Numerous causeshave been described to explain this deformity. In our opinion it seemsto be reasonable to assumean imbalance between flexing and extending forces at the PIP-joint. Atypical insertion of the lumbrical muscles may be found, but is very rare. We report on the operative findings, methods of correction and late results of 26 patients that were operated upon for camptodactyly between 1982 and 1993. 13 were males, 13 were
9
females. Mean age at the time of the first oneration was 13.5 years (min I year, max. 39 years). Our concept of treatment includes functional reconstructions as well as static procedures. We recommend operative treatment in young children, since results are superior, if patients come to surgery before the age of 14. Still favorable results with full active range of motion are rare, even when correction has been performed in early childhood. Today there is no concept on the treatment of camptodactyly that is generally accepted. We therefore are interested in an open discussion on our practice.
The shamrock flap for centralization of radial club hand D. M. Evans, D. R. Gateley, J. S. Lewis Department of Plastic Surgery, St Thomas’ and Guy’s Hospital, London, UK
Various incisions have been used to approach the wrist joint in order to correct radial club hand by centralization or radialization. With most existing approaches there is some tension in the skin on the radial side and redundant skin on the ulnar side which requires excision to leave a satisfactory contour. The ideal incision would provide additional skin on the radial side and take up the slack on the ulnar side, as well as giving wide accessto the wrist and surrounding structures. We describe the use of a bilobed flap which fulfils these requirements. Redundant skin on the ulnar side is transposed into the dorsal midline over the wrist to replace a similar flap which moves to the radial defect created by straightening the wrist. The wide exposure afforded by this shamrock-shaped flap provides wider accessto all aspects of the joint than any other incision, and leaves a smoothly contoured wrist without discarding any skin. The approach has been used for centralization of radial club hand in five caseswithout complication and with excellent contour of the wrist.