- Email: [email protected]
A-0200 Thumb triplication; a rarity?
6
A-0200 2.1 THUMB TRIPLICATION; A RARITY?
S. Hovius, M. Ananta and J. Zuidam From the Department of Plastic and Reconstructive Surgery and Hand Surgery, Erasmus MC, University Medical Center, Rotterdam, The Netherlands Background: Radial polydactyly is a common form of a congenital hand anomaly; however, more complex forms like triplication of the thumb are supposed to be rare. We encountered an unusually high number of triplicated thumbs at our unit. Is triplication of the thumb indeed a rare condition and is it treated differently? Methods: In our series from 1993 till 2006 112 patients with radial polydactyly were recorded. In nine subjects (8%) of this Group, triplicated thumbs either unilateral (seven) or bilateral (two) were identified. Other associated congenital upper limb deformities were syndactyly, ulnar polydactyly, dislocation of the radial head and triphalangeal thumb. Results: A total of 11 (9%) triplicated thumbs are demonstrated, all cases were combined with triphalangeal components. Only one of these in total eleven triplicated thumbs could be classified according to currently most used classifications. In all cases aberrant rays were excised, thumb length and alignment restored by osteotomies, joints were stabilised, tendons reinserted and nails and nail walls corrected if necessary. Also in all cases a correction of triphalangeal components was performed. Discussion: In the literature triplications are a rarity, presented by single case reports. In our series, triplicated thumbs are not so rare probably because in the southwest region of the Netherlands a genetic isolate, a deformity located at chromosome 7q36, has been identified with radial polydactyly and triphalangeal thumbs. The molecular basis of this deformity is the up-regulation of Sonic Hedge Hog (SHH) leading to ectopic expression at the anterior margin of the growing limb bud, resulting in radial polydactyly. Treatment for the presented triplicated thumbs was based on the same general principles as for less complex forms of radial polydactyly, in essence to assemble useful elements of the separate (partial) thumbs to reconstruct one functioning, stable thumb. 10.1016/j.jhse.2007.02.077
THE JOURNAL OF HAND SURGERY VOL. 32E SUPPLEMENT 1
A-0092 2.2 AUTOLOGOUS CELL CULTURES IN THE SURGICAL MANAGEMENT OF THE HAND IN DYSTROPHIC EPIDERMOLYSIS BULLOSA
E. Betsi, M. Benathan and W. Raffoul From the Department of Plastic and Reconstructive Surgery, University Hospital of Lausanne, Switzerland Background: Epidermolysis Bullosa (EB) is a diverse group of genodermatoses and can be classified into three major categories: the simplex, the junctional and the dystrophic forms. Aim: The purpose of this presentation is to demonstrate the use of autologous cultured cells in treating an advanced cocoon formation of the hand and an extended squamous cell carcinoma (SCC) of the lower limb in a patient with recessive dystrophic EB. The preparation of these cells and their application in the operation room is being described. Method: The hands in these patients are subject to repeated erosions and blisters due to frequent minimal mechanical traumas. Cocoon formation of the hand is well known to arise as a complication in Recessive Dystrophic Epidermolysis Bullosa (RDEB) patients. A number of surgical approaches have been described to correct these deformities and improve function. We propose a new therapeutic approach of treating loss of motion and independent digital function in a patient with RDEB using autologous culture cells. Results: We managed to expand the interspace between the thumb and the rest of the digits and to create space between the fingers and the underlying palm, resulting in the hand becoming functional. We covered the cutaneous defect with homologous keratinocytes and fibroblasts. Furthermore, we treated the lower limb by surgical excision achieving clear margins and we covered the wound with homologous keratinocytes and fibroblasts. Conclusion: Many studies have been carried out concerning the surgical management in RDEB. The use of autologous keratinocytes and fibroblasts in Epidermolysis Bullosa is outlined for the first time. E-mails: [email protected]; [email protected]; [email protected] 10.1016/j.jhse.2007.02.078
A-0200 2.1 THUMB TRIPLICATION; A RARITY?
S. Hovius, M. Ananta and J. Zuidam From the Department of Plastic and Reconstructive Surgery and Hand Surgery, Erasmus MC, University Medical Center, Rotterdam, The Netherlands Background: Radial polydactyly is a common form of a congenital hand anomaly; however, more complex forms like triplication of the thumb are supposed to be rare. We encountered an unusually high number of triplicated thumbs at our unit. Is triplication of the thumb indeed a rare condition and is it treated differently? Methods: In our series from 1993 till 2006 112 patients with radial polydactyly were recorded. In nine subjects (8%) of this Group, triplicated thumbs either unilateral (seven) or bilateral (two) were identified. Other associated congenital upper limb deformities were syndactyly, ulnar polydactyly, dislocation of the radial head and triphalangeal thumb. Results: A total of 11 (9%) triplicated thumbs are demonstrated, all cases were combined with triphalangeal components. Only one of these in total eleven triplicated thumbs could be classified according to currently most used classifications. In all cases aberrant rays were excised, thumb length and alignment restored by osteotomies, joints were stabilised, tendons reinserted and nails and nail walls corrected if necessary. Also in all cases a correction of triphalangeal components was performed. Discussion: In the literature triplications are a rarity, presented by single case reports. In our series, triplicated thumbs are not so rare probably because in the southwest region of the Netherlands a genetic isolate, a deformity located at chromosome 7q36, has been identified with radial polydactyly and triphalangeal thumbs. The molecular basis of this deformity is the up-regulation of Sonic Hedge Hog (SHH) leading to ectopic expression at the anterior margin of the growing limb bud, resulting in radial polydactyly. Treatment for the presented triplicated thumbs was based on the same general principles as for less complex forms of radial polydactyly, in essence to assemble useful elements of the separate (partial) thumbs to reconstruct one functioning, stable thumb. 10.1016/j.jhse.2007.02.077
THE JOURNAL OF HAND SURGERY VOL. 32E SUPPLEMENT 1
A-0092 2.2 AUTOLOGOUS CELL CULTURES IN THE SURGICAL MANAGEMENT OF THE HAND IN DYSTROPHIC EPIDERMOLYSIS BULLOSA
E. Betsi, M. Benathan and W. Raffoul From the Department of Plastic and Reconstructive Surgery, University Hospital of Lausanne, Switzerland Background: Epidermolysis Bullosa (EB) is a diverse group of genodermatoses and can be classified into three major categories: the simplex, the junctional and the dystrophic forms. Aim: The purpose of this presentation is to demonstrate the use of autologous cultured cells in treating an advanced cocoon formation of the hand and an extended squamous cell carcinoma (SCC) of the lower limb in a patient with recessive dystrophic EB. The preparation of these cells and their application in the operation room is being described. Method: The hands in these patients are subject to repeated erosions and blisters due to frequent minimal mechanical traumas. Cocoon formation of the hand is well known to arise as a complication in Recessive Dystrophic Epidermolysis Bullosa (RDEB) patients. A number of surgical approaches have been described to correct these deformities and improve function. We propose a new therapeutic approach of treating loss of motion and independent digital function in a patient with RDEB using autologous culture cells. Results: We managed to expand the interspace between the thumb and the rest of the digits and to create space between the fingers and the underlying palm, resulting in the hand becoming functional. We covered the cutaneous defect with homologous keratinocytes and fibroblasts. Furthermore, we treated the lower limb by surgical excision achieving clear margins and we covered the wound with homologous keratinocytes and fibroblasts. Conclusion: Many studies have been carried out concerning the surgical management in RDEB. The use of autologous keratinocytes and fibroblasts in Epidermolysis Bullosa is outlined for the first time. E-mails: [email protected]; [email protected]; [email protected] 10.1016/j.jhse.2007.02.078