Epidermolysis bullosa (EB) comprises a group of illnesses genetically determined and characterized by the presence of bullous skin lesions which develop spontaneously or after even slight trauma. Epidermolysys bullosa dystrophica (EBD) has variations with autosomal recessive (EBDR) and variations with autosomal dominant transmission, but in both types of transmission the bullous lesions heal leaving scarring. Recessive epidermolysis bullosa is more severe and is the cause of serious deformities of the hand, leading to functional impairment. Since surgical treatment of scarring of the hands cannot alter the course of the disease, its purpose is to improve functioning by restoring grasp and pincer movement and to delay relapse of the deformity for as long as possible. Many authors have already reported that a decrease in recurrences is somehow dependent on treatment of the skin and hand and the use of braces. This work describes a new protocol for the surgical treatment of deformity of the hand in epidermolysis bullosa dystrophica. Over the period from 1990 to 1999, 18 patients affected by EBD were treated. Mean age was 6 years and 9 months and in 60% of cases both hands were treated simultaneously. Intra- and postoperative splinting of the thumb and long fingers was not rigidly stabilized with Kirschner's wires; rather a new dynamic device was utilized consisting of a plastic, horseshoe-shaped frame designed to provide tension by means of calibratable elastic bands fixed to the distal phalanx with intraosseous metal clips. This splint keeps the hand open and enaples a gradual increase in joint extension during the postoperative period. All these young patients and their parents were satisfied by the operation and were in all cases informed of the fact that the underlying skin disease remained unaltered by surgery and that recurrences were consequently inevitable. As regards functioning, the mean time interval elapsing before recurrence was 42 months. To conclude, our results show that an aggressive approach to the pathology, with adequate intra- and postoperative rehabilitation permits good functional recovery of the hand and a satisfactory delay in relapse.
|Translated title of the contribution||Surgical treatment of deformity of the hand in recessie-type epidermolysis bullosa dystrophica|
|Number of pages||5|
|Journal||Rivista Italiana di Chirurgia Plastica|
|Publication status||Published - 1999|
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