Artikel
Resolution of the pseudo-syndactyly and hand "bag" in patients with epidermolysis bullosa (EB)
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Veröffentlicht: | 27. September 2011 |
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Introduction: Epidermolysis bullosa (EB) is a rare congenital disorder with blistering skin caused by minimal trauma. The hand, exposed to repeated microtraumatic, is one of the bodies "target". Authors present the experience in treatment of pseudo-syndactyly and "hand bag" with microsurgical technique and advanced device.
Material and Methods: From January 2001-2010, were treated 21 patients (12M, 9F), mean 14.5 years (3-46). Under local anesthesia and sedation, short-term profilaxis, analgesia post-op, 51 hands treated with opening of interdigital spaces preserving vessels and nerves, allowing coverage of the nervous-vascular pedicle with vascularized connective-adipose local flaps. Immobilization: splint with elastic rebound until 2008 (16 cases) and Kirshner in remaining 5 cases. In recent cases (4 hands) model of "glove" with dermal inducer device (Integra ®), with more fast and efficient re-epithelialization in absence of spontaneous pain. Average hospitalization was twenty-two days (first series), four days (last series). Removing glove and Kirshner: 28-32 days.
Results: No major complications. Mean “free interval”: 2.5 to 4.6 years. Intregra® reduces postoperative pain, providing a faster recovery by the fourth week. Follow-up of 48 months shows partial recovery of the wrist-hand (MF> 40 °, IP> 25 °, ID> 10 °), resolution of pseudo-syndactyly of long fingers, opening of first interdigital space (> 60 °).
Conclusion: Authors believe that the protocol developed has the following advantages: it reduces risk of iatrogenic injury to the nervous-vascular pedicle, protects joints from digital ankylosis, reduces the hospital stay, improve patient compliance, allows long-term medications, allows a durable functional recovery.