Microsurgical Release of Pseudosyndactyly in Patients Affected by Epidermolisis Bullosa

Epidermolisis Bullosa (E.B.) is a rare congenital condition characterized by extremely fragile skin and recurrent blister formation subsequent to minor trauma. Main forms are epidermolytic EB, junctional EB, and dermolytic EB. Patients reach the third decade of life and exitus is related to the cachectic state related to multi-organ failure.

From January 2001 to May 2005, 13 patients were treated (7M, 6F). Patient ages ranged from 10–40 years. Nineteen hands were operated on electively, with local anesthesia and sedation, and with microsurgical technique: blunt releasing of adherences with scissors and/or bipolar radiofrequency under loop magnification, and close to the neurovascular pedicles under the microscope. The finger pedicles in the web spaces were covered by local subcutaneous flaps, avoiding exposure in the first web spaces (5 cases) and in other web spaces (3 cases). Four hands were repaired with dermo-epidermal grafts, 13 hands with only Vaseline gauze dressings and emollient ointments. All patients underwent perioperative short-term prophylaxis. The median hospital stay was 3 days. In the first postoperative week, occlusive dressings in extension with a splint were used; in the second postoperative week, packaging of the splint, and in the third week, active and passive mobilization.

The postoperative course was uneventful. The taking rate of the only dermal component of the split-thickness skin graft was 60%. Wound healing occurred on average in 3 weeks (2 to 5 weeks). Before the operation, wrists were locked in flexion (30° to 90°), fingers were flexed and enveloped in a skin bag. After the operation, the pseudosyndactyly was corrected; wrists were from 20° extension to 65° flexion; the MCP range of the fingers was 40°, the PIP range 25°, the DIP range 10°.

Experience suggested opening the digitals spaces, and use of dressings only with ointments and Vaseline gauze. Loops and/or the microscope were mandatory to preserve nerves and vessels in pseudosyndactyly. Advantages of the protocol are that operative and postoperative anesthesiological risks were reduced; joints were protected from anchilosis due to non-immobilization (dynamic splintage); the hospital stay was reduced with better compliance of patient and parents; the risk of iatrogenic lesions was eliminated. There was the possibility of performing several surgical treatments for small corrections. The authors observed a durable partial functional recovery of the hand (follow-up: 1 to 4 years; mean: 1.8 years).