A recurrence-tolerant strategy for epidermolysis bullosa-related pseudosyndactyly: A microscope-assisted minimally invasive approach

Abstract

Complete long-term prevention of recurrence in epidermolysis bullosa (EB)-related pseudosyndactyly remains challenging. We report a case applied a minimally invasive, recurrence-tolerant surgical strategy that allows repeatable intervention with reduced patient burden. An 8-year-old boy with recessive dystrophic EB (RDEB) presented with advanced pseudosyndactyly. The patient and his mother expressed concerns regarding pain associated with treatment and postoperative care, as well as the need for repeated surgeries due to recurrence. Based on these concerns, a less invasive intervention was planned, focusing on selective and minimal release of scar tissue using microsurgical techniques. Under general anesthesia, the epidermis and firm dermal layer were carefully incised using a microscope. Microsurgical perivascular dissection enabled additional release of contracture at the digital joints. A previously cryopreserved cultured epidermal autograft was applied to cover the wound surface, followed by non-adhesive dressing. The graft was secured with a tie-over dressing using a bandage, without pin fixation. The patient was discharged on the day after surgery. Engraftment of the cultured epidermis was confirmed on postoperative day 4. Complete epithelialization was achieved within 3 weeks, after which rehabilitation was initiated. At 6 months postoperatively, no recurrent contracture was observed, and the improved range of motion was maintained. Given the potential risk of recurrence in EB-related pseudosyndactyly, our surgical concept prioritizes repeatability in the event of contracture recurrence. The use of microsurgical techniques allowed real-time identification of the boundary between firm and soft dermal layers, achieving short-term functional improvement without recurrence.