Experience in the surgical management of the hand in Dystrophic Epidermolysis Bullosa

Extracutaneous manifestations and complications of inherited epidermolysis bullosa

“…The largest published cohort-other than that of the National Epidermolysis Bullosa Registry (NEBR)-represents the experience at St Thomas' Hospital in London over a 10-year period (1981-1990) in 45 patients, based on surgical experience in repairing hand deformities. 11 The same surgeons also reported on eight surgical procedures on the feet of dystrophic EB (DEB) patients. 12 Among the NEBR study population, pseudosyndactyly of the hands was most commonly observed in RDEB (41.2% of patients with the inversa subtype of RDEB [RDEB-I] and 95.0% of patients with RDEB-HS).…”

Limited Hand Surgery in Epidermolysis Bullosa

“…The abduction and extension of the fingers is then maintained by Kirshner wires. Others have suggested the continuous use of acrylic gloves for three months following surgery (Terrill et al, 1992). Today modern splints from thermplastic materials are recommended, that may be easily remodeled or discarded after they become polluted.…”

“…In this group, 5 or more operations are presumed to be the norm (Fine et al, 2005). It is estimated that recurrences appear, on average, every 2.4 years (1 to 5 years) (Terrill et al, 1992). In the experience of many surgeons, this time interval is even shorter.…”

“…Some authors introduce precise angle measurements, describing the extension and flexion deficits in the metacarpophalangeal (MCP) and interphalangeal (IP) joints (Terrill et al, 1992). Based on similar criteria, precise indications for surgery have been proposed (Ciccarelli et al, 1995).…”

Novel and emerging therapies in the treatment of recessive dystrophic epidermolysis bullosa

“…The rationale for their use, successful in releasing the contractures and separating the fingers, although recurrence of pseudosyndactyly typically occurs. Skin grafting is often required and post-surgical splinting to minimise the speed of recurrence is challenging (26,28).…”