Rehabilitación protésica implanto-soportada en un paciente con epidermolisis ampollar distrófica recesiva. 4 años de seguimiento clínico / Implant-supported dental prostheses in a patient with recessive dystrophic epidermolisis bullosa. Four-year followup

Se describe la evolución de una paciente con diagnóstico molecular de Epidermolisis Ampollar Distrófica Recesiva, quien a los 35 años de edad inicia una rehabilitación bucal mediante prótesis fija implanto-soportada, luego de haber realizado las exodoncias de las piezas remanentes en boca. Todos los implantes se mantuvieron oseointegrados a lo largo de cuatro años de seguimiento clínico y no se encontró flora periodontopática peri-implantar en los controles microbiológicos realizados. Este tipo de rehabilitación es un caso paradigmático para personas con este tipo de genodermatosis, constituyéndose en una posibilidad válida para una mejor calidad de vida frente a las circunstancias adversas que plantea la enfermedad. (AU)

Oral rehabilitation of a 35-years old female patient with Recessive Dystrophic Epidermolisis Bullosa is described. After removal of all remaining teeth, cemented implantasisted prosthesis with shortened arches were installed over three implants in the mandible and four implants in the upper jaw. At the four- year follow up, all the implants conserved osseointegration as well as absence of peri-implantar periodontopatic biofilm was observed. The success achieved in this case becomes a paradigmatic landmark for the oral rehabilitation of patients with such genodermatoses, providing a new resource to improve their quality of life. (AU)

Implants placed simultaneously with particulated bone graft in patients diagnosed with recessive dystrophic epidermolysis bullosa.

PURPOSE: To describe the rehabilitation with implants placed simultaneously with particulated bone graft in 4 patients diagnosed with recessive dystrophic epidermolysis bullosa. MATERIALS AND METHODS: A retrospective study was conducted of 4 patients diagnosed with recessive dystrophic epidermolysis bullosa and treated with dental implants and simultaneous particulate bone graft from January 2005 to December 2009. All patients had marked oral involvement, with devastating alterations in the soft and hard tissues and were rehabilitated with a fixed prosthesis. RESULTS: Eighteen implants showed dehiscence or fenestration and were placed simultaneously with particulated bone grafts to cover exposed threads: 14 received autologous bone and 4 tricalcium betaphosphate. In 16, the bone graft was covered with resorbable collagen membranes and in 2 with a nonresorbable titanium-reinforced membrane. Of the 18 implants, 8 were placed in the maxilla combining drills and osteotomes and 10 in the mandible with the conventional drilling procedure. All implants survived after a minimum follow-up of 12 months (range 12 to 48). CONCLUSIONS: The results of this small-sample clinical study suggest that endosseous implants can be placed simultaneously with particulated bone graft, providing support for a fixed prosthesis in patients with recessive dystrophic epidermolysis bullosa and considerably improving these patients' quality of life.

[Hand management in recessive dystrophic bullous epydermolysis]. / Il trattamento della mano nel paziente affetto da epidermolisi bollosa distrofica recessiva.

PURPOSE: Recessive distrofic epidermolysis bullosa creates severe hand deformities with disabling functional limitations. Hand surgeon should perform surgery when deformity inibits function, in order to restore the pinch. MATERIALS AND METHOD: We present our experience on 44 patients and 58 operated hands, with the following schema: hand degloving, grafting of the first web and intraoperative dynamic splinting. RESULTS: In 30 patient with an 8 years follow up, 25 had had good or excellent results, and the 5 remaining shows early recurrence. CONCLUSION: Association of a correct surgical approach and adequate intra and post-operative rehabilitation improve hand function and a slow down inevitable recurrence.

Postoperative hand treatment in children with recessive dystrophic epidermolysis bullosa.

The purpose of this study is to give an overview of the postoperative hand treatment options in children with recessive dystrophic epidermolysis bullosa (EB) and to introduce a treatment protocol and discuss the indications and timing. Recessive dystrophic EB is a rare hereditary blistering skin condition, which leads to severe hand deformities. The aim of surgical intervention is to temporarily increase hand function and delay the recurrence of deformation. The aim of postoperative treatment is to maintain optimal range of motion of the wrist, fingers, and thumb and to delay recurrence of deformity to enlarge the possibilities of hand function. Two postoperative treatment programs are described in the literature: a program with static splinting and a program with dynamic splinting. Both splinting programs include exercises. This postoperative treatment program for EB starts with dynamic splinting, followed by static splinting in combination with exercises.

Surgical treatment and postoperative splinting of recessive dystrophic epidermolysis bullosa.

Digital contractures and pseudosyndactyly, common manifestations in recessive dystrophic epidermolysis bullosa, cause significant functional impairment. The deformities progress with time, although surgery may delay the progression. The role of surgical intervention, hand therapy, and the use of prolonged splinting was examined in seven children (nine hands) with recessive dystrophic epidermolysis bullosa with an average age of 5 years, 8 months (range, 1 year, 10 months to 16 years, 4 months). The technique of surgery, postoperative regimen, and splinting differ from those previously reported. Surgery includes "de-cocooning" the hand and fingers, manipulating contracted joints, and full-thickness skin grafting to dermal defects. Surgery and the postoperative regimen of rigid night splints and web-retaining gloves for day wear has allowed arrest or minimal progression of contractures in complaint patients in short-term follow-up study of an average of 17 months (range, 12-28 months). An interdisciplinary team of physicians and surgeons, therapists, and nurses makes this care regimen possible and influences family compliance.