A new protocol for the treatment of hand deformities in recessive dystrophic epidermolysis bullosa (13 cases).

The recessive form of dystrophic epidermolysis bullosa creates severe hand deformities with disabling functional limitations in the main daily activities. Typically, the thumb is contracted in adduction, the first web space is obliterated, the palm and digits are contracted in flexion and interdigital spaces are lost (pseudo-syndactyly). In this paper, we present our experience with a protocol based on the association of various internationally developed techniques: brachial plexus anesthesia with ketamine sedation, dynamic splinting and coverage of the wounds with allogenic keratinocytes sheets. The overall results obtained in the first 13 patients showed a good tolerance of the procedure, no anesthesiologic complication and marked improvement of the hand deformities. The long-term follow-up revealed a recurrence before 2 years in 2 hands, between 2 and 4 years in 7 hands and after 4 years in 6 hands. The conclusion is that an aggressive surgical attitude, along with an adequate intra and post-operative rehabilitation, ensures a good restoration of hand function and a satisfying delay of inevitable recurrence.

[Ear prostheses in burns of the external ear. Technical notes]. / Les épithèses d'oreille dans les brûlures du pavillon auriculaire. Notes techniques.

Ear reconstruction is best performed with autologous tissue. However, there are selected cases in which a prosthesis may be preferred. Some patients are unwilling to undertake multiple surgical procedures, others do not accept the chest wall scar. More importantly, in severe post burn cases, the scars in the periauricular region can truly compromise the outcome of an autologous reconstruction. In such cases, the authors perform a prosthetic reconstruction which is anchored to the cranial bone by means of osteointegrated titanium screws. The method described here has been modified compared to the original Bränemark system. A new microscrew design allows the implants to be inserted in a single surgical procedure. A magnetic anchoring system avoids cumbersome external rods, and the overall size of the masses emerging from the skin is significantly reduced. These improvements increase patient comfort and compliance.