From: Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa
Key D = theoretical/foundational Quality of evidence: 1: systematic review with high bias risk, 3 = non-analytic studies, case reports, case series 4 = expert opinion ✓ = recommended best practice based on the clinical experience of the guideline development group Section 5A | |||
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Outcome/recommendation | Recommendation strength | Quality of evidence | Key references |
Hand surgery improves hand function for all adults and children, therefore worth doing for individuals (degree may be less or limited) | D✓ | 3 | |
Hand surgery with adherence to appropriate hand therapy can improve function and aesthetic appearance of DEB hands | D✓ | 3 | |
Most surgeons prefer to operate for first time before 11 years of age, i.e. before secondary changes of tendons and joints have developed | D✓ | 4 | |
Bilateral hand surgery is possible, effect on independence must be considered and long surgical, anaesthetic, hand therapy time. In some circumstances it may be appropriate, if requested, individuals need to understand they will not be independent for several months Unilateral procedure is recommended because of avoiding loss of independence Operating on both hands simultaneously gets the process over more quickly Can be done by two teams Avoids long travel for each operative session Lowers the risks of anaesthetic complications (one intubation v/s two) | D✓ | 4 | |
Hand surgery is complex, takes a long time. Requires a large, experienced team, has cost implications | D✓ | 4 | |
Sparing approach (i.e. opening only first web space) recommended in: Severe deformity (Glicenstein 4), Secondary joint and bone changes in II-V fingers, Poor medical home care for “overseas” individuals/or low adherence with postoperative hand therapy | D✓ | 4 |