The Adult hand and EB Rachel Box Clinical Specialist St Thomas Hospital

The Adult hand and EB Rachel Box Clinical Specialist St Thomas Hospital

All types of EB affect the Hand. Patients treated often have Junctional, Dystrophic or Recessive Dystrophic EB. Skin, mucosal linings of body blister abnormally easily, effects function of hands as causes blistering, scarring, contractures that result in deformities. From birth hands are soon subjected to a destructive cycle.

Each episode of relatively minor trauma to the hand: ulceration produces fibronous adhesions scarring,web spaces obliterated progressing to finger tips causing pseudosyndactyly. process also occurs in the first web space initially causing an adduction contracture until the thumb is no longer independent

All structures of the hand may be affected Cutaneous involvement results in: Dermal fibrosis Pseudosyndactyly Contractures Atrophic finger and thumb tips Nail loss due to subungual blistering Dermal cocooning

Musculotendinous involvement: Shortening of the flexor tendons Intrinsic muscle contractures With time lack of use ipj and mcpj develop flexion deformities Initially despite pain patients may be able to separate fingers using thread or paper

If left untreated the resulting pseudosyndactyly, together with trauma to and scarring of the flexion creases produces flexion contractures at the joints Finally the whole hand may become encased in an epithelial cocoon

Articular involvement produces: Stiff, subluxed or even destroyed joints in the older patients Generalised osteoporosis and thinned wedge shaped distal phalanges may be found

Wrists Recurrent flexion deformity at the wrist occurs for several reasons Stronger pull of flexor tendons compared with extensor tendons, powerful FCU and complex carpal bone movements that favour ulnar deviation in wrist flexion.

In early wrist flexion contractures which are often ulnarly deviated simple division of scarred skin will produce full release of the wrist

In more advanced contractures division of PL and FCR may be necessary In the most extreme and neglected cases the wrist joint is also involved and may need to be fused in a neutral position.

In some cases the potential amount of surgery may be too daunting and the individual may have learned to function so well that the surgeon is unable to guarantee an improved predictable outcome

Typical hand deformities in recessive DEB (RDEB) particularly where there is reduced or absent collagen V11, include: Adduction contractures of thumb Pseudosyndactyly of the digits Flexion contractures of ip & mcpj s & wrist joints.

Those with RDEB have the greatest degree of blistering, ulceration and deformity.

The risk of the hand developing a mitten deformity is 98% in RDEB by the age of 20.

Aim of surgery is to: Provide simple pinch grip and grasp by releasing the first web space and flexion contractures Independent finger movement by releasing pseudosyndactyly Improved appearance of the hand

Multidisciplinary Team Surgery is planned and discussed with the whole team Allowing condition to be optimised and admission planned Wounds are swabbed preoperatively and active infection treated

Growth of streptococcus is a contraindication to hand surgery Therapist spends time with patient discussing post operative treatment

Surgery is indicated when loss of hand function starts to compromise independence But cosmetic appearance also important

Surgery should be performed when individuals skin, medical and nutritional condition have been optimized and at a time when they will be able to attend regular post operative hand therapy sessions

Surgery needs careful planning whether to separate thumb from digits how to cover soft tissue defects, what type of anaesthetic

Most surgeons release all contractures and affected joints of hand in one procedure thumb contributes to 50% of hand function release of the first web space produces significant improvement can be useful if surgeon or patient wishes to limit extent of surgery

K-wires Often used when the ips are stiff Surgeon prefers not to use k-wires over pipj s unless absolutely necessary to avoid complications of prolonged postoperative joint stiffness in extension

potential pin site infection and damage to articular cartiledge. Furthermore forced extension to the ip joints may cause injury to the neurovascular bundles or subluxation of the joints.

Full release of dipjs may not be possible or useful Shortening of neurovascular structures is often the limiting factor in release of flexion contractures

Soft tissue cover Surgeon covers lateral defects of fingers after release of pseudosyndactytly. In adults graft these areas as denuded defects take longer to reepithelialise and most patients do not tolerate prolonged wounds on their hands

The grafts are held in place with tissue glue or absorbable sutures. The grafts are then covered with Vaseline gauze and Proflavine impregnated wool this helps to maintain abduction and extension of web spaces and digits. Splinted for the first few weeks using POP

Patients return to theatre for change of dressings at 1 and 2 weeks, wounds and grafts are carefully cleaned and redressed

Approximately five days or three weeks after surgery patients are seen in Hand Therapy

Treatment involves: Advice/education Wound care Splint fabrication Exercise prescription Measuring for gloves.

Wound care Remove post operative dressings Wounds reviewed and cleaned Redressing wounds ensuring that the web spaces are maintained

Thermoplastic splints Fabricated at initial appointment Advantages: Digits supported individually Adjustable Can be cleaned Can be lined Material is perforated

Splints need to be worn at for as long as possible Regular Splint review is essential

Web Spaces These must be maintained to try to prevent recurrence of pseudosyndactyly Achieved by: Dressings/bandages Wearing rings Interim gloves Neoprene sleeves Putty splints

Neoprene sleeve

Putty Splint

Range of Movement Exercise regime: Tendon gliding Passive extension and abduction Block and bend

Function Return to full use of hand at eight weeks post surgery

Main complications after surgery are: Joint stiffness in extension particularly if wires used Graft failure Infection

Outcome dependent on: Close collaboration between therapist and patient to maintain surgical results

Long term results are affected not only by the natural course of the disease but also by failure to wear splints long term and to maintain web spaces.

On average recurrence of contractures occur with in 2-5 years. The first web space has the poorest outcome as it can be difficult to splint and it is used in an adducted position.