Managing complexity in a rare condition: A single case report of novel forearm tendon transfers for Inclusion Body Myositis

Purpose

Inclusion body myositis (IBM) is an acquired, inflammatory myopathy presenting in the over 50´s. Characterised with progressive muscle weakness and atrophy in the quadriceps and long finger flexors. Currently, this complex health problem, that has a prevalence of 5-10 per million, does not have an effective treatment or cure, therefore forearm tendon transfers provide a viable option to address finger weakness in suitable patients. The marked finger flexor weakness poses a significant limitation to patients´ quality of life and functional abilities.

This case demonstrates how physiotherapists can be pivotal in managing complex and challenging conditions through multi-disciplinary team (MDT) working, demonstrating how our roles evolve in response to complex cases.

Approach

This is a single case study presenting forearm extensor to flexor tendon transfers in a 56 year old male with IBM. To our knowledge, this is the fourth time such an operation has been carried out on a patient with IBM in the UK. The gentleman was seen pre-operatively and post-operatively with primary outcomes taken at 12 weeks post-operatively. The quickDASH (disabilities of arm, shoulder and hand), strength testing and 9-hole peg test were used as outcome measures. The 9-hole peg test, is a brief, standardized, quantitative test of finger dexterity, chosen due to the larger peg size for the patient to be able to handle.

Outcomes

The operation involved transferring extensor carpi radialis longus (ECRL) to flexor pollicis longus (FPL) and extensor carpi ulnaris (ECU) to animate a mass flexor action to flexor digitorum profundus (FDP) of all four fingers in the non-dominant hand. Both hands were equally affected by flexor weakness pre-operatively.

Pre-operatively the quickDASH score was 61; at 12-weeks post-operatively it reduced to 48. The quickDASH is a reliable and validated outcome where a lowering of the score is favourable and an 8 point change is considered clinically important. While the 9-hole peg test pre-operatively was 64.9 seconds, post-operatively it was quicker at 45.8 seconds. Pinch and grip testing pre-operatively were 0Kgs, post-operatively pinch was 0.5Kgs and grip was 1Kg.

The patient stated that the surgery has been "life-changing" with improved quality of life, he would recommend the surgery and he has the same surgery planned for the dominant hand. However, it is not possible to predict how long lasting the functional improvements will last in this progressive disorder. The case demonstrates that MDT working (hand and neuro-muscular physiotherapists, neurologist and hand surgeon) is key to managing patients with complex conditions. Treatment of disabling finger flexor weakness with tendon transfers can be a very effective option in the treatment of some patients with IBM.

Implications

Reporting this novel case aims to raise awareness amongst the physiotherapy profession of an option to help manage the functional hand loss seen in IBM, when the rarity of the condition will not enable a clinical trial. MDT working pre-operatively, gaining an understanding of the patients’ current physical state and needs are key to planning such an intervention and providing the specialist hand therapy post-operatively.

Top three learning points

  1. Detailed, tailored and specific pre-operative multi-disciplinary planning with the patient at the centre is pivotal and fundamental to achieving the common goal of functional improvement in these complex clinical cases.
  2. Sharing knowledge and our experience of the rare condition, surgery, rehabilitation and outcomes has encouraged other staff to publish their work, setting an example to peers of the positive impact that contributing to conferences has.
  3. Having the confidence to submit an abstract (my first), then designing the poster, to representing my Trust with the poster at conference has been a huge positive learning curve and has continued to provide teaching/learning opportunities to continue my own professional development.

Fund acknowledgements

This work was unfunded. 

Additional notes

This work was presented at Physiotherapy UK 2019

Please see the attached Innovations poster below. 

 

For further information about this work please contact Karen Belward.