Intended for healthcare professionals

Rapid response to:

Practice Clinical Updates

Ehlers-Danlos syndromes

BMJ 2019; 366 doi: https://doi.org/10.1136/bmj.l4966 (Published 18 September 2019) Cite this as: BMJ 2019;366:l4966

Rapid Response:

A Call for Evidence Based Research in Wound Closure in Ehlers-Danlos Syndrome

Dear Editor,

We thank the authors for raising awareness of this group of conditions and the issues faced by patients. However, we would like to emphasise the importance of identifying gaps in our knowledge and the need to address them with robust good quality research that will help both clinicians and those who have Ehlers-Danlos Syndrome (EDS).

The authors recommend access to plastic surgery for specialist suturing of wounds. However, there is a dearth of evidence examining best practice in wound closure techniques in this subset of patients suggesting that even plastic surgeons may find such cases challenging [1-4]. This may be particularly relevant for those requiring emergency surgery.

Case reports in the literature cite a mixture of practices including combining suturing with steri-strips [1], collagen dressings [2] and layered closure [3]. Anecdotal evidence supports meticulous care in handling tissues, and if non-absorbable skin sutures are used, they should be left longer than usual [3]. There is very little knowledge of long-term outcomes, wound healing times, complications or the impact of different approaches on people with EDS [5]. This highlights a significant evidence gap for EDS patients who sustain injuries or undergo elective surgery. Without this evidence it is difficult to advise patients and clinicians on best practice.

We would encourage those involved in the care of people with EDS to address this knowledge with well-structured research and long-term data to share with the wider community so that patients can receive care based on best evidence.

References:
1. Shaharan, S., et al., Using the suture/adhesive strips combination technique for skin closure in an individual with Ehlers-Danlos Syndrome. JPRAS Open, 2019. 19: p. 82-84.
2. Baik, B.S., et al., Treatment of the wide open wound in the Ehlers-Danlos syndrome. Arch Craniofac Surg, 2019. 20(2): p. 130-133.
3. Whitaker, I.S., et al., Molecular genetic and clinical review of Ehlers-Danlos Type VIIA: implications for management by the plastic surgeon in a multidisciplinary setting. J Plast Reconstr Aesthet Surg, 2009. 62(5): p. 589-94.
4. Kumar, P., et al., Wound healing and skin grafting in ehlers-danlos syndrome. Plast Reconstr Surg, 2010. 126(4): p. 214e-215e.
5. Gupta, A. and P. Kumar, Possible simple measures for complex wound healing problems in ehlers-danlos syndrome. Plast Reconstr Surg Glob Open, 2014. 2(10): p. e241.

Competing interests: No competing interests

24 June 2020
Nikita Joji
Plastic Surgery Registrar
Mr Baljit Dheansa, Consultant Plastic Surgeon
Queen Victoria Hospital
East Grinstead, Holtye Road, East Grinstead RH19 3DZ