Mr Aresh Hashemi-Nejad and Mr Ivor Vanhegan, Royal National Orthopaedic Hospital, Stanmore
Please note: The following text cannot and should not replace advice from the patient’s healthcare professional(s). Any person who experiences symptoms or feels that something may be wrong should seek individual, professional help for evaluation and/or treatment. This sheet is for information only and is not intended to provide individual medical advice.
The information included in this sheet relates to EDS hypermobility type (EDS-HT) only. For information about any other types of EDS please see the respective information sheet(s).
Surgery in patients with EDS should be approached after appropriate conservative treatment has been exhausted. The mainstay of treatment for EDS remains physiotherapy to strengthen muscles and stabilise the joints. Patients learn techniques to protect their joints, particularly avoiding pushing their joints past the normal range of movement which may cause long term traumatic arthritis.
However, occasionally surgery is needed to treat fractures or, indeed, joint problems. Soft tissue surgery on its own only has short-term relief as the soft tissues can stretch and the problem may recur.
Quite often surgery of the soft tissue is combined with bone surgery and in certain circumstances arthritic joints maybe stiffened or, indeed, replaced. The type of surgery that is offered can be arthrodesis, which is stiffening of the joint using internal metal work. Joint replacements are performed when there is quite a lot of arthritis which is painful and the aim of the surgery is to prevent pain relief and regain function. Care regarding stability of the joint needs to be taken with measures which will be discussed with the surgeon and the physiotherapist.
When approaching the idea of surgery this should be done in full consultation with the patient and other medical and para-medical staff. Issues that need to be addressed pre-operatively include pain management, assessment of cardiac, lung and possible bleeding disorders. A scan of the heart (‘echocardiogram’) is advisable in all patients as aortic root dilatation has been reported in 33% of patients suffering from classic-type EDS. Antibiotics may also be required if a floppy heart valve is found as this can increase the risk of infection settling on the valve. With regards lung problems, EDS patients might have a restriction in lung capacity if a spinal curvature is present, there may be fragility of the lining of the mouth and upper airway, as well as an increased risk of lung collapse. Patients must also be counselled on the increased risk of poor scar appearance as delayed wound healing may result in widened or atrophic scars.
At the time of surgery attention should be made when transferring and positioning the patient on the operating table to protect the soft tissues and nerves. Excessive bruising can be seen with intramuscular injections and we would discourage their use in EDS patients. Particular attention should be paid when the patient’s limbs are elevated e.g. to apply a tourniquet so as to avoid injury or dislocation of the joint. During operations the surgeon must be respectful of the fragility of the skin and soft tissues, limiting unnecessary retraction or trauma to these structures. As mentioned, attention also needs to be paid to closure of wounds with a multilayered method suggested to reduce the tension on the skin edges and improve wound healing and scar appearance. Wounds should preferably be closed with subcuticular stitches and use of steristrips which should remain undisturbed for longer than usual to allow for the delay in healing time. Bleeding can also be a problem in patients and occasionally surgeons do allow extra operating time to account for this.
Bone fixation should be rigid with appropriate internal metal work and subsequent removal of metal work should be discussed to see if it is necessary and whether it will actually alter the long term outcome if the metal work is left in situ.
Pain control should be discussed pre-operatively with the patient and anaesthetist and the surgeon. We find that patients with EDS usually have a good idea of what works in terms of pain relief. Local anaesthetics do not work for all patients with EDS. A history of whether they have had successful anaesthetic blocks at the dentist will tell you whether the use of local anaesthetic would be successful. Doubt has been cast over the use of epidural anaesthetic techniques because of the increased risk of bleeding but these have been used safely and effectively in EDS patients during childbirth.
Occasionally intravenous ﬂuids may not ﬂow normally with EDS patients and IV pumps may be needed.
Most importantly the surgery is only successful if proper rehabilitation is undertaken. This needs to be individualised with less aggressive range of motion than standard protocols to avoid stretching of repaired or reconstructed tissue, as stiffness does not typically develop in these patients. It is also advisable to avoid excessive ‘open chain’ exercises where the affected limb is not in contact with the ground at all times. This can be a difficult issue as sometimes resources are not available for an intensive rehabilitation programme. Patients with EDS can initially deteriorate following surgery, particularly if advice has been given to spend time on crutches, and they should be encouraged to continue with the rehab to get the full benefit from surgical intervention.
Complication rates following surgery in EDS patients have been reported to be higher than in the general population and surgical intervention should be considered only as a last resort. This specialist care should be approached in a multi-disciplinary fashion addressing the issues pre-operatively, peri-operatively and post-operatively. Results can be good if the goals and expectations are discussed pre-operatively and a co-operative approach is undertaken to achieve the best outcome
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Peer reviewed by:
Dr Hanadi Kazkaz- Rheumatologist at University College London Hospital
Date of last review: April 2016
Date of next review: April 2019
The views expressed are those of the author(s) and should not be construed to represent the opinions or policy of the Ehlers-Danlos Support UK or its trustees