Mr A P Barabas MD FRCS
Patients with Ehlers-Danlos Syndrome (EDS) require surgical operations more often than the average person. In childhood, there may be numerous cuts and tears of the skin necessitating trips to the Casualty Department. In old age, joint disorders are more common than in the general population. Despite this greater frequency with which patients with EDS require surgery this does not mean that they ever get used to the idea. In fact, they remain worried and apprehensive. This concern is understandable because even individuals without an inborn weakness in the connective tissues find making a decision about undergoing surgery difficult.
This fear of the unknown and possibly painful surgery is much greater in a person with Ehlers-Danlos Syndrome because he or she may have had one or two unpleasant experiences in the past. He or she may have met doctors who knew nothing about the syndrome and may have developed complications because of inexpert management. It is my intention in this short information article to reassure and to explain that operations in the EDS need not have complications and can often be beneficial.
My main message is that, provided a few sensible precautions are taken, patients with Ehlers-Danlos Syndrome can undergo the full range of surgical operations available to the general population. Surgical magazines, journals and books contain reports and descriptions of operations of all kinds, from coronary bypass to major joint replacement, which have been carried out without any undue difficulty and with good results in patients with known EDS.
It is my contention; therefore, that surgery in EDS can be safe and bring great benefits. For these happy outcomes of operations, undoubtedly, the surgeon will have to alter his operating technique to some extent when handling the tissues of an EDS patient during an operation. These small alterations of routine surgical techniques are well within the capabilities of most surgeons, because similar adjustments have to be made in the course of other, more regularly performed surgical situations. An obvious example is when operating on small newborn babies. Also in the adult patient when inflamed tissues become fragile and vulnerable, for instance the removal of an inflamed gall bladder, or the repair of tissues around a joint in rheumatoid arthritis, require a great deal of care and gentleness, much more that when operating on the same organs when they are not inflamed. Most Surgeons have to treat cases with severely inflamed tissues every week and the lesson learnt in these common situations can be employed on the rare occasions when a patient with severe Ehlers-Danlos Syndrome requires an operation.
Problems can be avoided, therefore, if the Surgeon is aware of the diagnosis. Unfortunately, this has not always been the case in the past. Patients and Doctors knew much less about this syndrome 10 or 20 years ago. The awareness of this rare syndrome was thinly distributed in this country in the past but there has been an upsurge of interest recently and I find that even medical students today seem to have heard of EDS and few qualified doctors are totally ignorant of its implications. In most patients nowadays the diagnosis of EDS is made in childhood.
Once an individual is diagnosed to have EDS he or she should always make it his or her business to make this known to any medical or surgical attendant as soon as possible. Carrying a badge or a pendant around the neck with the diagnosis clearly stated is also a good idea. Patients with EDS should make it their business to learn as much as possible about their condition and try to find out which type of the syndrome they have whenever possible.
No Surgeon, however experienced and well-read could be expected to know about every rare condition which may affect his patients, because these run into tens of thousands. He or she may meet one of these rare conditions or diseases only once in their lifetime. Patients, therefore, should familiarise themselves with their own disease as much as possible and practice a kind of self defence. They should not be shy or embarrassed to tell their doctor or surgeon all they know about EDS and even request them to find out more before treatment is started.
Knowledge about EDS has increased enormously in the past 10 and 20 years and new discoveries are made all the time. One of the most important discoveries has been the realisation that EDS is made up of different types. Nowadays it is not enough to know that a patient suffers from EDS but it is essential to also know which type of the syndrome he or she has.
The most important sub-group is the Vascular type (formerly type IV). Only in this rare sub-group is surgery exceptionally hazardous. In all other groups, Surgeons often remark after an operation that they have hardly noticed any difference from the normal. The Vascular type (formerly type IV) or arterial type is characterised by an extreme tendency to bruising. All patients with Ehlers-Danlos Syndrome bruise easily but those with Vascular type (formerly type IV) often have huge bruises which may occur quite spontaneously and may lead to hospital admissions and blood transfusions. Joint and skin in Vascular type (formerly type IV) is not as elastic or hyperextensible as in other types but bruising completely dominates the picture.
Anyone with this type of easily bruisable and often very thin transparent thin skin must insist that doctors will take the diagnosis further and try to confirm Vascular type (formerly type IV) by taking small quantities of skin for special tests. Often patients with Vascular type (formerly type IV) have no other relative in the family with the syndrome. If they have an affected relative, he or she may also have had huge haematomas and serious complications after surgery. Patients with Ehlers-Danlos Syndrome who have relatives with the same condition could learn a lot about their own future outlook because EDS seems to run true in every family and if more than one person in a family is affected they tend to have similar types with similar symptoms and complications. Any patient with an affected relative, therefore, should bring this to the notice of any medical attendant and especially try to point out any problems or complications this affected relative may have had during or after surgery.
Apart from the extent of bruising a good guide to possible problems during surgery is the amount of scarring of the skin. The greater amount of this scarring, usually the result of childhood injuries around the knees, hands and forehead, the more easily all soft tissue inside the body will tear on operation. Patients should bring this connection between the skin and internal tissues to the attention of their surgeon.
In my experience more problems arise because of reluctance on the patient’s or the surgeon’s part to embark on necessary, or even life-saving, operations because of fear of complications during or after these operations. The fear of complications and problems make patients often ask for surgical advice too late and even refuse an operation. This means that a condition requiring surgery progresses unchecked and eventually surgery may have to be carried out in a much more advanced state of the disease. Not surprisingly, surgery in advanced disease does not have the same good results as when carried out early or in good time. I would like to urge, therefore, all patients with the EDS not to shy away from an operation when it is advised by a well-informed surgeon.
I can understand the reluctance of anybody facing an operation and I know many people, even without EDS, refuse because of fear of pain and complications. Nevertheless I must emphasize that anything from major heart surgery to minor cosmetic operations, such as removal of ugly scars from around the knees, all have been performed with great success in EDS.
To give detailed advice in this short article on the whole range of possible operations in quite impossible but patients could ask any interested doctor to initiate a search of the surgical literature through a medical library and they can obtain detailed and specific advice on how to carry out any specific operations in EDS. Any surgeon who has never operated on a case of the Ehlers-Danlos Syndrome is welcome to get in touch with me and I will always try to help.
In general, I will say that surgical techniques in EDS have to be adjusted by a factor of 2 or 3. This means that every pull on the patient’s tissues, and every cut with the scalpel, will require only about half or a third of the usual force used in normal people for the same operation. I will advise the use of lighter than usual surgical instruments, such as retractors and blood vessel clamps. When stitching layers of tissues together, the ligatures must not be pulled tight but gently approximated. Stitches in all layers and especially in the skin will need to stay in place for at least twice as long as normal. This will require the use of non-absorbable or slowly absorbable stitch materials inside the body. Skin stitches should be left in place for at least twice as long as in normal situations.
Skin wounds need careful management. Most stitches should be put just under the surface of the skin (sub-cutaneous or sub-cuticular sutures). Stitches should also be reinforced or even substituted by adherent surgical tapes, such as Steristrips.
My own surgical field is general and vascular surgery and not orthopaedic surgery. This means that I only operate on the soft internal organs and blood vessels of the body and not on joints and bones. Unfortunately, most Ehlers-Danlos patients require surgery on joints and bones and I would not be able to give specific advice in this field. Nevertheless, the general principles of handling tissues gently and leaving stitches in at least twice as long as normal will apply in orthopaedic surgery as well.
Angiography is not an operation but an investigation and it carries special hazards in patient’s with EDS. Angiography means putting a needle or a cannula into a vein (venography) or an artery (arteriography) and injecting a contrast medium into the vessel so that they can be seen on an x-ray. Occasionally this is done for problems around the neck and the head and this is called carotid arteriography or angiography. Blood vessels in EDS and especially vascular type (formerly type IV) do not take kindly to needles or cannulas inserted in them and angiography may cause severe bleeding and bruising. I would strongly advise all patients and doctors not to use angiography in EDS, unless absolutely essential. Fortunately, there are numerous other ways of investigating blood vessels using so-called non-invasive imaging. For instance, an ultrasound or a CAT scan can give almost the same information about blood vessels nowadays as angiography.
Varicose veins are common in Ehlers-Danlos Syndrome and can be unsightly or even painful. Often varicose veins occur in vascular type (formerly type IV), the arterial type of Ehlers-Danlos Syndrome. Operations on varicose veins are quite easy in normal people and are often delegated to relatively junior surgeons. Surgery on varicose veins in EDS is much more hazardous and should never be undertaken by a junior doctor. My advice would be that varicose vein surgery in always undertaken by a Consultant Vascular Surgeon who has knowledge of the patients EDS and has read the relevant surgical literature. Despite the undoubted hazards of varicose vein surgery in EDS I have myself carried out numerous successful operations, even in patients with vascular type (formerly type IV), with a great deal of relief and benefit to the individuals concerned.
Abdominal pains (tummy pains) are common and again the vascular type (formerly type IV) is more often the sufferer. Some of these pains are due to common conditions such as appendicitis, and should be treated surgically just as if the patient had no EDS. There are a number of abdominal pains, however, which are not due to inflammation, such as appendicitis, but are due to internal bruises. I would, therefore, advise that patients with abdominal pains always inform Surgeons that they have EDS and the possibility of a large internal bruise would then be considered and investigated by an ultrasound or CAT scan.
In summary, I must repeat that almost all known operations to Surgeons have been successfully carried out in cases of Ehlers-Danlos Syndrome. Surgery of all kinds, therefore, can be beneficial and safe. Problems will only arise if the patient or/and the Surgeon are ignorant of the diagnosis and its implications. Once the Surgeon is made aware of the patient’s EDS, and familiarises themself with the extensive surgical literature on treating these patients, he or she can go safely ahead and carry out a necessary operation to the patient’s benefit.