A recent series of articles in The Guardian again reveals the significant financial and debilitating cost of this terrible disease. My friend and colleague, Dr Geoff Reid outlines the tremendous impact this has on women and their partners. Increasingly it is becoming clear that advanced cases require multi-specialised teams to cope with the different facets of the disease. Far too many people are having repeat surgery. I am also coming to the opinion that our current definition is too simplistic and that we need to expand this to accommodate the reality of the clinical situation. Surgery alone is probably not optimal.
The current definition of endometriosis is the presence of endometrial glands and stroma outside the uterus. This limited definition of the disease unfortunately creates unrealistic expectations that simply removing the disease results in a “cure”.
The reality is that we are hopelessly lost in understanding this disease and the underlying pathological process. It is known that individuals who have histologically proven endometriosis will have other abnormalities. These include changes within the normal endometrium such as the presence of nerve fibres, other immunological abnormalities at the endometrial myometrial interface, changes within the immunological profile of the peritoneal fluid and possible changes in the egg quality of patients with endometriosis. Further there appears to be an association with certain cancers, (ovary breast and melanoma) and an association with increased risk of auto-immune diseases, asthma and atopy. Pregnancy complications including miscarriage, ectopic, haemorrhage and prematurity appear increased but interestingly endometriosis might be protective for the development of hypertensive diseases and pre-eclampsia. All of these issues suggest that the disease is more than just some abnormal cells outside the uterus.
Despite the best efforts of your surgeon to remove “all visible disease” sometimes it may not be possible or practical to remove all the disease. Even if you do remove all the disease surgery will not correct all the other associated phenomena currently not well understood. It is well known the staging system as currently exists often conflicts with symptoms and at times is not a useful predictor of fertility prospects or pain. The natural history clearly demonstrates that pregnancy is highly advantageous to symptoms in individuals with endometriosis and it appears clear to me that some form of progestegenic support is almost mandatory in the ongoing management of patients with endometriosis. To put that another way the management should not be surgery alone. In an ideal world multiple pregnancies starting at a young age is probably the answer but many if not most people are unlikely to embrace this option. Management plans really need to be individualized but if surgery is contemplated it should involve attempting to excise “all visible disease” and then ongoing treatment with either pregnancy or some form of “pseudo-pregnancy” state with hormonal manipulation.
I am coming to the realization that our current definition of endometriosis is far too simplistic and creates significant difficulties for both patients and medical practitioners as this simplistic model encourages unrealistic expectation of what is possible with our current therapeutic armamentarium. The current definition implies that simply removing the disease results in a “cure”. It is time to embrace a more encompassing definition extending to include the clinical aspects of endometriosis which are not well understood and are not simply a result of ectopic endometrium.
Over recent months I have seen an unusual number of patients with endometriosis in various scar sites. The most common situation follows the performance of a caesarian section with a deposit of endometriosis within the operative field. More rarely and usually without prior surgery (including laparoscopy) the umbilicus can be affected. Interestingly I have not seen any endometriotic deposits within trocar sites.
The most common presentation is of a nodule of endometriosis somewhere within a caesarian scar. Most caesarian section scars are Pfannenstiel or bikini type scars across the lower abdomen. Although the skin incision is horizontal the underlying incision through the rectus sheath or covering fascia over the abdominal musculature is vertical and it is in this area that the nodule will develop. As a consequence the nodule may not overly the actual skin incision and may be situated anywhere along this line or extended up and towards the umbilicus. These nodules usually cyclically swell and become painful and most patients take some time to appreciate the significance of what is occurring. They are usually imaged with ultrasound and fine needle aspiration biopsy will reveal the diagnosis. The endometriosis in this area presumably has arisen as a result of the deposition of endometrial tissue at the time of the caesarian embedded within the scar. The endometriosis behaves in this area in a similar fashion to endometriosis in other sites and tends to develop a cicatrizing or scarring type effect. It can be likened somewhat to the example of a table cloth which if it could be grasped by one hand in the middle and a small area pulled into a ball, if that area is then cut out the resulting defect can be quite large. Many of the patients I have seen have had incomplete excision of the nodular area with subsequent recurrence. The nodules usually involve a portion of the rectus sheath although occasionally they can be above or below the rectus sheath. If the rectus sheath is involved and a large area is removed the resulting defect will require primary closure and if it is significantly large it may not be possible to achieve this. In that setting some form of mesh repair similar to a hernia repair will be required otherwise a hernia will develop. Caesarian scar endometriosis is often an isolated finding and at laparoscopic investigation further endometriosis may not be encountered within the pelvis.
Umbilical endometriosis by contrast is usually associated with pelvic endometriosis and is dealt with in a similar fashion. Other areas of involvement include the cervix and episiotomy scars. The cervical disease usually follows a surgical procedure to deal with abnormal pap smears.
The answer seems simple. The bowel surgeon should be involved early in the process. I agree. The bowel surgeon should ideally have the seen the patient in a pre-operative consultation to discuss the planned procedure, results and potential complications. The intra-operative timing is the critical thing. If the gynaecologist has minimal surgical skills then invariably the bowel surgeon will be involved earlier. If that occurs, then the patient will receive a colo-rectal procedure, usually a segmental resection because that is what most bowel surgeons do since they are treating cancers. Unfortunately this will be too aggressive for most endometriosis patients who would have been adequately treated with either a shave or a disk excision. Segmental resection has significant long term functional effects and I believe should be avoided if at all possible.
A recent series describes an impressive 750 cases of segmental bowel resection for endometriosis. Unfortunately the series involved no disks or shaves and I believe it is highly likely that many of these patients did not require segmental resection.
If the gynaecologist has the appropriate skills then I believe the endometriosis should be removed from the side walls, the ureters should be exposed and then the bowel mobilised and shaved. At that point the bowel surgeon should be asked to consult and the issue then becomes what is the best procedure for that patient – shave, disk or segment.
Early recourse to the assistance of bowel surgeons often due to inexperienced gynaecological endometriosis surgeons is likely to result in more segmental resections than should occur.
I have recently been asked this (or a very similar) question at a number of meetings. I have given this issue considerable thought over the years. The most common response I receive from both trainee surgeons and colleagues is that yes the surgeon should call for assistance in the event that there is unplanned damage to an adjacent organ. The most common setting is inadvertent damage to the bowel or bladder. At first glance this seems appropriate – the specialist surgeon can repair the damage and decrease the chance that there will be an adverse outcome. This also plays into the hands of the lawyers who would argue that you need to get a specialist surgeon involved to decrease the litigation risk in the event that a legal suit is filed.
Increasingly I am starting to believe that this approach is flawed and potentially a significant step backwards for our capacity to train high quality endometriosis surgeons for the future. The process, like much of medicine, is driven by lawyers attempting to decrease or (even worse) remove risk from the surgical process. The difficulty is that it is not possible to remove all risk from a surgical procedure. Whilst we can usually predict what is going to happen in most circumstances it is not always possible to do so. New ultrasound imaging techniques and better equipment have certainly helped the predictive process in recent years, especially picking up deep invasive bowel and bladder endometriosis. There will, however, always be lesions and areas that were not predicted or anticipated.
Don’t get me wrong – I am not advocating a situation that does not involve a multi-skilled surgical team for the performance of the surgery. If bowel endometriosis is predicted then I firmly believe a bowel surgeon should be involved. The issue then is WHEN the surgeon is involved. A topic for another blog.
The endometriosis surgeon needs to be able to do as much of the procedure as possible before involving other surgeons. To gain the confidence to do this involves being able to dissect out spaces and divide dense adhesions. At times there will be damage to adjacent organs that was not planned. In the event that this happens, I believe the gynaecologist should be able to repair most (if not all) of these injuries. Often a surgical colleague may be able to assist or offer an opinion but this will not always be the case. There may not be any colleagues around. The procedure may have taken place out of hours or in a smaller hospital with minimal other back up. The real world dictates that the endometriosis surgeon must be able to effect these repairs.
A related problem is the situation where it is clearly in the best interests of the patient to have something done that was somewhat unexpected. The most obvious examples I see involve patients who are having difficulties conceiving and who are also limited with time because of their age. Consider a patient who is 40 with a low AMH and a small bowel lesion that was not predicted on scan that is readily able to be removed. The patient has been consented for removal of endometriosis and the surgeon has mentioned the risks, including colostomy. You could take the conservative, traditional approach and defer surgery and get them to come back with a bowel surgeon. But what if the wait for repeat surgery is another 6 months and then another 6 months if IVF is required. Most of these patients would not be happy to have repeat surgery that required a significant time lag and potentially shut down their fertility window.
There are also clearly times when the surgeon should appreciate that even with significant skills, if the appropriate surgical back up is not available (especially when the risk is high) then the case should be abandoned. This relates to the concept of surgical insight.
The development of advanced surgical skills is not easy and requires significant training but also the capacity to be somewhat flexible in approach and able to deal with changing and unforseen circumstances as they arise.
If our trainees operate with the belief that surgical back up is always available then I strongly believe we will not be training them appropriately for the future. Their surgical skills will not advance to the level required for exceedingly difficult cases. Decreasing risk by early recourse to surgical back up may have the perverse effect of potentially increasing risk to even more patients in future by decreasing surgical skill levels.
I admit these concepts are somewhat “messy” but I believe they are real.
Over the last few years I have travelled to Hong Kong and China to operate and run surgical workshops where I have had the honour of teaching and working with other specialist gynaecological surgeons. I am registered as a specialist gynaecologist in Hong Kong and have been accredited to operate as an advanced level laparoscopic surgeon. I have been asked to speak at a meeting in Shanghai later in the year and I am planning to return to Hong Kong in the next few months to consult and operate. After talking with a number of Hong Kong surgeons including conversations at our most recent AGES conference, it struck me that there are now some significant differences evolving in the management of endometriosis currently in Australia as compared to Hong Kong.
Recent advances in imaging technology that I have alluded to previously now allow the diagnosis of deep invasive endometriosis via ultrasound rather than laparoscopy. It is early days with this technique but in the hands of experienced practitioners there is a high probability of predicting this form of disease which is of immeasurable value to both the patient and the surgeon and potentially avoids the use of an unnecessary diagnostic laparoscopy. I am not aware of any units in Hong Kong at this stage utilising this technique but I believe it would be certainly a useful addition to their diagnostic armamentarium and I hope to discuss this with some of my colleagues on my next visit.
My general understanding of the practice of obstetrics and gynaecology in Hong Kong is that virtually everybody is a generalist obstetrician and gynaecologist with perhaps specific certain interests. Some surgeons that I have encountered are excellent with excision of deep invasive endometriosis although they would rarely resort to any form of bowel resection and this is certainly thought to be an uncommon surgical manoeuver. Given the amount of material recently published about the extent of deep invasive endometriosis, I am sure there is a significant cohort of women within Hong Kong who have both under-diagnosed and under-treated endometriosis. In Australia there are an increasing number of specialists, albeit small at this stage, who have focused virtually their whole practice on the treatment of endometriosis. Like many other surgical disciplines where specialization occurs it seems to me as self-evident that the outcomes for complex problems are superior in the hands of those who do little else other than work in that particular field. I believe there is an opportunity for this type of practice to be established in Hong Kong and I have been discussing these issues with junior colleagues.
There is somewhat of a dilemma in the management of endometriosis when fertility is an issue, as is often the case. Currently within Australia most practitioners either perform some form of surgery or IVF but very few would be capable of performing surgery for deep invasive endometriosis and also being involved in managing an IVF cycle. It is becoming apparent that some patients may require both surgery and IVF and the timing, organization and sequence of these procedures is critical to ultimate patient success. Within Australia there are a limited number of such practitioners although it is increasingly being realized by the younger IVF specialists in particular that to optimally treat patients with endometriosis their surgical skills must improve to be able to deal with deep invasive endometriosis. At this stage in Hong Kong I am unaware of any practitioners who perform both IVF and have the surgical skills to deal with deep invasive endometriosis and this is certainly a message that could be shared.
The ultrasound diagnosis of deep infiltrating endometriosis has evolved dramatically over the last few years. Much of the initial work was done at Professor Mauricio Abrao’s unit in San Paulo, Brazil. As it transpires he is the main guest speaker at our annual gynaecological surgical conference in Brisbane next year. A number of Australian gynaecologists have been to his unit and the technique is now spreading throughout Australia. SAFE. The technique utilises a different angle of approach for the ultrasound transducer such that the vagina, cul-de-sac and rectosigmoid can be imaged. In this fashion it is possible to see nodules of endometriosis within these areas which were previously not identified. Anecdotal discussion with some of the practitioners using the technique is that the disease appears more common than we had previously appreciated. There are significant advantages to this technique. If deep invasive endometriosis can be diagnosed prior to a laparoscopy it may mean that for many people one extra diagnostic laparoscopy can be avoided. Most importantly forward planning for the surgical procedure can be organized optimally so that expert sub-specialist assistance from either colorectal or urological surgeons can be organized pre-operatively for optimal outcome. In the past this has been a significant problem as the surgeon often had no way of predicting the degree of severity of disease until confronted with it. I believe these techniques will be of significant assistance in the management of patients with endometriosis. Laparoscopy has substantial limitations due to the lack of tactile feedback. Ultrasound will offer superior assessment of some types of lesions particularly those within the sigmoid colon where the lesions may be more obvious on ultrasound than at laparoscopy. The technique is going to add significantly to the management of patients with deep invasive endometriosis but will mean that even more than previously it will be important who does your ultrasound.
In 1995, McArthur Wheeler walked into two Pittsburgh banks and robbed them in broad daylight with no visible attempts at disguise. He was arrested later that night, less than an hour after video tapes of him were taken from surveillance cameras were broadcast on the eleven o’clock news. When police later showed him the surveillance tapes Mr Wheeler stared incredulously “but I wore the juice” he mumbled. Apparently, Mr Wheeler was under the impression that rubbing ones face with lemon juice rendered it invisible to video tape cameras. (Fuocco 1996)
This unfortunate affair serves to illustrate an effect known as the Dunning-Kruger effect after two psychologists Justin Kruger and David Dunning from Cornell University published an article entitled “Unskilled and unaware of it – how difficulties in recognizing ones own incompetence can lead to inflated self assessments.” (Kruger 1999) (WIkipedia reference) The authors note that people tend to hold overly favourable views of their abilities and this over-estimation occurs in part because people who are unskilled in these domains suffer a dual burden – not only do they reach erroneous conclusions and make unfortunate choices but their incompetence does not allow them the cognitive ability to realize it.
Another way of thinking about this is that of insight. People who lack insight or understanding may then over-emphasize their own abilities. These concepts have been known for some time as Charles Darwin noted in 1871 “ignorance more frequently begets confidence than does knowledge”.
“It is one of the essential features of such incompetence that the person so afflicted is incapable of knowing that he is incompetent. To have such knowledge would be to remedy a good portion of the offence” (Miller 1993).
Whilst these concepts are self evident and known in many walks of life it is particularly pertinent to the field of surgery and in my own area even more so in the setting of difficult endometriosis surgery. I believe the problem has largely arisen because of the generally poor quality of excisional surgery around the globe. Many surgeons remain of the view that diathermy is adequate to deal with endometriosis whilst multiple studies have confirmed that excisional surgery is preferable. At its most difficult and severe stage, endometriosis can be exceedingly difficult to assess. In the setting of complete cul-de-sac obliteration with the bowel adherent to the posterior aspect of the uterus in the more severe forms the uterus may not even be able to be visible at laparoscopy because of these adhesions. The surgeon who only occasionally confronts such a case may not recognize what they are dealing with and is unlikely to have the necessary surgical skills to clear the disease and achieve an optimal result. Many surgeons who come to my theatre as visitors have never seen the types of dissection that are required to deal with the disease. In the absence of such knowledge or experience they may attempt to deal with the situation themselves with sub-optimal results and high rates of morbidity.
As most people would appreciate, the more you practice at a task the better you tend to become. For this reason in many fields of surgery, particularly areas such as cancer treatment, the general trend has been to move the treatment of these cases to specialized units where high volume surgical load is performed ultimately resulting in optimal outcomes and minimal complication rates. It is also noteworthy and obvious that a skill level in one area of surgery may not be applicable in another area. In most parts of the world surgery is now becoming a highly sub-specialized skill so that within the varying disciplines people will sub-specialize in a particular area, procedure or disease process. This has not traditionally been the case with obstetrics and gynaecology but I believe is increasingly likely to be so.
There is a flip side to the Dunning-Kruger effect involving the opposite end of the spectrum. Whilst at the bottom end the incompetent tend to over-estimate their skills and possibly operate when that would be inadvisable, the highly skilled who are deeply aware of the difficulties and problems particularly with severe cases of endometriosis may be inclined to be less aggressive even when they ought not.
Fuocco, MA (1996, March 21). Trial and error: They had larceny in their hearts, but little in their heads. Pittsburgh Post-Gazette, pD1.
Darwin, C. (1871). The descent of man. London: John Murray.
Kruger J, Dunning D. Unskilled and unaware of it: how difficulties in recognizing one’s own incompetence lead to inflated self-assessments. Journal of personality and social psychology 1999; 77(6): 1121-34.
Miller, WI. (1993). Humiliation. Ithaca, NY: Cornell University Press