Born at just five-and-a-half months on Christmas day and weighing less than 500 grams, Peter Erasmus came into the world in a different era of medicine, faced with a long road of healthcare challenges ahead. Now, 49 years later he has at last received the diagnosis and treatment that has eluded him most of his life.
Suffering from severe abdominal pain on and off for decades, alongside several other already identified conditions, Peter was recently diagnosed with the extremely rare Dunbar syndrome, also known as median arcuate ligament syndrome.
According to Dr Keona Lakey, a general surgeon trained in minimally invasive surgery and practising at Netcare Sunward Park Hospital, the condition is exceptionally rare, affecting approximately two out of every 100 000 people. “It is a congenital condition, which means that Peter had been suffering with it since birth and it is quite unique for a person to receive the diagnosis at this stage of life, as it is usually identified earlier.
“However, Peter had a very complex medical history of numerous other healthcare issues. Furthermore, this syndrome is a challenging disease to diagnose because it is so rare and because the diagnosis must be done by exclusion. There are many other conditions that share symptomatic similarities, such as gastritis, gallstone and reflux disease, pancreatitis and others. It is therefore necessary to rule out any other possibilities before proceeding with surgical treatment.”
|Pic: Dr Keona Lakey is a general surgeon trained in minimally invasive surgery who practises at Netcare Sunward Park Hospital.
|Pic: Dr Lakey says that laparoscopic surgery can make all the difference in the life of a patient, highlighting that it should be strongly considered wherever possible in the surgical treatment of numerous conditions ranging from the common to the complex.
|Pic: Peter Erasmus had suffered from severe abdominal pain on and off for decades and was recently diagnosed with and surgically treated for the extremely rare Dunbar syndrome, also known as median arcuate ligament syndrome.
Dunbar syndrome affects the main artery supplying the upper part of the abdomen, the celiac artery, which normally feeds blood supply through the area but with Dunbar syndrome it is compressed by the diaphragm. This can cause long-term chronic stomach pain and general discomfort.
Peter notes that dealing with healthcare difficulties has been a part of his daily life since he can remember. “I had a rocky start, being born so underweight and with certain of my internal organs on the outside of my body. I spent my first three years on earth in hospital and have been in and out of medical facilities since then.
“I have always had numerous conditions, but my stomach began to give me more severe trouble in my twenties and after a number of tests and scopes, I still did not have a clear answer as to what the problem might be. It then eased off and for some time I did not experience such terrible pain.
“However, both my mother and my father recently passed away, which was very stressful, and this seemed to trigger the problem again, only this time the pain after eating was so severe that I felt like I was dying,” says Peter.
Dr Lakey notes that a scope was done to eliminate certain other issues but knowing that the pain was directly related to eating made it easier to pinpoint the issue and after a CT scan the condition was formally diagnosed.
“The only way to treat this condition is with surgery and traditionally that would mean open surgery, which would have required a very large incision and dissection of the abdomen. An endovascular approach might have been considered with angioplasty and/or stent placement, to keep the artery open, however this would not solve the root cause of the problem, which is the compression of the artery by the diaphragm.
“We therefore opted for laparoscopic surgery, which meant that the artery was far easier to see, there would be a great deal less trauma to Peter and he would experience a much quicker healing period and better outcomes all round.
“I invited my colleague Dr Imraan Sardiwalla – a hepatobiliary surgeon who heads up the surgical gastroenterology hepatobiliary unit at Dr George Mukhari Academic Hospital and with whom I completed my fellowship training – to conduct the surgery with me, as it was such a rare condition and in these cases two heads are often better than one. We had to consider all possible challenges when undertaking the procedure. We knew that we might run into some scar tissue in Peter’s abdomen, as he had had such a complicated medical history, which would have made the operation particularly difficult.
“Fortunately, we had a clear path when opening the anatomy and working between the liver and the stomach to identify the blood vessels connected to his celiac artery. Because the condition had not been attended to until now the constriction was very tight and we needed to work our way backwards, gradually releasing the nerve fibres, of the celiac plexus, which had become enlarged and needed to be released first. We then released all the nervous tissue and the diaphragmatic fibres around the artery and ensured that it was completely free.
“Such a complex operation requires extremely thorough knowledge and experience of the anatomy in that area, as you are dealing with the main blood supply coming off the aorta and any incorrect incisions could result in catastrophic bleeding. Caution is therefore key in safely freeing a blood vessel like this, which is also part of the reason why we felt laparoscopic surgery was the best option, as it can be so much more precise than open surgery,” says Dr Lakey.
Peter notes that following a short stay of just a few days at Netcare Sunward Park Hospital, he was able to go home and experienced immediate relief from the time of the operation.
“I no longer feel any pain or discomfort in my stomach and my day-to-day life is just easier to get through without that difficulty. I am now able to better manage my diabetes, as I can eat a proper, healthy diet. Honestly, I am just so glad to have had this condition diagnosed and treated at long last. It is a relief,” he says.
Dr Lakey concludes by noting that laparoscopic surgery can make all the difference in the life of a patient, highlighting that it should be strongly considered wherever possible as it can be applied in the treatment of numerous conditions, whether rare and complex – as in the case of Peter Erasmus – or common.
“There is a great deal of international research supporting the fact that a laparoscopic approach is equally effective, if not more so, than open surgery. I myself have certainly seen the positive results of this minimally invasive method among my patients for conditions ranging from colonic cancers and resections to hiatus and abdominal hernias and appendix removals, and many others.
“Laparoscopic surgery also means a far lower risk of future complications, which is not only beneficial to patients like Peter who have already been through a lot, but for anyone needing to go under the knife. Best possible patient outcomes are always the surgeon’s ultimate goal, not only at the time of operating but also into the future. Thanks to modern laparoscopic interventions it is so much easier to achieve this and make an impact in the lives of people needing surgical care,” he concludes.
Notes to editor
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