Rare condition diagnosed and treated after decades of difficulty

A patient who suffered from severe abdominal pain on and off for decades, in addition to other conditions, was recently diagnosed with the extremely rare Dunbar syndrome, also known as median arcuate ligament syndrome.

After 49 years, Peter Erasmus has at last experienced relief following minimally invasive laparoscopic surgery.

Born at just five-and-a-half months on Christmas Day and weighing less than 500g, Erasmus came into the world in a different era of medicine, faced with a long road of healthcare challenges ahead.

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 about 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,” said Lakey.

However, Lakey said Erasmus 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.”

Dunbar syndrome affects the main artery supplying the upper part of the abdomen, the coeliac artery, which normally feeds blood supply through the area, but with Dunbar syndrome is compressed by the diaphragm. This can cause long-term chronic stomach pain and general discomfort.

Erasmus said healthcare difficulties have always been a part of his life.

“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 20s 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,” he said.

“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,” he said.

Lakey said a scope was done to eliminate certain other issues but knowing the pain was directly related to eating made it easier to pinpoint the issue and, after a CAT scan, the condition was formally diagnosed.

He said the only way to treat it 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.”

Lakey said they opted for laparoscopic surgery, which meant the artery was far easier to see, there would be a great deal less trauma to Peter and he would experience quicker healing 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,” he said.

“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 coeliac 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 coeliac plexus, which had become enlarged and needed to be released first.

“We then released all the nervous tissue and the diaphragmatic fibres about the artery and ensured that it was completely free.”

According to Lakey, such a complex operation requires extremely thorough knowledge and experience of the anatomy in that area, as one is dealing with the main blood supply coming off the aorta and any incorrect incisions could result in catastrophic bleeding. 

Erasmus said after a short stay of just a few days in hospital, he was able to go home and experienced immediate relief.

“I no longer feel any pain or discomfort in my stomach and my day-to-day life is 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 said.

Lakey said laparoscopic surgery 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 — or common.

TimesLIVE

Support independent journalism by subscribing to the Sunday Times. Just R20 for the first month.