Kareem Abu-Elmagd, MD, PhD, FACS, Director, Gut Rehabilitation and Transplantation at the Cleveland Clinic talks about intestinal malrotation.
Interview conducted by Ivanhoe Broadcast News in July 2018.
What is intestinal malrotation?
Dr. Abu-Elmagd: Normally, when we develop in the womb as an embryo, the intestine grows so fast that the abdominal cavity won’t accommodate the size of the intestine. So the intestine comes out of the abdominal cavity covered with a membrane, and then during the developmental phase in utero, the intestine returns back into the abdominal cavity. During this dynamic process, the intestine rotates in a certain way to adapt into the new length of each part of the gut that we all are born with. Upon completion of the rotation, the organs are fixed in their position inside the abdominal cavity. When this doesn’t happen completely or partially, we call it malrotation. Malrotation means that the different parts of the small and large intestine failed to rotate in utero and stay free in the abdominal cavity without being fixed with the potential for twist and possible cutoff of its blood supply.
What are some statistics? How widespread of a problem is this?
Dr. Abu-Elmagd: Based on the current published data, which is at best incomplete, about one of 200 to 500 children are born with gut malrotation. Either the whole intestine or part of it didn’t rotate in-utero. I think the magnitude of the problem is underestimated because of lack of complete reported data and the lack of awareness among the public and health care professionals. Often, the diagnosis is mostly made accidentally, particularly in patients with absent or vague gastrointestinal symptoms. However, some infants are born with already twisted intestine and develop gut symptoms soon after birth. Unfortunately, most physicians and parents are unaware of the problem until the baby starts vomiting green bile with abdominal distension and failure to thrive. A good number of these children lose their intestine because of the delayed diagnosis with the intestine being twisted with cutoff of its blood supply. Some of these children eventually require intestinal transplantation.
What are some symptoms people should watch out for?
Dr. Abu-Elmagd: The diagnosis is often delayed because a good percentage of these patients, including the kids, do not have symptoms until they grow up and become adults. A child can have malrotation and not necessarily have symptoms. In some way, the development of symptoms is a good thing because it will let the physician and parents pay attention to the problem so patients will not lose the bowel. The reason for the innovative surgical procedure that we introduced over recent years was the fair number of patients, including children, who required small bowel and multivisceral transplantation after they lost their gut from the catastrophic complication of malrotation. As we all know, there’s nothing better than our own gut. Because of that, I’m proud of the malrotation awareness website because it really makes everyone, particularly parents and patients, aware of the problem with impressive scope of knowledge. Patients with nonspecific and vague symptoms usually undergo unnecessary surgery for removal of the gallbladder or appendix. All the patients that I have had the pleasure to treat and operated on had scopes or prior removal of the gallbladder or appendix. Those unnecessary surgeries could be avoided by increasing awareness of the congenital malrotation among physicians and performing the appropriate test for the correct diagnosis before surgery.
How is the condition diagnosed?
Dr. Abu-Elmagd: The diagnosis can be simply made by radiologic contrast studies of the digestive tract. With an abdominal CT or follow-through with oral contrast, you see the first part of the intestine that is connected to the stomach and called the duodenum is located on the right side of the abdomen rather than on the left side. Also, the large bowel that is supposed to make a frame for the intestine in the abdominal cavity is all on the left side. So the duodenum and large bowel are on the wrong side. In addition, the small intestine is commonly roaming in the abdominal cavity and located most of the time on the right side because there is no fixation of the intestine to the back of the abdominal cavity. In addition to that, a CT scan with intravenous contrast commonly show reversal of the position of the artery and vein of the intestine with the vein to the left rather than to the right of the artery. These vascular anomalies are corrected with our new operation. My message to physicians and surgeons, particularly those working with kids, is if the patient has vague symptoms, a high index of suspicion of malrotation should be entertained.
What are some of those vague symptoms that you should be looking for?
Dr. Abu-Elmagd: Most of the kids develop nausea, vomiting, and severe abdominal pain when they develop an acute episode of volvulus. If the diagnosis is made quickly and the surgery is done promptly, the intestine could be saved. For the adults, most physicians believe that a congenital malrotation could not present that late without early symptoms in life. Some of these patients continue to have non-specific vague symptoms and seek care, including multiple visits to the emergency room for recurrent sudden onset abdominal pain. In most instances, physicians cannot find anything on the routine x-ray or the blood work and they send patients back home. A few of the patients I transplanted in the 90s experienced such a scenario with missed diagnosis that resulted in catastrophic loss of the intestine. Common chronic symptoms are vague abdominal discomfort with intermittent nausea and vomiting with constipation alternating with diarrhea. In some patients, constipation is severe with one bowel movement every one to two weeks that requires habitual use of stool softener. Another thing that physicians and patients should be aware of is when there is one congenital anomaly, you should look for other congenital abnormalities such as imperforate anus, gastrochisis, patent foramen oval, absent gallbladder, abnormal position of the cava and other organ abnormalities.
How is intestinal malrotation treated? Are there new treatments available?
Dr. Abu-Elmagd: The first operation for congenital malrotation was done for babies with bowel obstruction due to a band between the duodenum or the first part of the intestine and the beginning of the colon called the cecum. Darcy and other similar patients did not develop the 270 degree of full rotation. The operation is called the Ladd procedure, named after Dr. Ladd, the pediatric surgeon at Boston Children’s Hospital who did the first operation in 1936. What surgeons usually do and still do is just cut the band without any major gut reconstruction including repositioning of the intestine. The release of the band and other associated adhesions usually improve the patient’s initial symptoms but does not prevent the intestine from future twisting with the potential risk of cutting off the blood supply to the intestine with the development of small bowel necrosis. The definitive solution is to reconstruct the whole gut by rotating the bowel another 180 degrees to complete the natural 270 degree rotation and surgically fix all the organs in their new position into the posterior and lateral abdominal wall. At the same time, the major intestinal blood vessels are rearranged in their normal anatomic position.
Is this surgery something you invented?
Dr. Abu-Elmagd: Right. We’ve done more than fifty patients from the United States, Canada, and Europe so far. Most of these patients had prior Ladd procedure and redeveloped or continued to have symptoms with the life threatening potential risk of intestinal twist.
What advantages does your procedure have over previous treatments?
Dr. Abu-Elmagd: The new operation has the advantage of treating the chronic gastrointestinal symptoms including food intolerance, gut failure if present, and above all preventing the development of gut volvulus and subsequent loss of the intestine. As was mentioned earlier, the different steps of the procedure involve reconstructing the gut with repositioning and fixing each organ in its anatomical position. In addition, a piece of the colon is resected in patients with very redundant and convoluted large bowel. With the outdated Ladd procedure, only the duodenal band is released and the intestine is freed with no repositioning or fixation.
What happens if this condition is not treated?
Dr. Abu-Elmagd: This is a good question. In my opinion, NO treatment is not an option. With no treatment, the patients will continue to have symptoms that range from abdominal discomfort to gut failure with the need for intravenous nutrition. More importantly, the development of catastrophic intestinal volvulus will end in demise or bowel loss with the need for intestinal or even multivisceral transplantation. The new operation is proven to fix most of these problems so far with no to minimal postoperative complications. The intestine is an unforgivable organ and if you cut off its blood supply, it will die off in no time.
After surgery, how soon can a person get back to their regular routine? Will there be limitations?
Dr. Abu-Elmagd: Since the operation is currently done as an open procedure, the recovery period ranges from 4 to 6 weeks with an average of one week hospital stay. A few patients with chronic illness require a longer recovery period at home before returning back to their routine. The full recovery of the gastrointestinal function is commonly slower than usual because of the required manipulation of the gut during surgery. The only limitation is avoiding heavy lifting and vigorous exercise during the first three months after surgery.
Let’s talk a little bit about Darcy’s case. Can you give a little background on why she came to you?
Dr. Abu-Elmagd: Darcy is a member of the malrotation website and she took to social media in search of a solution. During an appointment with her local gastroenterologist, it was suggested that she should get a second opinion from Cleveland Clinic. I reached out to her after reviewing her files. I think she had her gallbladder taken out at 25 years of age and I’m not sure if they discovered the malrotation at that time or not. At a later time and over nearly 15 years, she developed recurrent episodes of bowel obstruction and underwent more than 8 abdominal operations. She was in and out of the hospital, which was challenging for her family including her two young children. She came to Cleveland Clinic and she underwent a successful surgery. One week after the operation, she was so happy and couldn’t believe the way she felt. She went home and she had a follow-up appointment a few months later while I was in Egypt on a charity mission and she was seen by one of our Gut Rehabilitation and Transplantation Center (CGRT) physicians. She recently came back for a follow-up visit with her happy family including her husband and the two growing children. This is another example that inspires all of us at Cleveland Clinic to continue doing our best to serve patients that cannot be helped elsewhere.
Why do you think it took her so long to find proper treatment? It said she was diagnosed around 26, but didn’t find a solution until she was 40.
Dr. Abu-Elmagd: We need more published data in the medical literature. In medical school teaching, a malrotation equals a Ladd procedure. Such an operation is like putting a band aid on the problem. I am working on publishing the new technique and outcome results very soon to help many surgeons across the globe taking care of patients like Darcy. This growing and rewarding experience reminds me of the early days when we developed intestinal and multivisceral transplantation in the 90s.
How far away are you from submitting and publishing it?
Dr. Abu-Elmagd: Very soon. We are in the process of finalizing the medical art work, collecting the clinical material, and drafting the manuscript.
What treatment options are there for babies when intestinal malrotation is discovered early on?
Dr. Abu-Elmagd: The only option is surgical correction of the malrotation with fixing the organs in their anatomic position. The Ladd procedure could be a temporary solution until the technical details and the therapeutic benefits of the new operation are published. Currently, we receive national and international patients who are seeking our help after failure of the Ladd procedure.
So when a physician suspects malrotation and confirms it with imaging, what are the options in a baby?
Dr. Abu-Elmagd: The option is to fix it as soon as possible. It’s like a time bomb. Once you diagnose a malrotation, you don’t know what’s going to happen tomorrow. Although the risk of developing intestinal volvulus is very low, it is catastrophic when it happens. So when the diagnosis is made, malrotation should be surgically corrected for both kids and adults. The newly developed reconstructive operation rather than the Ladd procedure should always be considered as a definitive treatment.
If a significant portion of the intestine is removed, is a colostomy a treatment option or are babies too young to have a colostomy? What effects will it have on a baby long term?
Dr. Abu-Elmagd: If the surgeon is confronted in the operating room with a baby, child or an adult that has volvulus with non-viable bowel, a conservative approach should be adopted and only the gangrenous bowel needs to be removed. Most of the time, the surgeon has to take the patient back to surgery for a second look 24-48 hours after the first operation. In stable patients, both ends of the intestine can be connected together. Under certain circumstances, a temporary stoma is created regardless of age. If a baby, child or an adult lost most of their intestine, total parenteral nutrition known as TPN is required to save their life and be later considered for intestinal transplantation. In patients with retained reasonable length of bowel and short bowel syndrome, gut rehabilitation with a bowel lengthening procedure and or a new medication called teduglutide (Gattex) is commonly successful in restoring their nutritional autonomy and quality of life.
Is there anything else that we didn’t talk about that you feel is important for people to know?
Dr. Abu-Elmagd: I think we covered almost everything. The take home message is to increase public awareness of malrotation among patients, physicians, and health care providers. We should always listen and learn from our patients. I believe that the Ladd procedure is not enough to solve the problem. Parents and pediatricians should always be aware of the possibility of malrotation in babies and kids with vague gastrointestinal symptoms including chronic abdominal discomfort, oral intolerance with nausea and vomiting, and sudden onset of abdominal pain. A high index of suspicion may save the intestine and rescue the life of these patients.
END OF INTERVIEW
This information is intended for additional research purposes only. It is not to be used as a prescription or advice from Ivanhoe Broadcast News, Inc. or any medical professional interviewed. Ivanhoe Broadcast News, Inc. assumes no responsibility for the depth or accuracy of physician statements. Procedures or medicines apply to different people and medical factors; always consult your physician on medical matters.
If you would like more information, please contact:
Caroline Auger
Sign up for a free weekly e-mail on Medical Breakthroughs called
First to Know by clicking here.
