Short gut syndrome, also known as short bowel syndrome, occurs when the body does not properly absorb and digest food normally because a large length of the small intestine is missing or non-functional. This can be due to a birth defect or surgical removal.
People with short gut syndrome cannot absorb enough water, vitamins, and other nutrients from food to thrive and grow.
Here to discuss Short Bowel Syndrome and when to refer, is Brad Warner, MD. He is a Washington University pediatric general surgeon and surgeon-in-chief at St. Louis Children's Hospital.
Short Bowel Syndrome
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Learn more about Brad Warner, MD
Brad Warner, MD
Brad Warner, MD, Washington University pediatric general surgeon and surgeon-in-chief at St. Louis Children’s Hospital. He has a special interest in pediatric gastroenterology and nutrition including short bowel syndrome and inflammatory bowel disease.Learn more about Brad Warner, MD
Transcription:
Short Bowel Syndrome
Melanie Cole (Host): Today’s topic is short bowel syndrome. My guest is Dr. Brad Warner, he’s a Washington University Pediatric General Surgeon and Surgeon and Chief at St. Louis Children’s Hospital. He has a special interest in pediatric gastroenterology and nutrition including short bowel syndrome and inflammatory bowel disease. Welcome to the show Dr. Warner. So, explain a little bit about short bowel syndrome or short gut syndrome as it is known in both ways.
Dr. Brad Warner, MD (Guest): So, it really is if there is inadequate intestinal length to be able to sustain growth and maintain health. So, in children that have short bowel syndrome, they have had either their intestine removed of sufficient length to leave them inadequate for growth and development and they require supplemental nutrition by vein for a period of time.
Melanie: So, what causes it? You mentioned that if they have had surgery or maybe even trauma; are there other causes that you know about or can it be prevented in some way?
Dr. Warner: So, the most common cause for short bowel syndrome in the pediatric population is necrotizing enterocolitis and this is a condition that affects about 4-6% of premature infants that are feeding and growing and doing well and all of the sudden for whatever reason, there are multiple factors; but their intestine dies and they become very ill and as pediatric surgeons; one of the things that we have to do is take these babies to the operating room, open their abdomen and determine how much intestine has died. And we have to remove the dead and dying intestine to save the life of the baby. And sometimes that requires removing more than half of their intestine and that leaves them with an inadequate amount of intestine length to survive just on feeding orally alone. So, that’s the most common cause. Other causes are what’s called a mid-gut volvulus where a child maybe otherwise fine, but their intestine has not developed correctly in that it is has not fixed itself in the abdominal cavity or rotated normally and so sometimes, and it’s most common in the first year of life but can be at any age; is the intestines suddenly twists on itself and cuts off its blood supply and everything dies and then again, that’s a situation where we take the child to the operating room and they are very ill and in order to save their life; we have to remove what’s dead and oftentimes, that would leave them with an amount of intestine that is insufficient for them to survive on oral feeding alone.
Melanie: How scary. Are there some red flags that pediatricians and or parents should be looking for in that first couple of years of life?
Dr. Warner: Yes, the big thing is what we call bilious emesis, so if a child throws up green; it ought to be very, very alarming. Bilious emesis is really the opportunity to investigate urgently whether the intestine has twisted or not. Now there are a lot of other causes of bilious emesis, throwing up green; but one of the few causes that really is associated with loss of life and loss of intestine would be bilious emesis related to volvulus that occurs. So, the important thing is not all bilious emesis is a surgical condition, in fact it’s less frequent but it is the one sort of thing that can develop rapidly and if you act judiciously; you can save a life and save intestinal length.
Melanie: So, this is an emergent situation and if you see that or if the pediatrician – if you bring your child in for because you think they are sick and the pediatrician notices this; then it becomes emergent. How do you diagnose it Dr. Warner?
Dr. Warner: So, the best way to diagnose this is an urgent upper GI contrast study, where they administer barium by mouth and they can see the course of the small intestine and sometimes they can actually see the twist, but a normal upper GI is extremely reassuring and then you can really start thinking of other causes that might be nonsurgical. So, an upper GI is the gold standard way to make the diagnosis. Sometimes though, children have had this happen and they have gone for hours and they are so sick that they wouldn’t tolerate going even for the upper GI and those are the patients that we take to the operating room right away and those are the patients that end up not doing as well.
Melanie: Does it always become surgical?
Dr. Warner: The twist, yes, it absolutely does. There is no way to untwist it without surgery and no real way to assess the viability of the intestine without looking at it directly. And even then, sometimes what we think might look good, over the next 24 hours or so, does not or sometimes what we think doesn’t look good improves overs 24 hours. So sometimes, we will untwist the intestine and if there are areas that are marginal, we prefer to leave those areas, hoping that they improve in their color, improve in their perfusion, blood flow and so sometimes we have to do second look operations, the following day, take the baby back to the operating room and have another look and see how things are progressing.
Melanie: Well, so if it’s a result of having part of their small intestine removed from trauma or surgery or whatever; then are you keeping an eye for short bowel syndrome then for however long? Is this something that now is going to follow this child through life?
Dr. Warner: Yes. So, what is interesting, and this is what our laboratory has been focused on for over 20 years is when you remove at least half of the intestine; the intestine that’s left in place tries to grow back to compensate for what’s been lost. And this is a process that is called adaptation. So, small bowel adaptation generally results in growing taller villi that line the surface of the bowel, the luminal surface, so that there is greater mucosal surface area for each unit length of bowel. So, the bowel does try to grow back and compensate, and it turns on proliferation of a bunch of cells and it grows structurally a little bit and along that period of time, while that is occurring, we can notice that patients that might tolerate a small amount of feeding into the intestine before having severe diarrhea are now tolerating more and more amounts of feeding with less and less diarrhea. And so that is a process that’s ongoing and so as long as patients are increasing the amount that they tolerate, over a period of time; then you can reduce the amount that they need by vein to supplement; then hopefully and sometimes it may take up to a year or two that patients are able to come off completely from the need for supplemental nutrition by vein and they can tolerate everything orally at least with food and or tube feeding. And that’s ideally what you want to achieve is a slow period where you transition from feeding by vein to feeding orally and it sometimes may take a year or two, but as long as it’s improving; that’s good.
I think the major problem is when you hit a wall in terms of every time you go beyond this amount of feeding by mouth that patients get severe diarrhea, they start losing weight and you are obviously not making the goal that you need. And that’s when things become a little bit more dicey. The overall survival rate for patients that require nutrition by vein with short bowel syndrome is 50% of those patients will ultimately be able to come off of the need for nutrition by vein. Of the remaining 50%, half of those will require a small bowel transplant and the other half will die. So, overall, a 25% mortality rate in patients with short bowel syndrome and of the patients that get a small bowel transplant; that has a 50% five-year survival rate. So, it’s a very, very lethal type of condition if you don’t have enough intestine length.
So, there’s a lot of research being done now to try to grow the bowel back even further to enhance the adaptation responses and there’s investigative work looking at how to better improve the survival of patients that require a small bowel transplant, but also now there is a whole body of work that is being done with regard to tissue engineering and I think the pie in the sky ultimate goal would be to take a biopsy of the patient’s intestine at the time that they lose all of their bowel and grow the bowel in a dish and put it back in the same patient with their own tissue. So, they don’t have to have any sort of transplant antirejection medicines and things. So, we are a way away from being able to do that successfully, but there are some things that are developing now that we are learning a lot about tissue engineered intestine and things like that. So, that’s what we are working toward is being able to grow our own bowel and put it back in to replace what’s been lost.
Melanie: And before we summarize, Dr. Warner, is dehydration a big issue with this syndrome and then summarize it up for us what you would like other physicians to know about short bowel syndrome and when to refer to a specialist.
Dr. Warner: So, dehydration is absolutely a problem in these patients. They take in a little bit and they stool out more than they are taking in, so they can dehydrate readily and so part of the supplementation that these kids require is nutrition by vein but also fluid and electrolytes by vein. So, that’s something that we monitor very, very carefully.
The big sort of take home message for patients with short bowel syndrome, I think the big thing in an otherwise normal child; bilious emesis is a surgical emergency until proven otherwise and if a child has a history of bilious emesis and or crampy abdominal pain with bilious emesis; then an urgent, emergent upper GI needs to be done and that child needs to be cared for under the supervision of a board certified general pediatric surgeon. In terms of managing short bowel syndrome, the data is pretty clear now that patients do far better when they are part of a multidisciplinary short bowel syndrome center and I think these are generally at major university associated children’s hospitals. These are not something that should be managed by a single physician in a community that I think bringing these patients together because they are very complicated. You need to have social work. You need to have pharmacy. You need to have surgeons with expertise in short gut syndrome and doing intestinal lengthening surgical procedures. You need to have pediatric GI medicine. There’s a whole list of dieticians and all that type of support all working singularly focused on making this child optimal. And that really can only be done in a multidisciplinary center.
Melanie: Thank you so much for being on with us today, Dr. Warner. A physician can refer a patient by calling Children’s Direct Physician Access line 1-800-678-HELP. That’s 1-800-678-4357. You’re listening to Radio Rounds with St. Louis Children’s Hospital. For more information on resources available at St. Louis Children’s Hospital, you can go to www.stlouischildrens.org , that’s www.stlouischildrens.org . This is Melanie Cole. Thanks so much for listening.
Short Bowel Syndrome
Melanie Cole (Host): Today’s topic is short bowel syndrome. My guest is Dr. Brad Warner, he’s a Washington University Pediatric General Surgeon and Surgeon and Chief at St. Louis Children’s Hospital. He has a special interest in pediatric gastroenterology and nutrition including short bowel syndrome and inflammatory bowel disease. Welcome to the show Dr. Warner. So, explain a little bit about short bowel syndrome or short gut syndrome as it is known in both ways.
Dr. Brad Warner, MD (Guest): So, it really is if there is inadequate intestinal length to be able to sustain growth and maintain health. So, in children that have short bowel syndrome, they have had either their intestine removed of sufficient length to leave them inadequate for growth and development and they require supplemental nutrition by vein for a period of time.
Melanie: So, what causes it? You mentioned that if they have had surgery or maybe even trauma; are there other causes that you know about or can it be prevented in some way?
Dr. Warner: So, the most common cause for short bowel syndrome in the pediatric population is necrotizing enterocolitis and this is a condition that affects about 4-6% of premature infants that are feeding and growing and doing well and all of the sudden for whatever reason, there are multiple factors; but their intestine dies and they become very ill and as pediatric surgeons; one of the things that we have to do is take these babies to the operating room, open their abdomen and determine how much intestine has died. And we have to remove the dead and dying intestine to save the life of the baby. And sometimes that requires removing more than half of their intestine and that leaves them with an inadequate amount of intestine length to survive just on feeding orally alone. So, that’s the most common cause. Other causes are what’s called a mid-gut volvulus where a child maybe otherwise fine, but their intestine has not developed correctly in that it is has not fixed itself in the abdominal cavity or rotated normally and so sometimes, and it’s most common in the first year of life but can be at any age; is the intestines suddenly twists on itself and cuts off its blood supply and everything dies and then again, that’s a situation where we take the child to the operating room and they are very ill and in order to save their life; we have to remove what’s dead and oftentimes, that would leave them with an amount of intestine that is insufficient for them to survive on oral feeding alone.
Melanie: How scary. Are there some red flags that pediatricians and or parents should be looking for in that first couple of years of life?
Dr. Warner: Yes, the big thing is what we call bilious emesis, so if a child throws up green; it ought to be very, very alarming. Bilious emesis is really the opportunity to investigate urgently whether the intestine has twisted or not. Now there are a lot of other causes of bilious emesis, throwing up green; but one of the few causes that really is associated with loss of life and loss of intestine would be bilious emesis related to volvulus that occurs. So, the important thing is not all bilious emesis is a surgical condition, in fact it’s less frequent but it is the one sort of thing that can develop rapidly and if you act judiciously; you can save a life and save intestinal length.
Melanie: So, this is an emergent situation and if you see that or if the pediatrician – if you bring your child in for because you think they are sick and the pediatrician notices this; then it becomes emergent. How do you diagnose it Dr. Warner?
Dr. Warner: So, the best way to diagnose this is an urgent upper GI contrast study, where they administer barium by mouth and they can see the course of the small intestine and sometimes they can actually see the twist, but a normal upper GI is extremely reassuring and then you can really start thinking of other causes that might be nonsurgical. So, an upper GI is the gold standard way to make the diagnosis. Sometimes though, children have had this happen and they have gone for hours and they are so sick that they wouldn’t tolerate going even for the upper GI and those are the patients that we take to the operating room right away and those are the patients that end up not doing as well.
Melanie: Does it always become surgical?
Dr. Warner: The twist, yes, it absolutely does. There is no way to untwist it without surgery and no real way to assess the viability of the intestine without looking at it directly. And even then, sometimes what we think might look good, over the next 24 hours or so, does not or sometimes what we think doesn’t look good improves overs 24 hours. So sometimes, we will untwist the intestine and if there are areas that are marginal, we prefer to leave those areas, hoping that they improve in their color, improve in their perfusion, blood flow and so sometimes we have to do second look operations, the following day, take the baby back to the operating room and have another look and see how things are progressing.
Melanie: Well, so if it’s a result of having part of their small intestine removed from trauma or surgery or whatever; then are you keeping an eye for short bowel syndrome then for however long? Is this something that now is going to follow this child through life?
Dr. Warner: Yes. So, what is interesting, and this is what our laboratory has been focused on for over 20 years is when you remove at least half of the intestine; the intestine that’s left in place tries to grow back to compensate for what’s been lost. And this is a process that is called adaptation. So, small bowel adaptation generally results in growing taller villi that line the surface of the bowel, the luminal surface, so that there is greater mucosal surface area for each unit length of bowel. So, the bowel does try to grow back and compensate, and it turns on proliferation of a bunch of cells and it grows structurally a little bit and along that period of time, while that is occurring, we can notice that patients that might tolerate a small amount of feeding into the intestine before having severe diarrhea are now tolerating more and more amounts of feeding with less and less diarrhea. And so that is a process that’s ongoing and so as long as patients are increasing the amount that they tolerate, over a period of time; then you can reduce the amount that they need by vein to supplement; then hopefully and sometimes it may take up to a year or two that patients are able to come off completely from the need for supplemental nutrition by vein and they can tolerate everything orally at least with food and or tube feeding. And that’s ideally what you want to achieve is a slow period where you transition from feeding by vein to feeding orally and it sometimes may take a year or two, but as long as it’s improving; that’s good.
I think the major problem is when you hit a wall in terms of every time you go beyond this amount of feeding by mouth that patients get severe diarrhea, they start losing weight and you are obviously not making the goal that you need. And that’s when things become a little bit more dicey. The overall survival rate for patients that require nutrition by vein with short bowel syndrome is 50% of those patients will ultimately be able to come off of the need for nutrition by vein. Of the remaining 50%, half of those will require a small bowel transplant and the other half will die. So, overall, a 25% mortality rate in patients with short bowel syndrome and of the patients that get a small bowel transplant; that has a 50% five-year survival rate. So, it’s a very, very lethal type of condition if you don’t have enough intestine length.
So, there’s a lot of research being done now to try to grow the bowel back even further to enhance the adaptation responses and there’s investigative work looking at how to better improve the survival of patients that require a small bowel transplant, but also now there is a whole body of work that is being done with regard to tissue engineering and I think the pie in the sky ultimate goal would be to take a biopsy of the patient’s intestine at the time that they lose all of their bowel and grow the bowel in a dish and put it back in the same patient with their own tissue. So, they don’t have to have any sort of transplant antirejection medicines and things. So, we are a way away from being able to do that successfully, but there are some things that are developing now that we are learning a lot about tissue engineered intestine and things like that. So, that’s what we are working toward is being able to grow our own bowel and put it back in to replace what’s been lost.
Melanie: And before we summarize, Dr. Warner, is dehydration a big issue with this syndrome and then summarize it up for us what you would like other physicians to know about short bowel syndrome and when to refer to a specialist.
Dr. Warner: So, dehydration is absolutely a problem in these patients. They take in a little bit and they stool out more than they are taking in, so they can dehydrate readily and so part of the supplementation that these kids require is nutrition by vein but also fluid and electrolytes by vein. So, that’s something that we monitor very, very carefully.
The big sort of take home message for patients with short bowel syndrome, I think the big thing in an otherwise normal child; bilious emesis is a surgical emergency until proven otherwise and if a child has a history of bilious emesis and or crampy abdominal pain with bilious emesis; then an urgent, emergent upper GI needs to be done and that child needs to be cared for under the supervision of a board certified general pediatric surgeon. In terms of managing short bowel syndrome, the data is pretty clear now that patients do far better when they are part of a multidisciplinary short bowel syndrome center and I think these are generally at major university associated children’s hospitals. These are not something that should be managed by a single physician in a community that I think bringing these patients together because they are very complicated. You need to have social work. You need to have pharmacy. You need to have surgeons with expertise in short gut syndrome and doing intestinal lengthening surgical procedures. You need to have pediatric GI medicine. There’s a whole list of dieticians and all that type of support all working singularly focused on making this child optimal. And that really can only be done in a multidisciplinary center.
Melanie: Thank you so much for being on with us today, Dr. Warner. A physician can refer a patient by calling Children’s Direct Physician Access line 1-800-678-HELP. That’s 1-800-678-4357. You’re listening to Radio Rounds with St. Louis Children’s Hospital. For more information on resources available at St. Louis Children’s Hospital, you can go to www.stlouischildrens.org , that’s www.stlouischildrens.org . This is Melanie Cole. Thanks so much for listening.