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Mm, good afternoon, good evening. Uh good morning, wherever you are joining from, welcome to the Zoom Academic Meeting of the Department of Pediatric Surgery in East London, South Africa. Uh Today we have Doctor Paul Stevens who is going to speak to us about a role of the pediatric surgeon in the management of intestinal failure. Doctor Paul Stevens did his pediatric surgical training at the University of Pretoria. He received the medal from the South African College of Surgeons in 2012. He did a two year fellowship at the Birmingham Children's Hospital in the UK and where he uh had special experience in pediatric surgical oncology, hepatobiliary surgery and transplant surgery. Doctor Stevens is currently in full time private practice at midstream Medic Clinic and Life Urs Hospitals in Pretoria. He is the surgical lead for the pediatric intestinal failure unit at midstream Medic Clinic. And to my knowledge, this is the only pediatric intestinal failure unit in the country. He maintains a keen interest in pediatric vascular access, short bowel management, neonatal surgery, and surgical oncology. So welcome Paul. I will stop sharing and you can share and then you can uh share your slides and share your experience. Thank you. Thanks very much, Melody. Ok. Oh, there we go. Can everyone see that? Yeah. Right. Ok. So I'm gonna chat about, um, pediatric intestinal failure. Um, and the role of the pediatric surgeon in intestinal failure. Um, it's a rather long and fairly complex topic. I'll try and get through it relatively quickly. So there's a few things we sort of going to skim over, um, as opposed to focus on and try and try and really get down more to the, the, the surgical aspects than anything else. So a few things we need to get through before we can understand the surgeon's role is actually what are the definitions of a number of things? One being intestinal failure itself. Um The definition uh sort of varies from place to place and you can look at academic definitions where intestinal failure is defined as requiring parenteral nutrition for a period of longer than 60 days. Um And it's due to a functional gut mass that is below the level that's necessary for adequate gut absorption of nutrients and fluids to allow Children to grow and maintain health. Some units, uh more of the sort of uh prominent international units have used a specific bowel length of length than 25% of predicted for gestational age or the need for parenteral nutrition for more than 42 days. I find a more practical um, definition would be any patient where you early on in their management can already see that they are not going to be coming off parenteral nutrition soon. Those patients need to be considered as being intestinal failure patients, because your actions early on often dictates um their outcomes later on in life, particularly in units where you don't have access to um long term home parenteral nutrition as well as um transplant programs. The context of intestinal failure is also very important. The the definition may be different in, in different countries or different uh uh socioeconomic circumstances depending on your resources. Um Obviously, a multidisciplinary team is, is very necessary or is necessary for the the adequate treatment of these patients. But you often may not have that in your setting and, and it, and it often falls on either the surgeon or the gastroenterologist to do a number of things um beyond their, their, their standard practice um uh in these patients and the resources one has needs to, you really need to be cognizant of what your resources are because, you know, reading international best practice on intestinal failure, you'll often um read about management options that you just really don't have in your in your setting. And this may determine, you know, which patients you actually can manage as intestinal failure patients or which patients are actually palliated compared to what they would be in, in other um international settings. So there are various causes of intestinal failure and we'll go through a few of them but the majority of causes that surgeons are involved in are, are, are short bowel causes. Um, we're responsible for the initial management as well as the long term management of the patients. And they, they, they say it often, often responsible for the short bowel itself. Um, the most common cause being ne gastrosis, intestinal A and gut ULV in short bowel. And then we have motility disorders such as your, um, visceral myopathy that we see in South Africa, relatively frequently. Um your other sources of uh chronic intestinal pseudo obstruction, like long segment, uh total colonic or, or, or, or total bowel Hirschprung's disease and then your mucosal enteropathies such as um microvillus inclusion disease and Tufting enteropathies which slightly more um uh slightly less involvement from uh your, your standard surgical um or, or pediatric surgeon and more more involved with um your, your transplant surgeons in those patients. Um Just a few things to go over in terms of the pathophysiology that one needs to understand before you can talk about. The role of the surgeon is the specific function of each gut segment. And, and they are listed there um for you to have a look at when we talk about intestinal failure and its management, generally, we're talking about small bowel. So we're looking at from the ligament of rights to the IC valve and then the colon as well. So when we talk about the Jejunum, its primary function is obviously absorption of micro and macronutrients. But important to know it is a limited capacity for adaptation uh compared to the ilium. So, while the ilium may not be as involved with absorption of most macronutrients, it's very involved with fluid and electrolyte absorption as well as it's a adaptive capacity being uh far more pronounced. And that has important um factors when we consider um surgical outcomes in these patients. The colon, I think the role of the colon perhaps initially was sort of perhaps underestimated when we started talking about intestinal failure. And the role of the colon is is becoming far more recognized, particularly with fluid and electrolyte management, um which can be a problem in the intestinal failure patients. So when we talk about outcomes in um intestinal failure patients, particularly short bowel patients, there are a number of factors listed there that, that determine those outcomes and the pathophysiology around um loss of, of, of bowel. Um and certain segments of bowel is, is usually the determinant when it comes to, to the outcomes, particularly in settings where uh intestinal transplant and long term parenteral nutrition may not be available. We will go through all of those. Um The first being residual bowel length, it's important for us to understand that um Children are growing patients. So it's very difficult to give a uh outcome or a prognosis based on absolute gut length. Um Initially, your your your outcomes were based on, on measurements of absolute gut length, but we know that a neonate obviously doesn't have the same length of bowel as a five year old child. So there are a number of studies that have looked at quantifying gut length as a percentage of expected age, weight, height and, uh, the two that are displayed here, um, this over here as well as this or, um, uh, postmortem studies where they looked at, um, um, small gut length, um, and, uh related it to gestational age. And you'll see that roughly at about term, you've got about 300 centimeters of small gut length and it's similar in this study as well, about 300 centimeters. The problem is postmortem, gut length changes. Um Initially, they thought it may shrink, others thought it may, may increase in size. So, moving along, this is a study that was published in 2009 from um uh Wales at all, which looked at the gut length in alive patients that were taken to theater for surgeries other than intestinal failure surgery. The majority of patients were uh N EC or patients having tumor surgery, worms, tumors, neuroblastomas. That's that uh those kinds of diagnoses. Um And they related um gut length at various um age weight and height um from uh preterm babies up until about five years of age. And um the uh measurements are slightly different than what you find in postmortem um findings. So a uh term child has roughly about 100 and 65 centimeters above which is almost half of what, what was initially expected. Um Using gestational age or overall age versus weight versus height. You'll see that the, the height graph has a, a relatively steady um curve to it, which makes it ideal um rather than using a post conception age versus weight. So this calculation down here can be used um to calculate um the expected um length of small bowel. And that gives us a prognosis when we work out, um the uh um uh overall bile versus expected bile um ratio when we look at the segments of B, this becomes important and particularly ensure.