Pediatric gastroenterologist Sabina Ali, MD, and pediatric surgeon Sarah Cairo, MD, MPH, discuss how multidisciplinary, evidence-based care for pediatric inflammatory bowel disease (IBD) improves long-term outcomes for patients at UCSF Benioff Children’s Hospitals. They describe a recent case involving a 13-year-old boy who presented with with abdominal pain, diarrhea, unintentional weight loss and fatigue, highlighting key clinical indicators for referral. Drs. Ali and Cairo explain how the UCSF team utilizes advanced diagnostic methods, medical management strategies, surgical interventions, nutritional support and monitoring techniques, including intestinal ultrasound, which requires no sedation or bowel preparation.
Hi, my name is Sabina Lee. I'm one of the pediatric gastroenterologists at UCSF Benioff Children's Hospitals. I'm the medical director of the inflammatory bowel Disease Program at BCH Oakland and specialize in caring for children with inflammatory bowel disease. My name is Sarah Cairo, and I'm one of the pediatric general surgeons at UCSF Benioff Children's Hospitals in Oakland and San Francisco. Today we're going to talk a little bit about our pediatric inflammatory bowel disease programs and the services offered across the Bay Area. We will speak generally about the program and dive into a case example to help paint a picture of what care looks like for a patient with suspected or confirmed IBD. While I'm a surgeon and love to do surgery, I will leave the bulk of the presentation with Doctor Ali to describe the incredible advances in medical management, making the role of surgery much, much smaller. That said, throughout this talk, as in the real world, your surgery team will always be here when problems arise. Thank you, Doctor Cairo. As stated, I have a particular interest in IBD management and improving the quality of care of our patients. I'm actively involved locally and nationally in examples of Crohn's and Colitis Foundation, Improved Care Now, and part of multi-center collaborations. Let's discuss a quick case. Here we have a patient in your clinic who had complained of abdominal pain, diarrhea, and weight loss. He's 13 years old, male, previously healthy, presents to you with a 6 month history of intermittent abdominal pain. Diarrhea is actually non bloody, but has a lot of fatigue and unintentional weight loss. His parents are also really worried about his declining school attendance. You refer him to the pediatric GI service for further evaluation. Trend of his weight has been down from the 30th percentile to the 10th. Height has been trending at the 10th percentile. Abdominal exam was normal when we did a perianal exam. Multiple skin tags, and there was a possible concern of a fistula. Initial labs reveal anemia, elevated blood inflammatory markers, which are either ESR or CRP, and also noted to have a low albumin. Stool infection testing was done because of the concern of diarrhea, which was negative. But stool calprotectin was elevated at 750. We consulted surgery as we planned to schedule him for an upper endoscopy, and ileocolonoscopy, because of the concern of possible fistula, and if our surgeons can assess under anesthesia, and also requested for MR enterography. Endoscopy or colonoscopy was performed. Noted first was skin tags, which were large, almost looked like elephant ears. External perianal opening was noted, which noted to have discharge and drainage, which was purulent or serous. There was patchy discontinuous inflammation, we call it skip lesions in the colon, and deep linear ulcers also were noted in the colon. MR enterography noted segmental circumferential wall thickening of the terminal ileum, and a complex perianal fistula was identified with tracts extending through the sphincter complex. Pediatric inflammatory bowel disease, chronic immune mediated inflammation of the GI tract, it can be relapsing and remitting. Onset is common in childhood and adolescence, and patients require lifelong multidisciplinary care. There are two types, ulcerative colitis or Crohn's disease, and how do we differentiate them? Ulcerative colitis, the inflammation is noted mostly in the colon, it is continuous. And in Crohn's disease, as we described earlier, there can be skipped lesions, deep ulcers, with perianal findings, or fistulas, or sometimes abscesses. There is also an entity called very early onset IBD. This is IBD diagnosed before 6 years of age, and infantile IBD, which is diagnosed less than 2 years of age. This is a distinct entity compared with our older onset, pediatric or adult IBD. Why does it matter? There's highly likelihood of monogenic immune dysregulation in this population. The disease can be more severe and sometimes refractory, requires different diagnostic and therapy approach. We also need to have these patients undergo immunological workup. Rates of pediatric onset IBD continues to rise around the world. This is a study published in 2022. You can see 7 out of 7 studies reported 100% increasing prevalence. And 84% of studies reported increasing incidence throughout the world. There is an interplay between genetic and environmental influences. We are looking at immune responses, genetics, microbiome, and environment that interplay when we are looking at what causes IBD, when to suspect IBD. It's not only gastrointestinal symptoms, your patients, yes, can have abdominal pain, diarrhea, blood in stool, urgency to stool, nighttime stooling, but they can also have other symptoms like unusual fatigue, fevers, weight loss, recurrent mouth ulcers. Anemia, growth failure or stunting, or even delayed puberty. IBD can also present beyond the gut. These are the extraintestinal manifestations. We are looking at signs in the skin, musculoskeletal, like arthralgias, joint pains, joint swelling, liver inflammation, eye inflammation. Kidney involvement or anemia. How do we evaluate? Starting with obtaining a blood count, looking at white cell count, your platelets, your anemia, if you have one, looking at our inflammatory markers, which can be ESR, the sedimentation rate, or C reactive protein, CRP. Also important to look at the protein level called albumin. Stool infection testing, if indicated, if someone is having diarrhea, checking for stool cultures and clostridium. And also obtaining a stool test called fecal calprotectin. Other diagnostic workup includes an upper endoscopy and colonoscopy, along with biopsies, and also small bowel imaging, or called cross-sectional imaging, MR enterography. Intestinal ultrasound is a newer tool, which is available now for our patients who have inflammatory bowel disease. It's a point of care testing, which means it can be done right in a clinic. It helps to monitor your disease activity, assess your response to treatment, and check for disease reoccurrence if patient has undergone surgery for inflammatory bowel disease. So, what is the overview of our treatment? We have medical therapy, which includes use of immunomodulators or biologic therapy. We have nutritional therapy or surgery. Why pediatric IBD is so unique, it impacts growth, puberty, bone health. There is a higher risk of extensive and more aggressive disease. It affects psychosocial health. Early disease control alters long term outcomes. Thank you, Doctor Ali, for all that information. As mentioned early on, the role of surgery has fortunately decreased in the management of IBD. This is due in large part to the advances in medical management we just reviewed, as well as an enhanced understanding of disease progression. That said, there are still some general and disease-specific indications for surgical involvement. The most common indication for surgery is failure of medical treatment or significant ongoing symptoms despite medical therapy or unacceptable side effects to medical treatment. Looking at IBD a little closer, there are some cases where emergency surgery is needed. As referring providers, these things are likely to be identified in the hospital setting, but still useful to keep in mind when working with patients with confirmed or suspected IBD. The most common indications for emergency surgery are shown here and are often related to complications of toxic megacolon, obstruction and perforation. In patients with long-standing disease, even well controlled, surgery may be needed for growth restriction and stricture or risk of cancer. While patients with long-standing disease are usually closely followed by our gastroenterology and nutrition teams, these may be things picked up in primary care ordering evaluations for other indications. As you know, IBD is a spectrum, but I wanted to give a couple of examples of how the surgery team may be involved in our two main categories of disease as our role is a bit different. For Crohn's disease, for example, surgery focuses on symptom management. One common example is the management of strictures, which can present as a type of bowel obstruction involving the distal ileum or small bowel. While anti-inflammatory regimens can often treat these, long-standing strictures develop fibrosis or stiffening of the intestine wall that is less responsive to medical management. In these cases, the surgeons are sometimes asked to perform isolated bowel resection or strictureoplasty, which can be done using a combination of minimally invasive and traditional approaches. Another place the surgeons get involved in Crohn's disease is in the management of perianal disease. This can be one of the most challenging areas for the multidisciplinary team, given the sensitivity and impact on quality of life, and the interventions range from incision and drainage of an abscess to diverting stomas to allow for healing of complex wounds and fistula. As Doctor Ali has described, while Crohn's disease and ulcerative colitis both live under the umbrella of inflammatory bowel disease, they're actually quite different, and that includes the role of surgery. One key difference is the extent of bowel involved, and given that ulcerative colitis, when pathologically confirmed, is confined to the colon, surgical excision can play a more definitive role in disease management and prevention of malignant transformation. For some patients, the decision to proceed with surgery comes as a relief after years of severe colitis and complications or side effects of medical management. Depending on the patient and family, surgery often includes multiple stages starting with colectomy and ileostomy creation, a reconstructive surgery with a J pouch or ileoanal anastomosis, and closure of a diverting stoma. We don't need to get into the details here, but as listed, there are a lot of factors that play into the decision about what surgery will look like for a patient with ulcerative colitis. One of my favorite things about the IBD program here at UCSF is the early engagement of the multidisciplinary team. That means that a child early in their disease course will have the opportunity to meet with a surgeon and learn about these options early and often in the course of their treatment. I think this increases transparency and helps frame the goals for each step of the way. Thank you, Doctor Cairo for giving us an overview of how surgeons play a role in IBD care. Goals of IBD care is timely diagnosis, effective and early treatment, treat to target strategy, which is remission. Reviewing our program highlights, we offer leading edge medical and surgical treatment options, world class diagnostic and monitoring tools, specialized nutritional therapy for pediatric IBD. Interdisciplinary care, have an infusion program, partner with families and advocacy, national and international collaboration, research clinical trials, and have a transition transfer program to our adult gastroenterology. To make a referral, please call our San Francisco office at 415-353-2813. If you're looking for access to our Oakland office or East Bay locations, please call 510-428-3058. For urgent appointment or requests, please call 1-877-822-4453 or 1-877-UCHL. There are 13 clinical pediatric surgeons at UCSF practicing across the bay with a variety of expertise. Well, we are all general surgeons, we collaborate closely to take care of patients. With specialized problems to ensure all our patients receive thoughtful and individualized care. We also have a team of advanced practice providers and nurses who help coordinate the care of our patients and provide short and long-term follow-up so you and your patients don't have extended wait times. As always, please feel free to contact our team whenever questions or concerns arise. We look forward to hearing from you and collaborating on all your surgical needs. For more information, On pediatric IBD care at UCSF, please visit our website at www.UCSF Benioff Children's.org and search for inflammatory bowel disease.
