Imaging and endoscopic tools in pediatric inflammatory bowel disease: What's new?

Pediatric inflammatory bowel disease (IBD) is a chronic inflammatory disorder, with increasing incidence and prevalence worldwide. There have been recent advances in imaging and endoscopic technology for disease diagnosis, treatment, and monitoring. Intestinal ultrasound, including transabdominal, transperineal, and endoscopic, has been emerging for the assessment of transmural bowel inflammation and disease complications (e.g., fistula, abscess). Aside from surgery, IBD-related intestinal strictures now have endoscopic treatment options including through-the-scope balloon dilatation, injection, and needle knife stricturotomy and new evaluation tools such as endoscopic functional lumen imaging probe. Unsedated transnasal endoscopy may have a role in patients with upper gastrointestinal Crohn's disease or those with IBD with new upper gastrointestinal symptoms. Improvements to dysplasia screening in pediatric patients with longstanding colonic disease or primary sclerosing cholangitis hold promise with the addition of virtual chromoendoscopy and ongoing research in the field of artificial intelligence-assisted endoscopic detection. Artificial intelligence and machine learning is a rapidly evolving field, with goals of further personalizing IBD diagnosis and treatment selection as well as prognostication. This review summarized these advancements, focusing on pediatric patients with IBD.

Outcomes of Therapy for Vulvar Manifestation of Inflammatory Bowel Disease in Adolescents.

BACKGROUND: Vulvar manifestations of inflammatory bowel disease (IBD) are variable in presentation and challenging to treat. We describe vulvar manifestations and treatment response in female adolescents with IBD. CASES: We identified 6 patients with vulvar manifestations of IBD and documented treatments using retrospective chart review. Vulvar symptoms occurred without gastrointestinal (GI) symptoms in 1 patient. For the remaining 5 patients, 2 had GI symptoms before the onset of vulvar symptoms (mean time difference, 4.5 years); 3 patients had vulvar symptoms precede the onset of GI symptoms (mean time difference, 3.3 years). Vulvar IBD manifestations included pain, 100% (n = 6); enlargement, "fullness" or "edema" of the labia minora or majora, 66% (n = 4); ulcers, 50% (n = 3); and abscess, 50% (n = 3). Gynecologic procedures included biopsies, incision and drainages, and partial vulvectomies. All patients were treated with multiple systemic therapies. None of the patients responded to surgical or medical treatment alone; all had recalcitrant vulvar symptoms. SUMMARY AND CONCLUSION: Vulvar manifestations of IBD might precede GI symptoms in adolescents with IBD. Treatment is challenging and in this series, systemic therapies were the most successful in achieving symptomatic improvement.

Lack of Mucosal Healing From Modified Specific Carbohydrate Diet in Pediatric Patients With Crohn Disease.

Exclusive enteral nutrition is effective in pediatric Crohn disease but challenging as maintenance therapy. There is interest in food-based therapies such as the specific carbohydrate diet (SCD) but paucity of data on efficacy and effect on mucosal healing, an evolving target of IBD therapy. We conducted a retrospective review of the mucosal healing effect of the SCD in pediatric Crohn disease (CD). The endoscopic findings for children younger than 18 years with CD treated exclusively with the SCD or modified SCD (mSCD; SCD + addition of "illegal foods") were reviewed before and after the diet. Ileocolonoscopic examinations were scored according to the Simple Endoscopic Score for CD and findings on upper endoscopy were described. Seven subjects were identified, all on mSCD. The average age at starting the SCD was 11 ±â€Š3.4 years and median duration of SCD/mSCD therapy was 26 months. All subjects reported no active symptoms before repeat endoscopic evaluation on mSCD, the majority had consistently normal C-reactive protein, albumin and hematocrit assessments, and mildly elevated fecal calprotectin (>50 µg/g, median 201, range 65-312) at any point within 3 months before the repeat endoscopy. One patient showed complete ileocolonic healing but persistent upper gastrointestinal tract ulceration. Complete macroscopic mucosal healing of both the ileocolon and upper gastrointestinal tract was not seen in any patient.

Resource utilization after implementing a hospital-wide standardized feeding tube placement pathway.

BACKGROUND/PURPOSE: Children requiring gastrostomy/gastrojejunostomy tubes (GT/GJ) are heterogeneous and medically complex patients with high resource utilization. We created and implemented a hospital-wide standardized pathway for feeding device placement. This study compares hospital resource utilization before and after pathway implementation. METHODS: We performed a retrospective cohort study comparing outcomes through one year of follow-up for consecutive groups of children undergoing GT/GJ placement prepathway (n=298, 1/1/2010-12/31/2011) and postpathway (n=140, 6/1/2013-7/31/2014) implementation. We determined the change in the rate of hospital resource utilization events and time to first event. RESULTS: Prior to implementation, 145 (48.7%) devices were placed surgically, 113 (37.9%) endoscopically and 40 (13.4%) using image guidance. After implementation, 102 (72.9%) were placed surgically, 23 (16.4%) endoscopically and 15 (10.7%) using image guidance. Prior to implementation, 174/298 (58.4%) patients required additional hospital resource utilization compared to 60/143 (42.0%) corresponding to a multivariate adjusted 38% reduced risk of a subsequent feeding tube related event. CONCLUSIONS: Care of tube-feeding dependent patients is spread among multiple specialists leading to variability in the preoperative workup, intraoperative technique and postoperative care. Our study shows an association between implementation of a standardized pathway and a decrease in hospital resource utilization.

Precolectomy Therapy Does Not Alter Histologic Findings Distribution in Resected Colon in Pediatric Ulcerative Colitis.

OBJECTIVES: Medically intractable pediatric ulcerative colitis can lead to colectomy after which patients commonly receive an ileoanal pouch. Postoperative complications are more common in patients with Crohn disease, a diagnosis that may be rendered after the colectomy specimen is examined. Because most children are likely to be exposed to medications before colectomy, we sought to examine whether such exposure influences the distribution of the inflammation within the resected colon and therefore potentially raise questions about the diagnosis accuracy. METHODS: We conducted a retrospective cohort study of 32 pediatric ulcerative colitis cases undergoing colectomy from 2007 to 2014 for clinical data and precolectomy treatment history. The resected colon histology was reviewed independently by 2 blinded pathologists. The acute/active inflammation was scored using the modified Riley score for 3 colonic segments (proximal, transverse, and distal colon) for each patient. Linear mixed-effects models were used to evaluate possible association between acute/active inflammation scores at various sites and medication use. RESULTS: Twelve cases (38%) showed decreasing acute inflammation score distally to proximally, 8 (25%) had increasing scores, and 12 cases showed no change. Patients were most commonly exposed to corticosteroids, followed by anti-tumor necrosis factor antibodies. There was no statistically or clinically significant change in the histologic scores across the colonic segments of the resected colon in association with exposure to any specific medication or combination of medications, sex, age at diagnosis and surgery, or duration of disease. CONCLUSIONS: Precolectomy therapy does not seem to influence the distribution of inflammation within the resected colon.

Total colectomy for ulcerative colitis in children: when are we operating?

PURPOSE: Ulcerative colitis (UC) in children is frequently severe and treatment-refractory. While medical therapy is well standardized, little is known regarding factors that contribute to surgical indications. Our aim was to identify factors associated with progression to colectomy in a large cohort of pediatric UC patients. METHODS: We conducted a retrospective cohort study using the Pediatric Health Information System database. We identified all patients under age 18 discharged between January 1, 2004 and September 30, 2011 with a primary diagnosis of UC. Primary outcome was odds of total colectomy. RESULTS: Of 8,688 patients, 240 (2.8 %) underwent colectomy. Compared with non-operative patients, a greater proportion of colectomy patients received advanced therapies during admission, including corticosteroids (84.2 vs. 71.3 %) and biological therapy (25.4 vs. 13.6 %). Odds of colectomy were increased with malnutrition (OR 1.86), anemia (OR 2.17), electrolyte imbalance (OR 2.31), and Clostridium difficile infection (OR 1.69). TPN requirement also independently predicted colectomy (OR 3.86). Each successive UC admission significantly increased the odds of colectomy (OR 1.08). CONCLUSION: These data identify factors associated with progression to colectomy in children hospitalized with UC. Our findings help to identify factors that should be incorporated into future studies aiming to reduce the variability in surgical treatment of childhood UC.

The pediatric pouch in inflammatory bowel disease: a primer for the gastroenterologist.

Pediatric severe ulcerative colitis that is resistant to current medical treatment can successfully be managed surgically with a colectomy, ileal pouch creation and pouch-anal anastomosis. Key issues that should be considered and discussed before the pouch option can be offered include alternative surgical procedures, pouch function expectations, risk of surgical leak, pelvic sepsis, anastomotic strictures, acute and chronic pouch inflammation, Crohn's disease of the pouch and risk of reduced fertility for females. A long-term risk is malignancy of the residual colonic tissue. The decision to proceed with a pouch or not poses a substantial emotional burden to the child and family. Despite the risk of surgical complications and pouch inflammatory and functional challenges, the overwhelming majority of children and their families are satisfied with their pouch surgery outcomes. Further study is needed to assess preoperative risk predictors, prevention and treatment of complications.

Loss of interleukin-10 signaling and infantile inflammatory bowel disease: implications for diagnosis and therapy.

BACKGROUND & AIMS: Homozygous loss of function mutations in interleukin-10 (IL10) and interleukin-10 receptors (IL10R) cause severe infantile (very early onset) inflammatory bowel disease (IBD). Allogeneic hematopoietic stem cell transplantation (HSCT) was reported to induce sustained remission in 1 patient with IL-10R deficiency. We investigated heterogeneity among patients with very early onset IBD, its mechanisms, and the use of allogeneic HSCT to treat this disorder. METHODS: We analyzed 66 patients with early onset IBD (younger than 5 years of age) for mutations in the genes encoding IL-10, IL-10R1, and IL-10R2. IL-10R deficiency was confirmed by functional assays on patients' peripheral blood mononuclear cells (immunoblot and enzyme-linked immunosorbent assay analyses). We assessed the therapeutic effects of standardized allogeneic HSCT. RESULTS: Using a candidate gene sequencing approach, we identified 16 patients with IL-10 or IL-10R deficiency: 3 patients had mutations in IL-10, 5 had mutations in IL-10R1, and 8 had mutations in IL-10R2. Refractory colitis became manifest in all patients within the first 3 months of life and was associated with perianal disease (16 of 16 patients). Extraintestinal symptoms included folliculitis (11 of 16) and arthritis (4 of 16). Allogeneic HSCT was performed in 5 patients and induced sustained clinical remission with a median follow-up time of 2 years. In vitro experiments confirmed reconstitution of IL-10R-mediated signaling in all patients who received the transplant. CONCLUSIONS: We identified loss of function mutations in IL-10 and IL-10R in patients with very early onset IBD. These findings indicate that infantile IBD patients with perianal disease should be screened for IL-10 and IL-10R deficiency and that allogeneic HSCT can induce remission in those with IL-10R deficiency.

Health supervision in the management of children and adolescents with IBD: NASPGHAN recommendations.

Ulcerative colitis (UC) and Crohn disease (CD), collectively referred to as inflammatory bowel disease (IBD), are chronic inflammatory disorders that can affect the gastrointestinal tract of children and adults. Like other autoimmune processes, the cause(s) of these disorders remain unknown but likely involves some interplay between genetic vulnerability and environmental factors. Children, in particular with UC or CD, can present to their primary care providers with similar symptoms, including abdominal pain, diarrhea, weight loss, and bloody stool. Although UC and CD are more predominant in adults, epidemiologic studies have demonstrated that a significant percentage of these patients were diagnosed during childhood. The chronic nature of the inflammatory process observed in these children and the waxing and waning nature of their clinical symptoms can be especially disruptive to their physical, social, and academic development. As such, physicians caring for children must consider these diseases when evaluating patients with compatible symptoms. Recent research efforts have made available a variety of more specific and effective pharmacologic agents and improved endoscopic and radiologic assessment tools to assist clinicians in the diagnosis and interval assessment of their patients with IBD; however, as the level of complexity of these interventions has increased, so too has the need for practitioners to become familiar with a wider array of treatments and the risks and benefits of particular diagnostic testing. Nonetheless, in most cases, and especially when frequent visits to subspecialty referral centers are not geographically feasible, primary care providers can be active participants in the management of their pediatric patients with IBD. The goal of this article is to educate and assist pediatricians and adult gastroenterology physicians caring for children with IBD, and in doing so, help to develop more collaborative care plans between primary care and subspecialty providers.