Obstructing Sigmoid Volvulus: An Unusual Complication in a Pediatric Patient With Ulcerative Colitis.

Acute colonic dilation in pediatric patients with ulcerative colitis (UC) raises a concern for toxic megacolon, but other rare conditions such as sigmoid volvulus may present in a similar manner. We report a rare case of a teenager with UC without prior surgery who developed an obstructing sigmoid volvulus managed with endoscopic detorsion and decompression. Colonic inflammation in patients with UC may result in a volvulus in the absence of other predisposing factors and should be considered in the differential diagnosis of patients with UC who present with obstructive symptoms with an atypical presentation.

Adipokine Resistin Levels at Time of Pediatric Crohn Disease Diagnosis Predict Escalation to Biologic Therapy.

BACKGROUND: Hypertrophy of visceral adipose tissue (VAT) is a hallmark of Crohn disease (CD). The VAT produces a wide range of adipokines, biologically active factors that contribute to metabolic disorders in addition to CD pathogenesis. The study aim was to concomitantly evaluate serum adipokine profiles and VAT volumes as predictors of disease outcomes and treatment course in newly diagnosed pediatric patients with CD. METHODS: Pediatric patients ages 6 to 20 years were enrolled, and their clinical data and anthropometric measurements were obtained. Adipokine levels were measured at 0, 6, and 12 months after CD diagnosis and baseline in control patients (CP). The VAT volumes were measured by magnetic resonance imaging or computed tomography imaging within 3 months of diagnosis. RESULTS: One hundred four patients undergoing colonoscopy were prospectively enrolled: 36 diagnosed with CD and 68 CP. The serum adipokine resistin and plasminogen activator inhibitor (PAI)-1 levels were significantly higher in patients with CD at diagnosis than in CP. The VAT volume was similar between CD and CP. Baseline resistin levels at the time of diagnosis in patients with CD who were escalated to biologics was significantly higher than in those not treated using biologic therapy by 12 months (29.8 ng/mL vs 13.8 ng/mL; P = 0.004). A resistin level of ≥29.8 ng/mL at the time of diagnosis predicted escalation to biologic therapy in the first year after diagnosis with a specificity of 95% (sensitivity = 53%; area under the curve = 0.82; P = 0.015 for model with log-scale). There was a significantly greater reduction in resistin (P = 0.002) and PAI-1 (P = 0.010) at the 12-month follow-up in patients on biologics compared with patients who were not treated using biologics. CONCLUSIONS: Serum resistin levels at diagnosis of pediatric CD predict the escalation to biologic therapy at 12 months, independent of VAT volumes. Resistin and PAI-1 levels significantly improved in patients with CD after treatment using biologics compared with those not on biologics. These results suggest the utility of resistin as a predictive biomarker in pediatric CD.

A Review of Autoimmune Enteropathy and Its Associated Syndromes.

Autoimmune enteropathy is an extremely rare condition characterized by an abnormal intestinal immune response which typically manifests within the first 6 months of life as severe, intractable diarrhea that does not respond to dietary modification. Affected individuals frequently present with other signs of autoimmunity. The diagnosis is made based on a characteristic combination of clinical symptoms, laboratory studies, and histological features on small bowel biopsy. Autoimmune enteropathy is associated with a number of other conditions and syndromes, most notably immunodysregulation polyendocrinopathy enteropathy X-linked (IPEX) syndrome and autoimmune polyglandular syndrome type 1 (APS-1). Diagnosis and treatment is challenging, and further research is needed to better understand the pathogenesis, disease progression, and long-term outcomes of these conditions.

Pediatric Colonoscopic Polypectomy Technique.

Colonoscopy with polypectomy is frequently performed in pediatric patients based on symptoms, with the majority of polyps identified being benign juvenile pedunculated polyps with a vascular stalk. This is in distinction to adults where polypectomy is often performed as part of a colon cancer screening and prevention strategy and a higher fraction of polyps are sessile and or dysplastic. In adults, polypectomy techniques emphasize a need for deeper resection to ensure complete resection of adenomas or potential carcinoma in situ. Adenomatous polyps can occur in the pediatric age group and may be associated with an underlying polyposis, hereditary or chronic inflammatory conditions. Polypectomy techniques include use of cold biopsy forceps for very small polyps, cold snare polypectomy for small sessile polyps and hot snare polypectomy for the majority of polyps in the pediatric age group. Adjuvant techniques include epinephrine volume reduction, saline-assisted polypectomy and hemostatic techniques including injection, clip application and loop application to prevent or treat post-polypectomy bleeding. Electrosurgical principles guide the settings and type of current utilized during hot snare polypectomy. Polypectomy utilizing thermal techniques is associated with a higher risk of complications compared with diagnostic colonoscopy.

A Single-center Review of Pediatric Colonoscopy Quality Indicators.

OBJECTIVES: Colonoscopy with terminal ileal (TI) intubation is an important diagnostic and therapeutic tool in the care of children with digestive diseases, especially in those with inflammatory bowel disease. Ileal intubation rate is a recognized quality indicator for pediatric colonoscopy. Our primary aim was to identify our single-center ileal intubation rate and to secondarily identify specific factors, including bowel preparation quality, procedure duration, and cecal intubation rates which affect successful ileal intubation and by extension, complete colonoscopy. METHODS: A retrospective chart review of all colonoscopies in 2015 was completed, identifying 458 procedures. Sixty-seven patients were excluded, resulting in 391 colonoscopies reviewed. RESULTS: We analyzed 391 colonoscopy procedures with a mean patient age of 14.4 ±â€Š5.3 years. The most frequent primary indications for colonoscopy included abdominal pain with "red flag" symptoms (35.5%), known inflammatory bowel disease (25.1%), and isolated abdominal pain (11.5%). Ileal intubation was achieved in 91% of all colonoscopies, with a 94.4% cecal intubation rate. Failure of ileal and cecal intubations was classified into 4 categories: disease-related conditions, bowel preparation, technical aspects, and miscellaneous issues. Potentially modifiable factors accounted for the majority of cases of failed TI intubation. The mean colonoscopy time with and without successful TI intubation were 39 and 48.1 minutes, respectively. CONCLUSIONS: Completion of colonoscopy to the TI is an essential part of a complete colonoscopy. TI intubation was possible in 91% of patients. This rate could potentially improve to 95% with optimization of modifiable factors such as improving bowel preparation or further refinement of endoscopic skills.

Biologics Delay Progression of Crohn's Disease, but Not Early Surgery, in Children.

BACKGROUND & AIMS: Up to 30% of patients with Crohn's disease (CD) require surgery within the first 5 years from diagnosis. We investigated the recent risk of bowel surgery in an inception cohort of pediatric patients with CD and whether early use of biologics (tumor necrosis factor antagonists) alters later disease course. METHODS: We collected data from the Pediatric Inflammatory Bowel Disease Collaborative Research Group registry on 1442 children (age, ≤16 y) diagnosed with CD from January 2002 through December 2014. Data were collected at diagnosis, 30 days following diagnosis, and then quarterly and during hospitalizations for up to 12 years. Our primary aim was to determine the 10-year risk for surgery in children with CD. Our secondary aim was to determine whether early use of biologics (<3 mo of diagnosis) affected risk of disease progression. RESULTS: The 10-year risk of first bowel surgery was 26%. The 5-year risk of bowel surgery did not change from 2002 through 2014, and remained between 13% and 14%. Most surgeries occurred within 3 years from diagnosis. The only predictor of surgery was disease behavior at diagnosis. CD with inflammatory behavior had the lowest risk of surgery compared to stricturing disease, penetrating disease, or both. We associated slowing of disease progression to stricturing or penetrating disease (but not surgery) with early use of biologics, but this effect only became evident after 5 years of disease. Our results indicate that biologics slow disease progression over time (hazard ratio, 0.85; 95% CI, 0.76-0.95). CONCLUSIONS: In an analysis of data from a registry of pediatric patients with CD, we found that among those with significant and progressing disease at or shortly after presentation, early surgery is difficult to prevent, even with early use of biologics. Early use of biologics (<3 mo of diagnosis) can delay later disease progression to stricturing and/or penetrating disease, but this affect could become evident only years after initial management decisions are made.

Caustic Ingestions and Foreign Bodies Ingestions in Pediatric Patients.

Children inevitably swallow foreign material accidentally or intentionally. Each type of ingestion carries their own set of risks and complications, short and long term, some requiring immediate attention while others close monitoring. Alkalotic household cleaning products and lithium button batteries are increasingly common and damage the esophagus quickly. While many toys with rare-earth metals are banned, they are already present in many households and can cause necrosis of bowel that is between the magnets. This article reviews the incidence and assessment along with current literature to provide guidelines for management of pediatric patients with suspected caustic or foreign body ingestion.

Long-term follow up of ileal pouch anal anastomosis in a large cohort of pediatric and young adult patients with ulcerative colitis.

AIM: The study's aim is to determine long-term outcomes in a large cohort of pediatric and young adult patients who underwent proctocolectomy with ileal pouch anal anastomsis (IPAA) for ulcerative colitis (UC). METHODS: Patients diagnosed with UC in childhood or adolescence (age≤21years) who underwent IPAA in childhood, adolescence, or young adulthood between 1982 and 1997 were contacted to determine pouch history, complications, and quality of life. RESULTS: Data were obtained from 74 patients out of a previously reported cohort. Median age at diagnosis of UC was 15years and at surgery was 18years. Median follow-up was 20years. Complications during follow-up were pouchitis (45%), strictures (16%), fistulae (30%), obstruction (20%), and change of diagnosis to Crohn's (28%). Twenty-three percent reported no complications. Fourteen percent had pouch failure, with Crohn's and fistulae reported to be the most frequent complications. Seventy-nine percent reported being very satisfied at 20years follow-up. CONCLUSION: To our knowledge, this study represents the largest cohort with the longest follow-up of pediatric and young adult patients undergoing IPAA for UC. Change in diagnosis to Crohn's and development of fistulae are risk factors for pouch failure. Despite reported complications, IPAA remains an excellent option for pediatric patients with UC.

Colonic polyps and polyposis syndromes in pediatric patients.

PURPOSE OF REVIEW: Gastrointestinal polyps are commonly encountered during childhood and are one of the most common causes of rectal bleeding in this age group. Most polyps are benign and located in the colon, with the most frequent type being juvenile polyps. However, in older pediatric patients, if multiple polyps are present, in patients who have a positive family history, or if polyps are located outside of the colon, either adenomatous polyps or polyps associated with genetic abnormalities are more common. RECENT FINDINGS: Imaging techniques such as ultrasound and computed tomographic colonoscopy have recently been utilized to identify simple juvenile colonic polyps in children with rectal bleeding in whom there is a high index of suspicion. Colonoscopy with polypectomy is still required for histologic evaluation and resection of the polyp. There have been significant advances in genetic testing and management of hereditary gastrointestinal cancer syndromes with onset in childhood or adolescence that may ultimately reduce long-term morbidity and mortality. In addition to enhanced gastrointestinal and extraintestinal malignancy screening for affected individuals, specific gene mutations within a given condition such as adenomatous polyposis coli may predict clinical course and timing of specific interventions such as colectomy. In other conditions such as phosphatase and tensin homolog hamartoma tumor syndrome, phenotype may not be predicted by genotype. SUMMARY: Pediatricians, pediatric gastroenterologists, and adult gastroenterologists caring for children should understand how to differentiate benign polyps in the pediatric age group from those associated with a higher risk of complications including recurrence risk and risk of development of intestinal or extraintestinal malignancy. Recent advances in genetic testing, as well as development of consensus guidelines, are key in the identification, screening, and follow-up of children and adolescents with polyposis syndromes.

Clinical Presentation and Five-Year Therapeutic Management of Very Early-Onset Inflammatory Bowel Disease in a Large North American Cohort.

OBJECTIVE: To evaluate the presentation, therapeutic management, and long-term outcome of children with very early-onset (VEO) (≤ 5 years of age) inflammatory bowel disease (IBD). STUDY DESIGN: Data were obtained from an inception cohort of 1928 children with IBD enrolled in a prospective observational registry at multiple centers in North America. RESULTS: One hundred twelve children were ≤ 5 years of age with no child enrolled at <1 year of age. Of those, 42.9% had Crohn's disease (CD), 46.4% ulcerative colitis (UC), and 10.7% had IBD-unclassified. Among the children with CD, children 1-5 years of age had more isolated colonic disease (39.6%) compared with 6- to 10-year-olds (25.3%, P = .04), and 11- to 16-year-olds (22.3%, P < .01). The change from a presenting colon-only phenotype to ileocolonic began at 6-10 years. Children 1-5 years of age with CD had milder disease activity (45.8%) at diagnosis compared with the oldest group (28%, P = .01). Five years postdiagnosis, there was no difference in disease activity among the 3 groups. However, compared with the oldest group, a greater proportion of 1- to 5-year-olds with CD were receiving corticosteroids (P < .01) and methotrexate (P < .01), and a greater proportion of 1- to 5-year-olds with UC were receiving mesalamine (P < .0001) and thiopurine immunomodulators (P < .0002). CONCLUSIONS: Children with VEO-CD are more likely to have mild disease at diagnosis and present with a colonic phenotype with change to an ileocolonic phenotype noted at 6-10 years of age. Five years after diagnosis, children with VEO-CD and VEO-UC are more likely to have been administered corticosteroids and immunomodulators despite similar disease activity in all age groups. This may suggest development of a more aggressive disease phenotype over time.

Concomitant Use of Immunomodulators Affects the Durability of Infliximab Therapy in Children With Crohn's Disease.

BACKGROUND & AIMS: It is important to determine the effects of immunomodulators on the ability of children to remain on infliximab therapy for Crohn's disease (durability of therapy), given the potential benefits and risks of concomitant therapy-especially with thiopurines in male patients. We investigated how immunomodulatory treatment affects the durability of infliximab therapy. METHODS: We collected data from the Pediatric Inflammatory Bowel Disease Collaborative Research Group Registry, from January 2002 through August 2014, on 502 children with Crohn's disease who participated in a prospective multicenter study. Data were collected from patients who received at least a 3-dose induction regimen of infliximab, and their concomitant use of immunomodulators: no thiopurine or methotrexate treatment, treatment for 6 months or less during infliximab therapy, or treatment for more than 6 months during infliximab therapy. RESULTS: The probabilities (± standard error) that children remained on infliximab therapy for 1 year, 3 years, and 5 years after the treatment began were 0.84 ± 0.02, 0.69 ± 0.03, and 0.60 ± 0.03, respectively. Age, sex, and disease extent or location did not affect the durability of infliximab therapy. Greater length of concomitant use of immunomodulators was associated with increased time of infliximab therapy. The probability that patients with more than 6 months of immunomodulator use remained on infliximab therapy for 5 years was 0.70 ± 0.04, compared with 0.48 ± 0.08 for patients who did not receive immunomodulators and 0.55 ± 0.06 for patients who received immunomodulators for 6 months or less (P < .001). In boys who received immunomodulators for 6 months or more after starting infliximab, the overall durability of infliximab therapy was greater among patients receiving methotrexate than thiopurine (P < .01); the probabilities that they remained on infliximab therapy for 5 years were 0.97 ± 0.03 vs 0.58 ± 0.08, respectively. CONCLUSIONS: In children with Crohn's disease, concomitant treatment with an immunomodulator for more than 6 months after starting infliximab therapy increases the chances that patients will remain on infliximab. In boys, methotrexate appears to increase the durability of infliximab therapy compared with thiopurine.

Impact of abdominal visceral adipose tissue on disease outcome in pediatric Crohn's disease.

BACKGROUND: Increased abdominal visceral adipose tissue (VAT) is associated with systemic inflammation. The influence of VAT on pediatric inflammatory bowel disease (IBD) has not been studied. The objective of this study was to investigate the differences in VAT between pediatric patients with IBD and age-matched controls and identify associations between VAT and Crohn's disease (CD) outcomes. METHODS: Single-center retrospective cohort study of 114 pediatric patients with IBD (101 CD and 13 ulcerative colitis) who had abdominal computed tomography at diagnosis. VAT volumes were measured from computed tomography images. A control group of 78 age-matched patients without IBD who had abdominal computed tomography was selected for comparison. RESULTS: Median VAT was 634 cm (interquartile range, 411-1041) in the IBD group and 659 cm (interquartile range, 394-1015) in the controls. IBD group had 33% higher VAT than controls (95% confidence interval [CI], 11-58) P = 0.002 after adjusting for body mass index and age. In patients with CD, higher VAT was associated with fistulizing or fibrostenotic disease (odds ratio [OR], 1.7; 95% CI, 1.1-2.9; P = 0.03), CD hospitalizations (OR, 1.9; 95% CI, 1.2-3.4; P = 0.01), moderate or severe disease activity scores (OR, 1.8; 95% CI, 1.1-3.1; P = 0.02), and shorter intervals from diagnosis to surgery (hazard ratio, 1.4; 95% CI, 1.0-2.0; P = 0.05) after adjusting for body mass index and age. CONCLUSIONS: At diagnosis, pediatric patients with IBD have higher adjusted VAT volumes than age- and body mass index-matched controls. Higher VAT volumes in pediatric patients with CD predicted more hospitalizations, increased likelihood of complicated disease, shorter interval from diagnosis to surgery, and higher disease activity scores at diagnosis.

Metastatic osteosarcoma to the stomach and ascending colon in a pediatric patient causing gastrointestinal hemorrhage.

Osteosarcoma metastasis to the gastrointestinal tract is a rare phenomenon (Horiuchi A, Watanabe Y, Yoshida M, et al.: Metastatic osteosarcoma in the jejunum with intussusception: report of a case. Surg Today 2007;37:440-2). Gastrointestinal metastases may cause intussusception, bowel obstruction, or hemorrhage (Horiuchi A, Watanabe Y, Yoshida M, et al.: Metastatic osteosarcoma in the jejunum with intussusception: report of a case. Surg Today 2007;37:440-2; Chondramohan K, Somanathan T, Kusamakumary P: Metastatic osteosarcoma causing intussusception. J Pediatr Surg 2003;38(E44):1-3; Hung GY, Chiou TJ, Hsieh YL, et al.: Intestinal metastasis causing intussusception in a patient treated for osteosarcoma with history of multiple metastases: a case report. Jpn J Clin Oncol 2001;31:165-167). We report a case of a 17 year old male with osteosarcoma metastatic to the stomach and ascending colon, causing significant chronic gastrointestinal hemorrhage. Surgical resection was performed due to persistent, symptomatic anemia. The patient is free of recurrent hemorrhage at 24months after metastectomy. Resection of gastrointestinal metastases of osteosarcoma offers good palliation of chronic hemorrhage related to these lesions.

Outcome following aminosalicylate therapy in children newly diagnosed as having ulcerative colitis.

OBJECTIVES: Despite a paucity of published supporting data, 5-aminosalicylate (5-ASA) use in pediatric ulcerative colitis (UC) is common. The present study describes the use and outcome of a large multicenter inception cohort of children with UC treated with 5-ASA. METHODS: Data were obtained from the Pediatric Inflammatory Bowel Disease Collaborative Research Group Registry, a prospective North American observational study of children newly diagnosed as having inflammatory bowel disease ages 16 years or younger. Patient data are recorded at diagnosis, 30 days, and then quarterly. Patients are managed by physician dictate, not protocol. Disease activity is classified by physician global assessment. The primary outcome examined was corticosteroid (CS) free, inactive UC at 1 year following initiation of 5-ASA within 30 days of diagnosis (with or without concomitant CS use) without the need for rescue therapy (immunomodulators, biologics, or colectomy). RESULTS: Study subjects included 213 patients newly diagnosed as having UC who received oral 5-ASA compounds (115 of whom also received CS) during the first 30 days after diagnosis, and no other oral therapies for the treatment of UC. Of these 213 patients, 86 (40%) were CS free and physician global assessment inactive at 1 year without rescue. Outcome was not associated with disease severity at diagnosis, demographic or laboratory factors examined, or initial dose of 5-ASA used. CONCLUSIONS: Forty percent of children taking 5-ASA as primary maintenance therapy at diagnosis are in CS-free remission after 1 year of treatment. Further pediatric studies will be needed to address whether increased adherence and/or higher dosing schedules will improve outcomes.

Budesonide use in pediatric Crohn disease.

BACKGROUND AND AIM: Budesonide (BUD) is being used in pediatric Crohn disease (CD) because it is believed to have the potential to reduce corticosteroid-related toxicity; however, few data are available describing its use. The aim of the present study was to describe BUD use in an inception cohort of pediatric patients with CD. METHODS: Data were derived from the prospective Pediatric IBD Collaborative Research Group Registry established in 2002 in North America. Use of BUD in children with CD was examined. RESULTS: BUD was used in 119 of 932 (13%) of children with newly diagnosed CD, with 56 of 119 (47%) starting BUD ≤ 30 days of diagnosis (26/56 with ileum and/or ascending colon [IAC] disease). BUD was used as monotherapy (9%), in combination with 5-aminosalicylates (77%), or in combination with immunomodulators (43%). Forty-three percent (24/56) went on to receive conventional corticosteroid at some point following their first BUD course. For the 63 of 119 (53%) who started BUD beyond the diagnosis period, 51 of 63 (81%) also received prednisone, with BUD used as a means of weaning from prednisone in 17 of 63 (27%). Patients with IAC disease who received BUD ≤ 30 days of diagnosis were just as likely to have received conventional corticosteroids by 1 year as were those who did not receive BUD ≤ 30 days of diagnosis. Two-thirds (77/119) of patients received BUD for ≤ 6 months. CONCLUSIONS: BUD is being used among pediatric patients newly diagnosed as having CD, although the majority does not have disease limited to the IAC. BUD monotherapy was rare, and further data are required to better define the role of BUD in the treatment of pediatric CD.