Pediatric inflammatory bowel disease (IBD) in children requires endoscopic intervention for initial diagnosis and for continued disease surveillance. Intestinal ultrasound (IU) has the potential to be less invasive while also accurate when used as a diagnostic modality for IBD. The authors of this study evaluated IU in comparison to pediatric endoscopy and fecal calprotectin testing in the diagnosis of pediatric IBD via a prospective study performed over one year.
Pediatric patients (age 2-21 years) who had previously undergone colonoscopy (including terminal ileum intubation) were divided into 2 groups consisting of patients with IBD and patients without IBD. A subsequent IU occurred one month after colonoscopy, and five segments of the intestine were evaluated (terminal ileum, ascending colon, transverse colon, descending colon, and rectum). IU studies were read by three pediatric radiologists, and all radiologists and gastroenterologists involved in the study were blinded to each other’s findings. IU documentation included bowel wall thickness, hyperemia, presence of fatty proliferation, and absence of wall stratification. A modified ultrasound-ulcerative colitis index was used as a scoring system to determine bowel wall inflammation, and bowel wall thickness was scored based on millimeter thickness. For example, a score of “0” was given for normal thickness; “1” was given for a thickness of 3 to 5 millimeters; “2” was given for a thickness of 5 to 7 millimeters; and “3” was given for a thickness greater than 7 millimeters. The presence of hyperemia, fatty proliferation, and absence of wall stratification were each given a score of “1” if present. A Mayo score was used to document endoscopic colon inflammation for ulcerative colitis while a Simple Endoscopic Score for Crohn Disease (SES-CD) was used to determine endoscopic colon inflammation for Crohn disease. Individual patient data including assessments for disease activity were collected. Disease activity was assessed by fecal calprotectin level, the Pediatric Ulcerative Colitis Activity Index (PUCAI), and the Pediatric Crohn Disease Activity Index (PCDAI).
In total, 50 patients were included in the study (median age 13.5 years; 58% female). Half of these patients were diagnosed with IBD in which 12 patients had ulcerative colitis, 12 patients had Crohn disease, and 1 patient had IBD unclassified. The other 25 patients had no IBD, and there was no difference in age distribution between these two groups. Patients with ulcerative colitis had a median PUCAI of 52.5 and a Mayo score of 8.5. Patients with Crohn disease had a median PCDAI of 42.5 and a SES-CD of 16.5. IU demonstrated bowel wall thickness in 23 patients (46%) for which 19 of these patients had IBD (82.6%). A bowel inflammation score of at least “1” was associated with 76% sensitivity, 84% specificity, 83% positive predictive value (PPV), 78% negative predictive value (NPV), and a Pearson’s chi-square being <0.001when patient with IBD were compared to patients without IBD.
Sensitivity, specificity, PPV, and NPV improved further if only moderate or severe endoscopic disease was included. Pearson correlation coefficient testing demonstrated a significant correlation between IU scoring and fecal calprotectin levels as well as IU and endoscopic scoring (Mayo score and SES-CD). No such correlation existed between IU and clinical scoring (PUCAI and PCDAI).
IU appears to have potential utility in the diagnosis of pediatric IBD, especially since this study noted good correlation with fecal calprotectin levels and endoscopic scoring systems. It also may prove to be a good non-interventional testing technique to follow up on disease activity after a diagnosis of IBD is made in a child.
Khan H, Munden M, Spence L, Jones R, Whatley J, Suppa C. Intestinal ultrasound at diagnosis of pediatric inflammatory bowel disease compared to endoscopy. Journal of Pediatric Gastroenterology and Nutrition. 2024; doi: 10.1002/jpn3.12444.\Online ahead of print.
More Data on Exclusive Enteral Nutrition as Treatment for Crohn Disease in Children
One potential therapy for some cases of Crohn disease in children is exclusive enteral nutrition (EEN). Although EEN can be effective in the treatment of Crohn disease, its associated dietary limitations potentially can lead to eating disorders. The authors of this study determined the risk of eating disorder development in the setting of EEN therapy for pediatric Crohn disease. This retrospective study occurred over a four-month period at a children’s hospital in Paris, France. A questionnaire was developed which covered basic patient information, EEN use (including duration and how it was administered), potential difficulties with EEN use, follow-up data, long-term risks, and patient opinion on EEN therapy.
Out of the 450 pediatric patients with Crohn disease that were evaluated, 92 patients (20%) were receiving EEN and only 32 patients (7%) completed the survey. Survey respondents were 32% female, and most patients (84%) were 10 years of age or older. Ileocolonic disease was present in 50% of patients, and 75% of patients had non-stricturing/non-penetrating disease. Normal growth was present in 84% of patients. All patients who completed the survey had at least one eating difficulty associated with EEN for which the main difficulty was loss of eating desire. Enteral therapy discontinuation occurred in 12 patients receiving EEN (38%) for which the primary cause was intolerance of EEN treatment. Gastrointestinal symptom continuation was present in 8 patients (25%). The average time for patients to have an improvement while on EEN was 6 weeks, and most patients (62%) received continuing support from a multidisciplinary healthcare team.
Persistent eating difficulties remained in 10 patients (32%) after a median of 5 years post EEN, and patients receiving behavioral therapy during EEN did not develop long-term feeding issues. It was noted that 19 patients (59%) recommended use of EEN as treatment for Crohn disease mainly because it avoided use of medication.
This small study demonstrated that although EEN was effective and tolerable for many pediatric patients with Crohn disease, a sizable group of patients did not tolerate EEN and had persistence of eating difficulties long after EEN had been stopped. These findings suggest that psychological therapy is essential during EEN for pediatric Crohn disease, and frequent screening for the development of possible eating disorders during EEN is required.
Sandrine C, Emmanuelle D, Jerome V, Christine M. Exclusive enteral nutrition for induction of remission in pediatric Crohn＊s disease: short- and long-term tolerance and acceptance. JPGN Reports. 2024; https://doi.org/10.1002/jpr3.12163. Online ahead of print.
Constipation in Children with Inflammatory Bowel Disease
Children with inflammatory bowel disease (IBD) typically present with abdominal pain, blood in the stool, diarrhea, and weight loss. However, such children also can have constipation which can delay a diagnosis of IBD. Since the worldwide prevalence of pediatric functional constipation (FC) may be as high as 29.6%, it is imperative to be able to delineate children with sole FC from patients with FC and organic gastrointestinal disease. The authors of this Italian study attempted to determine the prevalence of constipation in children who eventually were diagnosed with IBD.
This study was retrospective, observational, and cross-sectional in which children diagnosed with either Crohn disease or ulcerative colitis over an 8-year period were reviewed. Initial patient characteristics were obtained, including IBD symptom duration, disease location (per the Paris Classification), the Pediatric Crohn’s Disease Activity Index (PCDAI) score, the Pediatric Ulcerative Colitis Activity Index (PUCAI) score, the Ulcerative Colitis Endoscopic Index of Severity (UCEIS) score, and the Simplified Endoscopic Score for Crohn disease (SES-CD) score. Patients with IBD who also had FC based on Rome IV Criteria at the initial follow-up clinic visit or phone call were included in the study.
Full data regarding a patient’s IBD diagnosis was present in 238 patients for which 104 patients (44%) had Crohn disease, 130 patients (56%) had ulcerative colitis, and 4 patients (0.016%) had IBD Unclassified. The mean age of patients was 174 ± 47 months, and 53% of these patients were male. A total of 41 of all patients with IBD met Rome IV criteria for FC. The authors noted that 18.2% of patients with Crohn disease, 21.5% of patients with ulcerative colitis, and none of these patients with IBD Unclassified had FC. None of the patients with IBD and FC had other autoimmune diseases, and there was no statistical difference in age, gender, extra-intestinal manifestations of IBD, age at IBD diagnosis, surgery, use of induction therapy, and IBD relapse when patients with IBD with FC were compared to patients with IBD and no FC. However, patients with IBD and FC were statistically more likely to have a delay in IBD diagnosis. Patients with IBD and no FC were statistically more likely to have diarrhea or bloody diarrhea. Initial PUCAI scoring also was statistically higher for patients with ulcerative colitis and no FC. Patients with Crohn disease and FC were statistically more likely to have perianal disease changes. Patients with ulcerative colitis and FC were statistically more likely to have proctitis and left-sided colitis. Serum testing and stool testing, including fecal calprotectin levels, were similar between patients with IBD with or without FC.
Laxatives were used in 69% of patients prior to an IBD diagnosis in patients with IBD and FC with 65% of these patients receiving PEG3350 medication. A statistically significant higher serum erythrocyte sedimentation rate (ESR) level was present in patients with IBD and FC who had not received PEG3350. Interestingly, there was a statistically significant delay in IBD diagnosis in patients with IBD and FC who did not receive PEG3350 therapy.
This study demonstrates that FC can occur in a sizable percentage of children with IBD. If a child with FC is not improving on laxative therapy, further testing, including a good anal/rectal examination and possible endoscopy is warranted.
Sabrina C, Antonio C, Salomone S, Daniela P, Marianna C, Pietro B, Massimo M, Erasmo M, Annamaria S, Caterina S. The prevalence of constipation in children with new diagnosis of inflammatory bowel disease (IBD): a retrospective study. Journal of Pediatric Gastroenterology and Nutrition 2025; doi: 10.1002/jpn3.70005.\Online ahead of print.
PFAPA Outcomes in Children
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) affects children and typically is associated with recurring fevers, stomatitis, pharyngitis, and cervical adenitis. Pediatric gastroenterologists often see such patients as children with PFAPA also can have associated abdominal pain, emesis, and diarrhea. Although the cause of PFAPA is unknown, tonsillectomy seems to be effective in relieving PFAPA symptoms. The authors of this study evaluated the long-term symptoms of PFAPA after tonsillectomy.
This retrospective study occurred over a 14-year period and included all patients who had been diagnosed with PFAPA and also who had undergone a subsequent tonsillectomy. All patients with PFAPA met the Marshall criteria for this disorder. Patients who met inclusion criteria initially were contacted by mail or by phone and subsequently had data collected by phone interview. Initial symptoms prior to tonsillectomy were obtained by medical record review. Included patients and their families were queried regarding the presence of fever (defined as temperature greater than 380 Celsius) as well as the persistence of any other symptoms associated with PFAPA after tonsillectomy. Patients were divided into 4 groups: symptom resolution, persistence of PFAPA-symptoms but no fever, less/milder febrile episodes, or no clinical change.
A total of 101 patients had data available for which 82 patients participated in the study. The median patient age at the time of interview was 14.8 years (range 6 – 28.8 years). The median age of developing PFAPA symptoms was 1.8 years (range 0.1 – 16 years). The median age of tonsillectomy was 5.1 years (range 2.3 – 18.8 years). Five patients had undergone genetic testing for periodic fever syndromes in the setting of PFAPA, but no associated genetic syndromes were detected. Median long term follow-up time after tonsillectomy was 8.8 years (range 2.8 – 16.1 years). It should be noted that gastrointestinal symptoms prior to tonsillectomy included abdominal pain (35% of patients) and nausea and emesis (16% of patients).
Resolution of fever episodes 6 months after tonsillectomy occurred in 52% of patients. Persistence of non-febrile PFAPA symptoms 6 months after tonsillectomy was present in 22% of patients.The most common non-febrile PFAPA symptoms were low-grade fever, malaise, aphthous ulcers, pharyngitis, cervical adenitis, arthralgias, abdominal pain, and headache. Persistence of less/milder febrile episodes was present in 20% of patients 6 months post tonsillectomy while 1% of patients had no clinical change 6 months post tonsillectomy. Children with PFAPA symptom resolution at 6 months post tonsillectomy were significantly more likely to maintain symptoms response long term compared to those patients with a partial response 6 months post tonsillectomy. Long-term data demonstrated that 89% of patients who had initial symptom resolution post tonsillectomy still maintained symptom resolution. It was noted that 26% of patients with initial persistence of non-febrile PFAPA symptoms still had associated symptoms. Also, 35% of patients with initial less/milder febrile episodes still had symptoms. Long-term gastrointestinal symptoms after tonsillectomy consisted of abdominal pain (40%) and nausea and emesis (33%) in patients with persistent fever symptoms as well as abdominal pain (12%) and nausea and emesis (29%) in patients with non-febrile symptoms.
This study demonstrates that tonsillectomy can ameliorate periodic fever in most pediatric patients with PFAPA. However, non-febrile symptoms can continue long term, even after tonsillectomy. Such symptoms can be gastrointestinal in nature, and it is imperative to make sure non-PFAPA conditions, such as inflammatory bowel disease, are not occurring in this patient population.
Moberg T, Rydenman K, Berg S, Fasth A, Wekell P. Long-term symptoms in periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome after tonsillectomy. Journal of Pediatrics; 2025: 114424.