Four intestinal ultrasound scores and bowel wall thickness alone correlated well with pediatric ulcerative colitis disease activity.

OBJECTIVES: Intestinal ultrasound (IUS) is a noninvasive tool in ulcerative colitis (UC), but scoring systems have mostly been developed for adults, Crohn's disease, and flaring UC. Our aim was to evaluate the performance of bowel wall thickness (BWT) and four IUS scores in pediatric patients with newly diagnosed UC. METHODS: Patients <18 years old with suspected UC were prospectively enrolled. Baseline IUS was done, and ulcerative colitis intestinal ultrasound score (UC-IUS), Milan criteria, simple pediatric activity ultrasound score (SPAUSS), and Civatelli index were calculated. Mayo endoscopic segment subscore, pediatric ulcerative colitis activity index (PUCAI), and biomarkers were correlated with IUS using nonparametric and receiver operating characteristic analyses. RESULTS: Fifty-two patients (56% male, median age 13.9 years, interquartile range [IQR] 11.2-16.3) with 206 colon segments were included. Patients who needed hospitalization (n = 27/52) had significantly worse IUS (BWT and all scores) compared to those not hospitalized. For all patients, IUS scores and BWT significantly correlated with baseline endoscopic, clinical, and biochemical disease activity (rho = 0.32-0.67, p < 0.05). BWT (τb = 0.53), UC-IUS (τb = 0.55), and Milan (τb = 0.52) had the strongest endoscopic correlations. For differentiating between endoscopic disease severity, BWT, UC-IUS, and Milan, had the highest areas under the curve (0.89-0.93). Using BWT alone, a thinner cut-off had improved sensitivity while maintaining high specificity: ≥2.5 mm for moderate/severe endoscopic inflammation (sensitivity 66%; specificity 94%) and ≥3.5 mm for severe endoscopic inflammation (sensitivity 92%; specificity 86%). CONCLUSIONS: BWT and all four IUS scores correlated well with endoscopic, clinical, and biochemical disease activity, and was another useful marker of severity in identifying patients needing hospitalization. Pediatric patients needed a thinner BWT cut-off, which should be accounted for when developing pediatric-specific scores. BWT alone may be just as clinically useful as composite US scores.

Pediatric Patient and Caregiver Satisfaction With the Use of Transabdominal Bowel Ultrasound in the Assessment of Inflammatory Bowel Diseases.

OBJECTIVES: Transabdominal bowel ultrasound (TABUS) is emerging as an attractive, noninvasive tool in inflammatory bowel disease (IBD). Patient and caregiver experience with TABUS is not well described. We aimed to determine pediatric patient and caregiver satisfaction with TABUS and the impact of IBD severity, gender, age, and a history of anxiety on satisfaction. METHODS: Pediatric patients (0-18 years old) with suspected IBD prospectively underwent baseline TABUS, magnetic resonance enterography (MRE), blood work, stool studies, and endoscopy. Patients and their caregiver each completed a cross-sectional satisfaction questionnaire (5-point Likert scale) after the baseline investigations. RESULTS: There were 54 patients included (67% male). The majority were completely satisfied and strongly agree TABUS was better tolerated than other investigations, regardless of disease severity ( P > 0.05). Patients with higher Simple Endoscopic Score for Crohn Disease (SES-CD) scores felt that TABUS increased their understanding of their IBD ( P < 0.05) and disease location ( P < 0.05). Patients with Crohn disease had similar responses to those with ulcerative colitis, but more strongly agreed that TABUS was better than MRE and endoscopy ( P < 0.05). Those with anxiety did not have an increased level of worry about potential ultrasound findings ( P > 0.05). CONCLUSIONS: Pediatric patients and their caregivers were highly satisfied with TABUS, preferring it to other modalities. It did not lead to increased worry, and was particularly important in those with severe IBD. These findings support wider implementation of this well tolerated and preferred monitoring tool in pediatrics.

Dysphagia is a strong predictor of revision supraglottoplasty in pediatric laryngomalacia.

BACKGROUND: There is growing research on the association between swallowing dysfunction and laryngomalacia. Supraglottoplasty is the surgical intervention used to treat laryngomalacia, however a portion of patients who undergo this procedure will require a revision surgery. Predictive risk factors for revision supraglottoplasty in patients with laryngomalacia are not well understood, and previous studies failed to evaluate swallowing systematically. HYPOTHESIS: We predict a significant association between swallowing dysfunction and revision supraglottoplasty for patients with laryngomalacia. METHODS: This was a retrospective cohort study of consecutive patients between 2013 and 2023 at a tertiary pediatric care centre (Stollery Children's Hospital). All patients had an endoscopic diagnosis of laryngomalacia by a staff otolaryngologist and at minimum a systematic clinical swallowing assessment by a speech language pathologist, with an instrumental assessment as needed. Patients with genetic or neurological comorbidities, lack of follow up information, or age of >3 years were excluded. Clinical and instrumental swallow data, demographic information, surgical outcomes and revision surgeries were documented and collected. Univariate analysis was done to determine associations between variables and revision supraglottoplasty. Binary logistic regression was done to determine independent predictors of revision supraglottoplasty. RESULTS: 214 patients met the inclusion criteria and were analyzed in the study. 24 patients (11 %) required revision supraglottoplasty. 118 out of the 214 patients (55 %) had an instrumental assessment completed (FEES or VFSS). Of those, 92 (78 %) had abnormal findings on instrumental assessments. Univariate analysis showed Type 2 laryngomalacia (P = 0.017), presence of aspiration (P=<0.001), presence of cyanosis (P = 0.002) and abnormal findings on an instrumental assessment (P = 0.013) to be significantly associated with the need for revision supraglottoplasty. Binary regression analysis showed aspiration (OR = 5.6 {2.087-14.889}, P=<0.001) and cyanosis (OR = 5.3 {1.852-15.181}, P = 0.002) to be the only independent predictors of revision supraglottoplasty. CONCLUSION: Presence of aspiration is a strong predictive factor for revision supraglottoplasty in patients with laryngomalacia, when swallowing is evaluated systematically. More prospective research is needed to understand the relationship between swallowing dysfunction, laryngomalacia and surgery.

Pediatric cricopharyngeal achalasia: A systematic review.

BACKGROUND: Pediatric dysphagia is a commonly encountered clinical problem, with primary cricopharyngeal achalasia being a rare etiology. Management options for this condition include observation, medical and surgical management. The goal of this review paper was to summarize the current literature on the topic with respect to patient presentation, diagnosis and surgical management options. METHODS: The inclusion criteria were as follows: pediatric patients less than age 18 years, case series featuring equal to or greater than two patients, and any article that described surgical interventions pertaining to primary cricopharyngeal achalasia. Exclusion criteria included patients greater than 18 years of age, those with lower esophageal sphincter pathology (achalasia), non-English articles and case reports consisting of only one patient. A search was run on the PubMed/Medline, OVID, EMBASE, Cochrane, and Web of Science databases on November 1, 2023. The validated bias tool from the Institute of Health Economics was used for bias screening. The results were synthesized using pooled analysis and descriptive statistics. RESULTS: Ten case series were identified that met inclusion criteria. The most common symptoms at presentation were choking/regurgitation, aspiration and failure to thrive. The most utilized diagnostic test that identified cricopharyngeal achalasia abnormality was the video fluoroscopic swallow study. Management options are dictated by the patient's symptoms and include observation, treating concomitant co-morbidities, balloon dilation, botulinum injection, and endoscopic or open cricopharyngeal myotomies. While botulinum toxin injections are reported to be effective, they often need to be repeated and thus offer a temporizing strategy to allow the natural history of the condition to declare itself. Surgical myotomy represents a more definitive management strategy compared to balloon dilation but has associated risks. DISCUSSION: There are limited studies available regarding the diagnosis and management of CPA in children. The studies that are available are mainly case series, with low sample sizes and heterogeneous data. This systematic review highlights the importance of keeping this diagnosis within the differential for infants and pediatric patients with dysphagia to minimize delays in diagnosis and provides updated data on the presentation and surgical management of this condition. There was a limitation of evidence in this systematic review, including the small number of articles that were identified, and limited sample size of patients within the articles themselves. Consequently, the analysis was not amenable to a meta-analysis.

Prevalence and Risk Factors for Swallowing Dysfunction in Patients With Laryngomalacia Treated Medically and Surgically.

Objective: To identify the prevalence, pattern, and nature of swallowing dysfunction (SwD) in a consecutive cohort of patients with laryngomalacia (LM), and to determine factors associated with a higher burden of SwD. Study Design: This was a retrospective review of consecutive patients diagnosed with LM by 2 pediatric otolaryngologists between 2013 and 2022 and a minimum of 3-month follow-up. Setting: Tertiary care pediatric otolaryngology referral center. Methods: Consecutive cohort of patients less than 3 years old with LM diagnosed on flexible laryngoscopy were reviewed. Patients with incomplete follow-up, lack of swallowing assessment, and genetic conditions or syndromes were excluded. All patients underwent at minimum a systematic clinical swallowing evaluation by a speech-language pathologist specialized in pediatric dysphagia. Patients with concerning clinical exams underwent instrumental swallow evaluation (Videofluoroscopic Swallow Study [VFSS] or Flexible Endoscopic Evaluation of Swallowing [FEES]). The prevalence of abnormalities of clinical swallowing evaluation, instrumental swallow evaluation data, and details of management were collected. Results: Two hundred and twelve patients met criteria and were included in the final analysis. One hundred and fifteen patients (54%) had an instrumental assessment (VFSS or FEES). Of the instrumental assessments performed, 96 (69%) were abnormal. Of the total patient cohort, 55 (26%) had laryngeal penetration and/or aspiration. One hundred and seventeen (55%) had clinical or instrumental indications for intervention, with 18 (8%) requiring tube feeding. Patients with severe LM and those treated surgically had a statistically significant higher rate of penetration and aspiration. Conclusion: Patients with LM have a high burden of dysphagia requiring medical intervention. The authors advocate for routine and systematic assessment of all patients with LM for swallowing dysfunction.

Video Capsule Endoscopy in a Pediatric Patient With Hematemesis While on Ventricular Assist Device Support.

We report the first case of video capsule endoscopy usage to diagnose gastrointestinal bleeding in a pediatric patient on a ventricular assist device. The outcomes of this case are consistent with the findings of reports in adult patients, showing no patient complications, no pacemaker or ventricular assist device interactions, and successful identification of a gastrointestinal source of bleeding. Use of video capsule endoscopy in this patient changed the management plan and eliminated the need for further invasive investigations highlighting the potential utility of this diagnostic method in this patient population.