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.

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.

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.

Clinical Features of Children With Serology Negative, Biopsy Positive Celiac Disease.

The prevalence of pediatric serological negative celiac disease (SNCD) is poorly described, with few recognized clinical predictors beyond immunoglobulin A (IgA) deficiency or reduced gluten intake. The purpose of this retrospective review was to describe the prevalence of SNCD at the Stollery Children's Hospital and identify clinical features to help in recognition of these cases. Patients with a positive biopsy and negative serology (SNCD) were compared to those with positive biopsy and serology. SNCD diagnosis required clinical correlation and either confirmatory genetics or follow up endoscopy on a gluten-free diet. Of the 424 patients who met celiac disease (CD) criteria, 4.7% (n = 20) fulfilled our criteria for SNCD. There was a significant difference in the rates of IgA deficiency between the 2 groups, but no other clinical features were found that allowed for ready identification of SNCD patients.

Early Infliximab Clearance Predicts Remission in Children with Crohn's Disease.

BACKGROUND AND AIMS: Children with Crohn's disease have lower response rates to infliximab, lower infliximab levels, and higher infliximab clearance on weight-based dosing than adults. We hypothesize infliximab clearance is a predictive of later outcomes on infliximab in children with Crohn's disease. METHODS: In this single-center retrospective study, data were collected from charts on diagnosis, anthropometry, routine labs, infliximab therapeutic drug monitoring, infliximab dosing, disease activity, and other treatments. With these data we generated a population pharmacokinetic model using non-linear mixed effects modeling and calculated infliximab clearance for each patient over time. Patients were classified as in remission, responder-only or non-responder at 5, 10 and 16 months. Regression and ROC analyses were used to assess for early predictors of remission and response to infliximab. RESULTS: Eighty-five subjects were included, with a median follow-up of 22.3 months (IQR 10.1-36.8). Our pharmacokinetic model showed infliximab clearance was positively associated with CRP and weight, while negatively associated with albumin. In regression analyses, early infliximab clearance was the only significant, consistent predictor of remission. A 0.1 L/day increase in infliximab clearance predicted remission with an OR between 0.179 and 0.426. Differences in dosing did not account for differences in outcome. Infliximab clearance alone had moderate predictive accuracy of remission, with an AUC between 0.682 and 0.738. CONCLUSIONS: Early infliximab clearance is strongly associated with remission in children with Crohn's disease. It may be useful as a marker of response in proactive therapeutic drug monitoring to guide early dose optimization and/or changes in treatment for betterment of long-term outcomes.

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.

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.

Growth Delay in Inflammatory Bowel Diseases: Significance, Causes, and Management.

Growth delay with height and weight impairment is a common feature of pediatric inflammatory bowel diseases (PIBD). Up to 2/3 of Crohn Disease patients have impaired weight at diagnosis, and up to 1/3 have impaired height. Ulcerative colitis usually manifests earlier with less impaired growth, though patients can be affected. Ultimately, growth delay, if not corrected, can reduce final adult height. Weight loss, reduced bone mass, and pubertal delay are also concerns associated with growth delay in newly diagnosed PIBD patients. The mechanisms for growth delay in IBD are multifactorial and include reduced nutrient intake, poor absorption, increased fecal losses, as well as direct effects from inflammation and treatment modalities. Management of growth delay requires optimal disease control. Exclusive enteral nutrition (EEN), biologic therapy, and corticosteroids are the primary induction strategies used in PIBD, and both EEN and biologics positively impact growth and bone development. Beyond adequate disease control, growth delay and pubertal delay require a multidisciplinary approach, dependent on diligent monitoring and identification, nutritional rehabilitation, and involvement of endocrinology and psychiatry services as needed. Pitfalls that clinicians may encounter when managing growth delay include refeeding syndrome, obesity (even in the setting of malnutrition), and restrictive diets. Although treatment of PIBD has improved substantially in the last several decades with the era of biologic therapies and EEN, there is still much to be learned about growth delay in PIBD in order to improve outcomes.

Mixed Lipid, Fish Oil, and Soybean Oil Parenteral Lipids Impact Cholestasis, Hepatic Phytosterol, and Lipid Composition.

OBJECTIVES: In parenteral nutrition-dependent infants and children, intestinal failure (IF)-associated liver disease (IFALD) remains an important problem. A comparative study was undertaken of parenteral mixed lipid (ML), ω-3 predominant fish oil (FO), and ω-6 predominant soybean oil (SO) emulsions in regards to hepatic phytosterol, neutral lipid, fatty acid (FA) content, and the relationship to cholestasis in piglets. METHODS: Neonatal piglets received parenteral nutrition, varying in lipid dose (5 or 10 g·â€Škg ·â€Šday) and formulation: SO5 (n = 5), SO10 (n = 5), FO5 (n = 5), and ML10 (n = 5). On day 14, liver chemistry, bile flow, histology and neutral lipid staining were assessed. Hepatic triglyceride FA content was determined using thin layer and gas chromatography, and phytosterol content was assessed using gas chromatography-mass spectrometry. RESULTS: SO groups had higher prevalence of biochemical cholestasis (P < 0.04) and lower bile flow (P < 0.0001). Hepatic campesterol, stigmasterol, and ß-sitosterol were highest in SO10 (P < 0.0001). Hepatic FA (P < 0.03) and ω-6/ω-3 FA ratio (P < 0.0001) were higher in the SO groups. Neutral lipid accumulation (P = 0.3) and liver histology (P = 0.16) were not different between groups. Univariate predictors of bile flow were: campesterol (r = -0.77, P = 0.001), ß-sitosterol (r = -0.74, P = 0.002), stigmasterol (r = -0.74, P = 0.002), ω-6 FA (r = -0.72, P = 0.002), and ω-3 FA (r = 0.59, P = 0.02). Only campesterol independently predicted bile flow. CONCLUSIONS: ML and FO lipid emulsions reduce cholestasis in association with lowered hepatic phytosterol and lipid content. Lower hepatic phytosterol and ω-6 FA content, and higher ω-3 FA content are hepatoprotective. Multivariate analysis suggests reduced phytosterol accumulation may best explain the hepatoprotective effect of fish oil-containing lipids.

Integrating preventive dental care into general Paediatric practice for Indigenous communities: paediatric residents' perceptions.

This qualitative study aimed to explore paediatric residents' perceptions of the feasibility of incorporating preventive dental care into a general paediatric outreach clinic for a First Nations community. Four focus groups were conducted with paediatric residents and attending paediatricians. Interviews were recorded, transcribed verbatim and analysed using a basic interpretive qualitative approach. Three major themes emerged from the data: advantages of integration, barriers to integration and strategies for integration. Comprehensive care and service delivery were the two identified advantages of integration. Three categories of barriers emerged including patient and caregiver-related, resident-related and setting-related barriers. Training and practice, patient education, support and policy were the suggested strategies for successful integration. Providers were found to be open to integrating preventive dental care into their practice. However, barriers impeded the success of this integration. Multiple strategies including oral health care training for medical providers, office support and policy changes would facilitate successful integration.

Practices regarding human Papillomavirus counseling and vaccination in head and neck cancer: a Canadian physician questionnaire.

BACKGROUND: Human papillomavirus (HPV) has recently been implicated as a causative agent in a rapidly growing number of oropharyngeal cancers. Emerging literature supports the hypothesis that HPV vaccination may protect against HPV-related head and neck cancer (HNC) in addition to HPV-related cervical and anogenital disease. While the association between HPV infection and cervical cancer is widely understood, its relation to HNC is less well known. The purpose of this study was to better understand HPV counseling practices for infection and vaccination in relation to HNC of primary care physicians (PCPs), Obstetricians/Gynecologists (OBGYNs), and Otolaryngology - Head and Neck Surgeons (OHNSs) in Canada. METHODS: A Canada-wide electronic questionnaire regarding counseling practices on HPV infection, transmission, and vaccination was designed and distributed to PCPs, OBGYNs, and OHNSs across Canada through electronic and paper-based methods. Basic Descriptive statistics were used to analyze responses. RESULTS: In total, 337 physicians responded (239 family physicians, 51 OHNSs, 30 OBGYNs, and 17 pediatricians). Three out of four PCPs reported routine counseling of their patients regarding HPV infection, transmission, and vaccination. Among this group, 68% reported "never" or "rarely" counseling patients that HPV can cause HNC. The most commonly reported reason that PCPs cited for not counseling was a lack of knowledge. The majority of OHNSs (81%) and OBGYNs (97%) counseled patients regarding HPV infection, transmission, and vaccination. However, very few OHNSs (10%) regularly counseled patients with HPV-related HNC about HPV-related anogenital cancer. Similarly, very few OBGYNs (18%) regularly counseled patients with HPV related cervical/anogenital cancer about HPV related HNC. CONCLUSIONS: The rate of counseling on HPV infection, transmission, and vaccination in relation to HNC among PCPs is low. The most common reason is a lack of knowledge. Specialists rarely counsel patients with confirmed HPV-related cancer about other HPV-related malignancies. More research is needed on the relationship between different HPV-related cancers in order to better inform counseling practices.

Antitissue Transglutaminase Normalization Postdiagnosis in Children With Celiac Disease.

OBJECTIVES: Limited pediatric data exist examining the trend and predictors of antitissue transglutaminase (atTG) normalization over time in children with celiac disease (CD). We aimed to evaluate time to normalization of atTG in children after CD diagnosis, and to assess for independent predictors affecting this duration. METHODS: A retrospective chart review was completed in pediatric patients with CD diagnosed from 2007 to 2014 at the Stollery Children's Hospital Celiac Clinic (Edmonton, Alberta, Canada). The clinical predictors assessed for impact on time to atTG normalization were initial atTG, Marsh score at diagnosis, gluten-free diet compliance (GFDC), age at diagnosis, sex, ethnicity, medical comorbidities, and family history of CD. Kaplan-Meier survival analysis was completed to assess time to atTG normalization, and Cox regression to assess for independent predictors of this time. RESULTS: A total of 487 patients met inclusion criteria. Approximately 80.5% of patients normalized atTG levels. Median normalization time was 407 days for all patients (95% confidence interval [CI: 361-453]), and 364 days for gluten-free diet compliant patients (95% CI [335-393]). Type 1 diabetes mellitus (T1DM) patients took significantly longer to normalize at 1204 days (95% CI [199-2209], P < 0.001). Cox regression demonstrated T1DM (hazard ratio = 0.36 [0.24-0.55], P < 0.001) and higher baseline atTG (hazard ratio = 0.52 [0.43-0.63], P < 0.001) were significant predictors of longer atTG normalization time. GFDC was a significant predictor of earlier normalization (OR = 13.91 [7.86-24.62], P < 0.001). CONCLUSIONS: GFDC and lower atTG at diagnosis are predictors of earlier normalization. Patients with T1DM are less likely to normalize atTG levels, with longer normalization time. Additional research and education for higher-risk populations are needed.

Managing the pediatric patient with celiac disease: a multidisciplinary approach.

Celiac disease (CD) is an autoimmune reaction to gluten, leading to intestinal inflammation, villous atrophy, and malabsorption. It is the most common autoimmune gastrointestinal disorder, with an increasing prevalence. A life-long gluten-free diet (GFD) is an effective treatment to alleviate symptoms, normalize autoantibodies, and heal the intestinal mucosa in patients with CD. Poorly controlled CD poses a significant concern for ongoing malabsorption, growth restriction, and the long-term concern of intestinal lymphoma. Achieving GFD compliance and long-term disease control poses a challenge, with adolescents at particular risk for high rates of noncompliance. Attention has turned toward innovative management strategies to improve adherence and achieve better disease control. One such strategy is the development of multidisciplinary clinic approach, and CD is a complex life-long disease state that would benefit from a multifaceted team approach as recognized by multiple national and international bodies, including the National Institutes of Health. Utilizing the combined efforts of the pediatric gastroenterologist, registered dietitian, registered nurse, and primary care provider (general practitioner or general pediatrician) in a CD multidisciplinary clinic model will be of benefit for patients and families in optimizing diagnosis, provision of GFD teaching, and long-term adherence to a GFD. This paper discusses the benefits and proposed structure for multidisciplinary care in improving management of CD.

Satisfaction in open access versus traditional referral for upper endoscopy in children.

OBJECTIVES: In traditional access endoscopy (TAE), patients are booked for endoscopy following a gastroenterology clinic assessment. In contrast, open access endoscopy (OAE) patients are seen for the first time on the day of the procedure, providing same day procedural consent. Controversy exists over the use of OAE in adults, both with the consent process and with patient satisfaction. No literature exists describing satisfaction with OAE in pediatrics. We therefore aimed to assess pediatric patient and caregiver satisfaction in OAE compared with TAE. METHODS: Consecutive pediatric patients, and their caregivers, undergoing elective upper endoscopy from May to December 2012 at the Stollery Children's Hospital (Edmonton, Alberta, Canada) were consented for a cross-sectional survey. Seven preprocedure and 5 postprocedure questions were completed regarding mood and satisfaction with the wait time and the information provided. Group demographics and endoscopy wait times were collected. RESULTS: Median wait time with OAE was less compared with TAE (57 days vs 196 days, P < 0.001). OAE patients reported worse mood preprocedure than TAE patients (35.3% vs 10.7%, P = 0.046). OAE caregivers and patients reported more mood disturbance if required to wait longer for endoscopy by attending clinic preprocedure (OAE caregivers 62.2%, OAE patients 64.7%). CONCLUSIONS: OAE is associated with worse preendoscopy patient mood; however, children and caregivers seem concerned about longer wait times associated with TAE. Given the significantly shorter wait times in OAE, identifying methods to minimize present limitations of OAE will be useful to improve clinical practices in pediatric gastroenterology.