Esophagitis, treatment outcomes, and long-term follow-up in children with esophageal atresia.

OBJECTIVES: Survival rates in children born with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) have improved; however, morbidity associated with the disease remains high. This study aimed to assess the prevalence of gastroesophageal reflux disease (GERD), eosinophilic esophagitis (EoE), fungal esophagitis, esophageal strictures, and long-term outcomes in children with EA/TEF. METHODS: We conducted a retrospective chart review on patients with EA/TEF who were seen at Children's Wisconsin from January 2003 to January 2023. Patients born with EA/TEF were included if they underwent at least one endoscopy after 1 year of age. GERD was diagnosed based on abnormal findings on endoscopy, pH-metry, and/or history of fundoplication. EoE and fungal esophagitis were diagnosed based on abnormal endoscopy. Esophageal stricture diagnosis was based on findings on endoscopy and/or esophagram, and clinical symptoms necessitating esophageal dilation. RESULTS: Eighty-five patients (64.7% males, mean age 7.5 years) were included, the majority had type C EA/TEF (90.6%). GERD was diagnosed in 61.1% (n = 52), 49.4% (n = 42) by macro and/or microscopic endoscopic findings, 22.3% (n = 19) by abnormal pH-metry, and 21.1% (n = 18) by the need for fundoplication for refractory reflux and/or esophageal stricture. Risk of GERD increased with lower gestational age (p = 0.0030), lower birth weight (p = 0.023), and long-gap EA (p = 0.034). In children diagnosed with GERD, only 13.4% of patients (n = 7/52) were able to be weaned off proton pump inhibitor (PPI) without disease recurrence. However, overall, at the completion of the study, 44.7% (n = 38) of patients were successfully weaned off PPI without evidence of GERD. EoE was diagnosed in 20% of the patients (n = 17). All patients diagnosed with EoE required escalation of therapy from PPI alone to swallowed corticosteroids in 52.9% (n = 9), dupilumab in 23.5% (n = 4), elemental formula in 17.6% (n = 3), and elemental formula and swallowed steroids in 5.8% (n = 1). Fungal esophagitis was diagnosed in 15.3% of patients (n = 13). An esophageal stricture requiring dilation was diagnosed in 77.6% (n = 66) of patients at a mean age of 28.5 months, with over 60% diagnosed by 24 months of age. CONCLUSIONS: Children born with EA/TEF continue to be at high risk of developing GERD, EoE, fungal esophagitis, and esophageal stenosis. Diagnostic and therapeutic endoscopy remains a high-yield test to identify and treat these comorbidities.

Ineffective esophageal motility: Characterization and outcomes across pediatric neurogastroenterology and motility centers in the United States.

OBJECTIVES: Ineffective esophageal motility (IEM) on high-resolution manometry (HRM) is not consistently associated with specific clinical syndromes or outcomes. We evaluated the prevalence, clinical features, management, and outcomes of pediatric IEM patients across the United States. METHODS: Clinical and manometric characteristics of children undergoing esophageal HRM during 2021-2022 were collected from 12 pediatric motility centers. Clinical presentation, test results, management strategies, and outcomes were compared between children with IEM and normal HRM. RESULTS: Of 236 children (median age 15 years, 63.6% female, 79.2% Caucasian), 62 (23.6%) patients had IEM, and 174 (73.7%) patients had normal HRM, with similar demographics, medical history, clinical presentation, and median symptom duration. Reflux monitoring was performed more often for IEM patients (25.8% vs. 8.6%, p = 0.002), but other adjunctive testing was similar. Among 101 patients with follow-up, symptomatic cohorts declined in both groups in relation to the initial presentation (p > 0.107 for each comparison) with management targeting symptoms, particularly acid suppression. Though prokinetics were used more often and behavioral therapy less often in IEM (p ≤ 0.015 for each comparison), symptom outcomes were similar between IEM and normal HRM. Despite a higher proportion with residual dysphagia on follow-up in IEM (64.0% vs. 39.1%, p = 0.043), an alternate mechanism for dysphagia was identified more often in IEM (68.8%) compared to normal HRM (27.8%, p = 0.017). CONCLUSIONS: IEM is a descriptive manometric pattern rather than a clinical diagnosis requiring specific intervention in children. Management based on clinical presentation provides consistent symptom outcomes.

Esophageal Dilation in Children: A State of the Art Review.

Esophageal dilations in children are performed by several pediatric and adult professionals. We aim to summarize improvements in safety and new technology used for the treatment of complex and refractory strictures, including triamcinolone injection, endoscopic electro-incisional therapy, topical mitomycin-C application, stent placement, functional lumen imaging probe assisted dilation, and endoscopic vacuum-assisted closure in the pediatric population.

Pediatric Aerodigestive Medicine: Advancing Collaborative Care for Children With Oropharyngeal Dysphagia.

OBJECTIVES: Aerodigestive disorders encompass various pathological conditions affecting the lungs, upper airway, and gastrointestinal tract in children. While advanced care has primarily occurred in specialty centers, many children first present to general pediatric gastroenterologists with aerodigestive symptoms necessitating awareness of these conditions. At the 2021 Annual North American Society for Pediatric Gastroenterology, Hepatology and Nutrition meeting, the aerodigestive Special Interest Group held a full-day symposium entitled, Pediatric Aerodigestive Medicine: Advancing Collaborative Care of Children with Aerodigestive Disorders. The symposium aimed to underline the significance of a multidisciplinary approach to achieve better outcomes for these complex patients. METHODS: The symposium brought together leading experts to highlight the growing aerodigestive field, promote new scientific and therapeutic strategies, share the structure and benefits of a multidisciplinary approach in diagnosing common and rare aerodigestive disorders, and foster multidisciplinary discussion of complex cases while highlighting the range of therapeutic and diagnostic options. In this article, we showcase the diagnostic and therapeutic approach to oropharyngeal dysphagia (OPD), one of the most common aerodigestive conditions, emphasizing the role of a collaborative model. CONCLUSIONS: The aerodigestive field has made significant progress and continues to grow due to a unique multidisciplinary, collaborative model of care for these conditions. Despite diagnostic and therapeutic challenges, the multidisciplinary approach has enabled and greatly improved efficient, high-quality, and evidence-based care for patients, including those with OPD.

Efficacy of Auricular Neurostimulation in Adolescents With Irritable Bowel Syndrome in a Randomized, Double-Blind Trial.

BACKGROUND & AIMS: Auricular neurostimulation therapy, in which a noninvasive device delivers percutaneous electrical nerve field stimulation (PENFS) to the external ear, is effective in pediatric patients with functional abdominal pain disorders. Preclinical studies showed that PENFS modulates central pain pathways and attenuates visceral hyperalgesia. We evaluated the efficacy of PENFS in adolescents with irritable bowel syndrome (IBS). METHODS: We analyzed data from pediatric patients with IBS who participated in a double-blind trial at a tertiary care gastroenterology clinic from June 2015 through November 2016. Patients were randomly assigned to groups that received PENFS (n = 27; median age, 15.3 y; 24 female) or a sham stimulation (n = 23; median age, 15.6 y; 21 female), 5 days/week for 4 weeks. The primary endpoint was number of patients with a reduction of 30% or more in worst abdominal pain severity after 3 weeks. Secondary endpoints were reduction in composite abdominal pain severity score, reduction in usual abdominal pain severity, and improvement in global symptom based on a symptom response scale (-7 to +7; 0 = no change) after 3 weeks. RESULTS: Reductions of 30% or more in worst abdominal pain were observed in 59% of patients who received PENFS vs 26% of patients who received the sham stimulation (P = .024). The patients who received PENFS had a composite pain median score of 7.5 (interquartile range [IQR], 3.6-14.4) vs 14.4 for the sham group (IQR, 4.5-39.2) (P = .026) and a usual pain median score of 3.0 (IQR, 3.0-5.0) vs 5.0 in the sham group (IQR, 3.0-7.0) (P = .029). A symptom response scale score of 2 or more was observed in 82% of patients who received PENFS vs 26% of patients in the sham group (P ≤ .001). No significant side effects were reported. CONCLUSIONS: Auricular neurostimulation reduces abdominal pain scores and improves overall wellbeing in adolescents with IBS. PENFS is a noninvasive treatment option for pediatric patients with functional bowel disorders. ClinicalTrials.gov no: NCT02367729.

Endoscopic Removal of a Single, Painless, Juvenile Polyp in the Small Intestine Causing Anemia.

Juvenile polyps are the most common gastrointestinal polyps in childhood. Typically, they are located in the colon and present with intermittent and painless hematochezia. A few case reports have described juvenile polyps in the small intestine, all presenting as intussusception requiring surgery. We report an isolated juvenile polyp in the small intestine presenting with painless anemia, identified using video capsule endoscopy, and removed via enteroscopy.

Tiny patients, huge impact: a call to action.

The continuation of high-quality care is under threat for the over 70 million children in the United States. Inequities between Medicaid and Medicare payments and the current procedural-based reimbursement model have resulted in the undervaluing of pediatric medical care and lack of prioritization of children's health by institutions. The number of pediatricians, including pediatric subspecialists, and pediatric healthcare centers are declining due to mounting financial obstacles and this crucial healthcare supply is no longer able to keep up with demand. The reasons contributing to these inequities are clear and rational: Medicaid has significantly lower rates of reimbursement compared to Medicare, yet Medicaid covers almost half of children in the United States and creates the natural incentive for medical institutions to prioritize the care of adults. Additionally, certain aspects of children's healthcare are unique from adults and are not adequately covered in the current payment model. The result of decades of devaluing children's healthcare has led to a substantial decrease in the availability of services, medications, and equipment needed to provide healthcare to children across the nation. Fortunately, the solution is just as clear as the problem: we must value the healthcare of children as much as that of adults by increasing Medicaid funding to be on par with Medicare and appreciate the complexities of care beyond procedures. If these changes are not made, the high-quality care for children in the US will continue to decline and increase strain on the overall healthcare system as these children age into adulthood.

Addressing Self-Injurious Behavior in the Medically Complex Child: Identifying the Root Cause vs Blocking Resulting Behavior.

CASE: Gillian is a 7-year-old nonverbal, internationally adopted girl with significant visual impairment (sees shadows and objects with high contrast), moderate-severe hearing loss, autism spectrum disorder, profound intellectual disability, and a seizure disorder. She resided in an orphanage until age 4 years when she was adopted by her mother. She is referred to the multidisciplinary team in developmental-behavioral pediatrics by her gastroenterologist, who is managing her constipation, for evaluation and management of self-injurious behavior that occurs before bowel movements.Gillian's adoptive mother reported that Gillian has a history of repetitive and self-injurious behavior including hitting her head with the palm of her hand and poking her eyes. The head-hitting behavior has resulted in consistent bruising to her forehead and nasal bridge. The eye poking is of significant concern because of the possibility of resultant injury including corneal abrasion or retinal detachment. Eye poking occurs exclusively during the hour before stooling, at home and school, and is accompanied by screaming, pacing, and increased aggression toward her mother/teachers. Mother typically responds by blocking the eye poking, redirecting Gillian to the toilet, providing prompts to use calming strategies (e.g., deep breathing), or providing sensory input by rubbing her arms/back. Teachers respond in a similar way at school. Gillian's mother has tried to place goggles over Gillian's eyes during eye poking, but this resulted in increased aggressive behaviors.Gillian is not fully bowel trained but will sit on the toilet to urinate when prompted. She completes toilet-sits at home/school 5 to 7 times daily. Gillian passes small, soft bowel movements 2 to 4 times daily on a bowel management regimen consisting of polyethylene glycol 3350 17 grams twice daily and liquid senna 8.8 mg daily. Seizures are reportedly well controlled with an antiepileptic medication managed by her neurologist. Previous medical evaluation by otolaryngology, ophthalmology, neurology, and gastroenterology did not identify a source of pain or other cause for the eye poking. Physical examination reveals normal bowel sounds and a mildly distended abdomen but is otherwise unremarkable. What are your next steps for evaluation and treatment?

The Role of Endoscopy in the Diagnosis and Management of Small Bowel Pathology in Children.

Small bowel evaluation has been transformed by capsule endoscopy and advances in small bowel imaging, which provide reliable and noninvasive means for assessing the mucosal surface. Device-assisted enteroscopy has been critical for histopathological confirmation and endoscopic therapy for a wide range of small bowel pathology that conventional endoscopy cannot reach. The purpose of this review is to provide a comprehensive overview of the indications, techniques, and clinical applications of capsule endoscopy; device-assisted enteroscopy; and imaging studies for small bowel evaluation in children.

Advances in Endoscopic Procedures in Pediatric Patients.

Endoscopy has been a crucial part of the diagnostic and therapeutic modality in pediatric gastrointestinal disorders. This article outlines recent advances in pediatric gastrointestinal endoscopy, including transnasal endoscopy, functional luminal imaging probe, peroral endoscopic myotomy, mucosal impedance, endoscopic vacuum-assisted closure system, chromoendoscopy, artificial intelligence, and machine learning.

Esophageal Wound Vacuum Placement for Anastomotic Leak: Lessons Learned From First Time Use at a Tertiary Care Center.

Esophageal atresia and tracheoesophageal fistula (TEF) are rare birth anomalies typically requiring corrective surgery over the first few months of life. Esophageal surgery can lead to a life-threatening anastomotic leak. Esophageal wound vacuums have seen increased use in adults and one cohort of children as a therapeutic modality. This case study explores a tertiary care pediatric hospital's introductory experience in utilizing this technique. A 19-month-old male underwent staged repair for esophageal atresia/tracheoesophageal fistula requiring an esophageal stricture resection with primary anastomosis. An anastomotic leak was successfully managed with wound vacuums. Our experiences highlighted the need for individualized treatment plans with this therapy based on feeding capabilities, side effects of the vacuum, placement method, and replacement strategies.

Nutrition Management in Pediatric Gastrointestinal Motility Disorders.

Gastrointestinal (GI) motility disorders are associated with suboptimal nutrition in children, mainly because of malabsorption and symptoms limiting dietary intake. Apart from medical therapy, nutrition support has a crucial role in maintaining growth and improving clinical outcomes in children. Based on recent data and guidelines, this review provides an overview of nutrition assessment and specific interventions for common pediatric GI disorders including gastroesophageal reflux disease, esophageal motility disorders, gastroparesis, chronic intestinal pseudo-obstruction, and constipation. Several approaches including diet modification, enteral nutrition (gastric vs post-pyloric, temporary vs permanent access, bolus vs continuous), and parenteral nutrition need to be tailored based on patient's nutrition and clinical assessment.

Cyproheptadine: A Potentially Effective Treatment for Functional Gastrointestinal Disorders in Children.

Functional gastrointestinal disorders (FGIDs) negatively affect children's quality of life and health care costs. It has been proposed that alteration of gut serotonin leads to gastrointestinal dysmotility, visceral hypersensitivity, altered gastrointestinal secretions, and brain-gut dysfunction. Cyproheptadine, a serotonin antagonist, has been shown to be a potentially effective and safe treatment option in children who meet the clinical criteria for FGIDs. Well-designed multicenter trials with long-term follow-up are needed to further investigate its efficacy. [Pediatr Ann. 2017;46(3):e120-e125.].

Long-Term Study of Children With ROME III Functional Gastrointestinal Disorders Managed Symptomatically in a Biopsychosocial Model.

BACKGROUND: Our study evaluated progression of and identified potential factors contributing to outcomes of ROME III defined-functional gastrointestinal disorders (FGIDs) in children treated symptomatically in a biopsychosocial model of care with a long-term follow-up. METHODS: We performed a retrospective review of pediatric patients who were diagnosed with ROME III defined-FGIDs including functional abdominal pain, functional dyspepsia, irritable bowel syndrome and abdominal migraine. Patients were managed symptomatically in a biopsychosocial model of care from the time of initial diagnosis. Demographics, management, progression and response to treatment assessed as complete, partial, and no improvement were reviewed. RESULTS: Two hundred fifty-eight patients were included with mean age of 10.6 years, female 55.4%, mean number of encounters 3.3 visits, and mean follow-up was 18.7 months (range 2 - 59, SD 15.8). Diagnoses were functional abdominal pain 45%, irritable bowel syndrome 20.9%, multiple 13.2%, functional dyspepsia 12.8%, and abdominal migraine 8.1%. Investigations were performed in most patients: laboratory studies in 93.4% (non-contributory abnormal 23.6%), imaging studies in 45.3% (non-contributory abnormal 5%) and endoscopies in 43.0% (non-contributory abnormal 1.2%). Treatment included medication in 93.7%, and surgery in 1.9% (normal pathology). There were new functional gastrointestinal diagnosis in 11.6%, evolution of FGIDs, from one to another in 12.0%, and recurrence found in 35.7% of patients. There were 60.1% patients in the complete improvement group (CIG) and 39.1% in the partial/no improvement group (PIG/NIG). No statistical difference was found between CIG and PIG/NIG regarding demographics or evaluation. PIG/NIG had more encounters (mean 3.63 vs. 3.11; P = 0.03), had non-contributory lab abnormalities (34.4% vs. 20.0%; P = 0.01), needed more endoscopies (52.4% vs. 36.8%; P = 0.02), required more treatment changes (mean 1.41 vs. 0.81; P < 0.01) and developed new functional gastrointestinal diagnoses (19.4% vs. 6.5%; P < 0.01) with long-term follow-up. CONCLUSIONS: Patients with ROME III defined-FGIDs who experience partial or no improvement with treatment develop new FGID diagnosis, need more number of follow-up visits, require more number of endoscopies, need more treatment changes, and have more non-contributory laboratory abnormalities, compared to those who experience complete improvement. Symptomatic treatment offered in a biopsychosocial model of care is possibly beneficial in managing children with FGIDs.