Natural airway as an alternative to intubation for pediatric endoscopic esophageal foreign body removal: A retrospective cohort study of 326 patients.

BACKGROUND: Anesthesia is required for endoscopic removal of esophageal foreign bodies (EFBs) in children. Historically, endotracheal intubation has been the de facto gold standard for airway management in these cases. However, as more elective endoscopic procedures are now performed under propofol sedation with natural airway, there has been a move toward using similar Monitored Anesthesia Care (MAC) for select patients who require endoscopic removal of an EFB. METHODS: In this single-center retrospective cohort study, we compared endoscopic EFB removal with either MAC or endotracheal intubation. Descriptive statistics summarized factors stratified by initial choice of airway technique, including intra- and postanesthesia complications and the frequency of mid-procedure conversion to endotracheal intubation in those initially managed with MAC. To demonstrate the magnitude of associations between these factors and the anesthesiologist's choice of airway technique, univariable Firth logistic and quantile regressions were used to estimate odds ratios (95% CI) and beta coefficients (95% CI). RESULTS: From the initial search, 326 patients were identified. Among them, 23% (n = 75) were planned for intubation and 77% (n = 251) were planned for MAC. Three patients (0.9%) who were initially planned for MAC required conversion to endotracheal intubation after induction. Two (0.6%) of these children were admitted to the hospital after the procedure and treated for ongoing airway reactivity. No patient experienced reflux of gastric contents to the mouth or dislodgement of the foreign body to the airway, and no patient required administration of vasoactive medications or cardiopulmonary resuscitation. Patients had higher odds that the anesthesiologist chose to utilize MAC if the foreign body was a coin (OR, 3.3; CI, 1.9-5.7, p < .001) or if their fasting time was >6 h. Median total operating time was 15 min greater in intubated patients (11 vs. 26 min, p < .001). CONCLUSIONS: This study demonstrates that MAC may be considered for select pediatric patients undergoing endoscopic removal of EFB, especially those who have ingested coins, who do not have reactive airways, who have fasted for >6 h, and in whom the endoscopic procedure is expected to be short and uncomplicated. Prospective multi-site studies are needed to confirm these findings.

Paediatric cannabinoid hyperemesis.

PURPOSE OF REVIEW: The prevalence of adolescent cannabinoid hyperemesis syndrome (CHS) continues to grow, as clinicians increasingly recognize the presenting features of cyclical nausea, emesis, abdominal pain and relief of symptoms with hot showers, in the setting of chronic cannabinoid use. RECENT FINDINGS: Our understanding of the contributory mechanisms continues to grow, but high-quality evidence of effective treatment in adolescents remains lacking. Current best evidence in the treatment of acute paediatric CHS suggests intravenous rehydration and electrolyte correction, followed by 0.05 mg/kg haloperidol with or without a benzodiazepine. The only long-term treatment remains complete cessation of cannabinoid use. SUMMARY: This article reviews our growing knowledge of adolescent CHS and provides practical guidance for diagnosis, treatment and understanding the underlying mechanisms of the condition.

Prevalence of Gastroesophageal Reflux Disease in Infants With Congenital Muscular Torticollis: A Prospective Cohort Study.

PURPOSE: Clinical experience suggests that gastroesophageal reflux disease (GERD) occurs commonly in infants with congenital muscular torticollis (CMT). However, this is an understudied topic and prospective studies are absent. We determine the prevalence of GERD in infants with CMT, comparing clinical characteristics between CMT infants with and without GERD, and identifying infants with potentially undiagnosed GERD. METHODS: A prospective cohort study of 155 infants with CMT younger than 12 months with and without GERD was evaluated by pediatric physical therapists. RESULTS: GERD prevalence was 30.3%, including 6 (3.9%) infants with undiagnosed GERD. Demographic and clinical characteristics were similar in CMT infants with and without GERD. CONCLUSIONS: This is the first prospective cohort study determining the prevalence of GERD in infants referred for evaluation of CMT. Further prospective studies are needed to determine whether early intervention and treatment of GERD improves outcomes in infants with CMT (see Supplemental Digital Content 1, available at: http://links.lww.com/PPT/A369).

Percutaneous Endoscopic Gastrostomy Enhances Interstage Growth in Infants With Hypoplastic Left Heart Syndrome.

OBJECTIVES: Infants with hypoplastic left heart syndrome undergoing staged palliation commonly experience chronic growth failure and malnutrition. Greater patient weight at stage 2 palliation (Glenn) is thought to be associated with improved perioperative outcomes. We aimed to compare weight for age z score and interstage growth velocity in children with and without a percutaneous endoscopic gastrostomy prior to Glenn and hypothesize that those with a percutaneous endoscopic gastrostomy experience-enhanced interstage growth and reduced malnutrition rates. DESIGN: Single-center, retrospective cohort study. SETTING: A total of 259-bed, quaternary, pediatric referral center. PATIENTS: Infants with hypoplastic left heart syndrome from 2007 to 2016 with and without percutaneous endoscopic gastrostomy insertion after initial palliation (Norwood). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Primary outcomes were weight for age z score (at birth, Norwood, Norwood discharge, and Glenn), interstage growth velocity, and moderate-to-severe malnutrition (weight for age z score<-2) rates. Secondary outcomes were lengths of stay, mechanical ventilation rates after Glenn, and mortality. Statistical analyses included chi-square, Wilcoxon rank-sum, student's t, paired testing, and exploratory logistic regression. Of the 69 infants studied, 47 (68%) had percutaneous endoscopic gastrostomy insertion at a median of 156 (interquartile range, 115-158) days prior to Glenn. Among children with and without percutaneous endoscopic gastrostomy, we observed no differences in demographics, comorbidities, cardiothoracic surgical times, postoperative Glenn outcomes (length of stay, mechanical ventilation rate, peak 24-hr lactate, nitric oxide use, extracorporeal life support rate, or mortality), weight for age z score at birth, and weight for age z score at Norwood. At the time of percutaneous endoscopic gastrostomy insertion, weight for age z score was -2.5 ± 1.3 and subsequent growth velocity increased from 8 ± 7 to 40 ± 59 g/d (p < 0.01). From Norwood discharge to the date of Glenn, weight for age z score increased in infants with percutaneous endoscopic gastrostomy (-2.5 ± 1.1 to -1.5 ± 1.4 [p < 0.01]) with a large reduction in moderate-to-severe malnutrition rates (76-36%; p < 0.01). In general, weight for age z score at the time of Glenn was associated with reduced postoperative mortality (odds ratio, 0.3; 95% CI, 0.09-0.95; p = 0.04). CONCLUSIONS: Infants undergoing palliation for hypoplastic left heart syndrome with percutaneous endoscopic gastrostomy insertion prior to Glenn had improved growth velocity and dramatically reduced rates of moderate-to-severe malnutrition rates (40% reduction). In addition, we noted weight for age z score at when Glenn was associated with improved postoperative Glenn survival. No complications from percutaneous endoscopic gastrostomy were noted. Placement of a percutaneous endoscopic gastrostomy improved weight for age z score, enhanced interstage growth, and reduced malnutrition rates for this at-risk population of malnourished children.

Percutaneous Endoscopic Gastrostomy After Cardiothoracic Surgery in Children Less Than 2 Months Old: An Assessment of Long-Term Malnutrition Status and Gastrostomy Outcomes.

OBJECTIVES: Infants with critical congenital heart disease undergoing cardiothoracic surgery commonly experience chronic malnutrition and growth failure. We sought to determine whether placement of a percutaneous endoscopic gastrostomy was associated with reduced moderate-severe malnutrition status and to describe percutaneous endoscopic gastrostomy-related clinical and safety outcomes in this population. DESIGN: Single-center, retrospective cohort study. SETTING: Two hundred fifty-nine-bed, tertiary care, pediatric referral center. PATIENTS: Children with congenital heart disease less than 2 months old undergoing cardiothoracic surgery from 2007 to 2013 with and without percutaneous endoscopic gastrostomy. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Primary outcomes were weight for age z scores during hospitalization, at 6 months, and 1 year after cardiothoracic surgery. Secondary outcomes were frequency of percutaneous endoscopic gastrostomy revision, percutaneous endoscopic gastrostomy complications, and mortality. Statistical analyses included Wilcoxon rank-sum, Fisher exact, and Student t tests. Two hundred twenty-two subjects met study criteria, and 77 (35%) had percutaneous endoscopic gastrostomy placed at a mean of 45 ± 31 days after cardiothoracic surgery. No differences were noted for demographics, comorbidities, and weight for age z score at birth and at the time of cardiothoracic surgery. The percutaneous endoscopic gastrostomy cohort had greater Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery risk category (4 [4-5] vs 4 [2-4]) and length of stay (71 d [49-101 d] vs 26 d [15-42 d]). Mean weight for age z score at the time of percutaneous endoscopic gastrostomy was -2.8 ± 1.3. Frequency of moderate-severe malnutrition (weight for age z score, ≤ -2) was greater in children with percutaneous endoscopic gastrostomy at discharge (78% vs 48%), 6 months (61% vs 16%), and 1 year (41% vs 2%). Index mortality was lower in children with percutaneous endoscopic gastrostomy at 30 days (8% vs 0%) and hospital discharge (19% vs 4%). However, no mortality differences were observed after discharge. Growth velocity after percutaneous endoscopic gastrostomy was greater (44 ± 19 vs 10 ± 9 g/d). Children tolerated percutaneous endoscopic gastrostomy without hemodynamic compromise, minor percutaneous endoscopic gastrostomy complications, and anticipated percutaneous endoscopic gastrostomy revisions. Children without mortality had percutaneous endoscopic gastrostomy removal at a median duration of 253 days (133-545 d). Children with univentricular physiology had improved in-hospital mean growth velocity (6.3 vs 24.4 g/d; p < 0.01) and reduced 1-year rate moderate-severe malnutrition (66.7% vs 36.9%; p < 0.01) after percutaneous endoscopic gastrostomy placement. CONCLUSIONS: Percutaneous endoscopic gastrostomy placement was well tolerated and associated with improved postoperative growth velocity in children with critical congenital heart disease undergoing cardiothoracic surgery less than 2 months old. These findings were also noted in our subanalysis of children with univentricular physiology. Persistent rates of moderate-severe malnutrition were noted at 1-year follow-up. Although potential index mortality benefit was observed, definitive data are still needed.

Endoscopic closure of persistent gastrocutaneous fistula in children.

PURPOSE: The incidence of persistent gastrocutaneous fistula (GCF) after removal of gastrostomy tubes in pediatric patients is estimated to be up to 44 %. Our aim was to review the outcomes of GCF closure by an endoscopic technique that utilizes cautery and endoclips. METHODS: A retrospective analysis of patients who underwent endoscopic treatment for persistent GCF from January 2010 to September 2013 was performed. This technique utilized esophagogastroduodenoscopy with cauterization of the fistula track and endoclipping of the gastric mucosa. RESULTS: Sixteen patients underwent endoscopic treatment for persistent GCF. Mean age at time of endoscopy was 7.5 ± 5.5 (1.1-17) years. Gastrostomy tubes were in place for mean of 5.4 ± 5.2 (0.5-14.2) years prior to removal. The average time from gastrostomy tube removal to first endoscopic clipping was 6.7 ± 9 (0.1-28.9) months. Seven patients (44 %) had successful closure after one endoclipping procedure. Six patients underwent a second endoclipping procedure, with three successful closures. Four patients (25 %) required surgical closure for persistent fistulas and 2 (13 %) have continued drainage. CONCLUSIONS: While endoscopy with cautery and endoclipping proves to be safe, many patients require multiple procedures and may require surgical closure. Patient selection and refinement of this technique may improve outcomes.

Corrigendum: Navigating formula shortages: associations of parental perspectives on transitioning to alternative infant formulas for cow's milk protein allergy during the 2022 national formula shortage.

[This corrects the article DOI: 10.3389/falgy.2023.1333570.].

Managing cow's milk protein allergy during the 2022 formula shortage: decision-making among pediatric healthcare providers.

Introduction: Cow's milk protein allergy (CMPA) affects 2%-7% of infants and is managed with hypoallergenic formulas. The 2022 recalls of infant formulas due to factors including contamination led to specialty formula shortages, highlighting CMPA management challenges. Understanding healthcare providers' (HCPs) decision-making in transitioning to alternative formulas during shortages is crucial. Limited attention has been given to how pediatric physicians make these choices. Methods: This study utilized US HCPs' de-identified survey data to assess driving factors when switching extensively hydrolyzed formulas during shortages. Results: 104 eligible HCPs participated, including general pediatrics, pediatric allergy/immunology, and pediatric gastroenterology specialists. Safety, tolerability, and efficacy were identified as top factors for switching formulas. Formula 1 was considered well-tolerated, patient-accepted, and safe by all HCPs. Most expressed strong belief in Formula 1's safety and effectiveness. Discussion: Findings inform CMPA management during shortages, offering guidance to HCPs for suitable formula selection and enhanced infant care.

Impact of the 2022 national formula shortage on clinical decision-making of healthcare providers in switching amino acid formulas for infants with cow's milk protein allergy: a survey-based study.

Background: In 2022, the United States experienced a national shortage of infant formula due to a global supply chain crisis and a large-scale domestic formula recall. The existing literature on healthcare providers' (HCPs) clinical decision-making during formula shortages is limited. This study aims to analyze the factors influencing pediatric HCP clinical decision-making when switching between amino acid formulas (AAF) for managing cow's milk protein allergy (CMPA) in infants under 24 months of age during an unprecedented national formula shortage. Methods: The study included pediatric HCPs with experience managing CMPA in infants and toddlers under 24 months during the formula shortage from January 2022 to November 2022. A de-identified survey comprising 26 questions examining driving factors used in clinical decision-making was administered to pediatric HCPs using a real-time mobile data collection tool. Results: Among the surveyed pediatric HCPs (n = 75), the factors most frequently considered as "extremely important" when switching to another AAF included safety (85%), tolerability (73%), and efficacy (83%). No statistically significant differences were found in HCP ratings among the listed examined factors of the four AAFs. The availability of specific formulas was the only factor that exhibited a statistically significant difference in perceived performance among pediatric HCPs when comparing the four AAFs (p < 0.05). Discussion: This study elucidates the crucial aspects that influenced pediatric HCPs' selection of AAFs for CMPA management during the 2022 formula shortage. The findings highlight the significance of safety, tolerability, efficacy, and availability in the pediatric HCP decision-making processes.

Management Strategies and Outcomes of Distal Congenital Esophageal Strictures in the Setting of Long-gap Esophageal Atresia.

BACKGROUND: The management of neonates with long-gap esophageal atresia (LGEA) combined with distal congenital esophageal strictures (CES) is challenging. We sought to review our approach for this rare set of anomalies. METHODS: We reviewed children with LGEA + CES surgically treated at two institutions (2018-2024). LGEA repair was performed using the Foker technique (traction-induced esophageal lengthening). A CES strategy was chosen based on preoperative evaluations and intraoperative findings. The configuration and length of the CES were assessed using retrograde flexible esophagoscopy via gastrostomy with contrast fluoroscopy. RESULTS: Eight patients (75% male) with LGEA + CES were treated: Four had type A and four had type B EA. Median gap length was 3.5 cm. Three underwent thoracoscopic esophageal lengthening. After a median follow-up of 18 months (IQR: 9-25), all retained their native esophagus. However, those who had CES resection concurrent with the lengthening process or at the time of EA anastomosis had more challenging perioperative courses: one required additional time on traction and another required esophageal anastomotic stricture resection. CONCLUSIONS: Our experience with LGEA and distal CES emphasizes tailoring surgical approaches to each patient's unique condition, avoiding a one-size-fits-all strategy. However, if the esophageal tissue above the distal CES is in good condition, our preference has shifted towards retaining the CES during traction, performing gentle dilation at anastomosis time, and conducting definitive endoscopic management subsequently. We would caution against making the assumption that salvage of the native esophagus is not possible or that resection of the CES is always needed. LEVEL OF EVIDENCE: Level III.

Celiac disease and diabetes mellitus diagnosed in a pediatric patient with Hirschsprung disease.

Hirschsprung disease is a disorder of neural crest migration characterized by intestinal aganglionosis along a variable segment of the gastrointestinal tract. It is a complex disorder associated with several syndromes. Celiac disease is an autoimmune enteropathy characterized by dietary intolerance to gluten proteins and can be associated with autoimmune conditions such as diabetes mellitus. Celiac disease can mimic Hirschsprung disease when presenting with constipation and abdominal distention. We present the case of celiac disease diagnosed in a patient with Hirschsprung disease who subsequently developed type one diabetes mellitus.

Acute Treatment of Adolescent Cannabinoid Hyperemesis Syndrome With Haloperidol, Lorazepam, and/or Capsaicin: A Single Institution Case Series.

Cannabinoid hyperemesis syndrome (CHS), an under-recognized and seemingly paradoxical condition, arises in some adolescents and adults who chronically use cannabis. It presents acutely with intractable nausea, vomiting, and abdominal pain but standard antiemetic therapy leads to improvement for only a minority of patients. Randomized controlled trial evidence in adults indicates the superiority of haloperidol over ondansetron in alleviating the acute symptoms of CHS, but safe and effective treatment for adolescents with the disorder is currently unknown. The successful use of topical capsaicin has also been reported. We report a case series of 6 adolescent patients with CHS who presented to Johns Hopkins All Children's Hospital and were treated with haloperidol, lorazepam, and/or capsaicin. Four patients given 5 mg intravenous (IV) haloperidol and 2 mg IV lorazepam and 1 patient treated with 5 mg IV haloperidol and peri-umbilical topical capsaicin (0.025%) experienced full acute symptomatic relief. One patient, treated only with topical capsaicin, reported improvement of symptoms with some persistent nausea. Haloperidol/lorazepam, haloperidol/capsaicin, and topical capsaicin alone appear safe and effective in adolescents, but larger studies are required to confirm our findings.

Endoscopic Assessment and Serial Balloon Dilatation in a Toddler With Dyskeratosis Congenita-Hoyeraal-Hreidarsson Syndrome Following Bone Marrow Transplant: A Case Report.

We report a 3-year-old patient with suspected oropharyngeal graft-versus-host disease (GVHD) who developed progressive dysphagia to solids and liquids. The patient has a history of Dyskeratosis Congenita-Hoyeraal-Hreidarsson Syndrome with associated bone marrow failure requiring a nonmyeloablative matched sibling hematopoietic stem cell transplant. Esophagram revealed significant narrowing in the cricopharyngeal region. Subsequent esophagoscopy showed a proximal, high-grade pinhole esophageal stricture that was very difficult to visualize and cannulate. High-grade esophageal strictures are uncommon in very young children with GVHD. We believe the patient's underlying Dyskeratosis Congenita-Hoyeraal-Hreidarsson Syndrome in the setting of inflammatory changes seen in GVHD following hematopoietic stem cell transplant set the stage for a high-grade esophageal obstruction. The patient's symptoms improved with serial endoscopic balloon dilation.

Clinician Experience with Using Hypoallergenic Formulas to Treat Infants with Suspected Cow's Milk Protein Allergy: A Secondary Analysis of a Prospective Survey Cohort.

Purpose: Cow's milk protein allergy (CMPA) is a common condition in infants, but little is known about healthcare providers' clinical experience treating infants with CMPA. To address this gap, we analyzed prospectively collected data from healthcare providers (HCPs) who treated infants under six months old with suspected CMPA using hypoallergenic formulas. The study focused on a commercial extensively hydrolyzed formula containing Lactobacillus rhamnosus GG (ATCC53103) (eHF-LGG) or a commercial amino acid formula (AAF). Methods: In this secondary analysis of prospectively collected survey data, 52 HCPs treated 329 infants under six months old with suspected CMPA using hypoallergenic formulas. A series of two de-identified surveys per patient were collected by HCPs to assess short-term symptom relief in the patients and HCP's satisfaction with the management strategies. The initial survey was completed at the initiation of treatment of CMPA, and the second survey was completed at a follow-up visit. Results: The majority of HCPs (87%) in the study were general pediatricians, and most saw 2 to 10 CMPA patients weekly. Results showed that clinicians reported satisfaction with treatment in 95% of patients in the EHF cohort and 97% of patients in the AAF cohort and achieved expected clinical results in 93% and 97% of patients using eHF and AAF, respectively. Furthermore, few patients were switched from the hypoallergenic formula once initiated. Conclusion: The study provides new insights into HCP perspectives on treating infants with CMPA and supports using hypoallergenic formulas to manage this condition. However, additional prospective controlled studies are needed to confirm these initial findings.

Peeling Back the Layers: Sloughing Esophagitis in a Teenager with Chronic Vomiting.

Esophagitis dissecans superficialis (EsoDS) is a rare condition characterized by the shedding of superficial esophageal epithelium. Limited data exists on EsoDS in the pediatric population. We present a case of a 17-year-old female with chronic nausea and vomiting diagnosed with EsoDS. Endoscopy revealed esophageal mucosal sloughing, and histology confirmed esophagitis with mucosal necrosis. EsoDS is underrecognized, and its association with psychoactive medications remains unclear. Fortunately, EsoDS cases tend to resolve spontaneously without complications. Awareness of EsoDS is essential, and further research is needed to understand its prevalence and outcomes in pediatric patients.

Esophageal Heterotopic Pancreas in an Asymptomatic 2-Year-Old With VACTERL Association.

A 2-year-old male with VACTERL association and asthma presented to the emergency room due to asthma exacerbation. Chest radiography revealed lingular pneumonia and thickening of the left paraspinal line of the gastroesophageal junction. Chest computed tomography confirmed a heterogeneous fluid- and gas-filled structure at the left posterior lateral posterolateral aspect of the esophagus, which was suspected to be an esophageal diverticulum on an upper gastrointestinal series. The esophageal diverticulum was excised via left thoracoscopy, and pathological examination revealed pancreatic tissue. Heterotopic pancreas lacks anatomical, vascular, or ductal continuity with the native pancreas. It is usually asymptomatic, but when discovered, it usually occurs later in life. It has been described in the foregut, but is not as common in the esophagus, especially in the pediatric population. This case report highlights the rare occurrence, and importance of considering, esophageal heterotopic pancreas within an esophageal diverticulum in an asymptomatic patient with VACTERL association.

Avoiding Surgery: Endoscopic Treatment of Congenital Duodenal Stenosis.

Duodenal stenosis is a rare congenital anomaly that is typically treated surgically, although endoscopic incisional therapy (EIT) and balloon dilation are minimally invasive alternatives. We present a case of a 15-month-old male with vomiting and difficulty tolerating solid food due to severe congenital duodenal stenosis. The patient underwent EIT and serial duodenal dilation to a diameter of 20 mm, which resulted in significant symptom improvement. Intralesional corticosteroid injection (ISI) was administered to help prevent the duodenal septum from restricturing. The combination of EIT, balloon dilation, and ISI was successful in treating the patient's congenital duodenal stenosis and avoided the need for surgery. However, further studies are required to confirm the efficacy of this treatment approach in this patient population. This report highlights the potential of this minimally invasive approach as an alternative to surgical intervention in the management of congenital duodenal stenosis.