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BACKGROUND: Small bowel obstruction (SBO) is a known complication following congenital diaphragmatic hernia (CDH) repair, resulting in significant morbidity and potential mortality. Our study aims to evaluate the incidence and risk factors for SBO following CDH repair. METHODS: A single-institution retrospective review evaluated all CDH births between January 2010 and September 2022 (n = 120). Risk factors for SBO were analyzed, including operative approach, type of repair, need for extracorporeal membrane oxygenation (ECMO), and additional abdominal surgeries (gastrostomy tube and fundoplication). RESULTS: 120 patients were included. 16 (13%) patients developed an SBO, of which 94% were due to adhesive bands. The median time to SBO was 7.5 months. 15/16 (94%) patients required operative intervention. Need for ECMO (P < 0.01), prior gastrostomy tube (P < 0.01), and prior fundoplication (P < 0.01) were associated with an increased risk of SBO, as were longer time to initial CDH repair (6 days vs 3 days; P < 0.01) and longer length of initial hospitalization (63 days vs 29 days; P = 0.01). DISCUSSION: Neonates with increased acuity of illness (ie, those requiring ECMO, additional abdominal operations, longer time to repair, and longer initial hospitalizations) appear to have an increased risk of developing adhesive SBO after CDH repair. More than 90% of patients who developed SBO required surgery.
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BACKGROUND: Functional constipation (FC), a disorder of the gut-brain interaction of multifactorial pathophysiology that is prevalent in paediatrics. It is associated with bothersome symptoms, increased healthcare costs, disgruntled caregivers and impaired health-related quality of life. Paediatric FC is a clinical diagnosis based on the Rome IV criteria and is characterised by decreased bowel movement frequency and/or hard, painful stools and can be complicated by retentive faecal incontinence. Stressful life events, difficult temperaments and emotional and behavioural challenges have been implicated in increasing risk of developing paediatric FC. AIMS: To provide current concepts in pathophysiology, evaluation and management of paediatric FC. METHODS: We reviewed pertinent literature after a comprehensive search utilising PubMed with keywords FC, chronic childhood constipation and paediatric FC. RESULTS: In the last decade, advances in our understanding of paediatric FC have changed the landscape of diagnosing and treating this disorder. Although polyethylene glycol is the first-line treatment for maintenance of FC, the armamentarium of therapeutics has expanded including the first Food and Drug Administration- agent, linaclotide, for children 6-17 years of age in conjunction with more emphasis on behavioural and physical therapy interventions. CONCLUSIONS: Treatment approach to paediatric FC should be individualised and integrated focusing on parental education, lifestyle and behavioural modifications, and pharmacological therapy to maximise therapeutic success. This review highlights advances in pathophysiology, diagnosis and treatment of FC in children.
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Estreñimiento , Humanos , Estreñimiento/terapia , Estreñimiento/fisiopatología , Estreñimiento/diagnóstico , Niño , Calidad de Vida , AdolescenteRESUMEN
Transition of Care for Adolescents and Young Adults (AYA) with Neurogastroenterology & Motility (NGM) Disorders.
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Enfermedades Gastrointestinales , Transición a la Atención de Adultos , Humanos , Adolescente , Adulto Joven , Enfermedades Gastrointestinales/terapia , Gastroenterología/organización & administración , Motilidad GastrointestinalRESUMEN
BACKGROUND: Linaclotide, a guanylate cyclase C agonist, has been approved in the USA for the treatment of chronic idiopathic constipation and irritable bowel syndrome with predominant constipation in adults. We aimed to assess the efficacy and safety of linaclotide in paediatric patients aged 6-17 years with functional constipation. METHODS: This randomised, double-blind, placebo-controlled, multicentre, phase 3 study was done at 64 clinic or hospital sites in seven countries (USA, Canada, Israel, Italy, the Netherlands, Ukraine, and Estonia). Patients aged 6-17 years who met modified Rome III criteria for functional constipation were randomly assigned (1:1), with a block size of four and stratified by age (6-11 years and 12-17 years), to receive either oral linaclotide 72 µg or placebo once daily for 12 weeks. Participants, investigators, and data assessors were masked to assignment. The primary efficacy endpoint was change from baseline (CFB) in the 12-week frequency rate of spontaneous bowel movements (SBMs; occurring in the absence of rescue medication on the calendar day of or before the bowel movement) per week and the secondary efficacy endpoint was CFB in stool consistency over the 12-week treatment period; efficacy and safety were analysed in all patients in the randomised population who received at least one dose of study intervention (modified intention-to-treat population and safety population, respectively). The study is registered with ClinicalTrials.gov, NCT04026113, and the functional constipation part of the study is complete. FINDINGS: Between Oct 1, 2019, and March 21, 2022, 330 patients were enrolled and randomly assigned to linaclotide (n=166) or placebo (n=164). Two patients in the linaclotide group did not receive any treatment; thus, efficacy and safety endpoints were assessed in 328 patients (164 patients in each group). 293 (89%) patients completed the 12-week treatment period (148 in the linaclotide group and 145 in the placebo group). 181 (55%) of 328 patients were female and 147 (45%) were male. At baseline, the mean frequency rate for SBMs was 1·28 SBMs per week (SD 0·87) for placebo and 1·16 SBMs per week (0·83) for linaclotide, increasing to 2·29 SBMs per week (1·99) for placebo and 3·41 SBMs per week (2·76) for linaclotide during intervention. Compared with placebo (least-squares mean [LSM] CFB 1·05 SBMs per week [SE 0·19]), patients treated with linaclotide showed significant improvement in SBM frequency (LSM CFB 2·22 SBMs per week [0·19]; LSM CFB difference 1·17 SBMs per week [95% CI 0·65-1·69]; p<0·0001). Linaclotide also significantly improved stool consistency over placebo (LSM CFB 1·11 [SE 0·08] vs 0·69 [0·08]; LSM CFB difference 0·42 [95% CI 0·21-0·64]; p=0·0001). The most reported treatment-emergent adverse event (TEAE) by patients treated with linaclotide was diarrhoea (seven [4%] of 164 vs three [2%] of 164 patients in the placebo group) and by patients treated with placebo was COVID-19 (five [3%] vs four [2%] in the linaclotide group). The most frequent treatment-related TEAE was diarrhoea (linaclotide: six [4%] patients; placebo: two [1%] patients). One serious adverse event of special interest (treatment-related severe diarrhoea resulting in dehydration and hospitalisation) occurred in a female patient aged 17 years in the linaclotide group; this case resolved without sequelae after administration of intravenous fluids. No deaths occurred during the study. INTERPRETATION: Linaclotide is an efficacious and well tolerated treatment for functional constipation in paediatric patients and has subsequently been approved by the US Food and Drug Administration for this indication. FUNDING: AbbVie and Ironwood Pharmaceuticals.
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Estreñimiento , Péptidos , Adulto , Humanos , Masculino , Femenino , Niño , Resultado del Tratamiento , Estreñimiento/tratamiento farmacológico , Estreñimiento/inducido químicamente , Péptidos/efectos adversos , Diarrea/inducido químicamente , Método Doble CiegoRESUMEN
INTRODUCTION: To date, no international guidelines have been published for the treatment of paediatric functional abdominal pain disorders (FAPDs), subcategorised into functional abdominal pain-not otherwise specified (FAP-NOS), irritable bowel syndrome (IBS), functional dyspepsia and abdominal migraine (AM). We aim for a treatment guideline, focusing on FAP-NOS, IBS and AM, that appreciates the extensive array of available therapies in this field. We present the prospective operating procedure and technical summary protocol in this manuscript. METHODS: Grading of Recommendations, Assessment, Development and Evaluation (GRADE) will be followed in the development of the guideline, following the approach as laid out in the GRADE handbook, supported by the WHO. The Guideline Development Group (GDG) is formed by paediatric gastroenterologists from both the European Society for Pediatric Gastroenterology, Hepatology and Nutrition, as well as the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Also, one clinical psychologist with expertise in FAPDs is a voting member in the GDG. A final consensus list of treatment options is translated into 'patient, intervention, comparison, outcome' format options. Prospective agreement on the magnitude of health benefits or harms categories was reached through a Delphi process among the GDG to support grading of the literature.There will be a detailed technical evidence review with randomised controlled trial data that will be judged for risk of bias with the Cochrane tool. Recommendations are preferably based on GRADE but could also be best practice statements following the available evidence. A full Delphi process will be used to make recommendations using online response systems. This set of procedures has been approved by all members of the GDG.
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Dispepsia , Gastroenterología , Síndrome del Colon Irritable , Trastornos Migrañosos , Niño , Humanos , Dolor Abdominal , Dispepsia/tratamiento farmacológico , Estudios Prospectivos , Ensayos Clínicos Controlados Aleatorios como Asunto , Guías de Práctica Clínica como AsuntoRESUMEN
Infants born with esophageal atresia and tracheoesophageal fistula, a complex congenital malformation occurring in 1/2500-4000 live births, may suffer threats to their cardiac, respiratory, and digestive health in addition to anomalies that may exist in the genitourinary and musculoskeletal systems. Optimal care for these patients throughout their lives is best achieved through a coordinated, multidisciplinary approach that our health care system is not always well-equipped to provide. This review, though not exhaustive, highlights the components of care that pertain to initial surgical reconstruction and subsequent diagnosis and management of the complications that are most frequently encountered. Authors from among the many specialties involved in the care of these patients summarize the current best practice with attention to the most recent advances. Assessment and improvement of quality of life and transition to adult specialists as children grow to adulthood is also reviewed.
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Atresia Esofágica , Enfermedades del Recién Nacido , Fístula Traqueoesofágica , Lactante , Recién Nacido , Niño , Humanos , Fístula Traqueoesofágica/diagnóstico , Fístula Traqueoesofágica/genética , Fístula Traqueoesofágica/cirugía , Atresia Esofágica/complicaciones , Atresia Esofágica/diagnóstico , Atresia Esofágica/genética , Calidad de Vida , Estudios RetrospectivosRESUMEN
Our primary study objectives were to (i) determine the proportion of children admitted to the Pediatric Intensive Care Unit (PICU) with malnutrition diagnoses, (ii) compare healthcare utilization by malnourished and non-malnourished PICU patients, and (iii) examine the impact of implementing malnutrition screening and coding practices at a major academic urban tertiary care medical center. Using patient records, we conducted a retrospective analysis of 4106 children admitted to the PICU for severe illnesses between 2011 and 2019. Patients were identified as malnourished if records showed an ICD-9 or ICD-10 code for malnutrition. We compared malnourished and non-malnourished patients by age, admitting diagnoses, number of comorbid conditions, and clinical outcomes (length of stay, hospital readmission). About 1 of every 5 PICU-admitted patients (783/4106) had a malnutrition diagnosis. Patients with malnutrition were younger (mean age 6.2 vs. 6.9 years, p < 0.01) and had more comorbid conditions (14.3 vs. 7.9, p < 0.01) than those without. Malnourished patients had longer hospital stays (26.1 vs. 10.0 days, p < 0.01) and higher 30-day readmission rates (10% vs. 7%, p = 0.03). Implementation of malnutrition screening and coding practices was associated with an increase in malnutrition diagnosis. In this study of children admitted to the PICU, malnourished patients had more comorbid diagnoses and used more healthcare resources (prolonged hospitalizations and higher 30-day readmission rates), leading to higher healthcare costs. Such findings underscore the need for policies, training, and programs emphasizing identification and treatment of malnutrition at hospitals caring for critically ill children.
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Enfermedad Crítica , Desnutrición , Niño , Humanos , Niño Hospitalizado , Estudios Retrospectivos , Desnutrición/diagnóstico , Desnutrición/epidemiología , Desnutrición/terapia , Tiempo de Internación , Atención a la Salud , Estado NutricionalRESUMEN
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.
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Trastornos de Deglución , Gastroenterología , Medicina , Humanos , Niño , Trastornos de Deglución/diagnóstico , Trastornos de Deglución/etiología , Trastornos de Deglución/terapia , PulmónRESUMEN
Although pediatric neurogastroenterology and motility (PNGM) disorders are prevalent, often debilitating, and remain challenging to diagnose and treat, this field has made remarkable progress in the last decade. Diagnostic and therapeutic gastrointestinal endoscopy emerged as a valuable tool in the management of PNGM disorders. Novel modalities such as functional lumen imaging probe, per-oral endoscopic myotomy, gastric-POEM, and electrocautery incisional therapy have changed the diagnostic and therapeutic landscape of PNGM. In this review, the authors highlight the emerging role of therapeutic and diagnostic endoscopy in esophageal, gastric, small bowel, colonic, and anorectal disorders and disorders of gut and brain axis interaction.
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Acalasia del Esófago , Trastornos de la Motilidad Esofágica , Cirugía Endoscópica por Orificios Naturales , Humanos , Niño , Resultado del Tratamiento , Endoscopía Gastrointestinal/métodos , Esófago , Estómago , Tracto Gastrointestinal , Cirugía Endoscópica por Orificios Naturales/métodos , Esfínter Esofágico Inferior , Trastornos de la Motilidad Esofágica/diagnóstico , Trastornos de la Motilidad Esofágica/cirugíaRESUMEN
Children with Hirschsprung disease have postoperative long-term sequelae in defecation that contribute to morbidity and mortality and significantly impact their quality of life. Pediatric patients experience ongoing long-term defecation concerns, which can include fecal incontinence (FI) and postoperative obstructive symptoms, such as constipation and Hirschsprung-associated enterocolitis. The American Pediatric Surgical Association has developed guidelines for management of these postoperative obstructive symptoms and FI. However, the evaluation and management of patients with postoperative defecation problems varies among different pediatric gastroenterology centers. This position paper from the Neurogastroenterology & Motility Committee of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition reviews the current evidence and provides suggestions for the evaluation and management of postoperative patients with Hirschsprung disease who present with persistent defecation problems.
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Incontinencia Fecal , Gastroenterología , Enfermedad de Hirschsprung , Niño , Humanos , Enfermedad de Hirschsprung/complicaciones , Enfermedad de Hirschsprung/cirugía , Calidad de Vida , Incontinencia Fecal/diagnóstico , Incontinencia Fecal/etiología , Incontinencia Fecal/terapia , Sociedades Médicas , América del NorteRESUMEN
Esophageal atresia (EA) with or without trachea-esophageal fistula is relatively common congenital malformation with most patients living into adulthood. As a result, care of the adult patient with EA is becoming more common. Although surgical repair has changed EA from a fatal to a livable condition, the residual effects of the anomaly may lead to a lifetime of complications. These include effects related to the underlying deformity such as atonicity of the esophageal segment, fistula recurrence, and esophageal cancer to complications of the surgery including anastomotic stricture, gastroesophageal reflux, and coping with an organ transposition. This review discusses the occurrence and management of these conditions in adulthood and the role of an effective transition from pediatric to adult care to optimize adult care treatment.
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Atresia Esofágica , Estenosis Esofágica , Fístula Traqueoesofágica , Transición a la Atención de Adultos , Humanos , Adulto , Niño , Atresia Esofágica/cirugía , Atresia Esofágica/complicaciones , Fístula Traqueoesofágica/cirugía , Fístula Traqueoesofágica/complicaciones , Tráquea/cirugía , Complicaciones Posoperatorias/epidemiología , Estenosis Esofágica/etiología , Estenosis Esofágica/cirugíaRESUMEN
[This corrects the article DOI: 10.1016/j.xhgg.2022.100107.].
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Esophageal atresias/tracheoesophageal fistulas (EA/TEF) are rare congenital anomalies caused by aberrant development of the foregut. Previous studies indicate that rare or de novo genetic variants significantly contribute to EA/TEF risk, and most individuals with EA/TEF do not have pathogenic genetic variants in established risk genes. To identify the genetic contributions to EA/TEF, we performed whole genome sequencing of 185 trios (probands and parents) with EA/TEF, including 59 isolated and 126 complex cases with additional congenital anomalies and/or neurodevelopmental disorders. There was a significant burden of protein-altering de novo coding variants in complex cases (p = 3.3 × 10-4), especially in genes that are intolerant of loss-of-function variants in the population. We performed simulation analysis of pathway enrichment based on background mutation rate and identified a number of pathways related to endocytosis and intracellular trafficking that as a group have a significant burden of protein-altering de novo variants. We assessed 18 variants for disease causality using CRISPR-Cas9 mutagenesis in Xenopus and confirmed 13 with tracheoesophageal phenotypes. Our results implicate disruption of endosome-mediated epithelial remodeling as a potential mechanism of foregut developmental defects. Our results suggest significant genetic heterogeneity of EA/TEF and may have implications for the mechanisms of other rare congenital anomalies.
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ABSTRACT: To characterize the current availability and scope of pediatric neurogastroenterology and motility (PNGM) services in North America (NA), the NASPGHAN-NGM committee distributed a self-reporting survey through the NASPGHAN bulletin board and mailing listserv, to compile a list of NA centers offering PNGM services, PNGM training, and the types of diagnostic and therapeutic PNGM procedures and services. We received responses that 54 centers in NA offer some form of PNGM services. Previously, the NASPGHAN website had last updated information from 2015 listing 36 centers in the USA and 2 in Canada. The American Neurogastroenterology and Motility Society (ANMS) website had 16 PNGM centers listed in NA in 2021. Neither of these resources capture additional information regarding training, research, advanced diagnostics, and therapeutics, and all available PNGM services. Our data highlights the growth in the field of PNGM services, and the variability of their distribution throughout the continent.
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Gastroenterología , Canadá , Niño , Gastroenterología/educación , Humanos , América del Norte , Servicios Postales , Encuestas y Cuestionarios , Estados UnidosRESUMEN
ABSTRACT: The purpose of this document is to provide guidance for establishing a pediatric neurogastroenterology and motility (PNGM) program, including considerations for personnel, equipment, and physical space requirements, and business planning, from members of the neurogastroenterology and motility (NGM) Committee of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) who have developed PNGM programs at various institutions. A business plan defining the needs for required personnel, dedicated physical space, procedures, clinical care, and equipment storage is a prerequisite. Thoughtful logistical planning should address provider schedules, clinical visits, procedure coordination, and prior authorization processes. A business-plan outlining equipment purchase with projected costs, revenue generation, and goals for future growth is desirable for obtaining institutional support, which is imperative to building a successful PNGM program.
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Gastroenterología , Niño , Gastroenterología/métodos , HumanosRESUMEN
BACKGROUND: Autoimmune gastrointestinal dysmotility (AGID) is a limited form of dysautonomia that can be paraneoplastic or idiopathic. Some presentations can be preceded by a viral infection. METHODS: We report a case of a 17-year-old girl that developed intractable nausea and early satiety after SARS-CoV-2 infection. KEY RESULTS: Over ten months, she required nasogastric and nasoduodenal tube feedings and finally was advanced to total parenteral nutrition to meet her caloric needs. Her α3 nicotinic ganglionic acetylcholine and anti-striational antibodies were mildly elevated. Gastrointestinal transit scintigraphy studies showed delayed gastric emptying and slowed small bowel transit. Thermoregulatory sweat test showed areas of anhidrosis consistent with autonomic sudomotor impairment. After IVIG treatment the patient's symptoms improved dramatically and she was able to tolerate full oral diet. This was reflected by improvement of her baseline transit studies and the thermoregulatory sweat test. CONCLUSIONS AND INFERENCES: This is the first report of AGID occurring after SARS-CoV-2 infection. The dramatic response to IVIG emphasizes the importance of early recognition and the reversible and treatable nature of this condition.
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Enfermedades Autoinmunes , COVID-19 , Enfermedades Gastrointestinales , Inmunoglobulinas Intravenosas , Adolescente , Autoanticuerpos , COVID-19/complicaciones , Femenino , Enfermedades Gastrointestinales/inmunología , Tránsito Gastrointestinal , Humanos , SARS-CoV-2RESUMEN
Pediatric endoscopy has revolutionized the way we diagnose and treat gastrointestinal disorders in children. Technological advances in computer processing and imaging continue to affect endoscopic equipment and advance diagnostic tools for pediatric endoscopy. Although commonly used by adult gastroenterologists, modalities, such as endomicroscopy, image-enhanced endoscopy, and impedance planimetry, are not routinely used in pediatric gastroenterology. This state-of-the-art review describes advances in diagnostic modalities, including image-enhanced endoscopy, confocal laser endomicroscopy, optical coherence tomography, endo functional luminal imaging probes, wireless motility/pH capsule, wireless colon capsule endoscopy, endoscopic ultrasound, and discusses the basic principles of each technology, including adult indications and pediatric applications, safety cost, and training data.
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BACKGROUND/PURPOSE: As survival rates for patients with congenital diaphragmatic hernia (CDH) increase, long-term sequelae become increasingly prevalent. We present the outcomes of patients who underwent CDH repair at our institution and discuss standardization of follow-up care in our long-term multidisciplinary follow-up clinic. METHODS: A retrospective review of patients followed in multidisciplinary clinic after CDH repair at our institution from January 1, 2005 to December 1, 2020. RESULTS: A total of 193 patients met inclusion criteria, 73 females (37.8%) and 120 males (62.2%). Left-sided defects were most common (75.7%), followed by right-sided defects (20.7%). Median age at repair was 4 days (IQR 3-6) and 59.6% of all defects required patch repair. Median length of stay was 29 days (IQR 16.8-50.0). Median length of follow up was 49 months (IQR 17.8-95.3) with 25 patients followed for more than 12 years. Long-term outcomes included gastroesophageal reflux disease (42.0%), diaphragmatic hernia recurrence (10.9%), asthma (23.6%), neurodevelopmental delay (28.6%), attention deficit hyperactivity disorder (7.3%), autism (1.6%), chest wall deformity (15.5%), scoliosis (11.4%), and inguinal hernia (6.7%). CONCLUSION: As survival of patients with CDH improves, long-term care must be continuously studied and fine-tuned to ensure appropriate surveillance and optimization of long-term outcomes.
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Hernias Diafragmáticas Congénitas , Escoliosis , Pared Torácica , Femenino , Hernias Diafragmáticas Congénitas/complicaciones , Hernias Diafragmáticas Congénitas/cirugía , Herniorrafia , Humanos , Masculino , Estudios Retrospectivos , Escoliosis/complicaciones , Pared Torácica/anomalías , Resultado del TratamientoRESUMEN
Children with disorders affecting the sensory and motor functions of the esophagus will present primarily with swallowing dysfunction, dysphagia, and chest pain, and those with disorders affecting the normal function of the stomach will present with symptoms like abdominal pain, nausea, and vomiting. Recent advances in the mechanisms of disease and technology have increased our understanding of gastrointestinal physiology and that knowledge has been applied to develop new diagnostic studies and therapeutic interventions. We present an overview of the clinical presentation, diagnosis, and treatment of common primary and secondary functional and motility disorders affecting the upper gastrointestinal tract in children.