ABSTRACT
Crohn disease (CD) is often complicated by bowel strictures that can lead to obstructive symptoms, resistant inflammation, and penetrating complications. Endoscopic balloon dilatation of CD strictures has emerged as a safe and effective technique for relieving these strictures, which may obviate the need for surgical intervention in the short and medium term. This technique appears to be underutilized in pediatric CD. This position paper of the Endoscopy Special Interest Group of European Society for Pediatric Gastroenterology, Hepatology and Nutrition describes the potential applications, appropriate evaluation, practical technique, and management of complications of this important procedure. The aim being to better integrate this therapeutic strategy in pediatric CD management.
Subject(s)
Crohn Disease , Humans , Child , Crohn Disease/complications , Crohn Disease/therapy , Constriction, Pathologic/etiology , Constriction, Pathologic/therapy , Dilatation/methods , Public Opinion , Treatment Outcome , Endoscopy, Gastrointestinal/methodsABSTRACT
OBJECTIVES: A high prevalence of eosinophilic esophagitis (EoE) has been reported in children with repaired esophageal atresia (EA). Topical steroids proved to be an effective and safe therapy in EoE, although not approved in pediatrics. We report the results of the first clinical trial of oral viscous budesonide (OVB) performed in children with EoE after repaired esophageal atresia (EoE-EA). METHODS: This open-label, single-arm, phase 2 clinical trial with randomized pharmacokinetic sampling, was conducted at the Bambino Gesù Children's Hospital between September 2019 and June 2021. EoE-EA patients received an age-banded dose of OVB twice daily for 12 weeks and were endoscopically evaluated. The primary endpoint was the rate of patients achieving histological remission. Secondary endpoints included clinical and endoscopic benefit after treatment, and safety assessments. RESULTS: Eight consecutive EA-EoE patients were enrolled (median age 9.1 years, interquartile range 5.5). Of these, 5 received 0.8 mg and 3 received 1.0 mg twice daily of OVB. Histological remission was obtained in all but 1 patient (87.5%). The clinical score showed significant improvement at the end of treatment in all patients. No endoscopic features of EoE were found after treatment. No treatment-emergent adverse event occurred. CONCLUSION: OVB is an effective, safe, and well-tolerated formulation of budesonide for use in pediatric patients with EoE-EA.
Subject(s)
Eosinophilic Esophagitis , Esophageal Atresia , Child , Humans , Infant , Eosinophilic Esophagitis/pathology , Esophageal Atresia/drug therapy , Esophageal Atresia/surgery , Esophageal Atresia/complications , Treatment Outcome , Budesonide/therapeutic use , Glucocorticoids/therapeutic useABSTRACT
Previous studies have demonstrated the safety of performing endoscopic retrograde cholangiopancreatography (ERCP) in the pediatric population; however, few have addressed the outcomes of children undergoing ERCP during acute pancreatitis (AP). We hypothesize that ERCP performed in the setting of AP can be executed with similar technical success and adverse event profiles to those in pediatric patients without pancreatitis. Using the Pediatric ERCP Database Initiative, a multi-national and multi-institutional prospectively collected dataset, we analyzed 1124 ERCPs. One hundred and ninety-four (17%) of these procedures were performed in the setting of AP. There were no difference in the procedure success rate, procedure time, cannulation time, fluoroscopy time, or American Society of Anesthesiology class despite patients with AP having higher American Society of Gastrointestinal Endoscopy grading difficulty scores. This study suggests that ERCP can be safely and efficiently performed in pediatric patients with AP when appropriately indicated.
Subject(s)
Cholangiopancreatography, Endoscopic Retrograde , Pancreatitis , Child , Humans , Cholangiopancreatography, Endoscopic Retrograde/adverse effects , Cholangiopancreatography, Endoscopic Retrograde/methods , Pancreatitis/diagnostic imaging , Pancreatitis/surgery , Pancreatitis/epidemiology , Acute Disease , Retrospective Studies , FluoroscopyABSTRACT
This study examines the role of trainee involvement with pediatric endoscopic retrograde cholangiopancreatography (ERCP) and whether it affects the procedure's success, post-procedural adverse outcomes, and duration. A secondary analysis of the Pediatric ERCP Database Initiative, an international database, was performed. Consecutive ERCPs on children <19 years of age from 18 centers were entered prospectively into the database. In total 1124 ERCPs were entered into the database, of which 320 (28%) were performed by trainees. The results showed that the presence of trainees did not impact technical success ( P = 0.65) or adverse events rates ( P = 0.43). Rates of post-ERCP pancreatitis, pain, and bleeding were similar between groups ( P > 0.05). Fewer cases involving trainees were in the top quartile (>58 minutes) of procedural time (19% vs 26%; P = 0.02). Overall, our findings indicate trainee involvement in pediatric ERCP is safe.
Subject(s)
Cholangiopancreatography, Endoscopic Retrograde , Pancreatitis , Child , Humans , Cholangiopancreatography, Endoscopic Retrograde/adverse effects , Cholangiopancreatography, Endoscopic Retrograde/methods , Pancreatitis/epidemiology , Pancreatitis/etiology , Retrospective StudiesABSTRACT
Visceral myopathy is a rare, life-threatening disease linked to identified genetic mutations in 60% of cases. Mostly due to the dearth of knowledge regarding its pathogenesis, effective treatments are lacking. The disease is most commonly diagnosed in children with recurrent or persistent disabling episodes of functional intestinal obstruction, which can be life threatening, often requiring long-term parenteral or specialized enteral nutritional support. Although these interventions are undisputedly life-saving as they allow affected individuals to avoid malnutrition and related complications, they also seriously compromise their quality of life and can carry the risk of sepsis and thrombosis. Animal models for visceral myopathy, which could be crucial for advancing the scientific knowledge of this condition, are scarce. Clearly, a collaborative network is needed to develop research plans to clarify genotype-phenotype correlations and unravel molecular mechanisms to provide targeted therapeutic strategies. This paper represents a summary report of the first 'European Forum on Visceral Myopathy'. This forum was attended by an international interdisciplinary working group that met to better understand visceral myopathy and foster interaction among scientists actively involved in the field and clinicians who specialize in care of people with visceral myopathy.
Subject(s)
Intestinal Pseudo-Obstruction , Malnutrition , Animals , Child , Humans , Quality of Life , Models, Animal , Mutation , Rare DiseasesABSTRACT
OBJECTIVES: Endoscopic retrograde cholangiopancreatography (ERCP) in adults has been extensively studied through multicenter prospective studies. Similar pediatric studies are lacking. The Pediatric ERCP Database Initiative (PEDI) is a multicenter collaborative aiming to evaluate the indications and technical outcomes in pediatric ERCPs. METHODS: In this prospective cohort study, data were recorded for pediatric ERCPs performed across 15 centers. A pre-procedure, procedure, 2-week post-procedure follow-up, and adverse event form were completed for each ERCP performed. Univariate and stepwise linear regression was performed to identify factors associated with technically successful procedures and adverse events. RESULTS: A total of 1124 ERCPs were performed on 857 patients from May 1, 2014 to May 1, 2018. The median age was 13.5 years [interquartile range (IQR) 9.0-15.7]. Procedures were technically successful in the majority of cases (90.5%) with success more commonly encountered for procedures with biliary indications [odds ratio (OR) 4.2] and less commonly encountered for native papilla anatomy (OR 0.4) and in children <3 years (OR 0.3). Cannulation was more often successful with biliary cannulation (95.9%) compared to pancreatic cannulation via the major papilla (89.6%, P < 0.0001) or minor papilla (71.2%, P < 0.0005). The most commonly identified adverse events included post-ERCP pancreatitis (5%), pain not related to post-ERCP pancreatitis (1.8%), and bleeding (1.2%). Risk factors for the development of each were identified. CONCLUSIONS: This large prospective study demonstrates that ERCP is reliable and safe in the pediatric population. It highlights the utility of PEDI in evaluating the technical outcomes of pediatric ERCPs and demonstrates the potential of PEDI for future studies in pediatric ERCPs.
Subject(s)
Cholangiopancreatography, Endoscopic Retrograde , Pancreatitis , Humans , Child , Adult , Adolescent , Cholangiopancreatography, Endoscopic Retrograde/adverse effects , Cholangiopancreatography, Endoscopic Retrograde/methods , Prospective Studies , Retrospective Studies , Catheterization/adverse effects , Pancreatitis/etiologyABSTRACT
BACKGROUND AND AIMS: Ionizing radiation exposure during endoscopic retrograde cholangiopancreatography (ERCP) is an important quality issue especially in children. We aim to identify factors associated with extended fluoroscopy time (FT) in children undergoing ERCP. METHODS: ERCP on children <18âyears from 15 centers were entered prospectively into a REDCap database from May 2014 until May 2018. Data were retrospectively evaluated for outcome and quality measures. A univariate and step-wise linear regression analysis was performed to identify factors associated with increased FT. RESULTS: 1073 ERCPs performed in 816 unique patients met inclusion criteria. Median age was 12.2âyears (interquartile range [IQR] 9.3-15.8). 767 (71%) patients had native papillae. The median FT was 120âseconds (IQR 60-240). Factors associated with increased FT included procedures performed on patients with chronic pancreatitis, ERCPs with American Society of Gastrointestinal Endoscopy (ASGE) difficulty grade >3, ERCPs performed by pediatric gastroenterologist (GI) with adult GI supervision, and ERCPs performed at non-free standing children's hospitals. Hispanic ethnicity was the only factor associated with lower FT. CONCLUSION: Several factors were associated with prolonged FTs in pediatric ERCP that differed from adult studies. This underscores that adult quality indicators cannot always be translated to pediatric patients. This data can better identify children with higher risk for radiation exposure and improve quality outcomes during pediatric ERCP.
Subject(s)
Cholangiopancreatography, Endoscopic Retrograde , Radiation Exposure , Adult , Child , Cholangiopancreatography, Endoscopic Retrograde/adverse effects , Cholangiopancreatography, Endoscopic Retrograde/methods , Cohort Studies , Fluoroscopy/adverse effects , Humans , Radiation Exposure/adverse effects , Retrospective StudiesABSTRACT
OBJECTIVE: To analyze the findings of both multichannel intraluminal impedance with pH (MII-pH) and endoscopy/histopathology in children with esophageal atresia at age 1 year, according to current recommendations for the evaluation of gastroesophageal reflux disease (GERD) in esophageal atresia. STUDY DESIGN: We retrospectively reviewed both MII-pH and endoscopy/histopathology performed in 1-year-old children with esophageal atresia who were followed up in accordance with international recommendations. Demographic data and clinical characteristics were also reviewed to investigate factors associated with abnormal GERD investigations. RESULTS: In our study cohort of 48 children with esophageal atresia, microscopic esophagitis was found in 33 (69%) and pathological esophageal acid exposure on MII-pH was detected in 12 (25%). Among baseline variables, only the presence of long-gap esophageal atresia was associated with abnormal MII-pH. Distal baseline impedance was significantly lower in patients with microscopic esophagitis, and it showed a very good diagnostic performance in predicting histological changes. CONCLUSIONS: Histological esophagitis is highly prevalent at 1 year after esophageal atresia repair, but our results do not support a definitive causative role of acid-induced GERD. Instead, they support the hypothesis that chronic stasis in the dysmotile esophagus might lead to histological changes. MII-pH may be a helpful tool in selecting patients who need closer endoscopic surveillance and/or benefit from acid suppression.
Subject(s)
Esophageal Atresia/surgery , Esophagoplasty/adverse effects , Esophagus/physiopathology , Gastroesophageal Reflux/diagnosis , Postoperative Complications , Adolescent , Child , Electric Impedance , Endoscopy, Gastrointestinal , Esophageal pH Monitoring/methods , Esophagus/metabolism , Female , Follow-Up Studies , Gastroesophageal Reflux/etiology , Gastroesophageal Reflux/physiopathology , Humans , Male , Manometry , Retrospective Studies , Time FactorsABSTRACT
PURPOSE OF REVIEW: The present review aims at describing recent advances in therapeutic strategies for the treatment of benign esophageal strictures in children. We discuss current knowledge and practice on esophageal dilations, which are still the mainstream of treatment. We present new evidence about adjuvant treatments for recurrent and refractory strictures, including endoscopic incisional therapy, esophageal stenting, intralesional or topical mytomicin C and intralesional, systemic or topical steroids. RECENT FINDINGS: Current evidence on esophageal dilations is not sufficient to establish superiority of one of the available techniques, especially the use of balloon or bougie dilators, but a prospective international cohort study on anastomotic stricture in esophageal atresia is underway to address this issue. Recurrent and refractory strictures still represent a challenge, since none of the adjuvant pharmacological and mechanical interventions has shown to be enough feasible, effective and safe to revolutionize clinical practice. SUMMARY: In the last couple of years, several encouraging results have been published on management of esophageal strictures in children. Further research is needed, hopefully directed toward secure, easily reproducible and minimally invasive measures.
Subject(s)
Esophageal Stenosis , Child , Cohort Studies , Esophageal Stenosis/therapy , Esophagoscopy , Humans , Prospective Studies , Treatment OutcomeABSTRACT
OBJECTIVES: The aim of this study was to assess whether clinicians approached the management of children with esophageal atresia (EA) in accordance with the 2016 European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN)/North American Society of Paediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) guidelines on the management of gastrointestinal and nutritional complications in this cohort. METHODS: We invited expert physicians and surgeons closely involved in the care of children with EA (members of the International network on esophageal atresia [INoEA], ESPGHAN EA working group, French national EA registry, European pediatric surgical association (EUPSA), and European rare disease reference network [ERNICA]) to participate in an anonymous online survey containing 15 multiple choice questions concerning the management of gastrointestinal and nutritional complications in children with EA. Questions were based on the management of gastroesophageal reflux disease (GERD) dysphagia, cyanotic spells, feeding and nutrition, anastamotic strictures, and transition to adult care as detailed in the 2016 guidelines. RESULTS: Median concordance with ESPGHAN/NASPHAN EA Guidelines was 69% (16-100%, SD 16%) across all responders. Areas of greatest concordance were in the fields of surveillance endoscopy and medical management of GERD. Areas for potential educational opportunities include: the differential diagnosis and appropriate investigation of dysphagia and the diagnostic evaluation of extraesophageal symptoms. CONCLUSIONS: This survey highlights the importance of improving the understanding and adherence to the EA guidelines amongst clinicians involved in the care of these patients.
Subject(s)
Deglutition Disorders , Esophageal Atresia , Gastroenterology , Gastroesophageal Reflux , Adult , Child , Esophageal Atresia/complications , Esophageal Atresia/therapy , Gastroesophageal Reflux/complications , Gastroesophageal Reflux/diagnosis , Gastroesophageal Reflux/therapy , Humans , Surveys and QuestionnairesABSTRACT
BACKGROUND: The European Society for Paediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) position paper from 2015 on percutaneous endoscopic gastrostomy (PEG) required updating in the light of recent clinical knowledge and data published in medical journals since 2014. METHODS: A systematic review of medical literature from 2014 to 2020 was carried out. Consensus on the content of the manuscript, including recommendations, was achieved by the authors through electronic and virtual means. The expert opinion of the authors is also expressed in the manuscript when there was a lack of good scientific evidence regarding PEGs in children in the literature. RESULTS: The authors recommend that the indication for a PEG be individualized, and that the decision for PEG insertion is arrived at by a multidisciplinary team (MDT) having considered all appropriate circumstances. Well timed enteral nutrition is optimal to treat faltering growth to avoid complications of malnutrition and body composition. Timing, device choice and method of insertion is dependent on the local expertise and after due consideration with the MDT and family. Major complications such as inadvertent bowel perforation should be avoided by attention to good technique and by ensuring the appropriate experience of the operating team. Feeding can be initiated as early as 3âhours after tube placement in a stable child with iso-osmolar feeds of standard polymeric formula. Low-profile devices can be inserted initially using the single-stage procedure or after 2-3âmonths by replacing a standard PEG tube, in those requiring longer-term feeding. Having had a period of non-use and reliance upon oral intake for growth and weight gain-typically 8-12âweeks-a PEG may then safely be removed after due consultation. In the event of non-closure of the fistula the most successful method for closing it, to date, has been a surgical procedure, but the Over-The-Scope-Clip (OTSC) has recently been used with considerable success in this scenario. CONCLUSIONS: A multidisciplinary approach is mandatory for the best possible treatment of children with PEGs. Morbidity and mortality are minimized through team decisions on indications for insertion, adequate planning and preparation before the procedure, subsequent monitoring of patients, timing of the change to low-profile devices, management of any complications, and optimal timing of removal of the PEG.
Subject(s)
Gastroenterology , Child , Child Nutritional Physiological Phenomena , Enteral Nutrition , Gastrostomy , Humans , Nutritional StatusABSTRACT
OBJECTIVES: The main aim of this study was to determine the impact on clinical practice of the first European Society of Gastroenterology, Hepatology, and Nutrition (ESPGHAN) position paper on the diagnosis and management of nutritional and gastrointestinal problems in children with neurological impairment (NI). METHODS: In this pilot-study, a web-based questionnaire was distributed between November, 2019 and June, 2020, amongst ESPGHAN members using the ESPGHAN newsletter. Fifteen questions covered the most relevant aspects on nutritional management and gastrointestinal issues of children with NI. A descriptive analysis of responses was performed. RESULTS: A total of 150 health professionals from 23 countries responded to the survey. A considerable variation in clinical practice concerning many aspects of nutritional and gastrointestinal management of children with NI was observed. The most frequently used method for diagnosing oropharyngeal dysfunction was the direct observation of meals with or without the use of standardised scores (nâ=â103). Anthropometric measurements were the most commonly used tools for assessing nutritional status (nâ=â111). The best treatment for gastroesophageal reflux disease (GERD) was considered to be proton pump inhibitor therapy by most (nâ=â116) participants. Regarding tube feeding, nearly all respondents (nâ=â114) agreed that gastrostomy is the best enteral access to be used for long-term enteral feeding. Fundoplication was indicated at the time of gastrostomy placement especially in case of uncontrolled GERD. CONCLUSIONS: More studies are required to address open questions on adequate management of children with NI. Identifying knowledge gaps paves the way for developing updated recommendations and improving patient care.
Subject(s)
Gastroesophageal Reflux , Nutrition Disorders , Child , Fundoplication , Gastroesophageal Reflux/complications , Gastroesophageal Reflux/diagnosis , Gastroesophageal Reflux/therapy , Humans , Pilot Projects , Surveys and QuestionnairesABSTRACT
BACKGROUND: Endoscopic dilatation is the first-line treatment of stricture formation after esophageal atresia (EA) repair. However, there is no consensus on how to perform these dilatation procedures which may lead to a large variation between centers, countries and doctor's experience. This is the first cross-sectional study to provide an overview on differences in endoscopic dilatation treatment of pediatric anastomotic strictures worldwide. METHODS: An online questionnaire was sent to members of five pediatric medical networks, experienced in treating anastomotic strictures in children with EA. The main outcome was the difference in endoscopic dilatation procedures in various centers worldwide, including technical details, dilatation approach (routine or only in symptomatic patients), and adjuvant treatment options. Descriptive statistics were performed with SPSS. RESULTS: Responses from 115 centers from 32 countries worldwide were analyzed. The preferred approach was balloon dilatation (68%) with a guidewire (66%), performed by a pediatric gastroenterologist (n = 103) or pediatric surgeon (n = 48) in symptomatic patients (68%). In most centers, hydrostatic pressure was used for balloon dilatation. The insufflation duration was standardized in 59 centers with a median duration of 60 (range 5-300) seconds. The preferred first-line adjunctive treatments in case of recurrent strictures were intralesional steroids and topical mitomycin C, in respectively 47% and 31% of the centers. CONCLUSIONS: We found a large variation in stricture management in children with EA, which confirms the current lack of consensus. International networks for rare diseases are required for harmonizing and comparing the procedures, for which we give several suggestions.
Subject(s)
Esophageal Atresia , Esophageal Stenosis , Child , Constriction, Pathologic , Cross-Sectional Studies , Dilatation , Esophageal Atresia/surgery , Esophageal Stenosis/etiology , Esophageal Stenosis/surgery , Humans , Postoperative Complications , Retrospective Studies , Surveys and Questionnaires , Treatment OutcomeABSTRACT
OBJECTIVES: Disk battery esophageal retention in children younger than 6 years represents an increasing endoscopic emergency, followed by a relevant risk of life-threatening late complications. Surgical removal after a failed endoscopic approach is rarely reported in the literature. We describe our experience in this scenario. METHODS: Two female asymptomatic patients aged 26 and 29 months presented within 4 hours after a witnessed ingestion of a 2-cm, 3-V lithium battery (CR2032) retained in the cervical esophagus. Both patients underwent a prolonged unsuccessful emergent endoscopic removal with a flexible instrument performed by an adult gastroenterologist. Both batteries fused with the esophageal wall were extracted through a longitudinal left cervical esophagotomy combined with minimal resection of necrotic tissues and repaired over a 12F feeding tube. RESULTS: Patients were extubated after 12 and 72 hours, respectively. Contrast study was performed after 20 and 13 days, respectively, before resuming oral feeding. At endoscopy, the first patient developed a 3-cm-long severe esophageal stenosis (35th day), followed by an asymptomatic tracheoesophageal fistula (60th day), which was conservatively treated. After spontaneous resolution of the tracheoesophageal fistula, esophageal stenosis progressed, partially responsive to esophageal stenting. Short esophagectomy is under evaluation. The second patient developed an asymptomatic limited stenosis, not requiring dilatation. CONCLUSIONS: The emergent management of lithium battery ingestion needs a structured timely multidisciplinary approach in the emergency department, an experienced pediatric endoscopist, and a simultaneous engagement of pediatric surgical expertise, even in patients who do not show bleeding, to reduce esophageal exposure time to high-voltage current released by batteries, which represents the main factor conditioning tissue damage and prognosis.
Subject(s)
Foreign Bodies , Tracheoesophageal Fistula , Child , Electric Power Supplies , Female , Foreign Bodies/surgery , Humans , Lithium , Tracheoesophageal Fistula/surgeryABSTRACT
OBJECTIVES: Jejunal tube feeding (JTF) is increasingly becoming the standard of care for children in whom gastric tube feeding is insufficient to achieve caloric needs. Given a lack of a systematic approach to the care of JTF in paediatric patients, the aim of this position paper is to provide expert guidance regarding the indications for its use and practical considerations to optimize its utility and safety. METHODS: A group of members of the Gastroenterology and Nutrition Committees of the European Society of Paediatric Gastroenterology Hepatology and Nutrition and of invited experts in the field was formed in September 2016 to produce this clinical guide. Seventeen clinical questions treating indications and contraindications, investigations before placement, techniques of placement, suitable feeds and feeding regimen, weaning from JTF, complications, long-term care, and ethical considerations were addressed.A systematic literature search was performed from 1982 to November 2018 using PubMed, the MEDLINE, and Cochrane Database of Systematic Reviews. Grading of Recommendations, Assessment, Development, and Evaluation was applied to evaluate the outcomes.During a consensus meeting, all recommendations were discussed and finalized. In the absence of evidence from randomized controlled trials, recommendations reflect the expert opinion of the authors. RESULTS: A total of 33 recommendations were voted on using the nominal voting technique. CONCLUSIONS: JTF is a safe and effective means of enteral feeding when gastric feeding is insufficient to meet caloric needs or is not possible. The decision to place a jejunal tube has to be made by close cooperation of a multidisciplinary team providing active follow-up and care.
Subject(s)
Enteral Nutrition , Gastrointestinal Diseases/therapy , Jejunum , Practice Guidelines as Topic , Child , Child Nutritional Physiological Phenomena , Europe , Humans , Jejunostomy , Nutritional Requirements , Societies, MedicalABSTRACT
This Executive summary of the Guideline on pediatric gastrointestinal endoscopy from the European Society of Gastrointestinal Endoscopy (ESGE) and the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) refers to infants, children, and adolescents aged 0â-â18 years. The areas covered include: indications for diagnostic and therapeutic esophagogastroduodenoscopy and ileocolonoscopy; endoscopy for foreign body ingestion; endoscopic management of corrosive ingestion and stricture/stenosis; upper and lower gastrointestinal bleeding; endoscopic retrograde cholangiopancreatography, and endoscopic ultrasonography. Percutaneous endoscopic gastrostomy and endoscopy specific to inflammatory bowel disease (IBD) have been dealt with in other Guidelines and are therefore not mentioned in this Guideline. Training and ongoing skill maintenance will be addressed in an imminent sister publication.
Subject(s)
Digestive System Diseases/therapy , Endoscopy, Gastrointestinal/methods , Endoscopy, Gastrointestinal/standards , Foreign Bodies/therapy , Adolescent , Burns, Chemical/etiology , Burns, Chemical/therapy , Caustics/toxicity , Child , Child, Preschool , Cholangiopancreatography, Endoscopic Retrograde/standards , Endosonography/standards , Gastrointestinal Tract/injuries , Humans , Infant , Infant, NewbornABSTRACT
BACKGROUND: Chronic granulomatous disease (CGD) is a primary immunodeficiency of phagocytes, characterized by life-threatening infections and hyperinflammation. Due to survival improvement, inflammatory bowel disease (IBD) is becoming increasingly relevant. Here, we report our 20 year experience. METHODS: We retrospectively analyzed clinic, endoscopic, and histologic features, as well as the management of CGD-IBD patients referred to the Bambino Gesù Children's Hospital in Rome, Italy. RESULTS: Of 20 patients with CGD, 9 presented with CGD-IBD at diagnosis and/or during follow-up. Symptoms occurred at a median age of 16 years (range 3.2-42), with a median delay of 6 months for endoscopic confirmation. Patients mainly complained of nonspecific diarrhea (55%), with discrepancy between symptom paucity and severe endoscopic appearance, mainly represented by extensive colonic involvement (44%). Histology revealed at least 2 characteristic features (epithelioid granulomas, pigmented macrophages, and increased eosinophils) in 78% of patients. Eight of 9 patients received oral mesalamine, and 5 required systemic steroids. One patient received azathioprine due to steroid dependence. No patient required biological therapy or surgery. Clinical remission was obtained in all patients, but the majority complained of mild relapses. Two episodes of severe infection occurred early after steroid therapy. CONCLUSIONS: Penetrance of CGD-IBD increases with age. Clinical manifestations may be subtle, and clinicians should have a low threshold to recommend endoscopy. Treatment with NSAIDs and/or steroids achieves a good response, but relapses usually occur. Infection surveillance is mandatory during treatment, to prevent opportunistic infections. A close collaboration between pediatric immunologists and gastroenterologists is pivotal, including combined follow-up.
Subject(s)
Granulomatous Disease, Chronic/complications , Inflammatory Bowel Diseases/etiology , Adolescent , Adult , Anti-Inflammatory Agents/therapeutic use , Child , Child, Preschool , Female , Follow-Up Studies , Granulomatous Disease, Chronic/diagnosis , Humans , Inflammatory Bowel Diseases/diagnosis , Inflammatory Bowel Diseases/drug therapy , Male , Recurrence , Retrospective Studies , Treatment Outcome , Young AdultABSTRACT
OBJECTIVES: Feeding difficulties are frequent in children with neurological impairments and can be associated with undernutrition, growth failure, micronutrients deficiencies, osteopenia, and nutritional comorbidities. Gastrointestinal problems including gastroesophageal reflux disease, constipation, and dysphagia are also frequent in this population and affect quality of life and nutritional status. There is currently a lack of a systematic approach to the care of these patients. With this report, European Society of Gastroenterology, Hepatology and Nutrition aims to develop uniform guidelines for the management of the gastroenterological and nutritional problems in children with neurological impairment. METHODS: Thirty-one clinical questions addressing the diagnosis, treatment, and prognosis of common gastrointestinal and nutritional problems in neurological impaired children were formulated. Questions aimed to assess the nutritional management including nutritional status, identifying undernutrition, monitoring nutritional status, and defining nutritional requirements; to classify gastrointestinal issues including oropharyngeal dysfunctions, motor and sensory function, gastroesophageal reflux disease, and constipation; to evaluate the indications for nutritional rehabilitation including enteral feeding and percutaneous gastrostomy/jejunostomy; to define indications for surgical interventions (eg, Nissen Fundoplication, esophagogastric disconnection); and finally to consider ethical issues related to digestive and nutritional problems in the severely neurologically impaired children. A systematic literature search was performed from 1980 to October 2015 using MEDLINE. The approach of the Grading of Recommendations Assessment, Development, and Evaluation was applied to evaluate the outcomes. During 2 consensus meetings, all recommendations were discussed and finalized. The group members voted on each recommendation using the nominal voting technique. Expert opinion was applied to support the recommendations where no randomized controlled trials were available.
Subject(s)
Gastrointestinal Diseases/diagnosis , Gastrointestinal Diseases/therapy , Nervous System Diseases/complications , Nutrition Disorders/diagnosis , Nutrition Disorders/therapy , Body Composition , Body Weights and Measures , Child , Diet Therapy/methods , Enteral Nutrition/methods , Gastrointestinal Diseases/etiology , Humans , Myofunctional Therapy , Nervous System Diseases/therapy , Nutrition Assessment , Nutrition Disorders/etiology , Nutrition Policy , Nutritional Requirements , PrognosisABSTRACT
This guideline refers to infants, children, and adolescents ages 0 to 18 years. The areas covered include indications for diagnostic and therapeutic esophagogastroduodenoscopy and ileocolonoscopy; endoscopy for foreign body ingestion; corrosive ingestion and stricture/stenosis endoscopic management; upper and lower gastrointestinal bleeding; endoscopic retrograde cholangiopancreatography; and endoscopic ultrasonography. Percutaneous endoscopic gastrostomy and endoscopy specific to inflammatory bowel disease has been dealt with in other guidelines and are therefore not mentioned in this guideline. Training and ongoing skill maintenance are to be dealt with in an imminent sister publication to this.
Subject(s)
Endoscopy, Gastrointestinal/standards , Gastrointestinal Diseases/therapy , Adolescent , Caustics , Child , Child, Preschool , Cholangiopancreatography, Endoscopic Retrograde , Colonoscopy , Endoscopy, Digestive System , Endosonography , Europe , Female , Foreign Bodies , Gastroenterology , Gastrointestinal Diseases/diagnosis , Gastrointestinal Hemorrhage , Humans , Infant , Infant, Newborn , Male , Pediatrics , SocietiesABSTRACT
PURPOSE OF REVIEW: Guidelines were recently published highlighting why esophageal atresia (EA) patients are prone to complication risks, and the need for long-term follow-up. In this review, we will focus on how to investigate and treat potential complications, as well as the pros and cons of different investigative and treatment modalities, and what areas continue to need further research. RECENT FINDINGS: EA patients are at high risk for gastroesophageal reflux and esophageal strictures, and the sequela that result. Extraintestinal manifestations of gastroesophageal reflux disease (GERD) can appear similar to other pathologic diagnoses commonly found in EA patients, such as congenital stricture, eosinophilic esophagitis, esophageal dysmotility, tracheomalacia, recurrent fistula, aspiration, etc. Therefore, it is important to have a standardized way to monitor for these issues. pH impedance allows for detection of nonacid reflux and the height of reflux, which are important in correlating symptoms with reflux episodes. A multidisciplinary approach is beneficial in evaluating and monitoring EA patients in the long term.