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Recent studies have reported a higher prevalence of eosinophilic esophagitis in children with esophageal atresia. Under recognition of eosinophilic esophagitis in these patients may lead to excessive use of antireflux therapy and an escalation of interventions, including fundoplication, as symptoms may be attributed to gastroesophageal reflux disea...
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Objectives:
A high prevalence of eosinophilic esophagitis (EoE) has been reported in children with repaired esophageal atresia (EoE). 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...
Citations
... Common symptoms include dysphagia, gastro-oesophageal reflux disease (GORD), oesophagitis, oesophageal strictures, and Barrett's oesophagus (BE). [3][4][5] Current literature suggests that the incidence of BE and eosinophilic oesophagitis (EoE) is higher in children born with OA. 6 Furthermore, two longterm follow-up studies have shown an increased risk of both BE and oesophageal carcinoma at a relatively young age in patients born with OA. 2,5 Guidelines for adults born with OA recommend routine endoscopy and lifelong screening of the upper GIT. 5 Despite this and the potential long-term GI complications, uncertainty remains regarding the necessity and frequency of endoscopic surveillance for children born with this disease. ...
... There are increasing reports in the literature of children with OA who present with BE, EoE, and oesophageal carcinoma. 2,5,6 In our study all children born with OA with either a clinical indication for endoscopy or those who did not have any previous endoscopies (and had a surveillance endoscopy) had abnormal endoscopic findings, which highlights the need for further research and treatment protocols for these children. ...
... 14 Gorter et al. published two cases of patients (6 and 12 years old) post-OA who presented with EoE. 13 Horning et al. stated that in patients with OA early recognition and treatment of EoE can prevent the incidence of strictures and the need for dilatation. 6 The European Society for Paediatric Gastroenterology, Hepatology and Nutrition/ North American Society For Paediatric Gastroenterology, Hepatology & Nutrition (ESPGHAN/ NASPGHAN) guidelines recommend exclusion of EoE in all patients born with OA and presenting with refractory dysphagia, GORD, coughing or choking, or recurrent strictures, despite maximum medical treatment, before antireflux surgery. 10 Although we did not have any patients with features suggesting BE, the current literature does indicate that the prevalence is higher in children born with OA. ...
... EoE is an increasingly recognized childhood disease. Cases of EoE occurring in patients with oesophageal atresia have been reported, with an incidence of up to 17%, although the exact prevalence of EoE in oesophageal atresia remains unknown in adults 31 . The prevalence of EoE was reported to be 9.5% in a prospective ...
Oesophageal atresia-tracheoesophageal fistula (EA-TEF) is a common congenital digestive disease. Patients with EA-TEF face gastrointestinal, surgical, respiratory, otolaryngological, nutritional, psychological and quality of life issues in childhood, adolescence and adulthood. Although consensus guidelines exist for the management of gastrointestinal, nutritional, surgical and respiratory problems in childhood, a systematic approach to the care of these patients in adolescence, during transition to adulthood and in adulthood is currently lacking. The Transition Working Group of the International Network on Oesophageal Atresia (INoEA) was charged with the task of developing uniform evidence-based guidelines for the management of complications through the transition from adolescence into adulthood. Forty-two questions addressing the diagnosis, treatment and prognosis of gastrointestinal, surgical, respiratory, otolaryngological, nutritional, psychological and quality of life complications that patients with EA-TEF face during adolescence and after the transition to adulthood were formulated. A systematic literature search was performed based on which recommendations were made. All recommendations were discussed and finalized during consensus meetings, and the group members voted on each recommendation. Expert opinion was used when no randomized controlled trials were available to support the recommendation. The list of the 42 statements, all based on expert opinion, was voted on and agreed upon.
... GERD is commonly encountered despite EA/TEF repair and tends not to improve over time, with a reported incidence in 35 to 60% of post-operative patients [32,35]. Eosinophilic esophagitis is an increasingly recognized entity that has overlapping symptoms with GERD, but is best treated with an elemental diet and topical corticosteroids [36]. ...
Background
Improved survival of neonates with esophageal atresia with/without tracheoesophageal fistula (EA/TEF) has resulted in increased prevalence of gastro-esophageal and respiratory-related morbidities. However, long-term outcome data on these patients remains limited, making it difficult to substantiate any guidelines on their chronic care. The purpose of our study is to report on their post-operative outcomes especially the long-term gastro-esophageal and respiratory morbidities.
Methods
This was a retrospective review of 65 patients (39 males, 26 females) who underwent EA/TEF repair from 1996 to 2019 at a single tertiary institution. Follow up data pertaining to clinical characteristics, operative management and long-term gastro-esophageal and respiratory morbidities was analyzed.
Results
Fifty-seven patients (87.7%) had Gross Type-C anatomy, followed by 5(7.7%) patients with Type-A, 1(1.5%) with Type-B and 1 with Type-D. One patient had a late diagnosis of H-type fistula (Type-E). Thirteen (20%) patients had long-gap EA.
Median age at first surgery was day 1 (IQR 1–2) of life. All patients underwent bronchoscopy at their index surgery. All 52 non-long gap EA (LGEA) patients underwent primary anastomosis, while most (76.9%) LGEA patients underwent staged repair. Post-operatively, 4(6.2%) developed anastomotic leak which resolved with conservative management. Three (4.6%) had recurrent TEF, 2 underwent re-do ligation. Twenty (30.8%) patients developed anastomotic strictures, with 15 requiring serial dilatation.
Long-term burden of gastro-esophageal and respiratory morbidity was high (63.1%; 64.6% respectively). The majority ( n = 39,60%) of patients required active follow-up for a median duration of 5 years (IQR 1.5–10 years). Predominant conditions were gastroesophageal reflux disease ( n = 28, 43.1%), dysphagia ( n = 20, 30.8%), recurrent respiratory infections ( n = 23, 35.3%), chronic cough ( n = 19, 29.2%), and pneumonia ( n = 19, 29.2%). Tracheomalacia was diagnosed in 22(33.8%), 2 of whom required tracheostomy for severe disease. Overall mortality rate was 10.8% ( n = 7): 5 demised due to chronic respiratory failure, while 2 demised intra-operatively during the index surgery.
Conclusion
Despite successful surgical repair for EA/TEF, our data demonstrated significant morbidities among EA/TEF survivors, thus highlighting the importance of long-term multi-disciplinary care with collaboration between respiratory, gastroenterology, and otolaryngology specialists.
Level of evidence
Prognostic, Level IV.
... Up to 40% of patients are affected by respiratory symptoms [2]. Feeding difficulties are multifactorial, including esophageal, oropharyngeal and behavioural disorders [3]. Furthermore, esophageal atresia is associated with other congenital malformations in over half of all cases [4,5], such as cardiac abnormalities (28 to 42% [4,5]), which may influence growth and development [2]. ...
Background
Esophageal atresia (EA) is a rare malformation with a wide range of co-morbidity and associated malformations impairing weight gain and growth. The aim of this study was to calculate specific percentiles for body weight and height for children born with esophageal atresia according to sex from birth to the age of 6 years, accounting for prematurity and presence of congenital heart disease (CHD).
Methods
Data was extracted from an anonymized voluntary national registry for patients born with esophageal atresia between 2001 and 2021, from birth until the age of six years. Missing values were imputed using a multiple imputation approach. In premature infants, chronological age was corrected for gestational week until the age of one year. The impact of sex and additional congenital heart disease on weight gain and growth was analysed using quartile regression models.
Results
In total, 1812 examinations of 485 patients were considered and 1232 examinations of 301 patients were finally included. Most data was available for children at birth and during the first year of life. Body weight was imputed for 3.3% and height for 12.5% of examinations. The mean body weight-for-age and length-for-age at birth according to gestational age was lower in EA patients and median body weight developed along the tenth percentile compared to the general population. Median height-for-age was at the 50th percentile during the first months of life, before crossing to lower percentiles before the age of one year. CHD had an additional negative impact on growth and weight gain, especially during the first year of life.
Conclusions
Children with EA had a reduced bodyweight and -height compared to the general population. Therefore, specific percentile curves are helpful to evaluate growth and development. Especially during the first year of life, particular attention is necessary and complications leading to a reduced calorie intake should be treated without delay to promote timely development and growth. Cardiac co-morbidities may further compromise weight gain in these patients, implying that such patients should be under even closer surveillance.
... Modern discussion in the scientific community focuses on the long-term follow-up of the EA population. Most EA patients suffer from gastrointestinal (GI) conditions during their lifetime: 22 to 45% of EA patients have gastroesophageal reflux disease (GERD), up to 17% have eosinophilic esophagitis (EoE), and 9 to 41% have physical growth impairment, mainly in the first years of life [2][3][4][5][6][7]. It is thus well known that patients born with EA require a lifetime follow-up comprising endoscopic examinations from childhood [8]. ...
... Patients who presented allergic symptoms or EoE symptoms underwent full allergic skin tests. The diagnosis of EoE was made according to the updated criteria of the AGREE Conference [7,14]. We diagnosed EoE in patients who had clinical symptoms and presented esophageal samples with >15 eosinophils/High Power Field (HPF), in the absence of other possible causes. ...
... In EA patients, EoE is reported as 17% [4,7] and attributed to a possible genetic association and barrier defect in the esophageal mucosa caused by acid reflux or prolonged exposure to PPI therapy [3]. In this cohort of patients, symptoms of EoE are often misdiagnosed as GERD. ...
Follow-up of children born with esophageal atresia (EA) is mandatory due to high incidence of comorbidities. We evaluated endoscopic findings at follow-up of EA patients performed at our Centre according to ESPGHAN-NASPGHAN 2016 guidelines. A retrospective observational study was performed using data from January 2016 to January 2021. We included EA patients (age range: 1–18 years) who were offered a program of endoscopic and histological high gastrointestinal (GI) tract examinations as per ESPGHAN-NASPGHAN 2016 guidelines. Clinical, surgical, auxological, endoscopic, and histological data were reviewed; variables as polyhydramnios, EA type, surgical type, enteral feeding introduction age, growth data, and symptoms were correlated to endoscopic and histological findings. The population included 75 patients (47 males), with mean age of 5 ± 4 years. In 40/75 (53.3%) patients, we recorded oral feeding problems, and upper gastrointestinal or respiratory symptoms suspicious of gastroesophageal reflux. Eosinophilic esophagitis (EoE) incidence was 9/75 (12%), significantly higher than in general population (p < 0.0001), and 10/75 (13.3%) presented non-specific duodenal mucosal lesions. EoE represents a frequent comorbidity of EA, as previously known. EA is also burdened by high, never-described incidence of non-specific duodenal mucosal lesions. Embedding high GI tract biopsies in EA endoscopic follow-up should be mandatory from pediatric age.
... There are several contributing and underlying factors including food impaction, treatment-resistant strictures and increased allergen exposure due to motility problems underlying EoE development in children with EA. However, it is difficult to diagnose EoE in patients with EA due to similarities of symptoms with GERD, dysphagia and esophageal dysmotility (3). For instance, the study with the largest number of patients with EA and EoE suggested that in addition to fluoroscopic swallowing studies, endoscopic evaluation with multiple biopsies was important in children who suffered from increasing dysphagia (5). ...
... These findings suggest that most pediatric surgeons are aware of the considerable overlapping between GERD and EoE symptoms in children with EA. It has been reported that patients with long gap EA and EoE have 1.9 times higher relative risk to develop strictures and surgical treatment of strictures are needed if they become resistant to dilatation treatment (3). The results of survey confirm that majority of pediatric surgeons suspect EoE in case of recurrent strictures. ...
... When a diagnosis of EoE is made in preschool-age children with EA, the most representative symptoms of the disease in the EA-EoE cohort seem to be vomiting and food intolerance [23], while older children from the general population with only EoE manifest the usual symptoms of EoE, such as dysphagia, food impaction and chest pain. ...
Eosinophilic esophagitis (EoE) is a chronic, immune-mediated esophageal disease. Symptoms are related to mucosal eosinophilic-predominant inflammation that leads to esophageal dysfunction. Recent data suggest that esophageal atresia (EA) patients may have an increased incidence of EoE compared to the general population. As EoE symptoms may be confused with EA-related symptoms, they may significantly worsen morbidity in this specific group of patients. We investigated specific characteristics of patients with AE and EoE compared to those with EoE only. We conducted an observational retrospective monocentric study including all patients diagnosed with EoE from 1 January 2010 to 31 December 2021. For each patient, demographic, clinical and histopathological data were collected and then compared between the two cohorts (EA-EoE vs. EoE only). During the study period, 62 patients were included: 17 children were in the follow-up because of EA (18.1% of 94 EA patients screened in that period), while the other 45 presented EoE only. The demographic and clinical features of EA-EoE patients demonstrate a lower prevalence of allergic subjects (23.5% vs. 80%, p < 0.05), a lower age of presentation (3.1 vs. 12.2 years, p < 0.05), non-specific symptoms and a higher resolution rate with PPI therapy (64.7% vs. 17.8%, p < 0.05) compared to EoE-only patients. Our data confirm that EA patients are at high risk for developing EoE. As symptoms may overlap with the EA spectrum, early recognition of EoE may prevent patients from receiving unnecessary invasive therapeutic interventions and from developing complications from untreated EoE.
... Among those with oesophagitis 34/42 (81%), nearly half 16/34 (47%) were EoE with majority of cohort (11/16) failed to demonstrate histological response to dose-optimized PPI (1.5-2.0 mg/kg/day). Although overall frequency of EoE (16 %) is consistent with some recent reports, 30 however, only 1/11 patient with EoE (6%) was treated with topical steroids in our cohort. Therefore, it is important to consider EoE as a potential diagnosis and a trial of topical steroids and elimination diet should be commenced. ...
Objective
The aim of this study is to characterize long-term morbidities of oesophageal atresia (OA) with or without tracheoesophageal fistula (TOF).
Methods
Infants born with OA/TOF from 2000 to 2016 in Western Australia were included for analysis. Infants were categorized into high-risk and low-risk groups based on the presence of one or more perioperative risk factors [low birth weight, vertebraldefects, anal atresia, cardiac defects, TOF, renalanomalies, limb abnormalities (VACTERL), anastomotic leak, long gap OA, and failure to establish oral feeds within the first month] identified by a previous Canadian study. Frequency of morbidities in infants with perioperative risk factors was compared.
Results
Of 102 patients, 88 (86%) had OA with distal TOF (type C). The most common morbidities in our cohort were anastomotic oesophageal strictures (AS) (n=53, 52%), tracheomalacia (n=48, 47%), gastroesophageal reflux disease (GORD) (n=42, 41%) and recurrent respiratory tract infections (n=40, 39%). Presence of GORD (30/59 vs 12/43, p=0.04) and median frequency of AS dilatations (8 vs 3, n=59, p=0.03) were greater in the high-risk group. This study further confirmed that inability to be fed orally within the first month was associated with high morbidities.
Conclusions
Gastrointestinal and respiratory morbidities remain high in OA/TOF regardless of perioperative risk factors. Inability to be fed orally within the first month is a predictor of poor outcomes with high frequency of gastrointestinal and respiratory comorbidities.
... Also, type 1 diabetes, cystic fibrosis, adrenal insufficiency, autism, attention deficit hyperactivity disorder (ADHD) (8), and monogenic diseases (7) appear to be significantly associated with a non-atopic phenotype of EoE (2). An increasing amount of evidence showed that children with esophageal atresia (EA) (30)(31)(32) or with diaphragmatic hernia (34) are at higher risk to develop EoE (33,34,79). Several risk factors have been associated with the development of EoE in children with EA, such as early life factors, early exposure to acid suppressants and antibiotics, GERD, esophageal dysmotility, and epithelial injury (79). ...
... An increasing amount of evidence showed that children with esophageal atresia (EA) (30)(31)(32) or with diaphragmatic hernia (34) are at higher risk to develop EoE (33,34,79). Several risk factors have been associated with the development of EoE in children with EA, such as early life factors, early exposure to acid suppressants and antibiotics, GERD, esophageal dysmotility, and epithelial injury (79). Interestingly, Krishnan et al. demonstrated that children with EoE + EA share the same dysregulated genes (that encode for proteins involved in epithelial barrier functions and Th2 inflammation) compared to patients with EoE and without EA (33). ...
Eosinophilic esophagitis (EoE) is a chronic antigen-mediated inflammatory disease that affects the esophagus. In the last 20 years, a large number of epidemiological studies showed a significant increase in the incidence and prevalence of EoE, especially in developed countries. This phenomenon might correlate to the overall increase in pediatric allergic diseases or might be a result of improved medical awareness and knowledge through modern diagnostic instruments. Since 1993, when EoE was first recognized as a distinct clinical entity, several signs of progress in the pathophysiology of EoE were achieved. However, a few studies reported data on early risk factors for pediatric EoE and how these factors may interfere with genes. Currently, the most defined risk factors for EoE are male sex, Caucasian race, and atopic comorbidities. Other putative risk factors may include alterations in epithelial barrier function and fibrous remodeling, esophageal dysbiosis, variation in the nature and timing of oral antigen exposure, and early prescription of proton pump inhibitors and antibiotics. Notably, the timing and nature of food antigen exposure may be fundamental in inducing or reversing immune tolerance, but no studies are reported. This review summarized the current evidence on the risk factors that might contribute to the increasing development of EoE, focusing on the possible preventive role of early interventions.
