DOZ047.57: Eosinophilic esophagitis in an infant with repaired esophageal atresia and tracheoesophageal fistula

2019 ◽  
Vol 32 (Supplement_1) ◽  
Author(s):  
A Mustafa ◽  
A AlKhabaz ◽  
A A Mallik ◽  
S Kumar

Abstract Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) requires early surgical intervention in life, following which symptoms related to esophageal dysmotility, gastroesophageal reflux, and strictures may develop. Eosinophilic esophagitis (EoE) is an immune-mediated disorder presents with vomiting and dysphagia. The appearance of exudates, rings, and furrows during endoscopy and the presence of >15 eosinophils/HPF in biopsy confirm the diagnosis. Treatment options include elimination diet, proton pump inhibitors (PPI), and topical steroids. We report a full-term male who had EA and TEF repaired in the second day of life. At 11 months of age, he developed progressive vomiting, contrast study showed esophageal dysmotility with slow emptying and no stricture. The initial endoscopy showed a mild nonobstructive narrowing 5 cm above the gastroesophageal junction, below which the mucosa was erythematous, biopsies showed >25 eosinophils/HPF. Started treatment with PPI and endoscopy repeated three months later showed same endoscopic features and biopsies showed >45 eosinophils/HPF. Allergy testing was positive for milk, wheat, oat, and rice. Topical steroids and elimination diet were added to PPI. At 32 months of age, the child was asymptomatic, not on elimination diet and treatment was stopped. Repeat endoscopy showed longitudinal furrows and erosions of the lower esophagus, no stricture, and biopsies showed >15 eosinophils/HPF. Steroids, PPI, and elimination diet were all resumed. Despite moderate esophagitis due to EoE and poor compliance to treatment, our patient shows normal growth, development, and symptoms resolution. Asymptomatic children with repaired EA and treated EoE may require close follow-up and repeat endoscopy.

2021 ◽  
Vol 4 (Supplement_1) ◽  
pp. 16-18
Author(s):  
K A Bortolin ◽  
D Ashok ◽  
V Avinashi ◽  
J Barkey ◽  
D Burnett ◽  
...  

Abstract Background Eosinophilic esophagitis (EoE) is a chronic disorder treated by food elimination diet (FED), topical steroids and/or proton-pump inhibitors (PPI). Serial endoscopies and biopsies assess response to therapy. EoE management has evolved as guidelines are updated. Aims To identify practice variation among Canadian paediatric gastroenterologists (PG) who care for children with EoE. Methods An online survey using REDCap about decision-making in children with EoE was distributed to PG in Canada in November 2020. Results 62 PG completed the survey (response rate 69%, 62/94). The majority work in academic centres (92%). 3 centers indicated an accrual of >50 new patients per year; 9/16 centres have >100 patients in follow-up. An EoE Clinic is present in 5 centres. Diagnosis: Familiarity with the 2018 AGREE and 2020 AGA EoE guidelines was found to be 57% and 67% respectively. Criteria required to diagnose EoE according to current guidelines were correctly indicated by 42% of PG. (Figure 1). Endoscopy: The majority of PG (95%) adhere to guidelines in terms of required number and location of biopsies for the initial diagnosis. Ideal timing of repeat endoscopy after change in therapy in patients who are not in histological remission was 8–12 weeks by 67% of PG, timing in stable patients on maintenance therapy varied (33% only if patient is symptomatic). 25% used the EREFS Score in reporting endoscopic findings. Therapy: Improvement of symptoms was the highest ranked goal (64%), followed by remission of histologic findings (30%). A treatment algorithm was in place in 4 centers. The majority routinely assess adherence to therapy (73%) and consult a dietitian for FED (77%). Most (87%) do not consult an allergist for initial management. Preferred choices of 1st-line therapy varied among PG (Figure 2). When FED was selected, 32% of PG started with 1 food, 32% started with 2 foods, most frequently excluding dairy, followed by wheat. 14 (26%) start with ≥6 FED. Prescription of budesonide slurry was consistent among PG with doses of 1 and 2 mg/day in children <10 and >10 years, respectively. Conclusions The is the first Canadian study to assess the variation in management of children with EoE by PG. Overall, PG demonstrated good adherence to the guidelines in terms of initial diagnosis, but differences in maintenance therapy choice and timing of endoscopies. The results highlight a need for standardized management algorithms to deliver uniform care to this growing group. Grounding these guidelines in evidence will warrant a significant investment in further paediatric EoE research. Funding Agencies None


2018 ◽  
Vol 63 (9) ◽  
pp. 2381-2388 ◽  
Author(s):  
Craig C. Reed ◽  
Anca M. Safta ◽  
Shadi Qasem ◽  
M. Angie Almond ◽  
Evan S. Dellon ◽  
...  

2019 ◽  
Vol 57 (06) ◽  
pp. 745-752 ◽  
Author(s):  
Stephan Miehlke ◽  
Ulrike von Arnim ◽  
Christoph Schlag ◽  
Thomas Frieling ◽  
Ahmed Madisch ◽  
...  

Abstract Background Eosinophilic esophagitis (EoE) is an increasingly recognized immune-mediated esophageal disease and a common cause for dysphagia and food bolus obstruction. The aim of this study was to evaluate the current clinical management of EoE among adult gastroenterologists in Germany. Methods We performed a cross-sectional study of 1393 adult gastroenterologists using a questionnaire containing 22 questions to general, diagnostic, and therapeutic aspects of EoE. The self-administered online survey was conducted between November 2017 and February 2018. Data capture and analysis was performed using SurveyMonkey. Results The overall responder rate was 29.6 %. More than half of the responders (54.9 %) felt to observe a significant increase of EoE patients. The EREFS score was mostly either unknown (44.3 %) or not routinely used (52.2 %). If EoE was suspected, most responders obtained multiple esophageal biopsies (n = 3 – 4: 35.7 %; n > 4: 61.6 %). The preferred primary treatment was proton pump inhibitors (PPI) in 37.2 % and topical steroids in 35.0 % of responders. PPI regimens were highly diverse, with only half of responders using high-dose PPI regimens. Allergy testing was often initiated (always 25.4 %, sometimes 48.9 %). The most common dietary therapy was 6-food elimination diet (52 %), followed by allergy test-directed diets (16 %) and 2-food elimination diet (16.5 %). The majority of responders indicated a need for long-term treatment (i. e., 23 % of responders in > 50 % their patients and 47.7 % of responders in 25 – 50 % of their patients). Conclusions Among gastroenterologists in Germany, substantial variation in the adherence to published EoE guidelines appears to exist. This indicates the need for intensified education and national guidelines in order to optimize and harmonize the clinical management of EoE patients.


2019 ◽  
Vol 32 (Supplement_1) ◽  
Author(s):  
R Tambucci ◽  
F Rea ◽  
M Malamisura ◽  
F Torroni ◽  
E F Romeo ◽  
...  

Abstract Introduction Since recently, after detection of eosinophilic predominant inflammation of esophagus a trial with proton-pump inhibitors (PPIs) was needed to individuate children with PPI-responsive esophageal eosinophilia (PPI-REE), only those non-responders received eosinophilic esophagitis (EoE) diagnosis. In 2018 updated international consensus suggested removing the PPI trial as a diagnostic criterion and consider PPIs as a treatment together with diets and topical steroids. The role of PPIs is evaluated in children with esophageal atresia (EA) and EoE versus EoE from general population. Method A retrospective chart review of both children with EA and EoE followed-up from at January 2005 has been performed. According to ESPGHAN guidelines published in 2014 patients showing eosinophilic inflammation received high-dose PPI trial to identified PPI-REE. Those non-responders were labeled as EoE and underwent to dietary and/or topical steroid treatment. Demographics and disease characteristics of EA patients with EoE were analyzed and compared with those with EoE from general population. Results Overall, 370 EA and 118 EoE patients were analyzed. Of them 15 EA-EoE patients were detected. Consequently, in our cohorts, 4.0% of EA patients developed EoE. Male-to-female prevalence ratio was of 2.55 with no difference in gender prevalence between groups. At diagnosis EoE-EA children were significantly younger compared to EoE group (mean: 5.1 vs 10.8 years; P < 0.0001). Peak EOS/HPF at diagnosis did not differ between groups (50.1 ± 26 vs 59.8 ± 29 EOS/HPF). No difference was observed in allergy prevalence between groups (53.8 vs 68.0%). PPI-REE was significantly more prevalent in EA-EoE group that in EoE group (66.6% vs 19.4%; P = 0.0004). Conclusion Similar gender distribution and high prevalence of allergy suggest that common genetic susceptibility factors for EoE exist. However, high prevalence of PPI-REE coupled early EoE onset might also suggest that other factors (e.g. esophageal motility disorders) might play a physiopathological role in EoE development in EA children. Our study suggests that a stepwise approach with PPIs as a first-line treatment for EoE management in EA children should still be considered.


2016 ◽  
Vol 29 (3) ◽  
pp. 294-295 ◽  
Author(s):  
U.K. Krishnan ◽  
L.M. McLennan ◽  
J.C. Li Chan ◽  
C.C. Clarkson ◽  
J.M. Menzies ◽  
...  

2009 ◽  
Vol 2009 ◽  
pp. 1-7 ◽  
Author(s):  
Biagio Zuccarello ◽  
Antonella Spada ◽  
Nunzio Turiaco ◽  
Daniela Villari ◽  
Saveria Parisi ◽  
...  

Introduction and Aim. Disorders of esophageal motility causing dysphagia and gastroesophageal reflux are frequent in survivors to esophageal atresia (EA) and distal tracheoesophageal fistula (TEF). The aim of the present study was to investigate the histologic and immunohistochemical features in both esophageal atretic segments to further understand the nature of the motor disorders observed in these patients.Material and Methods. Esophageal specimens from 12 newborns with EA/TEF and 5 newborns dead of unrelated causes were examined. The specimens were fixed in 5% buffered formalin, included in paraffin and cut in 5 micron sections that were stained with hematoxilin and eosin (H and E), and immunohistochemical stainings for Actin, S-100 protein, Neurofilament, Neuron-Specific-Enolase, Chromogranin A and Peripherin were evaluated under the microscope.Results. In controls, the distribution of the neural elements was rather homogenous at both levels of the esophagus. In contrast, the atretic segments showed quantitative and qualitative differences between them with sparser nervous tissue in the distal one in comparison with the proximal one and with controls.Conclusions. These results further support the assumption that histomorphological alterations of the muscular and nervous elements within the esophageal wall might contribute to esophageal dysmotility in patients surviving neonatal operations for EA/TEF.


2018 ◽  
Vol 06 (01) ◽  
pp. e108-e110
Author(s):  
Takafumi Kawano ◽  
Oliver Muensterer

AbstractWe report the first use of a miniature stapler to divide a mucosal bridge at the gastroesophageal junction after complex esophageal atresia (EA) repair. A 4-year-old girl was referred to our center after treatment of EA elsewhere. On our initial enodoscopy, a large iatrogenic tracheoesophageal fistula had formed, prompting us to perform a tracheoplasty and gastric interposition. One year after recovery, she had dysphagia with solid food. Upon endoscopy, a mucosal bridge was noted at the gastroesophageal anastomosis. This bridge was divided under endoscopy using a 5 mm miniature stapler. No complications were noted. Upon follow-up, she had no more complaints with solid food. Our report shows that the mucosal bridges may cause dysphagia after EA repair and can be safely divided using a miniature stapler.


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