Introduction
Esophageal atresia (EA) is the most common birth defect of the esophagus and affects 1 in 2,500 live births (1). Children with EA experience a high rate of morbidity, which lasts lifelong. Patients have varying degrees of morbidities like esophageal dysmotility, anastomotic strictures, gastroesophageal reflux disease (GERD), dysphagia and feeding difficulties (2).
Eosinophilic esophagitis (EoE) is a chronic allergic disorder, which is characterized by esophageal eosinophilia and esophageal dysfunction. It causes vomiting, dysphagia, feeding difficulties, food allergy, and decreased quality of life (3). Children with EA are shown to have a significant prevalence of EoE ranging between 3-17% (4-7). 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). Vomiting, dysphagia or feeding problems were more common in patients with EA and EoE resulting in a higher likelihood of undergoing fundoplication and gastrostomy surgeries in comparison to those who did not have associated EoE (5). Pediatric surgeons not only have an important role in the management of children with EA but they also design their long-term follow-ups (1,2). Therefore, awareness of pediatric surgeons about the symptoms suggestive of EoE, and necessity to perform biopsies and referral of the patients to appropriate disciplines if needed are important. Pediatric surgeons are to be involved in developing standardized consensus and providing improvement in care.
In a recent study, dysphagia as one of the most common complication in these children from the perspective of pediatric surgeons was investigated, and it was suggested that there was a need for a standardized protocol in dysphagia management and their knowledge was found to be high (8). The importance of the awareness and knowledge of pediatric surgeons is also known to handle EoE in children with EA. However, there is no study to evaluate their current clinical practice and knowledge in terms of EoE in children with EA in literature. Determination of the current practice and knowledge may provide better understanding of the pitfalls of management delivered by pediatric surgeons, reveal good practices, and contribute to create consensus in national and international areas. Therefore, the current study aimed to define clinical practice and knowledge of pediatric surgeons from Turkey regarding diagnosis and treatment of EoE in children with EA.
Methods
The online survey study was carried out at Hacettepe University. The Hacettepe University Non-invasive Clinical Research Ethics Committee approved the study protocol (approval number = GO20/528).
Participants
Pediatric surgeons who currently worked in Turkey and were involved in diagnosis and treatment of EA were included in the study. The invitation was done by using a national e-mail platform already in use for online communication among pediatric surgeons in Turkey. The online survey was administered via Google forms. All volunteering participants were required to provide informed consent prior to reach survey questions by clicking the start button of the survey. All data were collected anonymously.
Procedure
Two pediatric surgeons with more than 20 years of clinical and research experience in the treatment of children with EA developed the draft questions. A pilot study was performed to test clarity of the questions before survey distribution. Two pediatric surgeons and one physical therapist who were university-affiliated experts with a minimum of 10-year work experience and routinely encountered patients with EA in practice discussed each question, and minor changes were made through their feedback. The survey included 26 questions, which was estimated to take 15 minutes to complete.
The survey included (i) 4 questions related to descriptive information of participants, (ii) 8 questions related to diagnosis, (iii) 4 questions related to treatment, and (iv) 10 questions related to knowledge. Descriptive section questioned the type of hospital, occupational experience, the number of children with EA in annual follow-up and the number of children with EA who had EoE. In the diagnosis section, there were questions related to consultations in the long-term follow-up, the endoscopy applications and the diagnosis of EoE. There were two questions related to the treatment of GERD and two questions regarding the treatment of EoE. In the knowledge section, participants were asked to state “yes” or “no” for the statements. Only one option was the correct answer, which received one point, and the incorrect answer received zero point. Therefore, the total knowledge score ranged between 0 to 10 points. In addition, the total score was assessed based on Bloom’s cut off point (9). According to Bloom’s cut off point, the level of knowledge was classified into three as “low-level”, “moderate-level’’, and “high-level” knowledge. The total score less than 60% (0-5 scores) refered to low-level knowledge, 60-79% (6-7 scores) indicated moderate-level knowledge, and 80-100% (8-10 scores) refered to high-level knowledge. The statements were related to time of occurrence of esophagitis (Q1), frequency by gender (Q2), risk related to type of atresia (Q3), condition in prematurity (Q4), genetic relationship (Q5), relationship between esophagitis and anti-acid therapy (Q6), relationship between esophagitis and motility impairment (Q7), relationship between esophagitis and atopic diseases and food allergies (Q8), basal analysis (Q9), and cautions before anti-reflux treatment (Q10). Appendix 1 indicates the survey questions in detail.
The survey was started in July 2020. A reminding e-mail message was sent after each four weeks, and the survey was closed twelve weeks after initial posting, and responses were analyzed.
Statistical Analysis
The statistical analysis was performed by using IBM-SPSS for Windows version 20. Descriptive statistics were expressed as number/percent for qualitative data, and mean, standard deviation, minimum and maximum values for quantitative data.
Results
A total of 57 pediatric surgeons were participated in the current study. The descriptive characteristics of participants are shown in Table 1.
Diagnosis: In the long-term follow-up of children who underwent EA operation, 73.7% (n=42) of pediatric surgeons got routine consultation from pediatric gastroenterology, 36.8% (n=21) from pediatric pulmonology and 26.3% (n=15) from pediatric allergy/immunology units. The rate of pediatric surgeons who did not get any support from other disciplines was 22.8% (n=13). The responses related to the endoscopy applications and the diagnoses of EoE are presented in Table 2 and Table 3, respectively.
Treatment: In treatment section, the questions were related to GERD and EoE. The answers are given in Table 4.
Knowledge: The percentage of correct answers for each questions was as follows: Q1: 66.7% (n=38), Q2: 71.9% (n=41), Q3: 70.2% (n=40), Q4: 64.9% (n=37), Q5: 47.4% (n=27), Q6: 56.1% (n=32), Q7: 89.5% (n=51), Q8: 96.5% (n=55), Q9: 21.1% (n=12), Q10: 93% (n=53). The mean knowledge score was 6.64±1.92 (min=1, max=9). The rate of pediatric surgeons with low-level knowledge was 17.5% (n=10), moderate-level knowledge was 45.6% (n=26) and high-level knowledge was 36.8% (n=21).
Discussion
The care of children with EA, which requires multidisciplinary follow-up should be handled systematically from childhood to adulthood (10,11). Therefore, standard management guidelines in terms of gastrointestinal and nutritional complications in children with EA are important to improve the quality of care to these patients and guide clinicians (12). In addition to clinical studies’ results and what is suggested in the guidelines, defining the clinical practice and knowledge of primary responsible clinicians regarding children with EA is also important to reveal what is exactly performed. Therefore, we aimed to determine the current practice and knowledge of pediatric surgeons in terms of EoE in children with EA.
In the long-term follow-up of children who were operated on for EA, pediatric surgeons reported that they mostly got help from pediatric gastroenterology unit. However, approximately one quarter of them could not get any collaboration from other disciplines. The percentage of clinicians who did not have any help from other discipline was remarkable. A multidisciplinary approach is necessary to have a favorable long-term outcome and better quality of life (12-14). Therefore, we believe that the awareness of other team members including pediatric gastroenterologists, pediatric pulmonologists, etc. should be increased as well as pediatric surgeons to improve clinical care of these children.
A substantial number of pediatric surgeons (42.1%) favor routine medical therapy for GERD in the first year of life in all patients with EA as recommended in ESPGHAN-NASPGHAN guidelines (12). One third of responders prescribe anti-reflux medication only in symptomatic patients and 12% of them prescribe after testing for reflux. Proton pump inhibitors (PPI) (35.1%) and alginate and/or prokinetics and H2 receptor blockers together (33.3%) were the first line of choice for GERD treatment by pediatric surgeons.
The most common indications for endoscopy for pediatric surgeons are esophageal dilatation, follow-up of reflux esophagitis, and suspicion of EoE or GERD in accordance with the literature (12). Only one pediatric surgeon reported that he/she planned endoscopy after stopping proton pump inhibitors. More than half of the pediatric surgeons preferred flexible endoscopy for children with EA, and approximately 25% preferred both rigid and flexible endoscopy. The results show that pediatric surgeons in Turkey follow international consensus regarding endoscopy applications (12).
The responding pediatric surgeons reported that they suspected EoE in patients with GERD symptoms resistant to treatment (87.7%), recurrent anastomotic strictures (77.2%) and food impaction (43.9%). In addition, they also reported that recurrent gastroesophageal reflux and airway aspiration were also signs of EoE in children with EA. 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.
Endoscopic evaluation with biopsies is crucial for diagnosis of EoE (12,15). EoE can be definitely diagnosed by esophageal biopsy revealing more than 15 eosinophils/HPF (12,15). In our survey, 89.5% of pediatric surgeons reported that they performed endoscopy and took biopsies whenever they suspected from EoE. The finding of “red streaks and white exudates” was the most frequently associated endoscopy finding with EoE (73.7%) by pediatric surgeons followed by crepe paper and fragile mucosa, mucosal bleeding centers and trachealization or ringing, in descending order. Interestingly, half of them consider mucosal bleeding as an endoscopic sign for EoE and 7% of them find varicose veins in the lower end of esophagus suggestive for diagnosis.
Since EoE is described as a patchy disease, it is important to take biopsies form widespread locations (3). In our study, 42.1% of surgeons preferred two biopsies each from the lower, middle and upper esophagus for suspicion of EoE, and 47.4% of them considered more than 15 eosinophils/HPF diagnostic for EoE. These results suggest that nearly half of the surgeons obtain appropriate biopsies and consider the results correctly according to the guidelines. However, 26% of responders obtain biopsies only from the suspected areas and 22.8% of them had no idea about the number of eosinophils in the biopsy specimens for EoE diagnosis. These percentages suggest that there is a need to increase awareness, knowledge and clinical applications of pediatric surgeons in terms of endoscopic evaluations and interpretations for EoE in children with EA. Pediatric gastroenterology (94.7%), pediatric allergy (78.9%) and pediatric immunology (33.3%) disciplines are the most commonly co-involved disciplines in patients with EA with suspected EoE. This may simply be dependent on the availability of the discipline in the institution at which the pediatric surgeon works or a personal preference depending on the enthusiasm of the other disciplines to collaborate in the care of the patients with EA in a given institution.
Although, pediatric surgeons do not directly dictate the treatment of EoE, they report diet elimination (73.7%), proton pump inhibitors (66.7%) and systemic steroids (43.9%) as most frequent treatment options in EoE. This is compatible with the recommended treatment protocols for children with EA (16). In a study performed in 2016, the authors reported that dilatations could be needed for strictures in patients with coexistent EoE with EA although seldom (17). The role of pediatric surgeons in the treatment of EoE is mainly limited to esophageal dilatations for strictures. About one fifth of pediatric surgeons in our study mentioned that they carried out dilatations for these patients. Interestingly, 10% of pediatric surgeons reported intralesional applications for the treatment for EoE. The use of any intralesional applications has not been reported in EoE before and possibly has no beneficial effect on EoE related esophageal strictures.
Study Limitations
Our study had a limitation because only volunteering pediatric surgeons answered the questions and they might be the ones with a special interest in EA and/or EoE. Bearing this fact in mind, the knowledge of pediatric surgeons was found to be above average in this study. Most participants had moderate (45.6%) to high (36.8%) level of knowledge. Wrong answers were mostly related to basal impedance analysis and genetic relationship. The most correct answers were related to relationship between esophagitis and atopic diseases and food allergies, and cautions before anti-reflux treatment. These results are also very important because determining the lack of knowledge as to the degree or aspect may provide clinicians to organize and participate in educational programs, improve their clinical applications, and increase the success of care regarding EoE in children with EA.
Conclusion
The EoE is a chronic inflammatory disease of the esophagus and can be seen in association with EA. Children with EA and also suffering from EoE have higher incidence of dysphagia, food impaction, vomiting and feeding difficulties, which result in decreased quality of life of both patients and their families. Therefore, the clinical practice and knowledge regarding its diagnosis and treatment are important in addition to clinical studies and management guidelines. The current study results suggest that pediatric surgeons have some strengths and weaknesses in terms of clinical practice and knowledge about this association. Therefore, their awareness, knowledge and clinical practice should be increased to improve the care of these children.
Ethics
Ethics Committee Approval: The online survey study was carried out at Hacettepe University. The Hacettepe University Non-invasive Clinical Research Ethics Committee approved the study protocol (approval number=GO20/528).
Informed Consent: provided informed consent prior to reach survey questions by clicking the start button of the survey.
Peer-review: Externally peer reviewed.
Authorship Contributions
Concept: S.S.A., Ç.U.D., T.S., Design: S.S.A., Ç.U.D., T.S., Data Collection or Processing: S.S.A., T.S., Analysis or Interpretation: S.S.A., Ç.U.D., T.S., Literature Search: S.S.A., Ç.U.D., T.S., Writing: S.S.A.
Conflict of Interest: No conflict of interest was declared by the authors.
Financial Disclosure: The authors declared that this study received no financial support.