Outcome Of Esophageal Atresia Research Articles

Management and Outcomes of Esophageal Atresia With or Without Tracheo-Esophageal Fistula Over 15 Years in South Africa

IntroductionTo determine the incidence, management and outcomes of esophageal atresia/tracheo-esophageal fistula (EA/TEF) over a 15-y period in South Africa. MethodsA retrospective chart review of neonates with EA/TEF presenting at the main tertiary referral hospital in the KwaZulu-Natal province between 2002 and 2017 was conducted. Data collection comprised patient and maternal demographics, clinical presentations, laboratory and radiologic investigations, surgical procedures, and outcomes. A multivariate logistic regression determined the risk factors associated with mortality. ResultsAmong 180 neonates, mean (SD) age of diagnosis was four (three) days postnatal with Gross Type C (n = 165, 92%) being the most common and the incidence was one per 10,000 live births. Majority were born term (n = 95, 53%) at peripheral hospitals (n = 167, 93%) with a mean birth weight of 2369 (736) grams. Overall HIV exposure rate was 27% (n = 48). Most (n = 138, 77%) patients presented with established pneumonia, 44% (n = 61) of whom required prolonged (>7 d) ventilator support. The median (IQR) hospital stay was 11 (8-20) d. Overall survival rate was 70% (n = 126). Birth weight <1500 g, life threatening anomalies, ventilation >30 d and postoperative sepsis contributed to mortality. ConclusionsIncidence, disease types and presentations were similar to developed countries. Despite advances in technology and neonatal care in Africa, EA/TEF surgical outcomes remain suboptimal likely due to caregivers’ inability to care for these infants in disadvantaged socioeconomic circumstances with poor sanitation, etc. Research is needed to identify strategies tailored for disadvantaged communities which may contribute to improved outcomes in the perioperative and postoperative period.

Outcome of esophageal atresia: inborn versus outborn patients.

Esophageal atresia (EA) is a rare congenital disease which is usually not of the detected prenatally. Due to the lack of prenatal diagnosis, some newborns with EA are born outside of specialized centers. Nevertheless, centralized care of EA has been proposed, even if a clear volume-outcome association in EA management remains unconfirmed. Furthermore, whether outcomes differ between outborn and inborn patients with EA has not been systematically investigated. Therefore, this single-center, retrospective study aimed to investigate EA management and outcomes with a special focus on inborn versus outborn patients. The following data were extracted from the medical records of infants with EA from 2009 to 2019: EA type, associated anomalies, complications, and long-term outcome. Patients were allocated into inborn and outborn groups. Altogether, 57 patients were included. Five patients were excluded (referral before surgery, loss of data, death before surgery [n = 1], and incorrect diagnosis [diverticulum, n = 1]). Among all patients, the overall survival rate was 96%, with no mortalities among outborn patients. The overall hospitalization period was shorter for outborn patients. The median follow-up durations were 3.8years and 3.2years for inborn and outborn patients, respectively. Overall, 15% of patients underwent delayed primary anastomosis (long-gap atresia [n = 4] and other reasons [n = 4]). Early complications included three anastomotic leakages and one post-operative fistula; 28% of patients developed strictures, which required dilatation, and 38% of patients showed relevant gastroesophageal reflux, which required fundoplication, without any differences between the groups. The two groups had comparable low mortality and expected high morbidity with no significant differences in outcome. The outborn group showed nonsignificant trends toward lower morbidity and shorter hospitalization periods, which might be explained by the overall better clinical status.

Evaluation of nebulized N-acetyl cysteine in outcome of esophageal atresia with tracheoesophegeal fistula

AimTo evaluate the role of nebulized N-acetyl cysteine (NAC) in liquefying the airway secretions and improving the outcome of patients of esophageal atresia with tracheoesophageal fistula (EA + TEF). MethodsIt was a non-randomized interventional study. Two milliliters of 10% NAC was given in a nebulized form (2:5 dilution, every six hourly) to patients of ET + TEF, along with regular suction of upper esophageal pouch. The group was compared with control, which comprised patients of EA + TEF receiving only saline nebulization. The consistency of the secretions was compared by hand held consistometer in unit of time (seconds) required to cross a predetermined distance along with gravity. ResultsSixty patients were assessed. Of these, 30 patients were present in both groups. The study group showed significant (p = 0.01–0.0001) decrease in consistency of secretions from the control group after day 2 of NAC nebulization. Patients' discharge was significantly (p = 0.01) earlier in cases. There was no significant (p = 0.41) difference in mortality between the groups. No specific adverse effects were observed in the study group. ConclusionIt appears that nebulized NAC decreases the consistency of secretions in EA + TEF patients. It is interesting to note that the group of patients that received NAC was discharged earlier than the control group and had a higher survival rate than the control group. Whether this is directly attributable to the use of NAC is unknown. A prospective double-blinded randomized clinical trial is warranted to confirm these results. Level of evidenceLevel II, prospective comparative study (non-randomized).

DOZ047.21: Esophageal atresia and long-term quality of life in Italian population: a pilot study

Abstract Introduction In Italy, long-term outcomes of esophageal atresia (EA) are poorly understood. The Italian Association ‘Family with Esophageal Atresia’ (F.AT.E.) and Tages Onlus, and some Italian pediatric centers of reference for AE, collaborated to define the first work about long-term outcomes and quality of life (QoL) in a cohort of Italian adults and young adults born with AE. As a rare disease, given the specificity of the reference population and the absence of similar research, this study was in any case designed to be a pilot study. Methods To date, 65 adults born with EA have participated in completing the questionnaire. Two survey formats were created: (i) a standard paper questionnaire and (ii) an online platform. The questionnaire included the following tools: (i) socio-personal data; (ii) QoL questionnaire (SF-36); (iii) a sub-battery of short questionnaires to investigate psychological adaptation to chronic diseases (HADS, IES-R, Brief-COPE); (iv) a questionnaire linked to the specific perception of social support (MOS-SSQ); and (v) four open questions developed to investigate the subjective experience of QoL and long-term morbidity. The use of quantitative and qualitative data (Mixed Methods) will allow us to deeply investigate the experience and perceptions related to the QoL. Results Our results indicated that AE type C is the most frequent, and the renal ones are the most common malformations associated with AE. According to the dysphagia score, more than half are suffering states of dysphagia. A significant negative statistical association emerges between the presence of dysphagia and perception of social support (MOSS-SSS). Gastroesophageal reflux symptoms are reported by more than half, in association with antireflux medications. At last, it emerges that the main psychological factors accused are sleep disorders and obsessive-compulsiveness symptoms. Conclusions Despite this study confirming that the frequency of respiratory, gastrointestinal, and musculoskeletal symptoms affects the QoL, it appears generally good. The information provided by this study could be an incentive to implement and improve long-term follow-up programs and to involve more specialist adult doctors in the care of these patients.

DOZ047.05: The flip side of the coin

Abstract Background Being two medical doctors (family practitioner and pediatric surgeon) with a son born with a rare type of esophageal atresia we felt the need to explore the nonmedical side of this disease. Aim The aim of this study was to emphasize the role of emotional support for parents with children born with all types of esophageal atresia and to realize the importance of the nonsurgical side—the flip side of the coin, to explore the need for support not only for the patient but also the family. Literature Review and Personal Experience The epidemiology, surgical management, and biological outcome of esophageal atresia with/without trachea-esophageal fistula are well known. The softer, more humane side of this condition, the flipside of the coin, being the cause of PTSD children with OA (mothers &gt; fathers).2 Severe anxiety also seems to be present in some of these parents. A meta-analysis done in 2013 concluded that psychological morbidity associated with esophageal atresia has important implications for clinical practise3; namely, if psychological support is being offered to parents during the neonatal period and continues as part of the long-term follow-up for both parents and the patients, the overall outcome is improved and the biopsychosocial morbidity of the parents decreases. Personal experience has taught us that a good support system is a critical part of a child's clinical treatment plan. Parents need good emotional support in order to be there for their child when he or she needs them. Conclusion Surgeons look at diseases from a technical point of view. They ask how they can surgically fix the problem and improve the patient's quality of life. They often forget the other side that parents need emotional support, someone to be there for them and to listen to their experience in hospital and at home. The family as another patient needs to be treated and included in the overall treatment plan if the intention is to improve the quality of life for both the patients and their families. There is a need to develop a multidisciplinary support system in South Africa to aid patients born with OA and their caring families. This includes the entire family and is not limited to the mothers but also includes fathers and other siblings often overlooked.

Predictive factors affecting the prognosis and late complications of 73 consecutive cases of esophageal atresia at 2 centers.

While the diagnosis and outcomes of esophageal atresia (EA) have improved, associated anomalies, the management of late complications and growth remain major issues. We analyzed factors that affected the prognosis, late complications and growth. We retrospectively reviewed EA patients treated at two centers from 1984 to 2016. Patient characteristics, complications (gastroesophageal reflux [GER], anastomotic stenosis, tracheomalacia, dysphagia) and growth were evaluated. Seventy-three EA patients were treated (overall survival rate:80.8%). The mean birth weight was 2514 ± 509g in the surviving group, and 2453 ± 567g in the fatal group excluded chromosomal abnormality (p = 0.76). Cardiac and chromosomal anomalies significantly affected mortality. Postoperative GER and anastomotic stenosis each occurred in 39% of the patients. Only GER was significantly affected by the Gross classification. The standard deviation (SD) values of the EA patients' growth were all lower than in the normal population. The SD of body weight was significantly lower in patients with extremity anomalies. Associated cardiac and chromosomal anomalies significantly affected the prognosis. GER and anastomotic stenosis were the most common late complications. The growth of the surviving cases was insufficient. These factors will help optimize the therapeutic strategies and postoperative management for EA.

Esophageal atresia: Early outcome analysis from a high-volume tertiary care institute in India

Context: Despite the progress made in the field of surgical techniques and neonatal care, conditions such as prematurity, very low birth weight, and associated anomalies compounded with delayed diagnosis may negatively influence the successful outcome of esophageal atresia (EA). Aims: The aim is to study the early outcome analysis of patients with EA and tracheoesophageal fistula (TEF) at a high-volume tertiary care institute. Settings and Design: A retrospective study performed from January 2016 to December 2016. Subjects and Methods: The study included all patients of EA and TEF admitted in the Neonatal Intensive Care Unit of our department. Results: There were 430 new cases of EA/TEF. Type C was the most common (90.23%), followed by Type A (8.37%), Type E (0.69%), and one case each of Type D, esophageal web, and esophageal stenosis. Two hundred and twenty-four (52.10%) remained undiagnosed on the 1st day of life. The average birth weight was 2200 g; 64.65% of patients were low birth weight. Associated major anomalies were present in 18.84% patients with gastrointestinal tract anomalies in 13.49% cases (anorectal malformation – 11.86%, duodenal atresia [DA] – 2.79%, and triple atresia – 0.93%) and VACTERL association in 11.63%. Surgical procedures were performed in 413 (96.05%) patients. Survival was seen in 29.77% cases, mortality in 68.84% and 1.39% patients left against the medical advice. The survival was better in Type C (29.89%) than Type A (27.78%) and also those without associated anomalies. VACTERL association had a very high (80%)-mortality rate. Septicemia (63.85%), severe pneumonia (53.71%), and congestive heart failure (24.32%) were main causes of mortality. Conclusions: Although there has been a marked improvement in the antenatal diagnosis of EA, most cases in our study remain undiagnosed in first 24 h of birth. Associated major anomalies were documented in approximately 1/5th patients. Survival was present in less than 1/3rd patients; survival outcomes were better in EA without associated anomalies. Early diagnosis with the help of red rubber catheter shortly after birth, measures to prevent pneumonia, strict infection control measures for prevention of septicemia, deployment of sufficient resources, and investigations for other associated anomalies are recommended to improve the outcomes of EA.

A patient led, international study of long term outcomes of esophageal atresia: EAT 1.

Long term outcomes of esophageal atresia (OA) are poorly understood. The Federation of Esophageal Atresia and Tracheo-Esophageal Fistula support groups (EAT), a collaboration of patient support groups aimed to define patient reported long term outcomes and quality of life (QoL) in a large international cohort of OA patients. Questionnaires were designed focusing on patient/parent reported outcomes including surgical history, current symptomatology and quality of life. Members of support groups within EAT were invited to complete questionnaires electronically via SurveyMonkey®. 1100 patients from 25 countries responded to the questionnaire and 928 were analyzed. 80% had type C anatomy, 19% type A and 1% type E. Patient ages were <5 years (42%), 5-10 years (26%), 11-17 years (16%) and 18 years and older (16%). 49% of all patients reported previous dilatations which was similar across age groups. Reflux symptoms affected 58% of patients and persisted into adulthood. Dysphagia also persisted in the adult population with 50% reporting sometimes or often getting food stuck. Reflux was significantly more frequent in 'long gap' versus 'standard gap' patients (p<0.005). Respiratory symptoms and chest infections decreased in frequency with age. In children median SDS for height was -0.41 (IQR -1.4 to 0.67) and that for weight was -0.63 (-1.6 to 0.67). BMI in adults was 21.5. Quality of life was described as significantly affected by OA in 18% of patients while 25% reported no effect on QoL. These results highlight the significant long term morbidity suffered by OA patients as children and into adulthood and suggest the need for quality transitional care. The patient designed and reported nature of the study gives a unique perspective to the results and emphasizes the benefits of collaboration.

Perioperative management and outcomes of esophageal atresia and tracheoesophageal fistula

Background/PurposeEsophageal atresia/tracheoesophageal fistula (EA/TEF) is a rare congenital anomaly lacking contemporary data detailing patient demographics, medical/surgical management and outcomes. Substantial variation in the care of infants with EA/TEF may affect both short- and long-term outcomes. The purpose of this study was to characterize the demographics, management strategies and outcomes in a contemporary multi-institutional cohort of infants diagnosed with EA/TEF to identify potential areas for standardization of care. MethodsA multi-institutional retrospective cohort study of infants with EA/TEF treated at 11 children's hospitals between 2009 and 2014 was performed. Over the 5year period, 396 cases were identified in the 11 centers (7±5 per center per year). All infants with a diagnosis of EA/TEF made within 30days of life who had surgical repair of their defect defined as esophageal reconstruction with or without ligation of TEF within the first six months of life were included. Demographic, operative, and outcome data were collected and analyzed to detect associations between variables. ResultsPrenatal suspicion or diagnosis of EA/TEF was present in 53 (13%). The most common anatomy was proximal EA with distal TEF (n=335; 85%) followed by pure EA (n=27; 7%). Clinically significant congenital heart disease (CHD) was present in 137 (35%). Mortality was 7.5% and significantly associated with CHD (p<0.0001). Postoperative morbidity occurred in 62% of the population, including 165 (42%) cases with anastomotic stricture requiring intervention, anastomotic leak in 89 (23%), vocal cord paresis/paralysis in 26 (7%), recurrent fistula in 19 (5%), and anastomotic dehiscence in 9 (2%). Substantial variation in practice across our institutions existed: bronchoscopy prior to repair was performed in 64% of cases (range: 0%–100%); proximal pouch contrast study in 21% (0%–69%); use of interposing material between the esophageal and tracheal suture lines in 38% (0%–69%); perioperative antibiotics ≥24h in 69% (36%–97%); and transanastomotic tubes in 73% (21%–100%). ConclusionContemporary treatment of EA/TEF is characterized by substantial variation in perioperative management and considerable postoperative morbidity and mortality. Future studies are planned to establish best practices and clinical care guidelines for infants with EA/TEF. Level of EvidenceType of study: Treatment study. Level IV.

Outcomes of esophageal atresia in a tier II referral centre

Background: To study the clinical profile, complications and outcomes of neonates with esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) in a tier II referral centre.Methods: This is a retrospective study of 54 patients with EA and/or TEF from 2008 to 2014.Results: Fifty four cases of EA/ TEF which were reviewed, 32 (59.26%) were male and 22 (40.74%) were female patients. The mean birth weight was 2.38 kg. 39(72.22%) babies were born at term and 15(27.78%) were preterm. Most common anatomical variant was type C. Most common associated anomaly was cardiac, seen in 8 patients (14.81%). Post operative barium study showed minor leaks in 7 patients. 22 of 54 patients (40.74%) died.Conclusions: Early recognition of symptoms and early referral to hospitals would improve the outcomes of neonates with EA/TEF. Sepsis remains the leading causes of mortality, and delayed referral with onset of pneumonia contribute to sepsis.

Long-term esophageal and respiratory outcomes in children with esophageal atresia and tracheoesophageal fistula.

Objectives: Few studies have evaluated the long-term complications and outcomes of esophageal atresia with or without tracheoesophageal fistula (EA/TEF) beyond childhood. The aim of our study was to characterize the esophageal and respiratory morbidity of EA/TEF through evaluation of clinical symptoms, diagnostic testing and therapeutic intervention at a tertiary care center.Methods: Patients with congenital EA/TEF evaluated from 2011 to 2014 were included. Demographic characteristics, type and mode of repair of EA/TEF, clinical symptoms, radiographic, endoscopic, bronchoscopic and medication use data were obtained.Results: A total of 43 patients were identified. The median age of this predominantly Caucasian population was 8 years (interquartile range: 3, 20). Twenty (62.5%) had type C (EA with distal TEF) abnormality. Twenty-one (48.8%) patients had heartburn, 19 (44.1%) had acid regurgitation, and 31 (72.1%) had dysphagia to solids. Barium swallow in 26 patients revealed strictures in 17 (65.4%), dysmotility in 20 (76.9%) and recurrent fistulas in four patients (15.4%). Thirty patients underwent upper endoscopy, of which 21 (70.0%) had a stricture, and six (20.0%) had recurrent fistula requiring surgical intervention. Eight (18.6%) patients underwent fundoplication. Pulmonary evaluation showed cough and choking in 31 (72.1%) patients and dyspnea and wheezing in 32 (53.4%) patients. Recurrent respiratory infections were reported in 19 (44.2%).patients. Other findings included tracheomalacia in 86.7% and restrictive lung disease in 54.5% of patients.Conclusion: There is a high burden of residual esophageal and pulmonary pathology in patients with EA/TEF. Ongoing follow-up is required to monitor both the clinical symptoms and treatment responses.

Outcome of esophageal atresia beyond childhood

Survivors of esophageal atresia are reaching their adulthood in large numbers for the first time enabling assessment of true long-term outcome among this group of patients. This review summarizes the current knowledge on the subject focusing on late symptoms and complications, esophageal pathology and pulmonary function. Relationships between esophageal dysmotility, gastroesophageal reflux, esophagitis and epithelial metaplastic changes including esophageal cancer are outlined. In addition to pertinent literature, institutional experience, and follow-up of patients with esophageal atresia for more than 60 years is included.

Modified prognostic criteria for esophageal atresia and tracheoesophageal fistulaEur J Pediatr Surg, 153–157

With the advances in technology, the outcome of esophageal atresia (EA) and tracheoesophageal fistula (TEF) has significantly changed. The aim of this study was to review the outcomes of EA and TEF with respect to the various prognostic criteria and to find out if there is a need for a further modification of the prevailing prognostic classification. The medical charts of 57 newborns with EA/TEF treated between 1996 and 2004 were reviewed retrospectively. Patient demographics, associated anomalies, and management were studied.

Phenotypic presentation and outcome of esophageal atresia in the era of the Spitz classification

Purpose: The aim of this study was to investigate contemporary patterns of presentation and outcome in newborns with esophageal atresia with or without tracheoesophageal fistula (OA) using modern prognostic criteria to appraise survival. Methods: Over a 12-year period (1986 through 1997), 134 patients with OA were admitted to a single institution. Patient demographics, the presence of cardiac defects, other associated abnormalities (VACTERL), surgical intervention, and patient outcome were recorded. To identify and evaluate changes in the pattern of clinical presentation, frequency of associated anomalies and outcome, patients were analyzed during 4 consecutive time periods, 1986 to 1988, 1989 to 1991, 1992 to 1994, and 1995 to 1997. Results: A primary repair or delayed primary repair was performed in 113 (84%) patients, with a staged procedure in 19 (14%). Two babies with trisomy 18 did not undergo surgery. Thirty-eight newborns (28%) had a major cardiac malformation (excluding patent ductus arteriosus, unless needing ligation), and 25 (19%) had recognized VACTERL associations. There was a significant increase in the proportion of infants with major cardiac defects diagnosed over the study period, 5 of 34 patients between 1986 and 1988 to 19 of 41 patients between 1995 and 1997 (χ2 test, P <.001), but the incidence of VACTERL associations remained unchanged. Overall survival rate was 86% in those who underwent surgery. The relative risk of mortality in patients with major cardiac disease and VACTERL associations was 3.47 (95% CI; 1.51 to 7.96) and 2.54 (95% CI; 1.14 to 4.86), respectively. Birth weight was significantly higher in infants who survived (2.68 kg) compared with those who died (2.16 kg, P =.003). Thirty percent of infants with more than one system abnormality died compared with 8% of infants with 1, system abnormality (P =.004). Conclusions: This study has found a significant increase in the frequency of cardiac abnormalities encountered in a cohort of OA patients during the period under review. Cardiac disease and multiple abnormalities carried a substantial increased risk of mortality. In the era of the Spitz classification, the phenotypic presentation is important to accurately assess caseload severity and prognosis. J Pediatr Surg 36:1419-1421. Copyright © 2001 by W.B. Saunders Company.