Surveillance Endoscopy in Pediatric Esophageal Atresia: Toward an Evidence-Based Algorithm.

BACKGROUND: Individuals with esophageal atresia (EA) have lifelong increased risk for mucosal and structural pathology of the esophagus. The use of surveillance endoscopy to detect clinically meaningful pathology has been underexplored in pediatric EA. We hypothesized that surveillance endoscopy in pediatric EA has high clinical yield, even in the absence of symptoms. STUDY DESIGN: The medical records of all patients with EA who underwent at least 1 surveillance endoscopy between March 2004 and March 2023 at an international EA referral center were retrospectively reviewed. The primary outcomes were endoscopic identification of pathology leading to an escalation in medical, endoscopic, or surgical management. Logistic regression analysis examined predictors of actionable findings. Nelson-Aalen analysis estimated optimal endoscopic surveillance intervals. RESULTS: Five hundred forty-six children with EA underwent 1,473 surveillance endoscopies spanning 3,687 person-years of follow-up time. A total of 770 endoscopies (52.2%) in 394 unique patients (72.2%) had actionable pathology. Esophagitis leading to escalation of therapy was the most frequently encountered finding (484 endoscopies, 32.9%), with most esophagitis attributed to acid reflux. Barrett's esophagus (intestinal metaplasia) was identified in 7 unique patients (1.3%) at a median age of 11.3 years. No dysplastic lesions were identified. Actionable findings leading to surgical intervention were found in 55 children (30 refractory reflux and 25 tracheoesophageal fistulas). Significant predictors of actionable pathology included increasing age, long gap atresia, and hiatal hernia. Symptoms were not predictive of actionable findings, except dysphagia, which was associated with stricture. Nelson-Aalen analysis predicted occurrence of an actionable finding every 5 years. CONCLUSIONS: Surveillance endoscopy uncovers high rates of actionable pathology even in asymptomatic children with EA. Based on the findings of the current study, a pediatric EA surveillance endoscopy algorithm is proposed.

Esophageal Atresia Associated with Congenital Duodenal Obstruction: Turkish Esophageal Atresia Registry (TEAR) Evaluation.

INTRODUCTION: Coexistent congenital duodenal obstruction and esophageal atresia (EA) is known to have significant morbidity and mortality. Management strategies are not well-defined for this association. The data from the Turkish EA registry is evaluated. MATERIALS AND METHODS: A database search was done for the years 2015 to 2022. RESULTS: Among 857 EA patients, 31 (3.6%) had congenital duodenal obstruction. The mean birth weight was 2,104 (± 457) g with 6 babies weighing less than 1,500 g. Twenty-six (84%) had type C EA. The duodenal obstruction was complete in 15 patients and partial in 16. Other anomalies were detected in 27 (87%) patients. VACTERL-H was present in 15 (48%), anorectal malformation in 10 (32%), a major cardiac malformation in 6 (19%), and trisomy-21 in 3 (10%). Duodenal obstruction diagnosis was delayed in 10 (32%) babies for a median of 7.5 (1-109) days. Diagnosis for esophageal pathologies was delayed in 2. Among 19 babies with a simultaneous diagnosis, 1 died without surgery, 6 underwent triple repair for tracheoesophageal fistula (TEF), EA, and duodenal obstruction, and 3 for TEF and duodenal obstruction in the same session. A staged repair was planned in the remaining 9 patients. In total, 15 (48%) patients received a gastrostomy, the indication was long-gap EA in 8. Twenty-five (77%) patients survived. The cause of mortality was sepsis (n = 3) and major cardiac malformations (n = 3). CONCLUSION: Congenital duodenal obstruction associated with EA is a complex problem. Delayed diagnosis is common. Management strategies regarding single-stage repairs or gastrostomy insertions vary notably depending on the patient characteristics and institutional preferences.

Diagnosis and management of congenital type D esophageal atresia.

This study was performed to describe the current clinical practice and outcomes of type D esophageal atresia. We retrospectively analyzed 10 patients who were diagnosed with type D esophageal atresia and underwent esophageal atresia and tracheoesophageal fistula repair in the Capital Institute of Pediatrics and Beijing Children's Hospital from January 2017 to May 2022. Ten patients include three newborns and seven non-newborns. Seven (70%) cases were misdiagnosed as type C esophageal atresia before the first operation. Three neonatal children underwent thoracoscopic distal tracheoesophageal fistula ligation and esophageal anastomosis: the proximal tracheoesophageal fistula was simultaneously repaired with thoracoscopy in one of these children, and the proximal tracheoesophageal fistula was not detected under thoracoscopy in the other two children. Among the seven non-neonatal children, one underwent repair of the proximal tracheoesophageal fistula through the chest and the other six underwent repair through the neck. Nine patients were cured, and one died of complications of severe congenital heart disease. Type D esophageal atresia lacks specific clinical manifestations. Misdiagnosis as type C esophageal atresia is the main cause of an unplanned reoperation. Patients without severe malformations have a good prognosis.

Impact of digestive-surgical cross-disciplinary management in patients with esophageal atresia. / Impacto del manejo multidisciplinar digestivo-quirúrgico en los pacientes con atresia de esófago.

OBJECTIVE: The objective of this study was to analyze whether patients undergoing esophageal atresia (EA) surgery benefit from a cross-disciplinary follow-up program, based on current clinical guidelines, implemented in our institution. MATERIALS AND METHODS: An observational, analytical, retrospective study of patients undergoing EA surgery from 2012 to 2022 was carried out. The results of a joint pediatric surgery and gastroenterology consultation program -which was implemented in 2018 and applies a protocol based on the new ESPGHAN-NASPGHAN guidelines- were analyzed. Patients were divided according to whether they had been treated before or after 2018. Quantitative variables -follow-up losses, anti-reflux treatment initiation and duration, and enteral nutrition initiation- and qualitative variables -prevalence of gastroesophageal reflux, anti-reflux surgery, respiratory infections, anastomotic stenosis, re-fistulizations, dysphagia, impaction episodes, need for gastrostomy, and endoscopic results- were compared. RESULTS: 38 patients were included. 63.2% had gastroesophageal reflux. 97.4% received anti-reflux treatment in the first year of life, with treatment being subsequently discontinued in 47.4%. Discontinuation time decreased by a mean of 24 months following program implementation (p< 0.05). A 4.6-fold increase in the frequency of pH-metries was noted following program implementation. The protocol standardized endoscopies in asymptomatic patients when they turn 5 and 10 years old. 25 endoscopies with biopsy were carried out after 2018, with histological disorders being detected in 28% of them. The number of follow-up losses significantly decreased following protocol implementation (p< 0.05). CONCLUSIONS: Digestive-surgical cross-disciplinary follow-up of EA patients has a positive impact on patient progression. Applying the guidelines helps optimize treatment and early diagnosis of complications.

OBJETIVOS: El objetivo de este estudio es analizar si los pacientes intervenidos de atresia de esófago (AE) se benefician de un programa de seguimiento multidisciplinar, basado en las guías clínicas actuales, implantado en nuestro centro. MATERIAL Y METODOS: Estudio retrospectivo, observacional y analítico incluyendo los pacientes intervenidos de AE entre 2012 y 2022. Se analizaron los resultados de la implantación en 2018 de un programa de consultas conjuntas de gastroenterología y cirugía pediátrica aplicando un protocolo basado en las nuevas guías ESPGHAN-NASPGHAN. Se dividieron a los pacientes tratados antes y después de 2018 y se compararon las variables cuantitativas: pérdidas de seguimiento, inicio y duración del tratamiento antirreflujo e inicio de nutrición enteral, y cualitativas: prevalencia de reflujo gastroesfoágico, realización de cirugía antirreflujo, infecciones respiratorias, estenosis de la anastomosis, refistulizaciones, disfagia, episodios de impactación, necesidad de gastrostomía y resultados de las endoscopias. RESULTADOS: Se incluyeron 38 pacientes. Un 63,2% presentaron reflujo gastroesofágico. El 97,4% tomaron tratamiento antirreflujo el primer año de vida que posteriormente se retiró en el 47,4%. El tiempo de retirada se redujo una media de 24 meses tras la aplicación del programa (p< 0,05). Se realizaron 4,6 veces más pHmetrías tras la implantación del programa. El protocolo estandarizó la realización de endoscopias en pacientes asintomáticos al cumplir 5 y 10 años. Se realizaron 25 endoscopias con tomas de biopsia después de 2018, detectando alteraciones histológicas en un 28%. El número de pérdidas de seguimiento se redujo de forma significativa tras la implantación del protocolo (p< 0,05). CONCLUSIONES: El seguimiento multidisciplinar digestivo-quirúrgico de los pacientes con AE genera un impacto positivo en su evolución. La aplicación de las guías facilita la optimización del tratamiento y el diagnóstico precoz de las complicaciones.

Type III congenital pulmonary airway malformation associated with oesophageal atresia and tracheoesophageal fistula. A case report and review of literature.

Congenital pulmonary airway malformation (CPAM) together with oesophageal atresia and tracheoesophageal fistula (TOF) is a very rare condition in neonates. We presented a case of an infant with Gross type C oesophageal atresia with TOF coexisting with Stocker Type III CPAM in our centre. It is interesting to know that TOF associated with type III CPAM has never been reported in the literature. The child was delivered through caesarean section, and because of respiratory distress post-delivery, endotracheal intubation was carried out immediately. CPAM was diagnosed by a suspicious finding from the initial chest X-ray and the diagnosis was confirmed through computed tomography scan of the chest. The patient was initially stabilised in a neonatal intensive care unit (NICU), and after the successful ligation of fistula and surgical repair of TOF, lung recruitment was started by high flow oscillatory ventilation. The patient recovered well without complications and able to maintain good saturation without oxygen support through the stay in the neonatal unit. Early recognition of this rare association is essential for immediate transfer to NICU, the intervention of any early life-threatening complications, and for vigilant monitoring in the postoperative period.

A newborn with ectrodactyly, tetralogy of Fallot, esophageal atresia, hypospadias and TP63 gene mutation: A new type of EEC Syndrome?

EEC syndrome is an autosomal dominant genetic disease with incomplete penetrance characterized by ectrodactyly, ectodermal dysplasia, and cleft lip/palate; these manifestations can differently occur in the affected subjects and can also be associated with other anomalies, such as in the urogenital tract.We reported the case of a newborn with prenatal diagnosis of EEC type 3 associated with severe cardiac abnormalities (Tetralogy of Fallot), high esophageal atresia with fistula and penoscrotal hypospadias.

[Combination of esophageal atresia with proximal tracheoesophageal fistula and meconium peritonitis: a case report]. / Sochetanie atrezii pishchevoda s proksimal'nym trakheopishchevodnym svishchom i mekonievogo peritonita.

We report a patient with combination of esophageal atresia, proximal tracheoesophageal fistula and meconium peritonitis. These two rare disorders have different etiology, pathogenetic mechanisms and require different diagnostic manipulations and surgical treatments. The authors discuss the features of diagnosis and surgical treatment of this disease.

[Epidemiological, clinical and therapeutic profile of esophageal atresia at the Mother and Child University Hospital of the Jeanne Ebori Foundation from 2019 to 2020]. / Profil épidémiologique, clinique et thérapeutique des atrésies de l'œsophage au Centre Hospitalier Universitaire Mère-Enfant Fondation Jeanne Ebori de Libreville de 2019 à 2020.

Esophageal atresia is a congenital malformation incompatible with life. Its management in our context is difficult. The purpose of this study was to determine the clinical, therapeutic and epidemiological profile of esophageal atresia at the Mother and Child University Hospital of the Jeanne Ebori Foundation from 2019 to 2020. We conducted a retrospective study in the Neonatal Medicine and Pediatric Surgery Department of Mother and Child University Hospital of the Jeanne Ebori Foundation over a 2-year period from January 1, 2019 to December 31, 2020. We collected data from the medical records of 10 newborns diagnosed with esophageal atresia. The prevalence of neonatal surgical emergencies was 11% (n=10/89). Mean gestational age of patients was 37 WA+2, ranging from 34WA+2 days to 40WA+2 days; mean life span was 3.7 days with extremes of D0 and D7. Sex ratio was 1. The average birth weight was 2636 grams, ranging from 1460 g to 3425g; mean time between symptom onset and diagnosis was 4 days. Type III accounted for 70% of anatomical shapes. Mean waiting time for surgery was 0.8 days, ranging from D0 to D3. The average length of stay in hospital was 9.3 days, ranging from 1 to 38 days. Nine out of 10 patients underwent surgery. Only 2 patients survived, with a follow-up period of at least one year without sequelae. The management of esophageal atresia is still precarious in our context. Diagnostic delay is the main challenge. Improvement in prognosis involves improvement in anesthetic, surgical and neonatal technical equipment.

Systematic review of long term follow-up and transitional care in adolescents and adults with esophageal atresia - why is transitional care mandatory?

PURPOSE:  to review recent literature concerning long-term health issues and transitional care in esophageal atresia (EA) patients. PubMed, Scopus, Embase and Web of Science databases were screened for studies regarding EA patients aged more than or equal to 11 years, published between August 2014 and June 2022. Sixteen studies involving 830 patients were analyzed. Mean age was 27.4 years (range 11-63). EA subtype distribution was: type C (48.8%), A (9.5%), D (1.9%), E (0.5%) and B (0.2%). 55% underwent primary repair, 34.3% delayed repair, 10.5% esophageal substitution. Mean follow-up was 27.2 years (range 11-63). Long-term sequelae were: gastro-esophageal reflux (41.4%), dysphagia (27.6%), esophagitis (12.4%), Barrett esophagus (8.1%), anastomotic stricture (4.8%); persistent cough (8.7%), recurrent infections (4.3%) and chronic respiratory diseases (5.5%). Musculo-skeletal deformities were present in 36 out of 74 reported cases. Reduced weight and height were detected in 13.3% and 6% cases, respectively. Impaired quality of life was reported in 9% of patients; 9.6% had diagnosis or raised risk of mental disorders. 10.3% of adult patients had no care provider. Meta-analysis was conducted on 816 patients. Estimated prevalences are: GERD 42.4%, dysphagia 57.8%, Barrett esophagus 12.4%, respiratory diseases 33.3%, neurological sequelae 11.7%, underweight 19.6%. Heterogeneity was substantial (> 50%).   Conclusion: EA patients must continue follow-up beyond childhood, with a defined transitional-care path by a highly specialized multidisciplinary team due to the multiple long-term sequelae. WHAT IS KNOWN: • Survival rates of esophageal atresia patients is now more than 90% thanks to the improvements in surgical techniques and intensive care, therefore patients' needs throughout adolescence and adulthood must be taken into account. WHAT IS NEW: • This review, by summarizing recent literature concerning long term sequelae of esophageal atresia, may contribute to raise awareness on the importance of defining standardized protocols of transitional and adulthood care for esophageal atresia patients.

Adequate timing of diagnostic tests for gastroesophageal reflux in children with esophageal atresia. / Momento adecuado para las pruebas diagnósticas de reflujo esofágico en pacientes con atresia de esófago.

OBJECTIVE: Recent guidelines made recommendations for the management of gastroesophageal reflux in patients with esophageal atresia (EA). However, the timing for some diagnostic tests remained somehow unclear. This investigation studied the tests for gastroesophageal reflux in children aged one year old and children aged two or three. MATERIALS AND METHODS: Patients with EA who underwent Multichannel Intraluminal Impedance-pH monitoring (MII-pH) and endoscopy-histology were studied retrospectively. Patients aged one when the test was performed were the YO group and patients aged two or three years old formed the OL group. Substantially impaired MII-pH was defined as total number of reflux episodes >105 or >85 (depending on age), or reflux index >10%. Substantially impaired endoscopy was defined as erosive esophagitis or Barrett's esophagus. Substantially impaired histology was defined as moderate-severe esophagitis or Barrett's esophagus. Conventional parameters and substantially impaired values of the tests were compared. RESULTS: Twenty-four patients were studied. Twenty-three MII-pH were performed (12 in YO and 11 in OL): percentages of abnormal conventional parameters of MII-pH were not significantly different in both groups. Twenty endoscopies with biopsies were performed (7 in YO and 13 in OL): percentages of esophagitis were not significantly different. Interestingly, 26.9% of all the tests performed in YO were substantially impaired vs. 10.8% of all the tests in OL (χ2 = 2.7; p = 0.1). CONCLUSION: Considering the percentage of alarming results of diagnostic tests in the YO group it would be advisable that patients with EA undergo MII-pH and endoscopy-histology at one year of age.

OBJETIVOS: Recientemente se han publicado recomendaciones para el manejo del reflujo gastroesofágico en pacientes con atresia de esófago (AE). Sin embargo, el momento de realización de algunas pruebas no está completamente aclarado. Esta investigación evalúa las pruebas para reflujo gastroesofágico en niños de 1 año y niños de 2-3 años. MATERIAL Y METODOS: Estudio retrospectivo de pacientes con AE sometidos a impedanciometría-phmetría (IMpH) y a endoscopia-histología. Los pacientes con 1 año en el momento de la prueba formaron el grupo MEN, y los pacientes con 2-3 años, el grupo MAY. Se consideró IMpH sustancialmente alterada aquella con un número total de reflujos >105 o >85 (según la edad), o un índice de reflujo >10%. La endoscopia se consideró sustancialmente alterada si presentaba esofagitis erosiva o esófago de Barrett. La histología se consideró sustancialmente alterada si presentaba esofagitis severa-moderada o esófago de Barrett. Se compararon los parámetros convencionales y los sustancialmente alterados. RESULTADOS: Se estudiaron 24 pacientes. Se realizaron 23 IMpH (12 en el grupo MEN y 11 en el MAY); los porcentajes de los parámetros convencionales patológicos no fueron estadísticamente diferentes en ambos grupos. Se realizaron 20 endoscopias (7 en el grupo MEN y 13 en el MAY); los porcentajes de esofagitis no fueron estadísticamente diferentes. El 26,9% de todas las pruebas en el grupo MEN resultaron sustancialmente alteradas, frente al 10,8% en el MAY (χ2 = 2,7; p = 0,1). CONCLUSION: Teniendo en cuenta el porcentaje de resultados alarmantes en el grupo MEN, sería recomendable realizar una IMpH y una endoscopia con biopsias a los pacientes con AE a la edad de un año.

Retrospective evaluation of clinical and molecular data of 148 cases of esophageal atresia.

Developmental abnormalities provide a unique opportunity to seek for the molecular mechanisms underlying human organogenesis. Esophageal development remains incompletely understood and elucidating causes for esophageal atresia (EA) in humans would contribute to achieve a better comprehension. Prenatal detection, syndromic classification, molecular diagnosis, and prognostic factors in EA are challenging. Some syndromes have been described to frequently include EA, such as CHARGE, EFTUD2-mandibulofacial dysostosis, Feingold syndrome, trisomy 18, and Fanconi anemia. However, no molecular diagnosis is made in most cases, including frequent associations, such as Vertebral-Anal-Cardiac-Tracheo-Esophageal-Renal-Limb defects (VACTERL). This study evaluates the clinical and genetic test results of 139 neonates and 9 fetuses followed-up at the Necker-Enfants Malades Hospital over a 10-years period. Overall, 52 cases were isolated EA (35%), and 96 were associated with other anomalies (65%). The latter group is divided into three subgroups: EA with a known genomic cause (9/148, 6%); EA with Vertebral-Anal-Cardiac-Tracheo-Esophageal-Renal-Limb defects (VACTERL) or VACTERL/Oculo-Auriculo-Vertebral Dysplasia (VACTERL/OAV) (22/148, 14%); EA with associated malformations including congenital heart defects, duodenal atresia, and diaphragmatic hernia without known associations or syndromes yet described (65/148, 44%). Altogether, the molecular diagnostic rate remains very low and may underlie frequent non-Mendelian genetic models.

Clinical Variables as Indicative Factors for Endoscopy in Adolescents with Esophageal Atresia.

INTRODUCTION: Gastro-esophageal reflux disease (GERD) occurs frequently in patients operated for esophageal atresia (EA). Longstanding esophagitis may lead to dysphagia, strictures, columnar metaplasia, and dysplasia with an increased risk of adenocarcinoma. Are clinical factors and non-invasive assessments reliable indicators for follow-up with endoscopy? MATERIAL AND METHOD: A follow-up study with inclusion of EA adolescents in Norway born between 1996 and 2002 was conducted. Clinical assessment with pH monitoring, endoscopy with biopsies, along with interviews and questionnaires regarding gastroesophageal reflux disease (GERD) and dysphagia were performed. RESULTS: We examined 68 EA adolescents. 62% reported GERD by interview, 22% by questionnaire. 85% reported dysphagia by interview, 71% by questionnaire. 24-hour pH monitoring detected pathological reflux index (RI) (>7%) in 7/59 (12%). By endoscopy with biopsy 62 (92%) had histologic esophagitis, of whom 3 (4%) had severe esophagitis. Gastric metaplasia was diagnosed in twelve (18%) adolescents, intestinal metaplasia in only one (1.5%). None had dysplasia or carcinoma. Dysphagia and GERD were statistically correlated to esophagitis and metaplasia, but none of the questionnaires or interviews alone were good screening instruments with high combined sensitivity and specificity. A compound variable made by simply taking the mean of rescaled RI and dysphagia by interview showed to be the best predictor of metaplasia (85% sensitivity, 67% specificity). CONCLUSION: The questionnaires and interviews used in the present study were not good screening instruments alone. However, combining dysphagia score by interview and RI may be helpful in assessing which patients need endoscopy with biopsy at each individual follow-up examination. LEVEL OF EVIDENCE: Level II prognostic study.

Prenatal diagnosis of distal 13q deletion syndrome in a fetus with esophageal atresia: a case report and review of the literature.

BACKGROUND: Chromosome 13q deletion syndrome shows variable clinical features related to the different potential breakpoints in chromosome 13q. The severely malformed phenotype is known to be associated with the deletion of a critical region in 13q32. However, esophageal atresia is a rare symptom and the relevant region is unknown. Thus, determining the association between accurate breakpoints and new clinical features is essential. CASE PRESENTATION: A 28-year-old Japanese primigravid woman was referred for fetal growth restriction, absence of a gastric bubble, cerebellar hypoplasia, overlapping fingers, and polyhydramnios at 31 weeks gestation. At 38 + 0 weeks, she delivered a 1774 g female infant. The infant presented with isolated esophageal atresia (Gross type A), Dandy-Walker malformation, right microphthalmia, left coloboma, overlapping fingers, pleurocentrum in the thoracic vertebrae, reduced anogenital distance, and hearing loss. Her karyotype was diagnosed as 46,XX,del(13)(q32.1-qter) by amniocentesis, but array comparative genomic hybridization after birth revealed the deletion of 13q31.3-qter. At 48 days after birth, the infant underwent surgery for esophageal atresia and was later discharged from the hospital at 7 months of age. CONCLUSION: This case report and the literature reviews supports the previous findings on the pathological roles of haploinsufficiency of the ZIC2/ZIC5 in Dandy-Walker malformation and the EFBN2 haploinsufficiency in eye malformation and hearing loss. Furthermore, the possible involvement of IRS2, COLA1, and COLA2 in eye malformation were identified. This is the first case of 13q deletion syndrome with esophageal atresia (Gross A), but it may be a symptom of VATER/VACTER association (vertebral defects, anorectal malformations, cardiac defects, tracheoesophageal fistula with or without esophageal atresia, renal malformations, and limb defects), as in the previous cases. These symptoms might also be associated with EFBN2 haploinsufficiency, although further research is required.

Columnar Metaplasia of the Esophagus Presenting as Iron Deficiency Anemia in Children with Neurologic Impairment or Congenital Esophageal Atresia.

BACKGROUND Columnar metaplasia of the lower esophagus includes both gastric and intestinal metaplasia. Children with severe neurologic impairment and congenital esophageal atresia often have gastroesophageal reflux disease, which can lead to Barrett's esophagus, a form of lower esophageal columnar metaplasia and precursor to esophageal adenocarcinoma, with some, but not all, guidelines specifically requiring the presence of intestinal metaplasia for diagnosis. This case series illustrates how iron deficiency anemia may be the primary symptom of esophageal columnar metaplasia in such children and how upper endoscopy is essential in their initial and ongoing evaluation. CASE REPORT We review 5 cases of columnar metaplasia of the lower esophagus in children, 3 with severe neurologic impairment and 2 with esophageal atresia. Each child presented with marked iron deficiency anemia and minimal-to-no gastrointestinal symptoms. CONCLUSIONS We conclude that columnar metaplasia of the esophagus may present with iron deficiency anemia in children with neurologic impairment or congenital esophageal atresia, even if without overt gastrointestinal symptoms. Accordingly, we propose that early endoscopic evaluation should be considered in this specific patient population. Based on our literature review, we also emphasize the need for guidelines on the endoscopic surveillance of such children with any type of columnar metaplasia of the lower esophagus, given the associated risk of malignant transformation.

Can 18-years of data from a tertiary referral center help to identify risk factors in esophageal atresia?

BACKGROUND: Esophageal atresia is a complex esophageal malformation with an incidence of 1 in 3,500-4,000 live births, and it usually occurs together with anomalies in other systems or chromosomes. This study aimed to investigate the short-term and long-term results of cases of esophageal atresia retrospectively in our institution and to analyze the factors affecting the outcome. METHODS: Charts of the patients managed for esophageal atresia in our tertiary pediatric surgery department were investigated retrospectively. Statistical analysis was performed to determine the risk factors for morbidity and mortality. RESULTS: One hundred and thirteen (95.8%) of 118 cases underwent a single-stage or staged esophagoesophagostomy procedure. In only five of the 40 patients with a long gap between the two atretic ends was an esophageal replacement procedure required. The most common early and late complications were anastomotic stenosis (41.6%) and gastroesophageal reflux (44.9%). In logistic regression analysis, the birthweight (OR [95% CI] = 0.998 [0.997, 0.999], P = 0.001) and preoperative inotrope requirement (OR [95% CI] = 13.8 [3.6-53.3], P < 0.001) were the two risk factors in the mortality prediction model obtained by multivariate analysis. The gap length between the two atretic ends (OR [95% CI] = 1.436 [1.010, 2.041], P = 0.044) and the number of sutures for anastomosis (OR [95% CI] = 1.313 [1.042, 1.656], P = 0.021) were the two risk factors in the gastroesophageal reflux prediction model obtained by multivariate analysis. CONCLUSIONS: Our study's early and late complication rates were like those found in other studies. Identifying risk factors would be beneficial and might help reduce the severity of potential complications in esophageal atresia patients. Prospective studies on large patient series would help develop registry-based, standardized management protocols.

Long term digestive outcome of œsophageal atresia.

Œsophageal atresia is a rare neonatal malformation consisting in an interruption of the continuity of the œsophagus, with or without a tracheo-œsophageal fistula. Although mortality rate is now low and most cases can benefit from successful surgical repair soon after birth, morbidity -specially digestive and nutritional-remains high. Many of the adults born with œsophageal atresia will suffer from dysphagia, gastro-œsophageal reflux and/or œsophageal dysmotility, leading to nutritional consequences and quality of life impairment. Barrett's œsophagus, potential risk of œsophageal cancer as well as risk of anastomotic stenosis and eosinophilic œsophagitis justify transition to adulthood and a lifelong prolonged follow-up.

Should all babies with oesophageal atresia have routine screening for midgut malrotation anomalies? A systematic review in search of evidence.

Background/Purpose Oesophageal Atresia (OA) is associated with co-existent anomalies. There is a controversy of literature pertaining to the risk (s) of intestinal malrotation. In order to guide management we critically evaluate the incidence of IM anomalies in OA newborns. Design MEDLINE and EMBASE databases were searched using keywords "(O)Esophageal Atresia and Malrotation/Associated Abnormalities/Associated Anomalies". Full texts of articles were screened if manuscripts exclusively reported patients with OA malrotation and/or associated anomalies. Larger case series (> 10patients) were included if abstract (s) showed that associated anomalies were systematically assessed. Full eligibility criteria required at least one case of malrotation in an OA index case. Data were collected on article type, number of patients and method (s) of diagnosis. Results 632 abstracts were screened of which 158 papers were analysed based on inclusion criteria-30 manuscripts documented the incidence (%) of malrotation. Incidence rate (s) were 0.5-13%. Malrotation was observed to have a higher incidence (10-44%) in OA babies with other gastrointestinal anomalies (VACTERL). Conclusion Newborns with OA appear to be at a higher risk (%) of having intestinal malrotation anomalies than healthy babies. Prospective studies are required to accurately quantify and define the ' true incidence ' of this association. Given the potential lethal consequences of midgut volvulus screening may be justified in OA babies. Consensus guidelines (DELPHI) exploring surgeons attitudes with regards management of ' asymptomatic malrotation ' disorders in OA newborns may further guide best practice.

Barrett's oesophagus and oesophageal cancer following oesophageal atresia repair: a systematic review.

BACKGROUND: Concern exists that patients born with oesophageal atresia (OA) may be at high risk for Barrett's oesophagus (BO), a known malignant precursor to the development of oesophageal adenocarcinoma. Screening endoscopy has a role in early BO identification but is not universal in this population. This study aimed to determine prevalence of BO after OA repair surgery, to quantify the magnitude of this association and inform the need for screening and surveillance. METHODS: A systematic review, undertaken according to PRISMA guidelines, was preregistered on PROSPERO (CRD42017081001). PubMed and EMBASE were interrogated using a standardized search strategy on 31 July 2020. Included papers, published in English, reported either: one or more patients with either BO (gastric/intestinal metaplasia) or oesophageal cancer in patients born with OA; or long-term (greater than 2 years) follow-up after OA surgery with or without endoscopic screening or surveillance. RESULTS: Some 134 studies were identified, including 19 case reports or series and 115 single- or multi-centre cohort studies. There were 13 cases of oesophageal cancer (9 squamous cell carcinoma, 4 adenocarcinoma) with a mean age at diagnosis of 40.5 (range 20-47) years. From 6282 patients under long-term follow-up, 317 patients with BO were reported. Overall prevalence of BO was 5.0 (95 per cent c.i. 4.5 to 5.6) per cent, with a mean age at detection of 13.8 years (range 8 months to 56 years). Prevalence of BO in series reporting endoscopic screening or surveillance was 12.8 (95 per cent c.i. 11.3 to 14.5) per cent. CONCLUSION: Despite a limited number of cancers, the prevalence of BO in patients born with OA is relatively high. While limited by the quality of available evidence, this review suggests endoscopic screening and surveillance may be warranted, but uncertainties remain over the design and effectiveness of any putative programme.

Primary repair of esophageal atresia is followed by multiple diagnostic and surgical procedures.

BACKGROUND: Children born with esophageal atresia (EA) face comorbidities and complications often requiring surgery and anesthesia. We aimed to assess all procedures performed under general anesthesia during their first 12 years of life. METHODS: We performed a retrospective cohort study about subsequent surgeries and procedures requiring general anesthesia in children born with type C EA between January 2007 and December 2017, with follow-up to March 2019. RESULTS: Of 102 eligible patients, 63 were diagnosed with comorbidities, of whom 18 had VACTERL association. Follow-up time for all patients varied between 14 months and 12 years (median 7 years). The patients underwent total 637 procedures, median 4 [IQR2-7] per patient. In the first year of life, 464 procedures were performed, in the second year 69 and in the third year 29. Thirteen patients underwent no other procedures than primary EA repair. In 57 patients, 228 dilatations were performed. Other frequently performed procedures were esophagoscopy (n=52), urologic procedures (n=44) and abdominal procedures (n=33). CONCLUSIONS: Patients with EA frequently require multiple anesthetics for a variety of procedures related to the EA, complications and comorbidities. This study can help care providers when counselling parents of a patient with an EA by giving them more insight into possible procedures they can be confronted with during childhood.

Radiation burden in patients with esophageal atresia: a systematic review.

Esophageal atresia (EA) is the most common congenital esophageal disorder. Radiological imaging facilitates diagnosis, surgical interventions, and follow-up. Despite this, standardized monitoring guidelines are lacking. We aimed to: (1) review the literature regarding radiation burden in children with EA; (2) establish the presence of guidelines for diagnosis and follow-up in children with EA. The systematic review was performed according to PRISMA protocol. Two investigators conducted independent searches (PubMed, Ovid, Cochrane Review) and data extraction. Analysis focused on pre- and post-operative imaging type and frequency to determine the radiation burden. Seven studies met the inclusion criteria (337 patients). All authors agreed upon the need to minimize radiation burden, recommending symptoms-guided management, use of dosimeters, and non-radiating imaging. One study identified a median 130-fold increase in cumulative lifetime cancer risk in children with EA compared with other babies in the special care unit. The most common investigations were X-ray and CT (pre-operatively), and X-ray and contrast swallow (post-operatively). Standardized guidelines focused upon the frequency and type of radiological imaging for children with EA are lacking. Children with EA are subjected to more radiation exposure than the general population. Implementation of non-radiating imaging (ultrasonography, manometry) is recommended.