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Acta Obstetricia Et Gynecologica... Jun 2019The primary aim of this systematic review was to quantify the diagnostic performance of ultrasound, magnetic resonance imaging and amniotic fluid analysis in detecting... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
The primary aim of this systematic review was to quantify the diagnostic performance of ultrasound, magnetic resonance imaging and amniotic fluid analysis in detecting esophageal atresia prenatally. The secondary aim was to explore the accuracy of individual imaging signs in identifying this anomaly.
MATERIAL AND METHODS
MEDLINE, Embase and Cochrane databases were searched. The quality of studies was assessed using the revised tool for the quality assessment of diagnostic accuracy studies. Summary estimates of sensitivity, specificity, positive and negative likelihood ratios, and diagnostic odds ratio for the predictive accuracy of ultrasound, magnetic resonance imaging and amniotic fluid analysis in detecting esophageal atresia were computed using the hierarchical summary receiver operating characteristic or DerSimonian-Laird random-effect model, according to the number of studies included in each analysis. PROSPERO registration number: CRD42017055828.
RESULTS
Twenty studies (73 246 fetuses, 1760 affected by esophageal atresia) were included. Overall, prenatal ultrasound had a sensitivity of 31.7%. Only two studies reported all data for diagnostic accuracy; based on these studies, prenatal ultrasound had a sensitivity of 41.9%, a specificity of 99.9%, a positive likelihood ratio of 88.1, a negative likelihood ratio of 0.58 and a diagnostic odds ratio of 153.7. Prenatal ultrasound correctly identified 77.9% of cases with esophageal atresia and 21.9% esophageal atresia with an associated tracheo-esophageal fistula. Polyhydramnios was present in 56.3% of cases affected by esophageal atresia, and a small or absent stomach was identified in 50.0% cases. When performed following a suspicious ultrasound, fetal magnetic resonance imaging had an good overall diagnostic accuracy for esophageal atresia, with a sensitivity of 94.7%, a specificity of 89.3%, a positive likelihood ratio of 8.8, a negative likelihood ratio of 0.06 and a diagnostic odds ratio of 149.3. Finally, amniotic fluid analysis with an esophageal atresia index ≥3 had a sensitivity of 89.9% and a specificity of 99.6% in detecting esophageal atresia.
CONCLUSIONS
Ultrasound alone is a poor diagnostic tool for identifying esophageal atresia prenatally, and has a high rate of false positive diagnoses. Magnetic resonance imaging and amniotic fluid analysis have high diagnostic accuracy for esophageal atresia. We would recommend their use following a suspicious ultrasound.
Topics: Esophageal Atresia; Humans; Prenatal Diagnosis; Reproducibility of Results
PubMed: 30659586
DOI: 10.1111/aogs.13536 -
Seminars in Pediatric Surgery Dec 2022Anomalies in tracheo-esophageal development result in a spectrum of congenital malformations ranging from, most commonly, esophageal atresia with or without...
Anomalies in tracheo-esophageal development result in a spectrum of congenital malformations ranging from, most commonly, esophageal atresia with or without trachea-esophageal fistula (EA+/-TEF) to esophageal web, duplication, stricture, tracheomalacia and tracheal agenesis. Despite the relative frequency of EA, however, the underlying etiology remains unknown and is likely due to a combination of genetic, epigenetic and environmental factors. In recent years, animal models have dramatically increased our understanding of the molecular and morphological processes involved in normal esophageal development during the key stages of anterior-posterior regionalization, dorsal-ventral patterning and morphogenic separation. Moreover, the use of animal models in conjunction with increasingly advanced techniques such as genomic sequencing, sophisticated live imaging studies and organoid models have more recently cast light on potential mechanisms involved in EA pathogenesis. This article aims to unravel some of the mysteries behind the anatomy and embryology of EA whilst providing insights into future directions for research.
Topics: Animals; Humans; Tracheoesophageal Fistula; Esophageal Atresia; Trachea; Tracheomalacia
PubMed: 36459913
DOI: 10.1016/j.sempedsurg.2022.151231 -
Pediatric Surgery International Oct 2023This study was performed to describe the current clinical practice and outcomes of type D esophageal atresia. We retrospectively analyzed 10 patients who were diagnosed... (Review)
Review
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.
Topics: Humans; Infant, Newborn; Child; Esophageal Atresia; Tracheoesophageal Fistula; Retrospective Studies; Thoracoscopy; Digestive System Surgical Procedures
PubMed: 37815659
DOI: 10.1007/s00383-023-05519-6 -
European Journal of Pediatrics May 2023to review recent literature concerning long-term health issues and transitional care in esophageal atresia (EA) patients. PubMed, Scopus, Embase and Web of Science... (Meta-Analysis)
Meta-Analysis Review
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.
Topics: Adolescent; Adult; Child; Humans; Middle Aged; Young Adult; Barrett Esophagus; Deglutition Disorders; Disease Progression; Esophageal Atresia; Follow-Up Studies; Gastroesophageal Reflux; Quality of Life; Transitional Care
PubMed: 36905437
DOI: 10.1007/s00431-023-04893-6 -
Diseases of the Esophagus : Official... Oct 2022Up to 45% of esophageal atresia (EA) patients undergo fundoplication during childhood. Their esophageal dysmotility may predispose to worse fundoplication outcomes...
Up to 45% of esophageal atresia (EA) patients undergo fundoplication during childhood. Their esophageal dysmotility may predispose to worse fundoplication outcomes compared with patients without EA. We therefore compared fundoplication outcomes and symptoms pre- and post-fundoplication in EA patients with matched patients without EA. A retrospective review of patients with- and without EA who underwent a fundoplication was performed between 2006 and 2017. Therapeutic success was defined as complete sustained resolution of symptoms that were the reason to perform fundoplication. Fundoplication indications of 39 EA patients (49% male; median age 1.1 [0.1-17.0] yrs) and 39 non-EA patients (46% male; median age 1.3 [0.3-17.0] yrs) included respiratory symptoms, brief resolved unexplained events, typical symptoms of gastroesophageal reflux disease, recurrent strictures and respiratory problems. Post-fundoplication, therapeutic success was achieved in 5 (13%) EA patients versus 29 (74%) non-EA patients (P<0.001). Despite therapeutic success, all 5 (13%) EA patients developed postoperative sustained symptoms/complications versus 12 (31%) non-EA patients. Eleven (28%) EA patients versus 3 (8%) non-EA patients did not achieve any therapeutic success (P=0.036). Remaining patients achieved partial therapeutic success. EA patients suffered significantly more often from postoperative sustained dysphagia (41% vs. 13%; P=0.039), gagging (33% vs. 23%; P<0.001) and bloating (40% vs. 17%; P=0.022). Fundoplication outcomes in EA patients are poor and EA patients are more susceptible to post-fundoplication sustained symptoms and complications compared with patients without EA. The decision to perform fundoplication in EA patients with proven gastroesophageal reflux disease needs to be made with caution after thorough multidisciplinary evaluation.
Topics: Child; Esophageal Atresia; Esophagoplasty; Female; Fundoplication; Gastroesophageal Reflux; Humans; Infant; Male; Postoperative Complications; Treatment Outcome
PubMed: 35211748
DOI: 10.1093/dote/doac006 -
Polski Przeglad Chirurgiczny Apr 2022Esophageal atresia is a congenital anomaly well known in the community of pediatric surgeons. Nonetheless some aspects of management remain vague and societies of... (Review)
Review
INTRODUCTION
Esophageal atresia is a congenital anomaly well known in the community of pediatric surgeons. Nonetheless some aspects of management remain vague and societies of gastroenterologists as well as surgeons have been gathering to determine pertinent ways to handle this condition. To make ground for unification, the guidelines of some most important societies were compared and gathered in one review.
MATERIALS AND METHODS
Literature review of online databases of the PubMed and the Cochrane Library with (o)esophageal atresia, guidelines and follow-up used as keywords.
RESULTS
Over the course of the last few years there was a lot of effort invested in making clear and accurate guidelines for management of EA, largely with good results. In the majority of important matters, opinions of specialists were consistent or complementary to each other. Because some of them described different phases of management, gathering them together led to obtaining the wider picture, which can help pediatric surgeons in making decisions while treating the patients with EA.
CONCLUSION
There is a necessity for careful following guidelines which have been changing quite fast along with new significant publications about EA. Some concerns remained debatable and their accentuation in this review was made to bring awareness to vague issues, such as postoperative antibiotic prophylaxis or duration of administering PPI. Additionally structured follow-up programs for the first 18 years and after reaching maturity should be made.
Topics: Child; Humans; Esophageal Atresia; Antibiotic Prophylaxis; Decision Making; Surgeons
PubMed: 36806162
DOI: 10.5604/01.3001.0015.8208 -
Paediatric Respiratory Reviews Jun 2016The current available literature evaluating feeding difficulties in children with esophageal atresia was reviewed. The published literature was searched through PubMed... (Review)
Review
The current available literature evaluating feeding difficulties in children with esophageal atresia was reviewed. The published literature was searched through PubMed using a pre-defined search strategy. Feeding difficulties are commonly encountered in children and adults with repaired esophageal atresia [EA]. The mechanism for abnormal feeding includes both esophageal and oropharyngeal dysphagia. Esophageal dysphagia is commonly reported in patients with EA and causes include dysmotility, anatomic lesions, esophageal outlet obstruction and esophageal inflammation. Endoscopic evaluation, esophageal manometry and esophograms can be useful studies to evaluate for causes of esophageal dysphagia. Oropharyngeal dysfunction and aspiration are also important mechanisms for feeding difficulties in patients with EA. These patients often present with respiratory symptoms. Videofluoroscopic swallow study, salivagram, fiberoptic endoscopic evaluation of swallowing and high-resolution manometry can all be helpful tools to identify aspiration. Once diagnosed, management goals include reduction of aspiration during swallowing, reducing full column reflux into the oropharynx and continuation of oral feeding to maintain skills. We review specific strategies which can be used to reduce aspiration of gastric contents, including thickening feeds, changing feeding schedule, switching formula, trialing transpyloric feeds and fundoplication.
Topics: Adult; Barium Sulfate; Child; Contrast Media; Deglutition Disorders; Esophageal Atresia; Esophagoscopy; Humans; Manometry; Nutritional Support
PubMed: 26164203
DOI: 10.1016/j.prrv.2015.06.002 -
Ultrasound in Obstetrics & Gynecology :... Jul 2021Esophageal atresia and/or tracheoesophageal fistula (EA/TEF) remains one of the most frequently missed congenital anomalies prenatally. The aim of our study was to...
OBJECTIVE
Esophageal atresia and/or tracheoesophageal fistula (EA/TEF) remains one of the most frequently missed congenital anomalies prenatally. The aim of our study was to elucidate the sonographic manifestation of EA/TEF throughout pregnancy.
METHODS
This was a retrospective study of data obtained from a tertiary center over a 12-year period. The prenatal ultrasound scans of fetuses with EA/TEF were assessed to determine the presence and timing of detection of three principal signs: small/absent stomach and worsening polyhydramnios, both of which were considered as 'suspected' EA/TEF, and esophageal pouch, which was considered as 'detected' EA/TEF. We assessed the yield of the early (14-16 weeks' gestation), routine mid-trimester (19-26 weeks) and third-trimester (≥ 27 weeks) anomaly scans in the prenatal diagnosis of EA/TEF.
RESULTS
Seventy-five cases of EA/TEF with available ultrasound images were included in the study. A small/absent stomach was detected on the early anomaly scan in 3.6% of fetuses scanned, without a definitive diagnosis. On the mid-trimester scan, 19.4% of scanned cases were suspected and 4.3% were detected. On the third-trimester anomaly scan, 43.9% of scanned cases were suspected and 33.9% were detected. An additional case with an esophageal pouch was detected on magnetic resonance imaging (MRI) in the mid-trimester and a further two were detected on MRI in the third trimester. In total, 44.0% of cases of EA/TEF in our cohort were suspected, 33.3% were detected and 10.7% were suspected but, eventually, not detected prenatally.
CONCLUSIONS
Prenatal diagnosis of EA/TEF on ultrasound is not feasible before the late second trimester. A small/absent stomach may be visualized as early as 15 weeks' gestation. Polyhydramnios does not develop before the mid-trimester. An esophageal pouch can be detected as early as 22 weeks on a targeted scan in suspected cases. The detection rates of all three signs increase with advancing pregnancy, peaking in the third trimester. The early and mid-trimester anomaly scans perform poorly as a screening and diagnostic test for EA/TEF. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.
Topics: Esophageal Atresia; Esophagus; Female; Fetal Development; Fetus; Humans; Longitudinal Studies; Polyhydramnios; Predictive Value of Tests; Pregnancy; Pregnancy Trimester, Second; Pregnancy Trimester, Third; Retrospective Studies; Stomach; Tracheoesophageal Fistula; Ultrasonography, Prenatal
PubMed: 32304613
DOI: 10.1002/uog.22050 -
Pediatric Pulmonology Jan 2020Respiratory morbidity after esophageal atresia (EA) is common. The aims of this study were to assess pulmonary function, to identify risk factors for pulmonary function... (Observational Study)
Observational Study
INTRODUCTION
Respiratory morbidity after esophageal atresia (EA) is common. The aims of this study were to assess pulmonary function, to identify risk factors for pulmonary function impairment (PFI), and to investigate the relations between respiratory morbidity, defined as medical treatment for respiratory symptoms or recent pneumonia and PFI after EA repair.
MATERIAL AND METHODS
Single center retrospective observational study including patients with EA who participated in the follow-up program for 8- or 15-year old patients from 2014 to 2018 and performed pulmonary function testing by body plethysmography, dynamic spirometry, impulse oscillometry, and diffusing capacity of the lungs. Univariate and multiple stepwise logistic regression with PFI as outcome were performed. Anastomotic leak, episodes of general anesthesia, extubation day, birth weight, age at follow up, gross classification, and abnormal reflux index were independent variables.
RESULTS
In total, 47 patients were included. PFI was found in 19 patients (41%) and 16 out of 19 patients (84%) had an obstructive pattern. Respiratory morbidity was found in 23 (52%, NA = 3) of the patients with no correlation to PFI. Birth weight, age at follow-up, and episodes of general anesthesia were identified as risk factors for PFI.
CONCLUSION
Respiratory morbidity and PFI were common in children and adolescents after EA repair. The major component of PFI was obstruction of the airways. The risk for PFI increased with lower birth weight and older age at follow up. The poor correlation between respiratory morbidity and PFI motivates the need of clinical follow up including pulmonary function tests.
Topics: Adolescent; Child; Esophageal Atresia; Female; Humans; Infant, Low Birth Weight; Logistic Models; Lung Diseases; Male; Risk Factors; Spirometry
PubMed: 31535483
DOI: 10.1002/ppul.24517 -
Frontiers in Pediatrics 2017
PubMed: 28913328
DOI: 10.3389/fped.2017.00190