Esophageal Atresia Type Research Articles

Esophageal atresia type III? Intraoperative surprise and different approaches.

A newborn was referred due to clinical and radiological suspicion of esophageal atresia (EA) type III. Surgery revealed an esophagus without evident interruptions; however, intraoperative advancement of the nasogastric tube was unsuccessful, and the distal esophagus inflated with each ventilation, indicating the presence of a distal fistula. An intraoperative esophago-tracheobronchoscopy showed a proximal esophageal pouch with a tiny tracheoesophageal fistula and a large distal tracheoesophageal fistula. The esophageal ends were blind but overlapping, with no external discontinuity observed. With the diagnosis of Krediet type IIIc2 esophageal atresia, we performed a meticulous esophago-tracheal dissection, distal fistula closure, and end-to-end anastomosis. Due to hemodynamic instability, the proximal fistula was closed two weeks later via cervicotomy without incidents.

Enhanced estimation strategy for determining the location of tracheoesophageal fistula in a preterm, low-birth-weight infant with congenital esophageal atresia type C and duodenal atresia: a case report

BackgroundIn esophageal atresia type C, identifying the tracheoesophageal fistula (TEF) location is crucial for airway management. However, a thin bronchoscope may not always be available.Case presentationWe report on a low-birth-weight neonate with esophageal atresia type C who required immediate gastrostomy after birth. With no suitable thin bronchoscope available, alternative methods were utilized to estimate the TEF location post-gastrostomy. Submerging the gastrostomy tube tip in water and applying positive pressure ventilation via a tracheal tube allowed for observation of air bubbles emerging from the gastrostomy tube. As the tracheal tube was advanced, the cessation of bubbles indicated that the TEF was sealed by the tracheal tube. The location of the tracheal tube tip, confirmed by chest radiographs, was consistent with the TEF location identified during corrective surgery for TEF.ConclusionsThis innovative technique facilitated successful estimation of the TEF location without bronchoscopy, demonstrating its efficacy in resource-limited settings.

Cardiovascular malformation associated with esophageal atresia

Abstract Background Congenital heart disease (CHD) is the commonest abnormality associated with esophageal atresia (EA). EA is a rare congenital malformation occurring in 3-4 in 10,000 births, in 87% occurs along with a distal tracheoesophageal fistula (TEF). The coexistence of a cardiac malformation and in addition, prematurity can be challenging and affects the further treatment and quality of life (QOL) of the child and family. Knowledge of the anatomical type and physiological consequences of CHD allows a well-coordinated treatment plan. Purpose The aim of this paper is to investigate whether the presence of underlying associated malformations, disease-specific problems and prematurity would have a considerable influence on the family of a child after surgical repair of EA. Methods We used a crossed-sectional questionnaire study. The impact of EA was assessed using an Authors-Designed Questionnaire (ADQ) to collect medical and sociodemographic background data (gestational week (HBD), comorbidities, type of EA, according to the Gross classification) as well as standardized questionnaire: the PedsQL™ Family Impact Module (PedsQL-FIM). Results Of the 76 children born with EA/TEF included in this study, 26% had a vascular malformation. Mean age was 5.3 years (SD = 3.7 years). Cardiovascular malformation associated with EA significantly (p=0.037) affects the functioning of the family in the emotional domain (FIM – ED). The QOL (p < 0,034) of children born before 37th HBD is lower that children born in term The presence of concomitant anomalies does affect the overall generic QoL. No statistical correlation was found between the type of anomaly (with/without TEF) and patients' QoL. Conclusions Cardiovascular malformation and prematurity significantly affect child’s QOL and the functioning of the family with EA. Patients who have undergone EA treatment require specialist outpatient care, not only in the first years of life, but also in adolescence and adulthood.

Management of long-gap esophageal atresia.

A uniquely challenging subset of infants diagnosed with esophageal atresia (EA) are those born with long-gap EA (LGEA). The common unifying feature in infants with LGEA is that the proximal and distal segments of the esophagus are too far apart to enable primary anastomosis via a single operation in the newborn period. Although any type of EA can technically result in a long gap, the Gross type A variant occurs in 8% of all EA cases and is most commonly associated with LGEA. In this review, we provide an evidence-based approach to the current challenges and management strategies employed in LGEA. There are fortunately a range of available surgical techniques for LGEA repair, including delayed primary repair, staged repair based on longitudinal traction strategies to lengthen both ends (e.g., Foker procedure, internal traction), and esophageal replacement using other portions of the gastrointestinal tract. The literature on the management of LGEA has long been dominated by single-center retrospective reviews, but the field has recently witnessed increased multi-center collaboration that has helped to increase our understanding on how to best manage this challenging patient population. Delayed primary repair is strongly preferred as the initial approach in management of LGEA in the United States as well as several European countries and is supported by the American Pediatric Surgery Association recommendations. Should esophageal replacement be required in cases where salvaging the native thoracic esophagus is not possible, gastric conduits are the preferred approach, based on the relative simplicity of the operation, low postoperative morbidity, and long-term durability. Long-term followup for monitoring of swallowing function, nutritional status, aspiration/respiratory illnesses, gastroesophageal reflux, and associated comorbidities is essential in the comprehensive care of these complex patients.

Kimura extrathoracic esophageal elongation for the treatment of complex esophageal atresia: Primary versus secondary treatment when other strategies failed

IntroductionThe Kimura´s extrathoracic esophageal elongation (KEEE) procedure uses upper esophageal pouch traction to induce its growth, as an alternative to repair long gap esophageal atresia (LGEA). It can be used as a first option in the neonatal period (primary Kimura) or as a rescue procedure when another strategy fails (secondary Kimura).The aim of our study is to compare the results of the primary versus secondary Kimura procedure after unsuccessful Puri procedure or end-to-end esophageal anastomosis. MethodsThe present work is a retrospective review of patients with complex esophageal atresia (CEA) who underwent Kimura procedure at our institution from 1997 to 2021. We divided the patients into two groups, primary group: patients who underwent primary Kimura extrathoracic esophageal elongation (KEEE), esophagostomized in the first surgical intervention; and secondary group: those who underwent secondary KEEE, esophagostomized due to complications of the primary end-to-end anastomosis (EEA), or due to failure of preservation of native esophagus (PNE) techniques. Demographic variables (gestational age, birth weight, type of esophageal atresia (EA) and associated malformations) and evolution variables related to the elongations and reconstruction (days of mechanical ventilation (MV), days of hospitalization and complications of the anastomosis, feeding/oral aversion, need for nutritional support, gastroesophageal reflux disease (GERD), and mortality) were evaluated. ResultsDuring the 24-year period, 31 patients were evaluated, nine patients belonged to the primary group (of which 7 were reconstructed) and the remaining twenty-two to the secondary group (of which 12 completed the procedure).Regarding demographic variables, there was a statistical difference in gestational age (p = 0.016). Regarding the variables associated with the elongations, there was a statistically significant lower number of elongations (p = 0.0547), longer hospitalization time (p = 0.045) and more complications (p = 0.027) for the secondary group. Regarding the final reconstruction, the primary group had a significantly shorter postoperative hospital stay (p = 0.03), and also fewer days on MV, although these variables did not reach a statistically significant difference.Two patients died before the final reconstruction (one from each group). DiscussionWe believe that KEEE is a useful tool for both primary treatment and rescue in case of failure of other esophageal preservation strategies. In addition, it allows preserving the native esophagus and also performing esophageal replacement strategies with other organs if it fails. Level of evidenceAccording to the Journal of Pediatric Surgery this research corresponds to type of study level III for retrospective comparative study.

Combined esophageal and duodenal atresia: A review of the literature from 1950 to 2020

The combination of duodenal atresia (DA) and esophageal atresia (EA) is very rare. With improvements in prenatal sonography and the use of fetal magnetic resonance imaging (MRI), these malformations can be diagnosed in a more accurate and timely manner; polyhydramnios remains the most common sign despite having a low specificity. The high rate of associated anomalies (in 85% of cases) can also impact neonatal management and increase the morbidity rate; thus, it is of paramount importance to look for every possible associated malformation, such as VACTERL and chromosomic anomalies. The surgical management of this combination of atresias is not well defined and changes according to the patient's clinical status, the type of EA, and the other associated malformations. Management ranges from a primary approach for one of the atresias with delayed correction of the other (56.8%) to a simultaneous repair of both atresias (33.8%) with or without gastrostomy, or total abstention (9.4%). We suggest that a simultaneous approach can be safely performed on patients in good physical condition, with a birth weight over 1500 g, and with no major respiratory distress; this method begins by closing the tracheoesophageal fistula to protect the lung and then repairing the DA. The mortality rate has decreased over the years, dropping from 71% before 1980 to 24% after 2001. In this review, we present the available evidence on these conditions, focusing mostly on the epidemiology, prenatal diagnosis, neonatal management strategies, and outcome, with the aim of determining how the different clinical features and surgical approaches may impact on morbidity and mortality.

Prenatal Diagnosis of Esophageal Atresia – Performance and Consequences

BackgroundPrenatal diagnosis of congenital malformations is considered favorable. Esophageal atresia (EA) is prenatally detected in 10–40% of patients. The aims of our study were to assess factors influencing the prenatal detection rate and to study the outcome in EA patients with and without prenatal diagnosis. MethodWe included 136 patients in two time periods, group 1 (1996–2002, n = 68) and group 2 (2014–2020, n = 68). We registered clinical variables; prenatal signs, perinatal and postnatal outcome from the electronic patient record. ResultsTwenty-five patients (18%) had a prenatal diagnosis of EA, significantly more during 2014–2020 (28%), than during 1996–2002 (9%). Patients with EA type A or B and with associated anomalies had increased likelihood of prenatal diagnosis, odds ratio (OR) 9.00 (1.99–40.69) and 3.53 (1.24–10.06), respectively. Among the 25 patients with prenatal diagnosis all had polyhydramnios and 16 had small/absent stomach. Prenatally diagnosed patients arrived significantly earlier at the surgical unit (median 2 h (2 h-1 days) vs 21 h (2 h-1275 days)), had more delayed primary anastomosis (OR 8.80 (2.68–28.92)) and anastomotic stricture (OR 3.11 (1.20–8.04)), longer length of stay (median 62 days (11–212 days) vs 20 days (2–270 days)) and longer time on ventilator (median 5 days (1–25 days) vs 1.5 days (0.5–33 days)) compared to patients without prenatal diagnosis. In multivariate analysis prenatal diagnosis predicts length of stay. ConclusionPrenatally diagnosed EA patients have more; type A and B malformations, associated anomalies and neonatal morbidity. Consequences of the assumed benefits of prenatal diagnosis; opportunity of early arrival to surgical care and prenatal counselling, must be further studied.

The Role of Bolus Residue and Its Relation with Respiratory Problems in Children with Esophageal Atresia.

Bolus residue is significant risk factor for postswallow aspiration. A retrospective study was performed to evaluate the role of bolus residue and its relation with respiratory problems in children with esophageal atresia. Children were evaluated for demographic features, type of esophageal atresia, associated anomalies, and respiratory problems. The videofluoroscopic swallowing evaluation (VFSE) was performed, and scored by using the penetration aspiration scale (PAS), bolus residual score (BRS) and normalized residual ratio scale (NRRS). Children with and without respiratory problems were also compared in terms of aspiration and bolus residue. Forty-one children with a median age of 15months (1-138months), male:female ratio of 26:15 was included. 65.9% (n = 27) of children were type-C and 24.4% (n = 10) were type-A EA. In 61% (n = 25) of children had liquid aspiration (PAS ≥ 6) and 9.8% (n = 4) had aspiration in pudding consistencies. Children with aspiration in liquids had significantly higher NRRS and BRS scores in vallecular residue for pudding consistencies when compared to children without aspiration (p < 0.05). No difference was detected in terms of PAS scores and bolus residual parameters between children with and without respiratory problems (p > 0.05). Children with aspiration in liquids have higher scores of BRS and NRRS at the level of vallecular especially in pudding consistencies. VFSE findings for bolus residue did not show significant relation with respiratory problems. Respiratory morbidity in children with EA is multifactorial and may not only explained by bolus residuals and aspiration.

Peri- and neonatal factors influencing mortality and morbidity 2years after esophageal atresia primary repair: a single center retrospective study.

Post-operative ventilation and feeding management strategies may represent an opportunity for quality-of-care improvement to positively impact long-term outcomes after primary esophageal atresia repair. • Children operated on for esophageal atresia experience long-term digestive, respiratory, and neurologic morbidity, especially after multiple-stage esophageal repair. • Exclusive oral feeding at discharge is associated with a decreased risk of medical complications in the first years of life, in studies including all types of esophageal atresia repair.Outcomes of children after primary repair (non-long gap populations) have been less documented. • In our retrospective cohort of children with one-stage esophageal atresia repair, ventilatory support for more than 8 days and inability to achieve full oral feeding before hospital discharge in the neonatal period were independently associated with adverse digestive, respiratory, and neurologic outcomes at 2 years in survivors. • Both these factors are potentially modifiable, representing an opportunity for quality-of-care improvement to positively impact long-term outcomes.These results might also help identify children at risk of unfavorable evolution, to customize a multi-disciplinary follow-up program.

Endoscopic Surveillance of Esophageal Atresia Population according to ESPGHAN-NASPGHAN 2016 Guidelines: Incidence of Eosinophilic Esophagitis and New Histological Findings.

Follow-up of children born with esophageal atresia (EA) is mandatory due to high incidence of comorbidities. We evaluated endoscopic findings at follow-up of EA patients performed at our Centre according to ESPGHAN-NASPGHAN 2016 guidelines. A retrospective observational study was performed using data from January 2016 to January 2021. We included EA patients (age range: 1-18 years) who were offered a program of endoscopic and histological high gastrointestinal (GI) tract examinations as per ESPGHAN-NASPGHAN 2016 guidelines. Clinical, surgical, auxological, endoscopic, and histological data were reviewed; variables as polyhydramnios, EA type, surgical type, enteral feeding introduction age, growth data, and symptoms were correlated to endoscopic and histological findings. The population included 75 patients (47 males), with mean age of 5 ± 4 years. In 40/75 (53.3%) patients, we recorded oral feeding problems, and upper gastrointestinal or respiratory symptoms suspicious of gastroesophageal reflux. Eosinophilic esophagitis (EoE) incidence was 9/75 (12%), significantly higher than in general population (p &lt; 0.0001), and 10/75 (13.3%) presented non-specific duodenal mucosal lesions. EoE represents a frequent comorbidity of EA, as previously known. EA is also burdened by high, never-described incidence of non-specific duodenal mucosal lesions. Embedding high GI tract biopsies in EA endoscopic follow-up should be mandatory from pediatric age.

Successful staged management of long gap esophageal atresia

Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus. There is little consensus on the definition of long gap esophageal atresia (LGEA). However, the term is used to describe cases in which a primary anastomosis of the proximal and distal ends of the esophagus cannot easily be performed under acceptable tension by the operating surgeon. LGEA remains a technically challenging subset of EA cases. We present a case of long gap esophageal atresia type IIIA (Vogt classification)/Type B (Gross classification) which had a successful final outcome after multiple procedures which allowed the maintenance of the original esophagus, thus not needing esophageal replacement. The patient is now 6 years old, is thriving well and does not have any complaints.

Nutritional status at age 1 year in patients born with esophageal atresia: A population-based, prospective cohort study.

ObjectiveDespite recent progress in caring for patients born with esophageal atresia (EA), undernutrition and stunting remain common. Our study objective was to assess nutritional status in the first year after birth with EA and to identify factors associated with growth failure.Study designWe conducted a population-based study of all infants born in France with EA between 2010 and 2016. Through the national EA register, we collected prenatal to 1 year follow-up data. We used body mass index and length-for-age ratio Z scores to define patients who were undernourished and stunted, respectively. Factors with P < 0.20 in univariate analyses were retained in a logistic regression model.ResultsAmong 1,154 patients born with EA, body mass index and length-for-age ratio Z scores at 1 year were available for about 61%. Among these, 15.2% were undernourished and 19% were stunted at the age of 1 year. There was no significant catch-up between ages 6 months and 1 year. Patients born preterm (41%), small for gestational age (17%), or with associated abnormalities (55%) were at higher risk of undernutrition and stunting at age 1 year (P < 0.05). Neither EA type nor surgical treatment was associated with growth failure.ConclusionUndernutrition and stunting are common during the first year after birth in patients born with EA. These outcomes are significantly influenced by early factors, regardless of EA type or surgical management. Identifying high-risk patient groups with EA (i.e., those born preterm, small for gestational age, and/or with associated abnormalities) may guide early nutritional support strategies.

From the Ground Up: Esophageal Atresia Types, Disease Severity Stratification and Survival Rates at a Single Institution.

Esophageal atresia (EA), although a rare congenital anomaly, represents one of the most common gastrointestinal birth defects. There is a gap in our knowledge regarding the impact of perioperative critical care in infants born with EA. This study addresses EA types, disease severity stratification, and mortality in a retrospective cohort at a single institution. Institutional Review Board approved our retrospective cross-sectional study of term-born (n = 53) and premature infants (28–37 weeks of gestation; n = 31) that underwent primary surgical repair of EA at a single institution from 2009–2020. Demographic and clinical data were obtained from the electronic medical record, Powerchart (Cerner, London, UK). Patients were categorized by (i) sex, (ii) gestational age at birth, (iii) types of EA (in relation to respiratory tract anomalies), (iv) co-occurring congenital anomalies, (v) severity of disease (viz. American Society of Anesthesiologists (ASA) and Pediatric Risk Assessment (PRAm) scores), (vi) type of surgical repair for EA (primary anastomosis vs. Foker process), and (vii) survival rate classification using Spitz and Waterston scores. Data were presented as numerical sums and percentages. The frequency of anatomical types of EA in our cohort parallels that of the literature: 9.5% (8/84) type A, 9.5% (8/84) type B, 80% (67/84) type C, and 1% (1/84) type D. Long-gap EA accounts for 88% (7/8) type A, 75% (6/8) type B, and 13% (9/67) type C in the cohort studied. Our novel results show a nearly equal distribution of sex per each EA type, and gestational age (term-born vs. premature) by anatomical EA type. PRAm scoring showed a wider range of disease severity (3–9) than ASA scores (III and IV). The survival rate in our EA cohort dramatically increased in comparison to the literature in previous decades. This retrospective analysis at a single institution shows incidence of EA per sex and gestational status for anatomical types (EA type A-D) and by surgical approach (primary anastomosis vs. Foker process for short-gap vs. long-gap EA, respectively). Despite its wider range, PRAm score was not more useful in predicting disease severity in comparison to ASA score. Increased survival rates over the last decade suggest a potential need to assess unique operative and perioperative risks in this unique population of patients. Presented findings also represent a foundation for future clinical studies of outcomes in infants born with EA.

Congenital Esophageal Atresia Long-Term Follow-Up—The Pediatric Surgeon’s Duty to Focus on Quality of Life

Esophageal atresia (EA) is the most common congenital esophageal malformation. An improvement in survival led to a focus on functional outcomes and quality of life (QoL). We analyzed the long-term outcomes and QoL of patients submitted to surgery for EA. Perinatal characteristics, surgical procedures, gastrointestinal and respiratory current symptoms and QoL were investigated. Thirty-nine patients were included. Long Gap patients had a higher rate of prematurity and low birth weight. The prevalent surgical procedure was primary esophageal anastomosis, followed by gastric pull-up. Twenty-four patients had post-operative stenosis, while gastroesophageal reflux (GER) required fundoplication in eleven cases. Auxological parameters were lower in Long Gap patients. The lowest scores of QoL were in the Long Gap group, especially in younger patients, which was the group with the highest number of symptoms. In the long term, the QoL appeared to be more dependent on the type of esophageal atresia rather than on associated malformations. Surgical management of GER was indicated in all patients with Long Gap EA, supposedly due to the prevalence of gastric pull-up for this type of EA. The assessment of QoL is part of surgeon’s management and needs to be performed in each phase of a child’s development.

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.

Esophageal atresia with proximal and distal tracheo-esophageal fistulae

The esophageal atresia (EA) with distal fistula is the most common type of EA and is easily diagnosed by frothy discharge from mouth and nose, respiratory distress and coiling of nasogastric tube in the upper esophagus. The EA with double fistula (proximal and distal) is the least common type of EA which can easily be ignored preoperatively and intraoperatively. So it offers diagnostic challenge. Herein, we present a case of EA with double fistula in which the proximal fistula was not preoperatively appreciated by us, but identified during dissection.

Thoracoscopic Repair of Esophageal Atresia.

Background: Esophageal atresia (EA) is a rare congenital malformation of the esophagus. Surgical treatment is required to restore the continuity of the esophagus. This can be performed through thoracotomy. However, an increasing number of hospitals is performing minimal invasive surgery (MIS). In this article, we describe the technique of thoracoscopic repair of EA in neonates in more detail and show the outcome of a patient cohort operated by young pediatric surgeons in training. Methods: Between 2014 and 2019 correction was performed in 64 EA type C patients at the UMC Utrecht, Wilhelmina Children's Hospital, mainly by young pediatric surgeons in training. Results: All patients were corrected through MIS, 3 days after birth. The median operation duration was 181 (127-334) minutes. Nasogastric tube feeding was started on the first postoperative day, and oral feeding 6 days postop. Postoperative complications included leakage (14.1%), stenosis (51.1%), and recurrent tracheoesophageal fistula (7.8%). Conclusion: Thoracoscopic repair of EA can be performed safely, with good outcome and all the benefits of MIS. However, it remains a challenging procedure and should be performed only in pediatric centers with a vast experience in MIS, especially when training young pediatric surgeons. These centers must have access to a multidisciplinary team of neonatologists, pediatric anesthesiologists, surgeons, and ENT specialists to ensure the best possible care in hemodynamic, respiratory, and cerebral monitoring and gastrointestinal and developmental outcome.

A rare congenital esophageal anomaly mimicking an isolated esophageal atresia: Kluth Type IV1a membranous esophageal atresia.

Congenital esophageal web is a very rare type of esophageal atresia (EA) and is considered as Type IV atresia in Kluth's classification. Type IV EA is further classified into subgroups according to the location of web and the presence of tracheoesophageal fistula (TEF). Herein, we present a newborn having Type IV1a EA who was initially suspected as isolated EA and has co-existing malrotation together with the review of the literature. A newborn having congenital esophageal web was admitted with antenatal ultrasonography revealing polyhydramnios, dilated stomach, and duodenum. Abdominal X-ray revealed a gasless abdomen suggesting isolated EA. The upper gastrointestinal series confirmed complete obstruction at the lower esophagus. Bronchoscopy performed excluding TEF. Laparotomy revealed intestinal malrotation and the absence of another GI webs. Gastrotomy was performed and mucosal web at GEJ was seen by pushing nasogastric catheter. Web excision and Ladd procedure were performed. Congenital esophageal web is an extremely rare type of esophageal anomaly that can be only classified in Kluth classification. A high index of suspicion is required for diagnosis especially in newborns with the gasless abdomen and esophageal obstruction at the level of GEJ. Preoperative bronchoscopy is mandatory to exclude an associating TEF. Surgical exploration via laparotomy is beneficial for exploring additional GIS anomalies.

Esophageal Atresia: Nutritional Status and Energy Metabolism to Maximize Growth Outcome.

Background: Long-term negative sequelae of esophageal atresia (EA) may induce poor growth and impaired nutritional status in childhood. We describe the nutritional profile and energy metabolism of children with repaired EA to identify malnutrition risk factors and optimize growth management. Methods: Twenty-one children (>4 years) were included, and anthropometric measurements, nutritional assessment, and energy metabolism were considered. The subjects were defined as undernourished if they met BMI < −2 standard deviation (SD). To grade undernutrition, we defined the prevalence of underweight, stunting, and wasting (cut-off level of <−2 SD). Medical records were reviewed for the type of EA and surgery and perinatal data. Results: Malnutrition was detected in 28.6% of children. Underweight was detected in 23.8% of patients (all with undernutrition p < 0.01). Wasting was noted in 28.6% of patients, of these 5 children were undernourished (p < 0.001) and stunting was noticed in only one patient with malnutrition (p = 0.5). Resting expenditure energy (REE) was lower in undernourished subjects compared to subjects with adequate nutritional status (p < 0.001). Malnutrition was associated to: type of EA (p = 0.003, particularly type A and C); intervention including deferred anastomosis due to long-gap repair (p = 0.04) with/or without jejunostomy (p = 0.02), gastric pull-up (p = 0.04), primary anastomosis (p = 0.04), pyloromyotomy in long-gap (p < 0.01); small for gestational age condition (p = 0.001). Conclusions: undernutrition risk factors, beyond the type of malformation, surgery, and perinatal factors, must be early considered to personalize nutritional programming. Energy metabolism is important to monitor the nutritional requirements. The management of nutritional issues is surely a contributory factor able to counteract the poor growth of children with EA.

Diagnosis of Fetal Esophageal Atresia by Prenatal MRI and Analysis of Prognostic Factors

Objective To investigate the value of prenatal ultrasound and MRI in the diagnosis of types I and III fetal esophageal atresia (EA), in addition to analyzing the factors related to prognosis. Methods The clinical data regarding six cases of type I and 39 cases of type III EA, with the diagnosis confirmed by postnatal autopsy, MRI, CT, or surgical operation from November 2012 to January 2018, were retrospectively analyzed. Moreover, the prenatal ultrasound and MRI features were summarized. According to the prognosis, 45 fetuses with EA were divided into study (poor prognosis) and control (better prognosis) groups. Logistic regression was used to analyze the prognostic factors. Results The imaging features of 45 EA cases were as follows: Ultrasound features of fetal EA: polyhydramnios (AFI ≥ 25), continuously absent stomach bubble (rechecked 48 hours later), and reduced or no fetal swallowing over 30 minutes; the measured values for fetal head circumference (HC), abdominal circumference (AC), and estimated fetal weight (EFW) were lower than expected according to the clinical gestational age. MRI features: appearance of line-like long T1 and long T2 signals from the fetal neck to the esophagus at the upper mediastinal spinal anterior area; esophageal disconnection, sagging, or the appearance of a visible blind-ended pouch, and disappearance of the stomach bubble. Three cases (6.67%, 3/45) were diagnosed accurately by prenatal ultrasound, while 45 cases (100%, 45/45) were diagnosed accurately by MRI after prenatal ultrasound suggested EA. The incidences of complex cardiovascular malformation, low birth weight ( &lt; 1200 g), EA type (type I), hypoproteinemia, and an esophageal deletion length &gt; 5 cm were significantly higher in the poor prognosis group than those in the better prognosis group ( P &lt; 0 05). Conclusions Due to interference factors such as fetal movement, excessive skin thickness, skeleton, and surrounding tissues and organs, only 6.67% of types I and III EA were directly diagnosed by prenatal ultrasound in the present study. The risk factors for poor prognosis of EA were complex cardiovascular malformation, low birth weight, EA type, hypoproteinemia, and esophageal deletion length &gt; 5 cm; when these ultrasound features were found, performing MRI to diagnose EA and predict prognosis was valuable.