Magnetic Resonance Imaging-Based Evaluation of Anatomy and Outcome Prediction in Infants with Esophageal Atresia.

INTRODUCTION: There is currently no validated diagnostic modality to characterize the anatomy and predict outcomes of tracheal esophageal defects, such as esophageal atresia (EA) and tracheal esophageal fistulas (TEFs). We hypothesized that ultra-short echo-time MRI would provide enhanced anatomic information allowing for evaluation of specific EA/TEF anatomy and identification of risk factors that predict outcome in infants with EA/TEF. METHODS: In this observational study, 11 infants had pre-repair ultra-short echo-time MRI of the chest completed. Esophageal size was measured at the widest point distal to the epiglottis and proximal to the carina. Angle of tracheal deviation was measured by identifying the initial point of deviation and the farthest lateral point proximal to the carina. RESULTS: Infants without a proximal TEF had a larger proximal esophageal diameter (13.5 ± 5.1 mm vs. 6.8 ± 2.1 mm, p = 0.07) when compared to infants with a proximal TEF. The angle of tracheal deviation in infants without a proximal TEF was larger than infants with a proximal TEF (16.1 ± 6.1° vs. 8.2 ± 5.4°, p = 0.09) and controls (16.1 ± 6.1° vs. 8.0 ± 3.1°, p = 0.005). An increase in the angle of tracheal deviation was positively correlated with duration of post-operative mechanical ventilation (Pearson r = 0.83, p < 0.002) and total duration of post-operative respiratory support (Pearson r = 0.80, p = 0.004). DISCUSSION: These results demonstrate that infants without a proximal TEF have a larger proximal esophagus and a greater angle of tracheal deviation which is directly correlated with the need for longer post-operative respiratory support. Additionally, these results demonstrate that MRI is a useful tool to assess the anatomy of EA/TEF.

Anatomy and embryology of tracheo-esophageal fistula.

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.

Quantifying Upper Aerodigestive Sequelae in Esophageal Atresia/Tracheoesophageal Fistula Neonates.

OBJECTIVES: We examined rates of upper aerodigestive tract (UADT) procedures in a multi-institutional cohort of neonates with esophageal atresia/tracheoesophageal fistula (EA/TEF) to estimate secondary UADT pathology. METHODS: A retrospective cohort study was performed using a previously-validated population of patients with EA/TEF within the Pediatric Health Information System (PHIS) between 2007 and 2015. ICD-9/10-CM codes for aerodigestive procedures were examined from 2007 to 2020: 1) diagnostic direct laryngoscopy and/or bronchoscopy (DLB), 2) DLB with intervention, 3) tracheostomy, 4) gastrostomy, 5) fundoplication, 6) aortopexy, 7) laryngotracheoplasty, and 8) esophageal dilation. Associations between procedures and demographics, length of gestation, and weight were estimated using generalized linear mixed models. RESULTS: We identified 2,509 patients with EA/TEF from 47 hospitals, 56.7% male and 43.3% female. Median length of stay for the first admission was 24 days (interquartile range: 12-55). Of these patients, 1,943 (77.4%) had at least one aerodigestive procedure within 14 admissions. Specifically, 1,635 (65.2%) underwent diagnostic DLB, 85 (3.4%) DLB with intervention, 167 (6.7%) tracheostomy, 1,043 (41.2%) gastrostomy, 211 (11.0%) fundoplication, 52 (2.1%) aortopexy, 161 (6.4%) laryngotracheoplasty, and 207 (8.3%) esophageal dilation. Preterm gestation increased odds of tracheostomy (adjusted odds ratio (OR) 2.4, 95% confidence interval (CI) 1.5-3.7), gastrostomy (OR 2.1, CI 1.7-2.7), fundoplication (OR 1.7, CI 1.1-2.4), aortopexy (OR 5.8, CI 2.1-16.1), and esophageal dilation (OR 2.0, CI 1.4-3.0). Very low birth weight (<1,500 g) increased odds of gastrostomy (OR 2.5, CI 1.6-3.8). CONCLUSION: Patients with EA/TEF frequently have aerodigestive sequelae. This work helps quantify aerodigestive needs in neonates with EA/TEF, suggesting early otolaryngology evaluation in their care. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:695-700, 2022.

Infant study of hemispheric asymmetry after long-gap esophageal atresia repair.

OBJECTIVES: Previous studies have demonstrated that infants are typically born with a left-greater-than-right forebrain asymmetry that reverses throughout the first year of life. We hypothesized that critically ill term-born and premature patients following surgical and critical care for long-gap esophageal atresia (LGEA) would exhibit alteration in expected forebrain asymmetry. METHODS: Term-born (n = 13) and premature (n = 13) patients, and term-born controls (n = 23) <1 year corrected age underwent non-sedated research MRI following completion of LGEA treatment via Foker process. Structural T1- and T2-weighted images were collected, and ITK-SNAP was used for forebrain tissue segmentation and volume acquisition. Data were presented as absolute (cm3 ) and normalized (% total forebrain) volumes of the hemispheres. All measures were checked for normality, and group status was assessed using a general linear model with age at scan as a covariate. RESULTS: Absolute volumes of both forebrain hemispheres were smaller in term-born and premature patients in comparison to controls (p < 0.001). Normalized hemispheric volume group differences were detected by T1-weighted analysis, with premature patients demonstrating right-greater-than-left hemisphere volumes in comparison to term-born patients and controls (p < 0.01). While normalized group differences were very subtle (a right hemispheric predominance of roughly 2% of forebrain volume), they represent a deviation from the expected pattern of hemispheric brain asymmetry. INTERPRETATION: Our pilot quantitative MRI study of hemispheric volumes suggests that premature patients might be at risk of altered expected left-greater-than-right forebrain asymmetry following repair of LGEA. Future neurobehavioral studies in infants born with LGEA are needed to elucidate the functional significance of presented anatomical findings.

Heritability and De Novo Mutations in Oesophageal Atresia and Tracheoesophageal Fistula Aetiology.

Tracheoesophageal Fistula (TOF) is a congenital anomaly for which the cause is unknown in the majority of patients. OA/TOF is a variable feature in many (often mono-) genetic syndromes. Research using animal models targeting genes involved in candidate pathways often result in tracheoesophageal phenotypes. However, there is limited overlap in the genes implicated by animal models and those found in OA/TOF-related syndromic anomalies. Knowledge on affected pathways in animal models is accumulating, but our understanding on these pathways in patients lags behind. If an affected pathway is associated with both animals and patients, the mechanisms linking the genetic mutation, affected cell types or cellular defect, and the phenotype are often not well understood. The locus heterogeneity and the uncertainty of the exact heritability of OA/TOF results in a relative low diagnostic yield. OA/TOF is a sporadic finding with a low familial recurrence rate. As parents are usually unaffected, de novo dominant mutations seems to be a plausible explanation. The survival rates of patients born with OA/TOF have increased substantially and these patients start families; thus, the detection and a proper interpretation of these dominant inherited pathogenic variants are of great importance for these patients and for our understanding of OA/TOF aetiology.

Exome survey of individuals affected by VATER/VACTERL with renal phenotypes identifies phenocopies and novel candidate genes.

The acronym VATER/VACTERL refers to the rare nonrandom association of the following component features (CFs): vertebral defects (V), anorectal malformations (ARM) (A), cardiac anomalies (C), tracheoesophageal fistula with or without esophageal atresia (TE), renal malformations (R), and limb anomalies (L). For the clinical diagnosis, the presence of at least three CFs is required, individuals presenting with only two CFs have been categorized as VATER/VACTERL-like. The majority of VATER/VACTERL individuals displays a renal phenotype. Hitherto, variants in FGF8, FOXF1, HOXD13, LPP, TRAP1, PTEN, and ZIC3 have been associated with the VATER/VACTERL association; however, large-scale re-sequencing could only confirm TRAP1 and ZIC3 as VATER/VACTERL disease genes, both associated with a renal phenotype. In this study, we performed exome sequencing in 21 individuals and their families with a renal VATER/VACTERL or VATER/VACTERL-like phenotype to identify potentially novel genetic causes. Exome analysis identified biallelic and X-chromosomal hemizygous potentially pathogenic variants in six individuals (29%) in B9D1, FREM1, ZNF157, SP8, ACOT9, and TTLL11, respectively. The online tool GeneMatcher revealed another individual with a variant in ZNF157. Our study suggests six biallelic and X-chromosomal hemizygous VATER/VACTERL disease genes implicating all six genes in the expression of human renal malformations.

Thoracoscopic repair of esophageal atresia with distal tracheoesophageal fistula: is it a safe procedure in infants weighing less than 2000 g?

BACKGROUND: Since Rothenberg first performed thoracoscopic repair for esophageal atresia with distal tracheoesophageal fistula (EA/TEF) successfully in 2000, thoracoscopic repair has achieved status as a routine procedure worldwide. Previously, an international multicenter study reported that this procedure was not inferior to conventional open surgery. However, thoracoscopic surgery is a highly difficult operation for surgeons and anesthesiologists; as a result, the safety and efficacy of the surgery is still under debate. Considering these circumstances, the purpose of this study was to analyze the results of single-center thoracoscopic surgery and to compare the outcomes relative to the patient's weight at the time of surgery. METHODS: We retrospectively analyzed patients with EA/TEF who underwent thoracoscopic surgery in a single center between October 2008 and February 2017. RESULTS: In total, 41 cases of thoracoscopic repair of EA/TEF were performed. Upon subgrouping by over and under 2000 g of body weight at the time of operation, 34 were found to be over 2000 g and seven were under 2000 g. Intraoperative factors and events were not significantly different between the two groups. Additionally, most of the postoperative outcomes, including the rate of postoperative leakage and strictures, showed no difference. On the other hand, the under 2000 g group had more gastroesophageal reflux requiring fundoplication than did the heavier group (P = 0.04). CONCLUSIONS: The results of this center's thoracoscopic repair of EA/TEF were not inferior to other centers' outcomes. Additionally, the intraoperative and postoperative outcomes were similar despite differences in weight at operation. Therefore, thoracoscopic repair might be a feasible surgical option for infants weighing less than 2000 g when performed by a surgeon and anesthesiologist team who are experienced in pediatric thoracoscopic surgery.

ERNICA Consensus Conference on the Management of Patients with Long-Gap Esophageal Atresia: Perioperative, Surgical, and Long-Term Management.

INTRODUCTION: Evidence supporting best practice for long-gap esophageal atresia is limited. The European Reference Network for Rare Inherited Congenital Anomalies (ERNICA) organized a consensus conference on the management of patients with long-gap esophageal atresia based on expert opinion referring to the latest literature aiming to provide clear and uniform statements in this respect. MATERIALS AND METHODS: Twenty-four ERNICA representatives from nine European countries participated. The conference was prepared by item generation, item prioritization by online survey, formulation of a final list containing items on perioperative, surgical, and long-term management, and literature review. The 2-day conference was held in Berlin in November 2019. Anonymous voting was conducted via an internet-based system using a 1 to 9 scale. Consensus was defined as ≥75% of those voting scoring 6 to 9. RESULTS: Ninety-seven items were generated. Complete consensus (100%) was achieved on 56 items (58%), e.g., avoidance of a cervical esophagostomy, promotion of sham feeding, details of delayed anastomosis, thoracoscopic pouch mobilization and placement of traction sutures as novel technique, replacement techniques, and follow-up. Consensus ≥75% was achieved on 90 items (93%), e.g., definition of long gap, routine pyloroplasty in gastric transposition, and avoidance of preoperative bougienage to enable delayed anastomosis. Nineteen items (20%), e.g., methods of gap measurement were discussed controversially (range 1-9). CONCLUSION: This is the first consensus conference on the perioperative, surgical, and long-term management of patients with long-gap esophageal atresia. Substantial statements regarding esophageal reconstruction or replacement and follow-up were formulated which may contribute to improve patient care.

Whole genome sequencing of familial isolated oesophagus atresia uncover shared structural variants.

BACKGROUND: Oesophageal atresia (OA) is a life-threatening developmental defect characterized by a lost continuity between the upper and lower oesophagus. The most common form is a distal connection between the trachea and the oesophagus, i.e. a tracheoesophageal fistula (TEF). The condition may be part of a syndrome or occurs as an isolated feature. The recurrence risk in affected families is increased compared to the population-based incidence suggesting contributing genetic factors. METHODS: To gain insight into gene variants and genes associated with isolated OA we conducted whole genome sequencing on samples from three families with recurrent cases affected by congenital and isolated TEF. RESULTS: We identified a combination of single nucleotide variants (SNVs), splice site variants (SSV) and structural variants (SV) annotated to altogether 100 coding genes in the six affected individuals. CONCLUSION: This study highlights rare SVs among candidate gene variants in our individuals with OA and provides a gene framework for further investigations of genetic factors behind this malformation.

Noggin regulates foregut progenitor cell programming, and misexpression leads to esophageal atresia.

Esophageal atresia (EA/TEF) is a common congenital abnormality present in 1 of 4000 births. Here we show that atretic esophagi lack Noggin (NOG) expression, resulting in immature esophagus that contains respiratory glands. Moreover, when using mouse esophageal organoid units (EOUs) or tracheal organoid units (TOUs) as a model of foregut development and differentiation in vitro, NOG determines whether foregut progenitors differentiate toward esophageal or tracheal epithelium. These results indicate that NOG is a critical regulator of cell fate decisions between esophageal and pulmonary morphogenesis, and its lack of expression results in EA/TEF.

Clinical Analysis of Azygos Vein Preservation Under Thoracoscope in the Operation of Type III Esophageal Atresia.

Purpose: The aim of this study was to investigate the clinical effectiveness of azygos vein preservation when using a thoracoscope in the operation for type III esophageal atresia for children. Materials and Methods: We retrospectively analyzed the clinical data of 34 patients (group B) who underwent the thoracoscopic operation of type III esophageal atresia with azygos vein preservation from January 2017 to April 2018. The clinical data of 36 patients (group A) who underwent the thoracoscopic operation of type III esophageal atresia with azygos vein mutilation from April 2015 to December 2016 were selected as a control group. Results: All patients were successfully operated on. There was no significant difference in the operation time, amount of bleeding, mechanical ventilation time, and intensive care time between the azygos vein mutilation group and azygos vein preservation group. Compared with the operation severing the azygos vein, preservation of azygos vein has obvious advantages in postoperative hospitalization time, the incidence of anastomotic leakage (P < .05). The follow-up results for 1 year after the operation showed that the incidence of anastomotic stricture with preservation of azygos vein was significantly lower than that with azygos vein transection (P < .05). Conclusions: The thoracoscopic operation of type III esophageal atresia with azygos vein preservation can not only improve the edema of esophageal tissue around the esophagus, but also reduce the occurrence of esophageal anastomotic leakage and accelerate the postoperative recovery of children, and it has the same safety as the operation with azygos vein mutilation.

Pathogenic copy number variants are detected in a subset of patients with gastrointestinal malformations.

BACKGROUND: Gastrointestinal atresias and urological defects are main causes of pediatric surgery in infants. As copy number variants (CNVs) have been shown to be involved in the development of congenital malformations, the aim of our study was to investigate the presence of CNVs in patients with gastrointestinal and urological malformations as well as the possibility of tissue-specific mosaicism for CNVs in the cohort. METHODS: We have collected tissue and/or blood samples from 25 patients with anorectal malformations, esophageal atresia, or hydronephrosis, and screened for pathogenic CNVs using array comparative genomic hybridization (array-CGH). RESULTS: We detected pathogenic aberrations in 2/25 patients (8%) and report a novel possible susceptibility region for esophageal atresia on 15q26.3. CNV analysis in different tissues from the same patients did not reveal evidence of tissue-specific mosaicism. CONCLUSION: Our study shows that it is important to perform clinical genetic investigations, including CNV analysis, in patients with congenital gastrointestinal malformations since this leads to improved information to families as well as an increased understanding of the pathogenesis.

An Unusual Lung Mass of Heterotopic Pancreatic Tissue in a Neonate With an Elevated Immunoreactive Trypsinogen on Newborn Screen.

We present a case of a neonate with tracheoesophageal fistula and esophageal atresia along with a suspicious lung mass who had a false-positive newborn screen for cystic fibrosis due to an elevated serum immunoreactive trypsinogen with an additionally elevated serum lipase. The infant's lung mass was found to contain heterotopic pancreatic tissue consisting of acini, ducts, and islet cells, without an associated gastrointestinal duplication cyst. This constellation of congenital abnormalities has not been described in previous literature. Also, this is the first reported case of a neonate with elevated serum pancreatic enzymes in which the underlying etiology was discovered to be heterotopic pancreas.

Congenital esophageal stenosis: a rare malformation of the foregut.

Congenital esophageal stenosis (CES) is a type of esophageal stenosis, and three histological subtypes (tracheobronchial remnants, fibromuscular thickening or fibromuscular stenosis, and membranous webbing or esophageal membrane) are described. Symptoms of CES usually appears with the introduction of the semisolid alimentation. Dysphagia is the most common symptom, but esophageal food impaction, respiratory distress or failure to thrive can be clinical manifestations of CES. Wide spectrum of differential diagnoses leads to delayed definitive diagnosis and appropriate treatment. Depends on hystological subtype of CES, some treatment procedures (dilation or segmental esophageal resection) are recommended, but individually approach is still important in terms of frequency and type of dilation procedures or type of the surgical treatment. Dysphagia can persist after the treatment and a long follow-up period is recommended. In 33% of patients with CES, a different malformations in the digestive system, but also in the other systems, are described.

Gastrointestinal disorders in Down syndrome.

Down syndrome is the most common human chromosomal disorder. Among clinical findings, one constant concern is the high prevalence of gastrointestinal system alterations. The aim of this study was to determine the prevalence of gastrointestinal disorders at a Down syndrome outpatient clinic during a 10-year follow-up period. Data from medical files were retrospectively reviewed from 1,207 patients. Gastrointestinal changes occurred in 612 (50.7%). The most prevalent disorder was chronic intestinal constipation. Intestinal parasite occurred in 22% (mainly giardiasis), gastroesophageal reflux disease in 14%, digestive tract malformations occurred in 5%: 13 cases of duodenal atresia, 8 of imperforate anus, 4 annular pancreases, 2 congenital megacolon, 2 esophageal atresias, 2 esophageal compression by anomalous subclavian and 1 case of duodenal membrane. We had 38/1,207 (3.1%) patients with difficulty in sucking and only three with dysphagia that resolved before the second year of life. Peptic ulcer disease, celiac disease, and biliary lithiasis were less prevalent with 3% each. Awareness of the high prevalence of gastrointestinal disorders promotes outstanding clinical follow-up as well as adequate development and greater quality of life for patients with Down syndrome and their families.

Sonographic demonstration of fetal esophagus using three-dimensional ultrasound imaging.

OBJECTIVE: To evaluate the feasibility of antenatal direct visualization of normal and abnormal fetal esophagus using three-dimensional ultrasound (3D-US) with Crystal Vue rendering technology. METHODS: Between February and April 2018, 3D-US volumes were collected from a non-consecutive series of singleton pregnancies, referred for clinically indicated detailed prenatal ultrasound at 19-28 weeks' gestation to one of two fetal medicine units in Italy. 3D volumes were acquired from a midsagittal section of the fetal thorax and upper abdomen with the fetus lying in supine position. Postprocessing with multiplanar mode was applied to orientate the volume and identify the esophagus. The region of interest was angled by approximately 30° to the spine and its thickness was adjusted in order to optimize visualization of the intrathoracic and intra-abdominal course of the esophagus. Crystal Vue software was used for image rendering of the fetal trunk in the coronal plane. Postnatal follow-up was available in all cases. RESULTS: During the study period, 91 pregnancies met the inclusion criteria and were recruited. The study cohort included two pregnancies with suspicion of esophageal atresia due to suboptimal visualization of the stomach. Of the 89 cases with normal stomach on two-dimensional (2D) imaging, 3D-US with Crystal Vue rendering technology allowed direct evaluation of the whole course of the esophagus in 74 (83.1%). In the two cases with small or absent stomach bubble on 2D imaging, esophageal atresia was demonstrated antenatally on 3D Crystal Vue imaging and was confirmed postnatally. The mean time required for offline postprocessing and visualization of the esophageal anatomy was 4 min. CONCLUSIONS: Using 3D-US with Crystal Vue rendering, it is possible to visualize antenatally the normal fetal esophagus and demonstrate presence of esophageal atresia. This should facilitate prenatal counseling and management of cases with suspected esophageal atresia. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

A new esophageal elongation technique for long-gap esophageal atresia: in vitro comparison of myotomy techniques.

BACKGROUND: Complications such as stricture, leakage, recurrent tracheoesophageal fistula and mucosal pouch are commonly seen in myotomy techniques used for long-gap esophageal atresia (LGEA) treatments. Therefore, we think that there is a clear need for other techniques which would enable us to create more robust and longer esophagus in such cases. In this study, we reviewed multiple V-myotomy (VM) technique and the differences of the said technique with Livaditis circular myotomy (LM) and Kimura spiral myotomy (KM) techniques using literature as an aid. METHODS: 21 esophagus samples from 21 male lambs aged 12 months were used in vitro for the study. All esophageal samples were matched to have a length of 120 mm. Samples were divided into 3 groups of 7 and VM, LM and KM techniques were used in each group, respectively. Post-op esophagus lengths, elongation amount with each incision and perforation pressures were measured. RESULTS: Post-op esophageal lengths were measured as 227, 210 and 200 mm for VM, LM and KM, respectively. Elongation amount per incision was measured as 5.1, 4 and 3.34 mm, again in previous order of VM, LM, and KM. Finally, perforation pressure following VM, LM, and KM was measured as 460, 400, and 410 mmHg. CONCLUSION: VM was found to significantly increase total esophagus length and elongation per incision over LM and KM. In addition, VM was also shown to have a higher perforation pressure. Although in vivo live animal studies are required, we can say that VM can be used to create longer and robust esophagus.

Closer location of the tracheoesophageal fistula to the carina in newborns with esophageal atresia and VACTERL association.

BACKGROUND: VACTERL is a complicated syndrome with an unknown etiology where many studies have failed to identify the cause. In esophageal atresia (EA) roughly 10%-23% also have concurrent anomalies that align with VACTERL disorder. The aim of this study is to investigate if there is a difference regarding localization of the tracheoesophageal fistula between patients with VACTERL and non-VACTERL patients. METHODS: Retrospective chart review of newborn operated for esophageal atresia between 2006 and 2016 at our Institution was performed. Children with a C-type fistula according to Gross and reliable preoperative tracheoesophageal fistula to carina distance measurement at rigid tracheoscopy were included in the study. RESULTS: A total of 90 patients were included in the study. Fifteen of those were diagnosed with VACTERL. Before and after adjusting for weight and gestational week patients with VACTERL had significantly shorter carina to fistula distance at perioperative rigid tracheoscopy (p = <0.001 nonadjusted, p = 0.016 adjusted). CONCLUSION: Patients with VACTERL born with EA type C had shorter carina to fistula distance as shown at perioperative rigid tracheoscopy. The significantly shorter distance may not only present surgical difficulties but may also suggest a structural or molecular difference in the development of the esophageal atresia between the two groups. LEVEL OF EVIDENCE: III.

Right or left thoracotomy for esophageal atresia and right aortic arch? Systematic review and surgicoanatomic justification.

INTRODUCTION: The optimal thoracotomy approach for the management of esophageal atresia and tracheoesophageal fistula (EA/TEF) with a right aortic arch (RAA) remains controversial. METHODS: Systematic review of complications and death rates between right- and left-sided repairs, including all studies on EA/TEF and RAA, apart from studies focusing on long-gap EA and thoracoscopic repairs. Review of right- and left-sided surgical anatomy in relation to reported complications. RESULTS: Although no significant differences were elicited between right- and left-sided repairs in complications (9/29 vs. 1/6, p = 0.64) and death rates (2/29 vs. 0/6, p = 0.57), unique anatomic complications - such as injury to the RAA covering the esophagus and intractable bleeding - associated with mortality were revealed in the right thoracotomy group. Left-sided repairs following failed repair through the right showed higher complications rate (3/3) than straightforward right- (9/29) or left-sided repairs (1/6) (p = 0.024). Right thoracotomies converted to left thoracotomies led to staged repairs more frequently (4/9) than straightforward right (5/38) or left thoracotomies (0/6) (p = 0.03). CONCLUSIONS: There is not enough evidence to support that right thoracotomy, characterized by unique surgicoanatomic difficulties, is equivalent to left thoracotomy for EA/TEF with RAA. Both approaches might be required, and, therefore, surgeons should be familiarized with surgical anatomy of mediastinum approached from right and left. Systematic review, Level of Evidence III.

Early Postnatal Ventricular Dysfunction Is Associated with Disease Severity in Patients with Congenital Diaphragmatic Hernia.

OBJECTIVE: To assess patterns of postnatal ventricular function and their relationship to prenatal and postnatal markers of disease severity in infants with congenital diaphragmatic hernia (CDH). STUDY DESIGN: In this observational case-control study of cardiac function in infants with CDH in the first 5 days of life, systolic and diastolic function in the right ventricle (RV) and left ventricle (LV) were assessed using speckle tracking echocardiography-derived global strain and tissue Doppler imaging. Correlation between cardiac function and prenatal observed:expected total fetal lung volume (TFLV), oxygenation index (OI), duration of intubation, and hospital length of stay were assessed. RESULTS: All measures of systolic and diastolic function were significantly reduced in the CDH group (n = 25) compared with controls (n = 20) at <48 hours, and were improved by 72-120 hours. LV global systolic longitudinal strain (GLS) correlated with prenatal TFLV (R2 = 0.32; P = .03), OI (R2 = 0.35; P < .001), duration of intubation (R2 = 0.24; P = .04), and length of stay (R2 = 0.4; P = .006). Mean (SD) LV GLS at <48 hours was significantly lower in infants with CDH who did not survive and/or required ECMO compared with those who did not: -11.5 (5.3)% vs -16.9 (5.3)% (P = .02). CONCLUSIONS: RV and LV function are impaired in the transitional period in infants with CDH. Early LV systolic function correlates with prenatal and postnatal markers of clinical disease severity and may be an important determinant of disease severity and therapeutic target in CDH. These findings support regular assessment of cardiac function in CDH and investigational trials of targeted cardiovascular therapies.