CHRND variant in a paternally inherited esophageal atresia and tracheoesophgageal fistula: Report of a case.

BACKGROUND: The familial occurrence of esophageal atresia and tracheoesophageal fistula (EA-TEF) is very rare and the genetic basis behind the isolated familial cases have not been identified. A male infant born with EA-TEF and his affected father were evaluated with whole genome sequence to define a genetic causative variation in paternally inherited EA-TEF. CASE REPORT: A male infant was born to 29-years-old, gravida 1, para 1 women by normal vaginal delivery. The patient was diagnosed as Type-C EA-TEF. In his family history, his father was also operated for EA-TEF during neonatal period. He had no associated anomaly despite patent foramen ovale. Genomic DNAs were extracted from peripheral blood of the patient and the father. When causative genes responsible for EA-TEF were filtered out, four different variants in NOTCH2, SAMD9, SUPT20H and CHRND were found. Except the variant found in CHRND (NM_000751.2, c.381C>G, p.(Tyr127Ter)), other three variants were not found to be segregated with the father who has EA-TEF also. This nonsense variant was not found in GnomAD database. CONCLUSION: CHRND variant found in both EA-TEF patient and his affected father suggest that CHRND variant might possibly be considered as one of the causative genetic variants in familial isolated EA-TEF patients.

The French Experience with a Population-Based Esophageal Atresia Registry (RENATO).

This paper presented a national register for esophageal atresia (EA) started in January 2008. We report our experience about the conception of this database and its coordination. Data management and data quality are also detailed. In 2023, more than 2,500 patients with EA are included. Prevalence of EA in France was calculated at 1.8/10,000 live birth. Main clinical results are listed with scientific publications issued directly from the register.

Perioperative anaesthetic management and short-term outcome of neonatal repair of oesophageal atresia with or without tracheo-oesophageal fistula in Europe: A sub-analysis of the neonate and children audit of anaesthesia practice in Europe (NECTARINE) prospective multicenter observational study.

BACKGROUND: Oesophageal atresia with or without a tracheo-oesophageal fistula is a congenital abnormality that usually requires surgical repair within the first days of life. OBJECTIVE: Description of the perioperative anaesthetic management and outcomes of neonates undergoing surgery for oesophageal atresia with or without a tracheo-oesophageal fistula, included in the 'neonate and children audit of anaesthesia practice in Europe' (NECTARINE) database. DESIGN: Sub-analyses of prospective observational NECTARINE study. SETTING: European multicentre study. PATIENTS: Neonates who underwent surgery for oesophageal atresia with or without a tracheo-oesophageal fistula in the NECTARINE cohort were selected. MAIN OUTCOME MEASURES: Incidence rates with 95% confidence intervals were calculated for peri-operative clinical events which required a predetermined intervention, postoperative complications, and mortality. RESULTS: One hundred and three neonates undergoing a first surgical intervention for oesophageal atresia with or without a tracheo-oesophageal fistula repair were identified. Their median gestational age was 38 weeks with a median birth weight of 2840 [interquartile range 2150 to 3150] grams. Invasive monitoring was used in 66% of the procedures. The incidence of perioperative clinical events was 69% (95% confidence interval 59 to 77%), of 30-day postoperative complications 47% (95% confidence interval 38 to 57%) and the 30- and 90 days mortality rates were 2.1% and 2.6%, respectively. CONCLUSION: Oesophageal atresia with or without a tracheo-oesophageal fistula repair in neonates is associated with a high number of perioperative interventions in response to clinical events, a high incidence of postoperative complications, and a substantial mortality rate.

Morbidity and mortality among neonates with esophageal atresia and/or tracheoesophageal fistula in the United States.

BACKGROUND: Although rare, esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) can lead to severe morbidity and mortality. A known complication of EA and/or TEF repair is vocal fold motion impairment (VFMI). OBJECTIVE: To characterize the prevalence of VFMI among repaired EA and/or TEF at a national level and to identify factors associated with in-patient mortality and common clinical endpoints in this population (tracheostomy and gastrostomy tube dependence). METHODS: This is a retrospective cohort study of inpatient neonate admissions with EA and/or TEF in the 2016 Kids' Inpatient Database (KID). Patients with a history of EA and/or TEF were identified with ICD-10 codes. VFMI prevalence was assessed by observed frequencies and percentages. Clinical endpoints included gastrostomy (G-)tube placement, tracheostomy status, and inpatient mortality. Potential predictors of these three primary outcomes included cardiac anomalies, airway disorders, chromosomal anomalies, surgery within 24 h of admission, prematurity, and low birthweight (LBW). Chi-squared analysis was performed between each parameter and the primary outcomes. Significant associations (p-value<0.05) were further assessed with multivariate logistic regression. RESULTS: Of 1062 neonates with history of EA and/or TEF, 24 (2.3%) had VFMI. Among this population, 27 patients underwent tracheostomies and 63 required G-tubes. There was a significantly higher likelihood of tracheostomy in patients with VFMI compared to patients without (16.7% versus 2.2%; p < 0.01). After multivariate analysis, birthweight less than 1.5 kg (BW < 1.5 kg; OR = 2.5, 95% CI: 1.03-6.10), extreme prematurity (OR = 7.5, 95% CI: 2.00-28.07), cardiac anomalies (OR = 1.8, 95% CI: 1.01-3.04) and chromosomal anomalies (OR = 4.2, 95% CI: 2.39-7.44) were significantly associated with in-hospital mortality. Bronchopulmonary dysplasia (BPD; OR = 4.7, 95% CI: 1.73-12.78) and BW < 1.5 kg (OR = 2.4, 95% CI: 1.05-5.49) were significant predictors for G-tube status. For tracheostomy, BPD (OR = 15.8, 95% CI: 4.70-52.83) and subglottic stenosis (SS, OR = 11.2, 95% CI: 1.85-68.02) remained significant predictors after multivariate regression. CONCLUSION: The national prevalence of VFMI among neonates following EA and/or TEF repair was 2.3% and was associated with an increased likelihood of tracheostomy in the KID 2016. Significant predictors of in-hospital mortality were cardiac anomalies, chromosomal anomalies, extreme prematurity, and BW < 1.5 kg. BPD and SS were significant predictors of tracheostomy, and BPD and BW < 1.5 kg were significant predictors for G-tube requirements.

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

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

Association between birth location and short-term outcomes for babies with gastroschisis, congenital diaphragmatic hernia and oesophageal fistula: a systematic review.

BACKGROUND: Neonatal care is commonly regionalised, meaning specialist services are only available at certain units. Consequently, infants with surgical conditions needing specialist care who are born in non-surgical centres require postnatal transfer. Best practice models advocate for colocated maternity and surgical services as the place of birth for infants with antenatally diagnosed congenital conditions to avoid postnatal transfers. We conducted a systematic review to explore the association between location of birth and short-term outcomes of babies with gastroschisis, congenital diaphragmatic hernia (CDH) and oesophageal atresia with or without tracheo-oesophageal fistula (TOF/OA). METHODS: We searched MEDLINE, CINAHL, Web of Science and SCOPUS databases for studies from high income countries comparing outcomes for infants with gastroschisis, CDH or TOF/OA based on their place of delivery. Outcomes of interest included mortality, length of stay, age at first feed, comorbidities and duration of parenteral nutrition. We assessed study quality using the Newcastle-Ottawa Scale. We present a narrative synthesis of our findings. RESULTS: Nineteen cohort studies compared outcomes of babies with one of gastroschisis, CDH or TOF/OA. Heterogeneity across the studies precluded meta-analysis. Eight studies carried out case-mix adjustments. Overall, we found conflicting evidence. There is limited evidence to suggest that birth in a maternity unit with a colocated surgical centre was associated with a reduction in mortality for CDH and decreased length of stay for gastroschisis. CONCLUSIONS: There is little evidence to suggest that delivery in colocated maternity-surgical services may be associated with shortened length of stay and reduced mortality. Our findings are limited by significant heterogeneity, potential for bias and paucity of strong evidence. This supports the need for further research to investigate the impact of birth location on outcomes for babies with congenital surgical conditions and inform future design of neonatal care systems. PROSPERO REGISTRATION NUMBER: CRD42022329090.

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.

Surgical outcome and predictors of neonates with esophageal atresia admitted at Tikur Anbesa Specialized Hospital.

BACKGROUND: Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus. This anomaly continues to cause considerable morbidity and mortality in Sub-Saharan Africa, presenting various concerns about how to treat esophageal atresia. Esophageal atresia-related neonatal mortality can be reduced by evaluating the surgical outcome and identifying associated factors. OBJECTIVE: This study aimed to assess the surgical outcome and identify predictors of neonates with esophageal atresia admitted at Tikur Anbesa specialized hospital. METHODS: Retrospective crossectional study design was employed on 212 neonates with esophageal atresia who were undergone surgical intervention in Tikur Anbesa specialized hospital. Data were entered into epi data 4.6 and exported to Stata version 16 software for further analysis. A logistic regression model with Adjusted odds ratio (AOR), confidence interval (CI) and p-value <0.05 were used to identify predictors of poor surgical outcome of neonates with esophageal atresia. RESULT: In this study, 25% of newborns who underwent surgical intervention at TikurAbnbesa specialized hospital had successful surgical outcomes, compared to 75% of neonates with esophageal atresia who had poor surgical outcomes. Significant predictors of the poor surgical outcome of neonates with esophageal atresia were severe thrombocytopenia (AOR = 2.81(1.07-7.34)), timing of surgery (AOR = 3.7(1.34-10.1), aspiration pneumonia (AOR = 2.93(1.17-7.38)) and related abnormalities (AOR = 2.26(1.06-4.82)). CONCLUSION: The results of this study showed that, when compared to other studies, a substantial percentage of newborn children with esophageal atresia had poor surgical outcomes. Early surgical management, aspiration pneumonia and thrombocytopenia prevention and therapy play a big part in improving the surgical prognosis for newborns with esophageal atresia.

Surgical management of acute life-threatening events affecting esophageal atresia and/or tracheoesophageal fistula patients.

BACKGROUND: Following surgical correction, many patients with esophageal atresia with or without tracheoesophageal fistula (EA/TEF) present to the emergency department (ED) with acute airway complications. We sought to determine the incidence and risk factors for severe acute life-threatening events (ALTEs) in pediatric patients with repaired congenital EA/TEF and the outcomes of operative interventions. METHODS: A retrospective cohort chart review was performed on patients with EA/TEF with surgical repair and follow-up at a single centre from 2000 to 2018. Primary outcomes included 5-year ED visits and/or hospitalizations for ALTEs. Demographic, operative, and outcome data were collected. Chi-square tests and univariate analyses were performed. RESULTS: In total, 266 EA/TEF patients met inclusion criteria. Of these, 59 (22.2%) had experienced ALTEs. Patients with low birth weight, low gestational age, documented tracheomalacia, and clinically significant esophageal strictures were more likely to experience ALTEs (p < 0.05). ALTEs occurred prior to 1 year of age in 76.3% (45/59) of patients with a median age at presentation of 8 months (range 0-51 months). Recurrence of ALTEs after esophageal dilatation was 45.5% (10/22), mostly due to stricture recurrence. Patients experiencing ALTEs received anti-reflux procedures (8/59, 13.6%), airway pexy procedures (7/59, 11.9%), or both (5/59, 8.5%) within a median age of 6 months of life. The resolution and recurrence of ALTEs after operative interventions are described. CONCLUSION: Significant respiratory morbidity is common among patients with EA/TEF. Understanding the multifactorial etiology and operative management of ALTEs have an important role in their resolution. TYPE OF STUDY: Original Research, Clinical Research. LEVEL OF EVIDENCE: Level III Retrospective Comparative Study.

Feeding and Swallowing Characteristics of Children With Esophageal Atresia and Tracheoesophageal Fistula.

OBJECTIVES: This study aimed to characterize feeding/swallowing difficulties in children with esophageal atresia and/or tracheoesophageal fistula (EA/TEF) and evaluate associations among feeding difficulties, pharyngeal dysphagia (PD), and other aerodigestive evaluation findings. METHODS: This was a retrospective cohort study of feeding/swallowing characteristics of 44 patients with EA/TEF treated in the aerodigestive program of a single academic medical institution from 2010 to 2015. Demographics, comorbidities, presence and characteristics of feeding/swallowing difficulties, and results of relevant diagnostic tests [videofluoroscopic swallow studies (VFSS), clinical feeding evaluations (CFEs), chest computerized tomography (CT) scans, pulmonary bronchoscopies, and upper GI (UGI)/esophagrams] were reviewed. RESULTS: Fifty percent of the cohort had PD and 88.6% had feeding difficulties. Across 118 encounters (87 VFSS and 31 CFEs), feeding difficulties suggestive of esophageal dysphagia were most frequently seen in children over 48 months and feeding difficulties suggestive of developmental feeding problems were most frequently seen in children from 24 to 48 months. Abnormal findings were present in 59.8% of VFSS, with aspiration (34.5%) and pharyngeal residue (26.4%) the most frequently observed signs of dysphagia. Abnormal UGI/esophagram findings were not associated with significantly increased risk of feeding difficulties during visits within 3 months (risk ratio, RR = 1.33). Presence of dysphagia was associated with increased risk for some abnormal CT findings (RR= 3.0 for airspace and 3.0 for bronchiectasis). CONCLUSIONS: Feeding/swallowing difficulties are common in EA/TEF, and types of feeding difficulties vary by patient age. The presence of abnormal findings on UGI/esophagram did not increase the risk of feeding complaints; however, the presence of dysphagia increased the risk of abnormal chest CT.

Thoracoscopy versus thoracotomy for esophageal atresia and tracheoesophageal fistula: Outcomes from the Midwest Pediatric Surgery Consortium.

BACKGROUND/PURPOSE: Controversy persists regarding the ideal surgical approach for repair of esophageal atresia with tracheoesophageal fistula (EA/TEF). We examined complications and outcomes of infants undergoing thoracoscopy and thoracotomy for repair of Type C EA/TEF using propensity score-based overlap weights to minimize the effects of selection bias. METHODS: Secondary analysis of two databases from multicenter retrospective and prospective studies examining outcomes of infants with proximal EA and distal TEF who underwent repair at 11 institutions was performed based on surgical approach. Regression analysis using propensity score-based overlap weights was utilized to evaluate outcomes of patients undergoing thoracotomy or thoracoscopy for Type C EA/TEF repair. RESULTS: Of 504 patients included, 448 (89%) underwent thoracotomy and 56 (11%) thoracoscopy. Patients undergoing thoracoscopy were more likely to be full term (37.9 vs. 36.3 weeks estimated gestational age, p < 0.001), have a higher weight at operative repair (2.9 vs. 2.6 kg, p < 0.001), and less likely to have congenital heart disease (16% vs. 39%, p < 0.001). Postoperative stricture rate did not differ by approach, 29 (52%) thoracoscopy and 198 (44%) thoracotomy (p = 0.42). Similarly, there was no significant difference in time from surgery to stricture formation (p > 0.26). Regression analysis using propensity score-based overlap weighting found no significant difference in the odds of vocal cord paresis or paralysis (OR 1.087 p = 0.885), odds of anastomotic leak (OR 1.683 p = 0.123), the hazard of time to anastomotic stricture (HR 1.204 p = 0.378), or the number of dilations (IRR 1.182 p = 0.519) between thoracoscopy and thoracotomy. CONCLUSION: Infants undergoing thoracoscopic repair of Type C EA/TEF are more commonly full term, with higher weight at repair, and without congenital heart disease as compared to infants repaired via thoracotomy. Utilizing propensity score-based overlap weighting to minimize the effects of selection bias, we found no significant difference in complications based on surgical approach. However, our study may be underpowered to detect such outcome differences owing to the small number of infants undergoing thoracoscopic repair. LEVEL OF EVIDENCE: Level III.

Distal Recurrent Tracheoesophageal Fistula after Repair of Esophageal Atresia-Incidence, Risk Factors, and Outcome.

OBJECTIVE: The aim of this study was to assess incidence, risk factors, and outcomes of distal recurrent tracheoesophageal fistula (RTEF) after repair of esophageal atresia (EA). METHODS: Ethical consent was obtained. Data collection was done by review of hospital records of 286 patients (268 in-house and 18 referred) who underwent repair of type C or D EA from 1980 to 2021. Spitz class, long-gap (tracheoesophageal fistula at carina), fundoplication, leakage, and stricture were assessed as RTEF risk factors. Outcome measures were long-term closure of RTEF, retainment of native esophagus, and survival. RESULTS: RTEF occurred in 23 patients (19 in-house) with type C (n = 22) or type D (n = 1) EA with median 4.4 (interquartile range [IQR]: 1.7-13) months after repair. Five patients had late RTEF 3.5 to 16 years after repair. Nineteen (7.3%) in-house patients developed RTEF. Presenting symptoms, age at diagnosis, and presence of anastomotic stricture (AS) are listed. No statistically significant risk factors were found, risk ratio of 0.5 to 2.7 (IQR: 0.1-8.7), p-value of 0.25 to 0.75. Detached closure clip and esophageal foreign body contributed to RTEF in two patients. Sixteen patients underwent rethoracotomy for closure of RTEF, 5 (22%) with AS eventually underwent esophageal reconstruction after a period with cervical esophagostomy. Two patients with late-manifested RTEFs underwent closure with laser cauterization. In 22 patients, treatment of RTEF succeeded, whereas 1 (4%) premature patient died of instant re-RTEF. CONCLUSION: RTEF had an incidence of 7% with diverse patterns of manifestation and predicting factors. Closure rate and patient survival were excellent, but RTEF with AS predicted loss of native esophagus.

Does the History of Tracheoesophageal Fistula Repair Alter Outcomes of Laryngeal Cleft Repair?

OBJECTIVE: Tracheoesophageal fistula and esophageal atresia (TEA) and laryngeal cleft (LC) can coexist in some patients. The surgery-specific success rate of LC repair in children with associated TEA has not been well described. The aim of the study is to determine if the history of TEA alters the LC repair outcomes. STUDY DESIGN: Case series with chart review. SETTING: Single-institution academic medical center. METHOD: A retrospective review was conducted of patients with LC with and without TEA repair between January 2001 and November 2020. Data collected and analyzed included demographics and clinical characteristics, LC type, and LC with TEA timing of repairs. RESULTS: An overall 282 patients met the inclusion criteria of LC repair: LC (n = 242, 85.8%) and LC + TEA (n = 40, 14.2%). Revision repair was required in 43 patients (15.2%) with 8 (2.8%) needing a second revision repair. The first LC revision rate in the LC group was 36/242 (14.9%) as compared with 7/40 (17.5%) in the LC + TEA group (P = .67). The second LC revision rate in the LC and LC + TEA groups was 7 (2.9%) and 1 (2.4%), respectively. The median time to revision was 5.1 months (interquartile range, 3.45-10.6) in the LC group as compared with 29.2 months (interquartile range, 4.8-44.2) in the LC + TEA group (P = .06). CONCLUSION: The incidence of TEA and LC was 14.2% in our study. Based on our findings, history of TEA repair is not associated with a higher revision rate vs LC alone. The history of TEA repair did not alter the outcomes of LC repair.

Outcome of long gap esophageal atresia at 6 years: A prospective case control cohort study.

BACKGROUND DATA: EA is the most frequent congenital esophageal malformation. Long gap EA remains a therapeutic challenge for pediatric surgeons. A case case-control prospective study from a multi-institutional national French data base was performed to assess the outcome, at age of 1 and 6 years, of long gap esophageal atresia (EA) compared with non-long gap EA/tracheo-esophageal fistula (TEF). The secondary aim was to assess whether initial treatment (delayed primary anastomosis of native esophagus vs. esophageal replacement) influenced mortality and morbidity at ages 1 and 6 years. METHODS: A multicentric population-based prospective study was performed and included all patients who underwent EA surgery in France from January 1, 2008 to December 31, 2010. A comparative study was performed with non-long gap EA/TEF patients. Morbidity at birth, 1 year, and 6 years was assessed. RESULTS: Thirty-one patients with long gap EA were compared with 62 non-long gap EA/TEF patients. At age 1 year, the long gap EA group had longer parenteral nutrition support and longer hospital stay and were significantly more likely to have complications both early post-operatively and before age 1 year compared with the non-long gap EA/TEF group. At 6 years, digestive complications were more frequent in long gap compared to non-long gap EA/TEF patients. Tracheomalacia was the only respiratory complication that differed between the groups. Spine deformation was less frequent in the long gap group. There were no differences between conservative and replacement groups at ages 1 and 6 years except feeding difficulties that were more common in the native esophagus group. CONCLUSIONS: Long gap strongly influenced digestive morbidity at age 6 years.

Assessment of MRI and Ultrasound Screening for Tethered Cord Syndrome in Patients Diagnosed With Esophageal Atresia/Tracheoesophageal Fistula.

INTRODUCTION: Infants with esophageal atresia and/or tracheoesophageal fistula (EA/TEF) undergo screening for tethered cord syndrome (TCS) via ultrasound and magnetic resonance imaging. Existing literature lacks data to guide optimal timing of screening and magnetic resonance imaging (MRI) is often delayed until 3-6 mo of age, when it is frequently forgotten. Detethering surgery has a high rate of success in patients with TCS and is often performed prophylactically due to potential irreversible deficits. This study aims to improve screening procedure for infants with EA/TEF. METHODS: A retrospective chart review was done of all EA/TEF patients treated over 6 y (n = 79). The study examined how often each imaging modality was performed and identified a TCS lesion, as well as age of screening/surgical intervention. RESULTS: Screening for TCS was done with MRI 58% of the time and US 15% of the time. However, 38% of patients did not undergo any screening. Out of the patients with TCS on MRI (n = 19, 41.3%), 73.7% had neurosurgery. Of patients who underwent ultrasound (US) (n = 12), nine patients also had MRI later: two reported TCS lesions and subsequently had neurosurgery. Surgical infection rates and complications were 0/14. CONCLUSIONS: MRI demonstrated a higher rate of detecting TCS lesions than US, and patients with TCS frequently had detethering. Patients with ≥3 VACTERL or vertebral anomalies had a higher incidence of TCS on MRI. Patients with vertebral anomalies reported false negative ultrasounds in two cases, suggesting the potential superiority of MRI screening in this subgroup. A third of children did not undergo any imaging and this will require a process improvement.

Current outcomes of infants with esophageal atresia and tracheoesophageal fistula: A multicenter analysis.

OBJECTIVE: This study aims to quantify mortality rates and hospital lengths of stay (LOS) in neonates with esophageal atresia and tracheoesophageal fistula (EA/TEF), and to characterize the effects of birth weight (BW) and associated congenital anomalies upon these. METHODS: Data regarding patients with EA/TEF were prospectively collected (2013-2019) at 298 North American centers. The primary outcome was mortality and secondary outcome was LOS.  Risk factors affecting mortality and LOS were assessed. RESULTS: EA/TEF was diagnosed in 3290 infants with a median BW of 2476 g (IQR 1897,2970). In-hospital mortality was 12.7%. Mortality was inversely correlated with BW. After adjustment, the risk of mortality decreased by approximately 11% with every 100 g increase in BW. A significant congenital anomaly other than EA/TEF was diagnosed in 37.9% of patients. Risk of mortality increased in patients with associated congenital anomalies, most notably in those with a severe cardiac anomaly. Lower BW was associated with an increased mean LOS among survivors. Similar to mortality risk, additional anomalies were associated with prolonged LOS. CONCLUSIONS: This study demonstrates an in-hospital mortality of over 10%. Both increased mortality and prolonged LOS are highly associated with lower birth weight and the presence of concomitant congenital anomalies.

Incidence of and risk factors for perioperative blood transfusion in infants undergoing index pediatric surgery procedures.

BACKGROUND: There is a paucity of data on the frequency of transfusion during pediatric surgery index cases and guidelines for pretransfusion testing, defined as type and screen and crossmatch testing, prior to operation are not standardized. This study aimed to determine the incidence of perioperative blood transfusions during index neonatal operations and identify risk factors associated with perioperative blood transfusion to determine which patients benefit from pretransfusion testing. METHODS: A retrospective review of infants who underwent index neonatal cases between 2013 and 2019 was performed. Data were collected for patients who underwent operations for Hirschsprung's disease, esophageal atresia/tracheoesophageal fistula (EA/TEF), biliary atresia, anorectal malformation, omphalocele, gastroschisis, duodenal atresia, congenital diaphragmatic hernia (non-ECMO) or pulmonary lobectomy. Infants under 6 months were included except in the case of lobectomy where infants up to 12 months were included. RESULTS: Analysis was performed on 420 patients. Twenty-five (6.0%) patients received perioperative blood transfusion. Patients who received perioperative transfusion most commonly underwent EA/TEF repair. Patients who received perioperative transfusion had higher rates of structural heart disease (52.0% vs 17.7%, p<0.001), preoperative transfusion (48.0% vs 8.9%, p<0.001), and prematurity (52.0% vs 25.6%, p = 0.005). Presence of all three risk factors resulted in a 48% probability of requiring perioperative transfusion. CONCLUSIONS: Blood transfusion during the perioperative period of neonatal index operations is rare. Factors associated with increased risk of perioperative transfusion include prematurity, structural heart disease, and history of previous blood transfusion. LEVEL OF EVIDENCE: III.

Prevalence of acid gastroesophageal reflux disease in infants with esophageal atresia/tracheoesophageal fistula.

BACKGROUND: Given the high prevalence and complication risks of acid gastroesophageal reflux (GERD) in the first months of life in infants with esophageal atresia, the ESPGHAN/NASPGHAN consensus statement recommends systematic treatment with proton pump inhibitors (PPIs) until the age of 1 year and checking for acid GERD thereafter. However, these recommendations have not been evaluated. METHODS: This prospective study was conducted from 2007 to 2016. We evaluated the prevalence of acid GERD in 100 consecutive infants presenting with esophageal atresia/tracheoesophageal fistula after the age of 18 months when PPI treatment was stopped. The diagnosis of acid GERD was based on positive pH-metry and/or evidence of complications (e.g., peptic esophagitis, need for jejunal nutrition, or antireflux surgery). Those with acid GERD at a median age of 18 months received a control examination every year or adapted to their clinical situation. RESULTS: The prevalence rates of acid GERD were 64.3% at 18 months and 22.8% at the last follow-up (median age 65 months).There is no risk factor for acid GERD identified. CONCLUSIONS: This study shows a high prevalence of acid GERD in late infancy and supports the recommendation of systematic checking for acid GERD when treatment with PPI is stopped. IMPACT: Acid gastroesophageal reflux disease (GERD) is a frequent complication of esophageal atresia in infants. The ESPGHAN/NASPGHAN consensus, which is based on expert opinion, recommends systematic treatment of children with PPI until the age of 1 year. The prevalence rates of acid GERD were 64.3% at 18 months and 22.8% at the last follow-up. This study shows a high prevalence of acid GERD in late infancy and supports the recommendation of systematic checking for acid GERD when treatment with PPI is stopped.

Clinical comparison between thoracoscopic and thoracotomy repair of Gross type C esophageal atresia.

BACKGROUND: To compare the clinical outcomes between thoracoscopic approach and thoracotomy surgery in patients with Gross type C Esophageal atresia (EA) and tracheoesophageal fistula (TEF). METHODS: Patients with Gross type C EA/TEF who underwent surgery from January 2007 to January 2020 at Beijing Children's Hospital were retrospectively analyzed. The patients were divided into two groups according to surgical approaches. The perioperative factors and postoperative complications were compared among the two groups. RESULTS: One hundred and ninety patients (132 boys and 58 girls) with a median birth weight of 2975 (2600, 3200) g were included. The primary operations were performed via thoracoscopic (n = 62) and thoracotomy (n = 128) approach. After comparison of clinical characteristics between the two groups, we found that there were statistically significant differences in associated anomalies, method of fistula closure, duration of mechanical ventilation after surgery, feeding option before discharge, management of pneumothorax, and prognosis (all P < 0.05). To a certain extent, thoracoscopic surgery reduced the incidence of anastomotic leakage and increased the incidence of anastomotic stricture in this study. However, there were no statistically significant differences between the two groups in terms of operative time, postoperative pneumothorax, anastomotic leakage, anastomotic stricture, and recurrent tracheoesophageal fistula (all P > 0.05). CONCLUSIONS: Thoracoscopy surgery for Gross type C EA/TEF is a safe and effective, minimally invasive technique with comparable operative time and incidence of postoperative complications.