Esophageal Atresia Tracheoesophageal Fistula Research Articles

A 3D printed esophageal atresia–tracheoesophageal fistula thorascopy simulator for young surgeons

We developed a 3D-printed thoracoscopic surgery simulator for esophageal atresia with tracheoesophageal fistula (EA-TEF) and assessed its effectiveness in educating young pediatric surgeons. Prototype production and modifications were repeated five times before producing the 3-D printed final product based on a patient’s preoperative chest computed tomography. A 24-item survey was used to rate the simulator, adapted from a previous report, with 16 young surgeons with an average of 6.2 years of experience in pediatric surgery for validation. Reusable parts of the thoracic cage were printed to combine with replaceable parts. Each structure was fabricated using diverse printing materials, and subsequently affixed to a frame. In evaluating the simulator, the scores for each factor were 4.33, 4.33, 4.27, 4.31, 4.63, and 4.75 out of 5, respectively, with the highest ratings in value and relevance. The global rating was 3.38 out of 4, with ten stating that it could be used with slight improvements. The most common comment from participants was that the esophageal anastomosis was close to the actual EA-TEF surgery. The 3D-printed thoracoscopic EA-TEF surgery simulator was developed and reflected the actual surgical environment. It could become an effective method of training young pediatric surgeons.

Thoracoscopic repair (TR) versus conventional open repair (COR) for esophageal atresia–tracheoesophageal fistula (TEF–EA): a pilot randomized controlled trial

Thoracoscopic repair (TR) versus conventional open repair (COR) for esophageal atresia–tracheoesophageal fistula (TEF–EA): a pilot randomized controlled trial

Ear anomalies and hearing loss in patients with VACTERL association and the effect of maternal diabetes.

VACTERL association is typically defined as the presence of three components among these birth defects: vertebral anomalies, anal atresia, cardiac anomalies, esophageal atresia/tracheoesophageal fistula (EA/TEF), renal anomalies, and limb defects. There is increasing recognition that VACTERL and other recurrent constellations of embryonic development often overlap clinically and might share pathogenesis. We conducted a comprehensive chart review of a large patient population with VACTERL association from two tertiary care centers in California. We included patients with incomplete VACTERL expression, which we denoted as "partial VACTERL" (pVACTERL). We assessed the occurrence of craniofacial (CF) findings in these two groups and the combined cohort. We collected data on potential risk factors and demographic information such as sex, Hispanic ancestry, pregnancy complications, and maternal age. The study included 409 participants, of whom 263 had VACTERL and 146 pVACTERL. CF abnormalities were found in 17.3% of VACTERL patients and 9.4% of pVACTERL patients. In the VACTERL group, ear anomalies were found in 10.2%, microtia in 5.9%, hearing loss (HL) in 13.90%, and orofacial clefts in 3.1%. In the pVACTERL group, ear anomalies were found in 7.2%, microtia in 5.0%, HL in 9.3%, and orofacial cleft in 2.2%. Maternal diabetes significantly increased the risk for HL in VACTERL (odds ratio [OR]: 3.71, 95% confidence interval [CI]: 1.5-7.3) and pVACTERL patients (OR: 6.7, 95% CI: 1.70-23.4). Poorly controlled maternal diabetes significantly increased the risk for all the outcomes in VACTERL patients including CF anomalies (OR: 4.2, 95% CI: 1.9-9.6), ear anomalies (OR: 4.7, 95% CI: 1.8-11.8), microtia (OR: 5.4, 95% CI: 1.7-16.6), and HL (OR: 8.1, 95% CI: 3.4-19.4). Twin status was significantly associated with the occurrence of microtia (p = 0.038) in VACTERL patients. Occurrence of CF features, particularly ear anomalies, microtia, and HL, might be considered as part of phenotypic diversity of VACTERL association. Diabetes and twinning might appear to play a role in increasing the risk for this phenotype in VACTERL association.

Utility of Infrared Technology in Minimally Invasive Procedures: A Novel Strategy to Identify a Esophagobronchial Fistula via Thoracoscopy

Esophagobronchial or esophagopulmonary fistulas are complications after esophageal atresia/tracheoesophageal fistula (EA/TEF) repair. Postoperative scarring and recurrent infections render surgical treatment a potentially hazardous undertaking.This report describes the use of infrared (IR) technology to locate and safely dissect a esophagobronchial fistula in a 11-year-old female with a history of EA/TEF. The patient, who had initially undergone primary repair via thoracotomy, was diagnosed with a late tracheoesophageal fistula (TEF) recurrence at the age of 6 after several pulmonary infections and was subjected to a left thoracoscopy with successful closure of the TEF. Despite both surgical procedures, she presented ongoing respiratory symptoms for several years and was referred to our center as an 11-year-old, where further examinations revealed the presence of a esophagobronchial fistula involving the right upper bronchus. After an unsuccessful attempt at endoscopic closure, surgical treatment via right thoracoscopy was planned. Initially, an endoscopic examination was performed, to catheterize the fistula tract in the mid-thoracic esophagus with the infrared probe. Subsequently, right thoracoscopy was performed in the prone position using four 5 mm trocars. Guided by the IR signal, the fistula tract was easily identified and dissected. Fistula closure was achieved using a 5 mm stapler. The staple line was reinforced with a pleural flap and a right thoracic tube was inserted. The patient was discharged after a four-day hospital stay, without complications. The patient remains asymptomatic to date and has returned to her native Ukraine.

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

IntroductionTo determine the incidence, management and outcomes of esophageal atresia/tracheo-esophageal fistula (EA/TEF) over a 15-y period in South Africa. MethodsA 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. ResultsAmong 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. ConclusionsIncidence, 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.

ARE LOW BIRTH WEIGHT AND MAJOR CONGENITAL HEART DISEASES CONTRAINDICATIONS FOR THORACOSCOPIC REPAIR OF ESOPHAGEAL ATRESIA WITH TRACHEOESOPHAGEAL FISTULA?

BACKGROUND Thoracoscopic repair of esophageal atresia (EA) with tracheoesophageal fistula (TEF) is becoming an increasingly widespread technique; there is still controversy about its indication in certain patients. Our objective is to analyze if potential risk factors such as major congenital heart disease (CHD) or low birth weight are a limitation to this approach. METHODS Retrospective study (2017-2021) of patients with EA and distal TEF who underwent thoracoscopic repair were included. Patients with low birth weight <2000g or major congenital heart disease were compared to the rest. RESULTS Twenty-five patients underwent thoracoscopic surgery. Nine patients (36%) had major CHD. Five of them (20%) were low birth weight <2000g and only 8% presented both risk factors (2/25). There were no differences in terms of operative time, conversion rate, tolerance evaluated with gasometric parameters (pO2, pCO2, pH) or complications (anastomotic leak and stricture, both early or during follow-up) in patients with major CHD and LBW (1473 ± 319g vs 2664 ± 402g). One conversion to thoracotomy was performed in a baby weighing 1050 g due to anesthetic intolerance. There was no recurrence of TEF. One patient died at the age of 9 months, due to major uncorrectable heart disease. CONCLUSIONS Thoracoscopic repair of EA/TEF is feasible technique in patients with CHD or LBW, with similar results to other patients. The complexity of this technique warrants individualizing the indication in each case.

Assessing the Feasibility of Predicting Intermediate Outcomes of Esophageal Atresia Using Okamoto Classification: A Prospective Study.

Several risk classifications based on various preoperative factors have been proposed to prognosticate the immediate survival of children operated for esophageal atresia. A major drawback of these classifications is that they only focus on immediate survival while ignoring the long-term morbidity and mortality in these children. Our study aims to bridge this gap in knowledge by studying the impact of one such classification (Okamoto's classification) on mortality and morbidity during a period of 1 year after discharge from the hospital in operated cases of esophageal atresia. After institutes ethical clearance, 106 children operated for esophageal atresia-tracheoesophageal fistula between 2012 and 2015 were studied prospectively for a period of 1 year after their discharge. The children were graded as per Okamoto classification. The primary objective was to determine the efficacy of this classification in predicting the survival rates in infancy and the secondary objective was to compare the complication rates in these children based on the classification. Sixty-nine children met the inclusion criteria. There were 40, 15, 10, and 4 children in Okamoto Classes I, II, III, and IV, respectively. Twenty-one patients (30%) died during the follow-up period with the maximum number of deaths occurring in Okamoto Class IV (75%) and the minimum in Okamoto Class I (17.5%) (P = 0.003). There was a significant correlation between the Okamoto classes with the incidence of poor weight gain (P = 0.001), lower respiratory tract infection (P = 0.007), and failure to thrive (P = 0.01) higher in Okamoto IV and III as compared to I and II. Okamoto prognostic classification during the initial hospitalization is relevant even at 1 year follow-up with increased mortality and morbidity in Okamoto Class IV as compared to Class I.

The Role of Postoperative Mechanical Ventilation in Neonates with Tracheoesophageal Fistula

Background: Many advances have been made in managing neonates with esophageal atresia/tracheoesophageal fistula (EA/TEF). This resulted in a progressive decrease in mortality as a result of improved neonatal intensive care and neonatal mechanical ventilation; however, this is not the case in developing countries. Objectives: We aimed to evaluate the efficacy of postoperative ventilation in neonates with EA/TEF in Iraq as a developing country. Materials and Methods: It is a prospective study for all neonates with EA and/or TEF admitted to the pediatric intensive care unit (PICU) in Alkhansaa Teaching Hospital between December 2012 to December 2014. Forty operated neonates were randomly divided into two groups according to the record number of presentations (even = G1, odd = G2): Twenty patients (group l) had been assigned to be post-operatively on a ventilator as a routine protocol. While group 2 planned to be extubated in the theater. We evaluated the complications and mortality rates in two groups. Results: For 24 months, 40 neonates with EA/TEF were admitted to PICU; Thirty-two were operated on within 24 hours of life. There were no significant differences between the two groups regarding fetal maturity (p-value 0.490), gender, and weight. The mean period for mechanical ventilation was four days. Six neonates had tube mal-positioning complications, leading to lung collapse, hypoxia, and pneumonia (p-value 0.250). Anastomotic leaks occur in 10 neonates (five in each group) (p-value 0.642), which sometimes leads to sepsis (p-value 0.347). Trachea-malacia was recorded after weaning in 5 intubated patients (p-value 0.078). The mortality rate in G1 was 35%, while in group 2 was 25% (p-value 0.366). Conclusions: Although the study’s sample is small, it is recommended to avoid mechanical ventilation in the postoperative care of neonates with EA and/or TEF.

IPSCs derived from esophageal atresia patients reveal SOX2 dysregulation at the anterior foregut stage.

A series of well-regulated cellular and molecular events result in the compartmentalization of the anterior foregut into the esophagus and trachea. Disruption of the compartmentalization process leads to esophageal atresia/tracheoesophageal fistula (EA/TEF). The cause of EA/TEF remains largely unknown. Therefore, to mimic the early development of the esophagus and trachea, we differentiated induced pluripotent stem cells (iPSCs) from EA/TEF patients, and iPSCs and embryonic stem cells from healthy individuals into mature three-dimensional esophageal organoids. CXCR4, SOX17 and GATA4 expression was similar in both patient-derived and healthy endodermal cells. The expression of the key transcription factor SOX2 was significantly lower in the patient-derived anterior foregut. We also observed an abnormal expression of NKX2.1 (or NKX2-1) in the patient-derived mature esophageal organoids. At the anterior foregut stage, RNA sequencing revealed the critical genes GSTM1 and RAB37 to be significantly lower in the patient-derived anterior foregut. We therefore hypothesize that a transient dysregulation of SOX2 and the abnormal expression of NKX2.1 in patient-derived cells could be responsible for the abnormal foregut compartmentalization.

Exome sequencing efficacy and phenotypic expansions involving esophageal atresia/tracheoesophageal fistula plus.

Esophageal atresia/tracheoesophageal fistula (EA/TEF) is a life-threatening birth defect that often occurs with other major birth defects (EA/TEF+). Despite advances in genetic testing, a molecular diagnosis can only be made in a minority of EA/TEF+ cases. Here, we analyzed clinical exome sequencing data and data from the DECIPHER database to determine the efficacy of exome sequencing in cases of EA/TEF+ and to identify phenotypic expansions involving EA/TEF. Among 67 individuals with EA/TEF+ referred for clinical exome sequencing, a definitive or probable diagnosis was made in 11 cases for an efficacy rate of 16% (11/67). This efficacy rate is significantly lower than that reported for other major birth defects, suggesting that polygenic, multifactorial, epigenetic, and/or environmental factors may play a particularly important role in EA/TEF pathogenesis. Our cohort included individuals with pathogenic or likely pathogenic variants that affect TCF4 and its downstream target NRXN1, and FANCA, FANCB, and FANCC, which are associated with Fanconi anemia. These cases, previously published case reports, and comparisons to other EA/TEF genes made using a machine learning algorithm, provide evidence in support of a potential pathogenic role for these genes in the development of EA/TEF.

Spiritual Counseling and Care Needs of Parents whose Children Diagnosed with Esophageal Atresia and Tracheoesophageal Fistula

Bu çalışmanın amacı, özofagus atrezisi-trakeaözofageal fistül tanısı alan çocuk sahibi anne ve babalara yönelik manevi danışmanlık ve rehberlik ihtiyacını ortaya koymaktır. Araştırmanın girişinde genel bir bilgi verildikten sonra yapılan çalışmanın amacına kısaca değinilmiştir. Aynı bölümde, çalışmanın metodolojik olarak yerli ve yabancı literatür taraması yapılarak dokümantasyon tekniğine göre hazırlandığına değinilmiştir. Öncelikle özofagus atrezisi- trakeaözofageal fistül hastalığının tanımı yapılmıştır. Özofagus atrezisi-trakeaözofageal fistül çeşitleri ele alınırken şekil ile konunun anlaşılırlığı desteklenmiştir. Özofagus atrezisi- trakeaözofageal fistül hastalığının tedavi yöntemleri ele alınmıştır. Hastalığın önce embriyolojisine sonra etiyolojisine yer verilerek hastalığa dair genel çerçeve çizilmiştir. Ardından özofagus atrezisi-trakeaözofageal fistül tanısı alan çocuk sahibi anne ve babaların değişen günlük rutinlere uyumsuzluk, kötü anne-baba olma hissi, toplumdan dışlanmışlık, sosyal değer düşüklüğü, savunmasızlık gibi birtakım psiko-sosyal uyum sorunlarının olduğu tespit edilmiştir. Özofagus atrezisi-trakeaözofageal fistül tanısı alan çocuk sahibi anne ve babaların, çocuklara yönelik temel bakım ve temel ilkyardımı öğrenme, mevcut durumla başa çıkmada maddi destek, sosyal destek, psikolojik anlamda başa çıkabilme konusunda din hizmetleri gibi ihtiyaç alanlarının olduğuna değinilmiştir. Dinsel başa çıkmanın ne olduğu açıklandıktan sonra bu anne ve babaların dinsel başa çıkma ihtiyaçları incelenmiştir. Ardından dinsel bağlanma üzerinde durulmuş ve özofagus atrezisi-trakeaözofageal fistül tanısı alan çocuk sahibi anne ve babaların dinsel bağlanma ihtiyaçları ele alınarak manevi danışmanlık ihtiyaçları bölümü sonlandırılmıştır. Daha sonra özofagus atrezisi-trakeaözofageal fistül tanısı alan çocuk sahibi anne-babaların rehabilitasyonunda anlam arayışı, dua ve ibadet, tevekkül ve kader gibi manevi danışmanlık temalarının önemine dikkat çekilmiştir. Anlam arayışı bölümünde ‘anlam arayışı’nın ne olduğuna yer verildikten sonra hastalıkla özdeşlik kurulmuştur. Özofagus atrezisi- trakeaözofageal fistül tanısı alan çocuk sahibi anne-babaların rehabilitasyonunda manevi içerikli psiko-eğitime olan ihtiyaç gözler önüne serilmiştir. Dua ve ibadet başlığında dua ve ibadetin tanımı yapılmış, konu ile ilişiği ayrı paragraflarda ele alınmıştır. Tevekkül ve kaderin tanımı yapılarak manevi danışmanlıktaki rehabilite özelliğine değinilmiştir. Araştırmanın sonuç bölümünde çalışma toparlanmış ve konuyla ilgili yapılabilecek çalışmalara değinen öneriler bölümüne yer verilmiştir.

Reliability and Validity of the Polish Version of the Esophageal-Atresia-Quality-of-Life Questionnaires to Assess Condition-Specific Quality of Life in Children and Adolescents Born with Esophageal Atresia.

Aim: This study reports the reliability and validity of the Polish version of the Esophageal Atresia Quality of Life (EA-QOL) questionnaires, which were originally developed in Sweden and Germany. Methods: A total of 50 families of children (23 aged 2 to 7, and 27 aged 8 to 17) with EA/TEF (esophageal atresia/tracheoesophageal fistula) participated in the study. The development and validation of the Polish version of the EA-QOL involved forward-backward translation of the survey items following the guidelines for cross-cultural translation, cognitive debriefing and evaluation of psychometric properties, including assessment of internal and retest reliability, linguistic validity, content validity, known-group validity and convergent validity. The medical records of patients and standardized questionnaires were used to obtain clinical data. The level of significance was p < 0.05. Results: The Polish versions of the EA-QOL questionnaires demonstrated strong linguistic and content validity, are slightly discriminative for esophageal and respiratory problems, but do not show convergent validity with the PedsQL 4.0 generic core scales. In terms of reliability, the internal consistency of the subscale and total scale of Polish versions as measured by Cronbach’s alpha is good, and retest reliability is excellent. Conclusions: The Polish versions of the EA-QOL questionnaires meet most psychometric criteria that confirm the EA-QOL questionnaires’ reliability and validity. This study enables application of these questionnaires in future research among children with EA in Poland and participation in international multicenter studies focusing on advancing knowledge of condition-specific QOL in this population. Future cross-cultural research using larger sample sizes is still needed to better address the relationship between condition-specific and generic QOL, as well as the discriminative ability of the EA-QOL questionnaires.

Social media communities for patients and families affected by congenital pediatric surgical conditions.

BackgroundSocial media has become a means to allow individuals affected by rare diseases to connect with others. This study aimed to characterize the use of three popular social media platforms by individuals affected by common pediatric surgery index conditions.MethodsA systematic search of Instagram, Facebook, and Twitter was performed using standardized terms for the following pediatric surgical conditions: anorectal malformation, biliary atresia, bronchopulmonary sequestration, congenital diaphragmatic hernia (CDH), congenital pulmonary airway malformation, duodenal atresia, esophageal atresia/tracheoesophageal fistula (EA/TEF), gastroschisis, Hirschsprung disease (HD), and omphalocele. Accounts active within the last year were analyzed and assessed.ResultsA total of 666 accounts were identified. Instagram was the most common platform, but accounts dedicated to support and story sharing were most common on Facebook. Biliary atresia and CDH had the largest communities identified. Support groups were most common among those dedicated to EA/TEF (43.3%, p < 0.001). Most accounts were created by parents, but accounts dedicated to HD had the greatest proportion founded by patients (22.4%, p = 0.04).ConclusionsSocial media use is common among patients and families affected by congenital surgical conditions. Certain diseases have larger communities and support networks. Knowledge about their existence may allow surgeons to direct patients towards supportive communities and resources.Level of evidenceIV.

The use of biological membranes for correction of congenital malformations

Many congenital malformations often require a multidisciplinary and multistep surgical treatment, including the use of biological membranes. Aims of the study were to describe the use of these membranes for the correction of malformations, their clinical performance at follow-up, and patient's tolerance to them. The study included patients treated between 2009 and November 2020 in two referral centers. They were affected by abdominal wall defects (AWD), esophageal atresia/tracheo-esophageal fistula (EA/TEF), diaphragmatic hernia (CDH), spinal defects (SD), and anorectal malformations (ARM). The human origin membranes used during surgery were amniotic membrane, fascia lata, and pericardium provided by the local tissue bank and the porcine-derived membrane available on the market. Thirty-one patients were retrieved. The sample included 10 AWD, 7 EA/TEF, 5 CDH, 4 SD, 2 ARM, and 3 miscellaneous defects. The median age at repair was 139 days (range: 10,5–1494). The median follow-up was 1021 days (range: 485,5–1535). Two patients were lost at follow-up. The defects were successfully repaired and the membranes perfectly tolerated in 28/29 cases. In 1 case of CDH the fascia lata was replaced with a Goretex patch due to recurrence of the defect. This is the largest series on the use of biological membranes in congenital malformations. The variety of tissues allows to choose the best material for each malformation. The excellent tolerance and performance of this first series of patients encourage the use of these membranes to correct different type of malformations at any age.

The largest report on thoracoscopic surgery for recurrent tracheoesophageal fistula after esophageal atresia repair

BackgroundAlthough thoracoscopic surgery has become the routine surgical method for esophageal atresia/tracheoesophageal fistula (EA/TEF), thoracoscopic treatment for recurrent tracheoesophageal fistula (rTEF) is far from popularized. ObjectivesWe aimed to explore the safety and efficacy of thoracoscopic treatment for rTEF with a large-cohort study. MethodsWe retrospectively analyzed the clinical characteristics and outcomes of 103 consecutive patients who underwent thoracoscopic surgery for rTEF by one surgeon after EA/TEF repair at two different institutions in China from 2014 to 2021. ResultsOne hundred and three pediatric patients (67 boys) were enrolled and the primary operations were performed via thoracoscopic (n = 75, 72.82%) or open surgery (n = 28, 27.18%). The median age at rTEF diagnosis was 5 (3, 10) months after the primary repair. Patients were diagnosed with recurrent fistula to the trachea (n = 97, 94.17%), bronchi (n = 4, 3.88%), and lung parenchyma (n = 2, 1.94%), and all of them underwent thoracoscopic surgery at a median age of 7 (5, 14) months with a median weight of 6200 (4870, 7650) g. After the repair of rTEF, the incidence of esophageal leakage, esophageal stricture, and TEF recurrence were 12.8%, 33.4%, and 10.8%, respectively. After the follow-up, 87 patients survived, 6 died, and 10 were lost to follow-up. ConclusionsThe results of thoracoscopic surgery for rTEF were comparable with previously reported thoracotomy surgery. Owing to the clear field during the operation, rapid patient recovery and esthetic results, the thoracoscopic approach could be a better choice for experienced pediatric surgeons. Level of EvidenceLEVEL IV

Musculoskeletal deformities in children with congenital thoracic malformations: a population-based cohort study.

It is unclear if musculoskeletal deformities observed in patients with congenital diaphragmatic hernia (CDH), congenital lung lesion (CLL) and esophageal atresia/tracheoesophageal fistula (EA/TEF) are associated with the anomaly or are a result of the surgery required to treat the anomaly. This study compared the prevalence of musculoskeletal deformities for: (1) children with congenital thoracic anomalies to controls; (2) CLL to EA/TEF both repaired via thoracotomy; and (3) CLL and EA/TEF to CDH repaired via laparotomy. We performed a retrospective study of children with CLL, CDH or EA/TEF between 1990 and 2016. Date-of-birth-matched control groups were generated from a population-based dataset. International Classification of Disease codes were used to identify scoliosis and pectus anomalies. We determined Hazard ratios (HR) for cases versus controls. We included 167 cases (CDH n = 82; CLL n = 29; EA/TEF n = 56) and 1670 controls. EA/TEF had a greater risk of scoliosis (HR 5.52, 95%CI 1.49,13.73) and pectus deformities (HR 4.07, 95%CI 1.96,8.45). CDH showed more scoliosis (HR 5.03, 95%CI 1.99,12.74) but not pectus anomalies. Musculoskeletal deformities were not more common in CLL. Children born with CDH or EA/TEF, but not CLL, had more musculoskeletal deformities than controls. The inconsistent association between musculoskeletal deformities and the surgical approach suggested a congenital predisposition.

Generation of three induced pluripotent stem cells lines from patients with esophageal atresia/tracheoesophageal fistula type C

Esophageal atresia/tracheoesophageal fistula (EA/TEF) is the most common congenital anomaly of the upper gastrointestinal tract affecting 1 in 3,000 which could stem from a developmental anomaly of the foregut. The cause is not fully understood. We generated three iPSC cell lines using peripheral blood mononuclear cells (PBMCs) from EA/TEF type C patients. Pluripotency and trilineage differentiation capacity of these three iPSC cell lines were confirmed by gene and protein expression profiles and the differentiation ability into the three germ layers. The generated disease-specific cell lines could serve as a tool to investigate the mechanisms of EA/TEF and acquired associated diseases.

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

BackgroundThere 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. MethodsA 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. ResultsAnalysis 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. ConclusionsBlood 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 EvidenceIII

Technical Innovations to Reduce Complication Rates in Esophageal Atresia with Particular Reference to Long-term Outcomes: A Single Surgeon's Experience of 22 Years.

Following esophageal atresia/tracheoesophageal fistula (EA/TEF) repair, the standard leak rate reported in the literature is 5%-10%, and stricture rate is 40%-72%. There is a global quest for surgical innovations to drive down these complication rates which can cause considerable morbidity. A prospectively maintained database of the senior author's patients who had esophageal atresia repair from 1995 to 2016 was reviewed. Two distinct innovations were implemented: (1) adequate or generous mobilization of the lower esophageal pouch and (2) a 2-5 mm slit in distal esophagus to widen its circumference. Forty-three patients with EA/TEF were reviewed. Of those, 40 underwent primary repair. The median follow-up was 12.5 years (range 4-26 years). There were no anastomotic leaks and only 8 (20%) patients developed anastomotic strictures requiring dilations (1-5 dilations/patients). One patient (2.5%) had a recurrent fistula. One early mortality was recorded. At the latest follow-up, 35 (87.5%) patients had normal oral feeding, while 1 (2.5%) patient had occasional food sticking episodes. Four syndromic patients (10%) were on jejunal or gastrostomy feeding. An adequate or generous mobilization of the distal esophageal pouch, together with a 2-5 mm slit in the distal esophagus, achieves a tension-free and wide anastomosis. All anastomoses eventually narrow, sometimes just a little, and starting on a higher scale with a small slit, helps. These seemingly minor innovations, when used together, contributed to a substantially lower complication rate sustained over a 22-year period - no leaks and only 20% stricture rate.

Retrospective analysis of pneumothorax after repair of esophageal atresia/tracheoesophageal fistula

BackgroundTo analyze the possible causes, treatment and outcomes of postoperative pneumothorax in patients with Gross type C esophageal atresia/tracheoesophageal fistula (EA/TEF).MethodsMedical records of patients with Gross type C EA/TEF who were diagnosed and treated in Beijing Children’s Hospital from January 2007 to January 2020 were retrospectively collected. They were divided into 2 groups according to whether postoperative pneumothorax occurred. Univariate and multivariate logistic regression analysis were performed to identify risk factors for pneumothorax.ResultsA total of 188 patients were included, including 85 (45 %) in the pneumothorax group and 103 (55 %) in the non-pneumothorax group. Multivariate logistic regression analysis showed that postoperative anastomotic leakage [P < 0.001, OR 3.516 (1.859, 6.648)] and mechanical ventilation [P = 0.012, OR 2.399 (1.210, 4.758)] were independent risk factors for pneumothorax after EA/TEF repair. Further analysis of main parameters of mechanical ventilation after surgery showed that none of them were clearly related to the occurrence of pneumothorax. Among the 85 patients with pneumothorax, 33 gave up after surgery and 52 received further treatment [conservative observation (n = 20), pleural puncture (n = 11), pleural closed drainage (n = 9), both pleural puncture and closed drainage (n = 12)]. All of the 52 patients were cured of pneumothorax at discharge.ConclusionsAnastomotic leakage and postoperative mechanical ventilation were risk factors for pneumothorax after repair of Gross type C EA/TEF, but the main parameters of mechanical ventilation had no clear correlation with pneumothorax. After symptomatic treatment, the prognosis of pneumothorax was good.