Early Morbidity and Perioperative Course of Neonates with Esophageal Atresia and Tracheoesophageal Fistula in a Tertiary Pediatric Surgical Center

Background: The management of infants (infs.) with esophageal atresia and tracheoesophageal fistula (EA ± TEF) is demanding and complex. The aim of this study was to evaluate early morbidity, the timing of surgery, and the results of surgery. Patients and Method: We collected data of 30 consecutive infs. treated for EA ± TEF between 2006 and 2014. Results: The median gestational age was 38 weeks (12 preterm), and the median Birth Weight (BW) was 2660 g (4 infs. had a BW 1500 g). The median Apgar score at 10 minutes was 10 (range 7 - 10). The median umbilical artery pH (UapH) was 7.30. According to the Spitz classification, 19 infs. were group 1, 9 infs. were group 2, and 2 infs. were group 3. Surgical repair was performed in 29 cases (25 EA;4 isolated TEF). Once the infs. arrived at the pediatric surgery department, surgery was postponed overnight in 11 cases. The duration of postoperative (p.o.) mechanical ventilation was significantly shorter for operations performed on day 2 after delivery. Twenty-four infs. (83%) underwent surgery within 2 days after delivery, and 5 infs. had later surgery. Chest drains (p.o.) for pneumothorax were inserted in 6 infs. (21%), and gastrostomy was performed in 6 cases (21%). No re-thoracotomy was required. The median length of hospital stay was 17.5 days (6 to 120). The incidence of p.o. mortality was 1 in 29 (3%). Discussion: The majority of the infs. presented growth retardation (indicated by low birth weight) and a stable immediate postnatal course. The data from this study support the concept of early but not emergent surgery for the majority of infs. with EA ± TEF. However, a remarkable rate of perioperative morbidity must be taken into account. Conclusion: Surgery for EA ± TEF can be performed safely during the first postnatal days with exception of very unstable preterm infants.

One Stage Reconstruction of Esophageal Atresia and Distal Tracheoesophageal Fistula in a 3250-gm Neonate: A Case Report

Esophageal atresia (EA) occurs when the upper part of the esophagus does not connect with the lower part of esophagus and stomach. Tracheoesophageal fistula (TEF) is an abnormal connection between the upper part of the esophagus and the trachea. Treatment for esophageal atresia has advanced over several decades due to improvements in surgical techniques and neonatal intensive care. The aim is to share our experience regarding the treatment of esophageal atresia with tracheoesophageal fistula. A 4-day-old neonate suffering from esophageal atresia with type IIIB tracheoesophageal fistula underwent one stage esophageal reconstruction and obtained good outcome without any complications. In this paper, a simple intra-operative technique for tracheal fistula repair and end to end esophageal anastomosis is discussed. We used a simple technique that we have found useful for ligation of tracheal fistula. Anastomosis of lower and upper esophagus without any complication like anastomotic leakage or stricture/stenosis of the neonate with EA/TEF (type IIIB), was proved to be safe and effective.

Simultaneous type III congenital esophageal atresia and patent ductus arteriosus in a low-weight patient: A case report

BACKGROUND We report a low-birth-weight child(1.8 kg)with neonatal type III congenital esophageal atresia(CEA)combined with symptomatic patent ductus arteriosus(PDA).After comprehensive evaluation,esophageal anastomosis was performed on postnatal day 11 after excluding surgical contraindications,and arterial catheter ligation was performed at the same time.Concurrent surgery for CEA combined with PDA has not been clearly reported in the literature.CASE SUMMARY We report a 6-day-old female child with type III CEA and PDA.The patient presented with foam at the mouth after birth,cough and shortness of breath after feeding.At another hospital,she was considered to have neonatal pneumonia,neonatal jaundice and congenital heart disease and transferred to our hospital.After iodine oil radiography of the esophagus and echocardiography we con-firmed diagnosis of CEA and PDA.The diameter of the PDA was 8 mm,with obvious left to right shunting.We performed right rear extrapleural orificium fistula ligation and esophageal anastomosis,and ligation of PDA via left axilla straight incision after 5 d of hospitalization.The operations were successful,and the incision healed after 12 d,and the patient was discharged.We re-examined the patient 1 mo after surgery.She did not vomit when she ate rice flour.Esophageal angiography showed no stricture of the anastomotic stoma.The patient weighed 3.2 kg.CONCLUSION For CEA patients with multiple risk factors,comprehensive,timely and accurate diagnosis and evaluation,and early treatment may improve prognosis.

Primary repair of esophageal atresia Gross type C via thoracoscopic magnetic compression anastomosis:Is it the best option?

Magnetic compression anastomosis is a promising treatment option for patients with complex esophageal atresia;but,at the present time,should not be the first therapeutic option in those cases where the surgeon can perform a primary anastomosis of the two ends of the esophagus with acceptable tension.

Primary repair of esophageal atresia gross type C via thoracoscopic magnetic compression anastomosis:A case report

BACKGROUND Esophageal atresia(EA)is a life-threatening congenital malformation in newborns,and the traditional repair approaches pose technical challenges and are extremely invasive.Therefore,surgeons have been actively investigating new minimally invasive techniques to address this issue.Magnetic compression anastomosis has been reported in several studies for its potential in repairing EA.In this paper,the primary repair of EA with magnetic compression anastomosis under thoracoscopy was reported.CASE SUMMARY A full-term male weighing 3500 g was diagnosed with EA gross type C.The magnetic devices used in this procedure consisted of two magnetic rings and several catheters.Tracheoesophageal fistula ligation and two purse strings were performed.The magnetic compression anastomosis was then completed thoracoscopically.After the primary repair,no additional operation was conducted.A patent anastomosis was observed on the 15th day postoperatively,and the magnets were removed on the 23rd day.No leakage existed when the transoral feeding started.CONCLUSION Thoracoscopic magnetic compression anastomosis may be a promising minimally invasive approach for repairing EA.

Graft dilatation and Barrett’s esophagus in adults after gastric pullup and jejunal interposition for long-gap esophageal atresia

BACKGROUND Esophageal replacement(ER)with gastric pull-up(GPU)or jejunal interposition(JI)used to be the standard treatment for long-gap esophageal atresia(LGEA).Changes of the ER grafts on a macro-and microscopic level however,are unknown.AIM To evaluate long-term clinical symptoms and anatomical and mucosal changes in adolescents and adults after ER for LGEA.METHODS A cohort study was conducted including all LGEA patients≥16 years who had undergone GPU or JI between 1985-2003 at two tertiary referral centers in the Netherlands.Patients underwent clinical assessment,contrast study and endoscopy with biopsy.Data was collected prospectively.Group differences between JI and GPU patients,and associations between different outcome measures were assessed using the Fisher’s exact test for bivariate variables and the Mann-Whitney U-test for continuous variables.Differences with a P-value<0.05 were considered statistically significant.RESULTS Nine GPU patients and eleven JI patients were included.Median age at follow-up was 21.5 years and 24.4 years,respectively.Reflux was reported in six GPU patients(67%)vs four JI patients(36%)(P=0.37).Dysphagia symptoms were reported in 64%of JI patients,compared to 22%of GPU patients(P=0.09).Contrast studies showed dilatation of the jejunal graft in six patients(55%)and graft lengthening in four of these six patients.Endoscopy revealed columnar-lined esophagus in three GPU patients(33%)and intestinal metaplasia was histologically confirmed in two patients(22%).No association was found between reflux symptoms and macroscopic anomalies or intestinal metaplasia.Three GPU patients(33%)experienced severe feeding problems vs none in the JI group.The median body mass index of JI patients was 20.9 kg/m^(2) vs 19.5 kg/m^(2) in GPU patients(P=0.08).CONCLUSION The majority of GPU patients had reflux and intestinal metaplasia in 22%.The majority of JI patients had dysphagia and a dilated graft.Follow-up after ER for LGEA is essential.

Current knowledge on esophageal atresia

Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus. The improvement of survival observed over the previous two decades is multifactorial and largely attributable to advances in neonatal intensive care, neonatal anesthesia, ventilatory and nutritional support, antibiotics, early surgical intervention, surgical materials and techniques. Indeed, mortality is currently limited to those cases with coexisting severe life-threatening anomalies. The diagnosis of EA is most commonly made during the first 24 h of life but may occur either antenatally or may be delayed. The primary surgical correction for EA and TEF is the best option in the absence of severe malformations. There is no ideal replacement for the esophagus and the optimal surgical treatment for patients with long-gap EA is still contro-versial. The primary complications during the postoperative period are leak and stenosis of the anastomosis, gastro-esophageal reflux, esophageal dysmotility, fistula recurrence, respiratory disorders and deformities of the thoracic wall. Data regarding long-term outcomes and follow-ups are limited for patients following EA/TEF repair. The determination of the risk factors for the complicated evolution following EA/TEF repair may positively impact long-term prognoses. Much remains to be studied regarding this condition. This manuscript provides a literature review of the current knowledge regarding EA.

Eosinophilic esophagitis in patients with esophageal atresia and chronic dysphagia

Esophageal atresia(EA)is defined as a discontinuity of the lumen of the esophagus repaired soon after birth.Dysphagia is a common symptom in these patients,usually related to stricture,dysmotility or peptic esophagitis.We present 4 cases of patients with EA who complained of dysphagia and the diagnosis of Eosinophilic esophagitis(Eo E)was made,ages ranging from9 to 16 years.Although our patients were on acid suppression years after their EA repair,they presented with acute worsening of dysphagia.Esophogastroduodenoscopy and/or barium swallow did not show stricture and biopsies revealed elevated eosinophil counts consistent with Eo E.Two of 4 patients improved symptomatically with the topical steroids.It is important to note that all our patients have asthma and 3 out of 4 have tested positive for food allergies.One of our patients developed recurrent anastomotic strictures that improved with the treatment of the Eo E.A previous case report linked the recurrence of esophageal strictures in patients with EA repair with Eo E.Once the Eo E was treated the strictures resolved.On the other hand,based on our observation,Eo E could be present in patients without recurrent anastomotic strictures.There appears to be a spectrum in the disease process.We are suggesting that Eo E is a frequent concomitant problem in patients with history of congenital esophageal deformities,and for this reason any of these patients with refractory reflux symptoms or dysphagia(with or without anastomotic stricture)may benefit from an endoscopic evaluation with biopsies to rule out Eo E.

Gastroesophageal reflux disease in pediatric esophageal atresia:Assessment of clinical symptoms and pH-impedance data

BACKGROUND Esophageal atresia(EA)is the most common congenital anomaly of the gastrointestinal tract.Gastroesophageal reflux disease(GERD)is a frequent and lifelong problem in these patients.GERD can be asymptomatic and the incidence of esophageal gastric and intestinal metaplasia(Barrett’s esophagus)is increased in adults with EA compared with the general population.Timely and accurate diagnosis of GERD is important to reduce long-term problems and this may be achieved by pH-impedance testing.AIM To assess symptoms and pH-impedance data in children after EA,in order to identify their specific features of GERD.METHODS This study was conducted from November 2017 to February 2020 and involved 37 children who had undergone EA via open surgical repair(51.35%boys,48.65%girls;age range:1-14 years,median:4.99 years).GERD diagnosis was made based on multichannel intraluminal impedance/pH study and two groups were established:EA without GERD,n=17;EA with GERD,n=20.A control group was established with 66 children with proven GERD(68.18%boys,31.82%girls;median age:7.21 years),composed of a nonerosive reflux disease(referred to as NERD)group(n=41)and a reflux esophagitis group(n=25).Upper gastrointestinal endoscopy with a mucosal esophageal biopsy was performed on all patients.RESULTS The most frequently observed symptom in EA patients with GERD and without GERD was cough(70%and 76.5%respectively).The number of patients with positive symptom association probability in the EA groups was significantly larger in the EA without GERD group(P=0.03).In the control reflux esophagitis group,prevalence of gastrointestinal symptoms was significantly higher than in the NERD group(P=0.017).For both EA groups,there was strong correlation with index of proximal events(IPE)and total proximal events(EA with GERD:0.96,P<0.001;EA without GERD:0.97,P<0.001)but level of IPE was significantly lower than in GERD patients without any surgical treatment(P<0.001).Data on distal mean nocturnal baseline impedance were significantly different between the EA with GERD group(P<0.001)and the two control groups but not between EA without GERD and the two control groups.CONCLUSION Mean nocturnal baseline impedance may have diagnostic value for GERD in EA children after open surgical repair.IPE might be an additional parameter of pHimpedance monitoring.

Detection of reflux-symptom association in children with esophageal atresia by video-pH-impedance study

BACKGROUND Children with esophageal atresia(EA)have risk of gastroesophageal reflux disease(GERD),suggesting reflux monitoring for prompt management.AIM To evaluate GERD in children with EA and specific symptom association from combined Video with Multichannel Intraluminal Impedance and pH(MII-pH)study.METHODS Children diagnosed with EA with suspected GERD and followed up at King Chulalongkorn Memorial Hospital between January 2000 and December 2018 were prospectively studied.All underwent esophagogastroduodenoscopy with esophageal biopsy and Video MII-pH study on the same day.Symptoms of GERD which included both esophageal and extra-esophageal symptom were recorded from video monitoring and abnormal reflux from MII-pH study based on the statement from the European Paediatric Impedance Group.Prevalence of GERD was also reported by using histopathology as a gold standard.Endoscopic appearance was recorded using Los Angeles Classification and esophagitis severity was graded using Esohisto criteria.RESULTS Fifteen children were recruited with age of 3.1(2.2,9.8)years(40%,male)and the common type was C(93.3%).The symptoms recorded were cough(75.2%),vomiting(15.2%),irritability or unexplained crying(7.6%)and dysphagia(1.9%)with the symptom-reflux association of 45.7%,89%,71%and 0%,respectively.There were abnormal endoscopic appearance in 52.9%,esophagitis in 64.7%and high reflux score in 47.1%.Video MII-pH study has high diagnostic value with the sensitivity,specificity and accuracy of 72.7%,100%and 82.4%,respectively.CONCLUSION Prevalence of GERD in children with EA was high.Video MII-pH study to detect GERD in children with EA had high diagnostic value with the trend of specific symptom association.

Endoscopic Detection and Surgical Repair of Congenital Tracheo-Esophageal-Fistula(TEF)±Esophageal Atresia(EA)

Purpose: This study was performed to evaluate the management of tracheoesophageal fistula (TEF) ± esophageal atresia (EA) under the guidance of preoperative tracheo-bronchoscopy (TrSc). Methods: Between 2007 and July 2014, a total of 26 consecutive newborns who underwent rigid TrSc for suspected TEF were identified. All associated charts and operation reports were retrospectively analyzed. Results: Distal TEF with EA (Gross C) predominated (n = 18). Furthermore, we managed 2 infants with proximal and distal TEF (Gross D) and 4 infants with isolated TEF (Gross E). In our hands, TrSc was feasible in infants with a birth weight above 1300 g. Twenty-five fistulas were identified by endoscopy in 23 patients. In one infant with a birth weight below 1000 g, an attempt to perform TrSc was interrupted, and urgent TEF closure was required. Fistula site at the carina was associated with a high rate of esophageal anastomosis under tension. During surgery, proximal TEF and isolated TEF were safely approached via right cervicotomy (n =5). Conclusion: This study supports the routine use of rigid TrSc at the time of surgery. Rigid TrSc allowed the surgical team to identify the number and location of TEFs, and the incidence of side effects was low.

Four cancer cases after esophageal atresia repair: Time to start screening the upper gastrointestinal tract

Esophageal atresia(EA) is one of the most common congenital digestive malformations and requires surgical correction early in life. Dedicated centers have reported survival rates up to 95%. The most frequent comorbidities after EA repair are dysphagia(72%) and gastroesophageal reflux(GER)(67%). Chronic GER after EA repair might lead to mucosal damage, esophageal stricturing, Barrett's esophagus and eventually esophageal adenocarcinoma. Several long-term follow-up studies found an increased risk of Barrett's esophagus and esophageal carcinoma in EA patients, both at a relatively young age. Given these findings, the recent ESPGHAN-NASPGHAN guideline recommends routine endoscopy in adults born with EA. We report a series of four EA patients who developed a carcinoma of the gastrointestinal tract: three esophageal carcinoma and one colorectal carcinoma in a colonic interposition. These cases emphasize the importance of lifelong screening of the upper gastrointestinal tract in EA patients.

Role of preoperative tracheobronchoscopy in newborns with esophageal atresia:A review

Preoperative tracheobronchoscopy(TBS) in the diag-nostic assessment of newborns affected by esophageal atresia(EA) was described in 1981. Nevertheless, the value of the procedure is actually much debated; only a few studies have clearly explored the advantages of TBS and this procedure is not yet routinely included inthe diagnostic and therapeutic assessment in many in-ternational pediatric surgery settings. Routine preoper-ative TBS is a safe procedure that enables the accurate examination of the tracheobronchial tree, the visualiza-tion of tracheoesophageal fistula and the diagnosis of tracheomalacia or associated respiratory anomalies. When a distal fistula is found, its occlusion with a Fog-arty balloon catheter improves mechanical ventilation and facilitates surgical repair. This review provides a detailed overview on the use of TBS in newborns with EA, focusing on technical aspects, anesthesiological management, indications and limits. The benefits and risks of the procedure are also compared with alterna-tive diagnostic tools, such as an esophageal contrast study, computed tomography scan and ultrasound.

Anesthetic Consideration on Neonatal Patient with Esophageal Atresia

Esophageal atresia is a disorder of the esophageal continuity with or without a connection with the trachea or the under-development esophagus. Most babies who suffer from esophageal atresia also have tracheoesophageal fistula (an abnormal connection between the esophagus and the trachea/windpipe). The possibility of these two conditions is accompanied by other anomalies such as congenital heart disease and anorectal malformations. Esophageal atresia can also be interpreted as malformations caused by the failure of the esophagus to develop a continual passage that may or may not establish a connection to the trachea (tracheoesophageal fistula) or it can be said that the esophageal atresia is the failure of the esophagus to form a channel from the pharynx to the stomach during embryonic development. Another understanding of esophageal atresia is an interruption in the growth of a segment of the esophagus and remains as thin sections without continual channels. Tracheal esophageal fistula is an abnormal connection between the trachea and esophagus. Miscellaneous types of esophageal atresia include: Type A. The incidence of Type A esophageal atresia was about 5% - 8%. Type A esophageal atresia occurs at each end of the esophageal sac, and is apart without fistula to the trachea. Type B incident is rare. Type B esophageal atresia occurs at each end of the esophagus, and there is fistula from the trachea to the upper segment of esophagus. Incidence of Type C esophageal atresia occurs about 80% - 95%. Type C esophageal atresia occurs at the proximal esophageal segment and ends at a dead-end sac, and distal segments are connected to the trachea or primary bronchus. Type D esophageal atresia is rare. In this type, upper and lower segment of the esophagus is connected to the trachea. Type E esophageal atresia is rare when compared with Types A and C esophageal atresia. In this type the esophagus and trachea are normal but they are connected with fistula.

Preoperative Tracheoscopy for Esophageal Atresia and/or Congenital Esoaerian Fistula: Experience at Cliniques Universitaires Saint Luc in Brussels

Introduction: Congenital septal anomalies between the trachea and the esophagus are rare conditions. It seemed to us interesting to recall the contribution of tracheoscopy in the diagnostic and therapeutic strategy of esophageal atresia and congenital esoaerian fistulas. Patients and methods: This is a retrospective study between June 1994 and June 2014 of children who underwent a tracheoscopy, at the Saint-Luc University Clinics in Brussels, the diagnostic set of esophageal atresia (EA) or a congenital esoaerian fistula was suspected. Results: A total of 43 children with esophageal atresia or congenital esoaerian fistula underwent tracheoscopy. Before the tracheoscopy, the diagnosis of the anatomical type of atresia of the esophagus and esoaerian fistula was as follows: type C, 34 (79.1%);type A, 4 (9.3%);type E, 5 (11.6%). After performing the tracheoscopy, the diagnosis was changed as follows: type C, 34 (79.1%);type A, 3 (7%);type E, 4 (9.3%);type B, 1 (2.3%), a patient with a tracheoesophageal laryngo cleft (2.3%). Tracheoscopy also made it possible to find 2 cases of tracheomalacia, 2 cases with 3 bronchial tubes and one case associating a diverticulum of the trachea. No complications were correlated with the performance of the tracheoscopy. Conclusion: Our study confirms the benefits of tracheoscopy in the laden price of esophageal atresia or congenital esoaerian fistula.

Surgical and Anaesthesiology Management of Esophageal Atresia: What Are the Mortality Factors in a Developing Country?

Esophageal atresia is an extreme neonatal surgical emergency whose mortality remains high in our country. We report 8 cases collected over 7 years in a tertiary hospital in Abidjan, C?te d’Ivoire. The purpose of the study is to identify the elements of surgical and anaesthesiological man-agement in our department that influences mortality.

Current profile and outcome of 100 esophageal atresia patients in the Kyushu area of Japan

Objectives: Since Spitz et al. reported the prognostic classification of esophageal atresia (EA) patients in 1994, decades have been past and there have been many advances in surgery and neonatology. Nevertheless, there have been very few reports according to the recent outcome of the neonates with EA, and otherwise, time has come to re-evaluate the credibility of this classification. The aim of this study was to validate the recent prognosis of the EA. Methods: Patient data were collected from 22 cooperative facilities during the 5 year period from 2005 to 2009 in Kyushu area, Japan. Total of 100 EA patients were retrospectively reviewed according to their characteristics and the outcome. Patient who missed the characteristics and outcome was excluded from the respective data. Results: Only 29.8% (28/94) was prenatally diagnosed and 52.0 (52/100) had associated anomalies including major congenital heart disease (CHD), abnormal chromosome, and others. According to the operation, primary anastomosis was performed 57.0% (57/100) and the staged operation was performed 34.0% (34/100). Survival rate in the neonatal period was 89.0% (89/ 100), and overall survival rate was 78.0% (78/100). According to the Spitz classification, if patients with associated anomalies were excluded, survival rate of Group 1 (>1500 g and no CHD) was 93.8% (61/65), Group 2 (<1500 g or CHD) was 68.4% (13/19), and Group 3 (<1500 g and CHD) was 50% (1/2). Conclusion: EA was proved to be rarely diagnosed prenatally. Primary outcome of the Group 1 and Group 3 in Spitz classification were fairly good, but Group 2 was worse as ever. The comprehensive treatment strategy for EA patients with birth weight under 1500 g or CHD should be reconsidered to improve the overall outcome.

High-Resolution Esophageal Manometry in Teenagers with Esophageal Atresia

Introduction: Children with surgically repaired esophageal atresia (EA) show esophageal dysmotility. Due to the performance of high-resolution manometry (HRM), three motility alteration patterns have been described, which allowed to know the segmental alterations. Objective: To describe the esophageal motility patterns found through HRM in teenagers with EA and to relate these with the associated esophageal pathology and its severity. Materials and Method: Ten teenagers were included with no history of esophageal blockage or dilations in the last six months, who were orally fed and asymptomatic. Through performance of HRM, we found surgical and endoscopic history, as well as of esophageal biopsies and pH monitoring. Results: We found the following patterns: aperistalsis, pressurization and distal contraction. 70% showed distal contraction, and 100% of esophageal endoscopies and biopsies were normal. 57% of the esophageal pH monitoring analyzed was pathologic. In the pressurization and aperistalsis groups, we observed severe esophagitis and requirement of Nissen antireflux procedure in 100% of the cases. Esophageal pH monitoring analyzed was 100% pathologic. Conclusion: We described the esophageal segmental alterations in teenagers with atresia by means of HRM. The distal contraction group showed better development, without severe esophagitis or requirement of antireflux procedure. Therefore, by performing an HRM in teenagers with EA, we could predict the future esophageal behavior, according to the peristaltic pattern, since there are significant differences among the groups in study.

Diagnose and treatment for Type D congenital esophageal atresia with tracheoesophageal fistula

Importance:Type D esophageal atresia(EA)with tracheoesophageal fistula(TEF)is characterized by EA with both proximal and distal TEFs.It is a rare congenital anomaly with a very low incidence.Objective:To investigate diagnostic and treatment strategies for this rare condition.Methods:We retrospectively reviewed the clinicopathological features of patients with EA/TEF treated at our institution between January 2007 and September 2021.Results:Among 386 patients with EA/TEF,14(3.6%)had type D EA/TEF.Only two patients were diagnosed with proximal TEF preoperatively.Seven patients were diagnosed intraoperatively.Five patients were missed for diagnosis during the initial surgery but was later confirmed by bronchoscopy.During the neonatal period,seven patients underwent a one-stage repair of proximal and distal TEF via thoracoscopy or thoracotomy.Due to missed diagnosis and other reasons,the other 7 patients underwent two-stage surgery for repair of the proximal TEF,including cervical incision and thoracoscopy.Ten of the 14 patients experienced postoperative complications including anastomotic leakage,pneumothorax,esophageal stricture,and recurrence.Patients who underwent one-stage repair of distal and proximal TEF during the neonatal period showed a higher incidence of anastomotic leak(4/7).In contrast,only one of seven patients with two-stage repair of the proximal TEF developed an anastomotic leak.Interpretation:Type D EA/TEF is a rare condition,and proximal TEFs are easily missed.Bronchoscopy may aim to diagnose and determine the correct surgical approach.A cervical approach may be more suitable for repairing the proximal TEF.

Conjugated hyperbilirubinemia presenting in first fourteen days in term neonates

AIM To describe the etiology and characteristics of earlyonset conjugated hyperbilirubinemia(ECHB) presenting within 14 d of life in term neonates.METHODS Retrospective review was performed of term infants up to 28-d-old who presented with conjugated hyperbilirubinemia(CHB) at a tertiary center over a 5-year period from January 2010 to December 2014. CHB is defined as conjugated bilirubin(CB) fraction greater than 15% of total bilirubin and CB greater or equal to 25 μmol/L. ECHB is defined as CHB detected within 14 d of life. "Late-onset" CHB(LCHB) is detected at 15-28 d of life and served as the comparison group.RESULTS Total of 117 patients were recruited: 65 had ECHB, 52had LCHB. Neonates with ECHB were more likely to be clinically unwell(80.0% vs 42.3%, P < 0.001) and associated with non-hepatic causes(73.8% vs 44.2%, P = 0.001) compared to LCHB. Multifactorial liver injury(75.0%) and sepsis(17.3%) were the most common causes of ECHB in clinically unwell infants, majority(87.5%) had resolution of CHB with no progression to chronic liver disease. Inborn errors of metabolism were rare(5.8%) but associated with high mortality(100%) in our series. In the subgroup of clinically well infants(n = 13) with ECHB, biliary atresia(BA) was the most common diagnosis(61.5%), all presented initially with normal stools and decline in total bilirubin but with persistent CHB. CONCLUSION Secondary hepatic injury is the most common reason for ECHB. BA presents with ECHB in well infants without classical symptoms of pale stools and deep jaundice.