Outcomes and prognostic factors for survival of children with oesophageal atresia.

INTRODUCTION: Oesophageal atresia (EA) is a life-threatening congenital oesophageal deformity that causes considerable newborn morbidity and death. Many prognostic variables have been linked to the survival of infants with EA, although the results of the studies are still conflicting. Furthermore, studies on EA effects in developing countries still need to be included. Here, we aimed to determine the survival of children with EA and link it to prognostic variables in a particular developing country. MATERIALS AND METHODS: A cross-sectional observational retrospective study was conducted using medical records of paediatric patients with EA at our institution from January 2014 to December 2020. RESULTS: A total of 53 children with EA were included in the study. Log-rank analysis showed that definitive surgery and thrombocytopenia were significantly associated with the survival of children with EA, with a p-value of 0.007 and 0.002, respectively, whereas, sex, EA type, pneumonia and sepsis were not (p = 0.898, 0.919, 0.255, and 0.499, respectively). Multivariate analysis revealed that thrombocytopenia and definitive surgery were strongly associated with the survival of children with EA with a pvalue of 0.014 (hazard ratio (HR) = 2.67 [95% confidence interval (CI) = 1.22-5.85]) and 0.022 (HR =0.39 [95% CI = 0.17- 0.87]), respectively. CONCLUSION: Our study shows that thrombocytopenia might increase mortality, while definitive surgery might be beneficial for the survival of paediatric patients with EA. It implies that definitive surgery should be performed as early as necessary to prevent further morbidity and mortality. Our study comprehensively provides the survival of children with EA and links it to prognostic variables in a particular developing country. It serves as a potential research project that can be applied to the clinical setting to help clinicians manage EA better.

Risk Factors of Early Mortality and Morbidity in Esophageal Atresia with Distal Tracheoesophageal Fistula: A Population-Based Cohort Study.

OBJECTIVE: To identify the risk factors for early mortality and morbidity in a population with distal esophageal atresia (EA)-tracheoesophageal fistula. STUDY DESIGN: Cohort study from a national register. Main outcomes and measures included early mortality, hospital length of stay (LoS), need for nutritional support at 1 year of age as a proxy measure of morbidity, and complications during the first year of life. RESULTS: In total, 1008 patients with a lower esophageal fistula were included from January 1, 2008, to December 31, 2014. The survival rate at 3 months was 94.9%. The cumulative hospital LoS was 31.0 (17.0-64.0) days. Multivariate analysis showed that intrahospital mortality at 3 months was associated with low birth weight (OR 0.52, 95% CI [0.38-0.72], P < .001), associated cardiac abnormalities (OR 6.09 [1.96-18.89], P = .002), and prenatal diagnosis (OR 2.96 [1.08-8.08], P = .034). LoS was associated with low birth weight (-0.225 ± 0.035, P < .001), associated malformations (0.082 ± 0.118, P < .001), surgical difficulties (0.270 ± 0.107, P < .001), and complications (0.535 ± 0.099, P < .001) during the first year of life. Predictive factors for dependency on nutrition support at 1 year of age were complications before 1 year (OR 3.28 [1.23-8.76], P < .02) and initial hospital LoS (OR 1.96 [1.15-3.33], P < .01). CONCLUSIONS: EA has a low rate of early mortality, but morbidity is high during the first year of life. Identifying factors associated with morbidity may help to improve neonatal care of this population.

Predictors of Mortality after Primary Discharge from Hospital in Patients with Esophageal Atresia.

OBJECTIVE: To describe esophageal atresia mortality rates and their associations in our cohort. STUDY DESIGN: Patients with esophageal atresia, managed at The Royal Children's Hospital, Melbourne (1980-2018), who subsequently died, were retrospectively identified from the prospective Nate Myers Oesophageal Atresia database. Data collected included patient and maternal demographics, vertebral anomalies, anorectal malformations, cardiovascular anomalies, tracheoesophageal fistula, renal anomalies, and limb defects (VACTERL) associations, mortality risk factors, and preoperative, operative, and postoperative findings. Mortality before discharge was defined as death during the initial admission. RESULTS: A total of 88 of the 650 patients (13.5%) died during the study period; mortality before discharge occurred in 66 of the 88 (75.0%); mortality after discharge occurred in 22 of the 88 (25.0%). Common causes of mortality before discharge were palliation for respiratory anomalies (15/66 [22.7%]), associated syndromes (11/66 [16.7%]), and neurologic anomalies (10/66 [15.2%]). The most common syndrome leading to palliation was trisomy 18 (7/66 [10.6%]). Causes of mortality after discharge had available documentation for 17 of 22 patients (77.3%). Common causes were respiratory compromise (6/17 [35.3%]), sudden unexplained deaths (6/17 [35.3%]), and Fanconi anemia (2/17 [11.8%]). Of the patients discharged from hospital, 22 of 584 (3.8%) subsequently died. There was no statistical difference in VACTERL association between mortality before discharge (31/61 [50.8%]) and mortality after discharge (11/20 [55.0%]), nor in incidence of twins between mortality before discharge (8/56 [14.3%]) and mortality after discharge (2/18 [11.1%]). CONCLUSIONS: We identified predictors of mortality in patients with esophageal atresia in a large prospective cohort. Parents of children with esophageal atresia must be counselled appropriately as to the likelihood of death after discharge from hospital.

30-Day Outcomes Following Esophageal Replacement in Children: A National Surgical Quality Improvement Project Pediatric Analysis.

INTRODUCTION: The optimal method of esophageal replacement remains controversial. The aim of this study was to evaluate 30-d outcomes of children in the National Surgical Quality Improvement Project Pediatric (NSQIP-P) database who underwent esophageal replacement from 2012 to 2018. METHODS: Demographics, comorbidities, and procedural technique was identified in NSQIP-P and reviewed. Thirty-day outcomes were assessed and stratified by gastric pull-up or tube interposition versus small bowel or colonic interposition. Categorical and continuous variables were assessed by Pearson's chi-square, Fisher's exact, and Wilcoxon rank-sum tests, respectively. Multivariate logistic regression was performed to estimate the effects of procedure technique and clinical risk factors on patient outcomes. RESULTS: Of the 99 cases of esophageal replacement included, 52 (52.5%) utilized a gastric conduit, whereas 47 (47.5%) involved small bowel/colonic esophageal interposition. Overall risk of complications was 52.5%, the most common of which were perioperative transfusion (30.3%), surgical site infection (11.1%), and sepsis (9.1%). Risk of unplanned reoperation was 17.2%, and risk of mortality was 3.0%. Risk for complications, reoperation, and readmission did not differ significantly between those who underwent gastric esophageal replacement and those who underwent small bowel or colonic interposition. Median operative time was shorter in the gastric esophageal replacement group (5.2 versus 8.1 h, P = 0.009). CONCLUSIONS: Among children in NSQIP-P who underwent esophageal replacement from 2012 to 2018, the risk of 30-d complications, unplanned reoperation, and mortality was relatively frequent and was similar across operative techniques. Opportunities exist to improve preoperative optimization, utilization of blood transfusion services, and infectious complications in the perioperative period irrespective of operative technique. LEVEL OF EVIDENCE: Level III, retrospective comparative study.

Esophageal atresia: Associated anomalies, mortality, and morbidity in Jordan.

BACKGROUND: Esophageal atresia is a developmental disorder in which the upper and lower esophagus fail to connect. It has an estimated prevalence of 1 in 2,500-4,500 live births and has poorer outcomes in low- and middle-income countries than in high-income countries. This study focused on the disorder's epidemiology, morbidity, and mortality in Jordan to address the lack of data regarding esophageal atresia in this country. METHODS: This was a retrospective study covering a 16-year period at a tertiary care academic hospital. Data were extracted from archived medical records and operative notes. All patients who had complete congenital esophageal atresia data were included. In total, the records of 55 patients were analyzed. RESULTS: Of the included patients, 9% were diagnosed prenatally and 47% were diagnosed with polyhydramnios. The mean gestational age was 37 weeks, the mean birthweight was 2,550 g, and 60% of patients were male. Isolated cases of esophageal atresia were reported in 58.2% of patients. There was a high rate of associated congenital anomalies (41.8%), with cardiac lesions the most common (20%), and 5.5% were syndromic. Parental consanguinity was found in 18.2% of patients. Postoperative surgical-related morbidities included stricture (18/24; 75%) and leakage (5/24; 20.8%). Fistula recurrence occurred in one patient (4.2%). The mortality rate was 12.8%. CONCLUSION: Esophageal atresia causes a high rate of mortality and exhibits post-operative morbidities. Moreover, associated anomalies were frequently observed. A high level of the malformation was found among offspring from consanguineous marriages.

Esophageal atresia and tracheoesophageal fistula associated with tetralogy of Fallot: a review of mortality.

INTRODUCTION: Oesophageal atresia ± tracheoesophageal fistula (EA/TEF) associated with congenital heart disease (CHD) carries a worse prognosis than EA/TEF alone. Though the Spitz classification takes major CHD into account, there are no data regarding survival with the specific combination of EA/TEF and Tetralogy of Fallot (TOF). With advances in postnatal care, we hypothesised that, survival is improving in these complex patients. This study reports morbidity and mortality outcomes of newborns with oesophageal atresia and TOF cardiac malformations METHODS: All patients with EA/TEF and TOF treated at Alder Hey Children's Hospital between the years 2000-2020, were identified. Data sets regarding gestation, birth weight, associated anomalies, operative intervention, morbidity, and mortality were analysed. RESULTS: Of a total of 350, EA/TEF patients 9 (2.6%) cases had EA/TEF associated with TOF (M:F 4:5). The median gestational age was 35/40 (range 28-41 weeks) with a median birth weight of 1790 g (range 1060-3350 g). Overall survival was 56% (5/9 cases) and all survivors remain under follow up (range 37-4458 days). Surgical strategies for managing EA/TEF with Fallot's tetralogy included 6/9 primary repairs and 3/9 cases with TEF ligation only (+ gastrostomy ± oesophagostomy). CONCLUSIONS: This study reports outcome data from one of the largest series of EA TEF patients with Fallot's tetralogy. Whilst outcomes may be challenging for this unique patient cohort, survival metrics provide important prognostic information that can be widely shared with health care teams and parents.

Management of esophageal atresia and early predictive factors of mortality and morbidity in a developing country.

The aim of this study is to investigate management and outcome in esophageal atresia (EA) and to identify early predictive factors of morbidity and mortality in a developing country. Charts of neonates with repaired EA from 2007 to 2016 were reviewed. Patients' characteristics, operative details, and postoperative outcomes were collected. Statistical analyses were performed to identify predictors of complicated evolution. Forty-two cases were collected. There were 14 girls and 28 boys. Only one patient had antenatal diagnosis (2.3%). The mean gestational age was 38 weeks. Nine patients (21.4%) weighed less than 2.5 kg. Seventeen (40.4%) patients had associated malformations most commonly cardiac (9/17). Thirteen patients had delayed diagnosis (30.9%). Thirty-nine (92.8%) patients underwent primary esophageal anastomosis. Overall survival was 76.2%. Nineteen patients (57% of survivals) had complicated evolution before the age of one year and 15 patients (46.8% of survivals) developed complications after the age of one year. Perinatal variables associated with mortality were prematurity (p = 0.004, OR = 5.4, IC95% = [1.13-25.80]), low birth weight (p = 0.023, OR = 7, IC95% = [1.38-35.47]), cardiac malformations (p = 0.006, OR = 10.5, IC95% = [2.03-54.27]) and delayed diagnosis (p = 0.005, OR = 10.11, IC95% = [2.005-50.980]). Variables associated with short-term and middle-term complications were duration of intubation (p = 0.019, OR = 0.118, IC95% = [0.019-0.713]) and the presence of short-term complications (p = 0.016, OR = 7.33, IC95% = [1.467-36.664]) respectively. These factors may be used to identify patients who will benefit from more intensive follow-up program.

Management and outcome of neonates with a prenatal diagnosis of esophageal atresia type A: A population-based study.

OBJECTIVE: Evaluate the neonatal management and outcomes of neonates with prenatal diagnosis of esophageal atresia (EA) type A. METHODS: This population-based study was conducted using data from the French National Register for infants with EA born from 2008 to 2014, including all cases of EA type A. We compared prenatal and neonatal characteristics and outcomes in children with prenatal diagnosis of EA type A with those with a postnatal diagnosis until the age of 1. RESULTS: A total of 1118 live births with EA were recorded among which 88 (7.9%) were EA type A. Prenatal diagnoses were performed in 75 cases (85.2%), and counselling with a prenatal specialist was conducted in 84.8% of the prenatal group. Still within that group, the gestational age at delivery was significantly higher than in the postnatal group (36 [35-38] versus 34 [32-36] weeks; P = .048). Inborn births were more frequent in the prenatal group (86.1% vs 7.7%, P < .0001), and mortality and outcome were similar in both groups. CONCLUSION: Prenatal diagnosis is high in EA type A, which enables to offer an antenatal parental counseling and which avoids postnatal transfers. Prognosis of EA types A does not appear to be influenced by the prenatal diagnosis.

New prognostic classification and managements in infants with esophageal atresia.

PURPOSE: The aim of this study was to investigate the risk factors for in hospital mortality in infants with esophageal atresia (EA) without critical chromosome abnormality disorders and develop a new prognostic classification to assess the influence of external risk factors on the prognosis of EA, which could provide more effective treatment strategies in clinical practice. METHODS: A retrospective chart review of all consecutive patients between January 1994 and December 2017, which included 65 EA infants who were admitted to Shizuoka Children's Hospital, was conducted. Based on multivariate analysis data and ROC analysis, the discrimination of the new prognostic classification was quantified and compared with that of the Spitz classification using the area under the ROC curve (AUC). RESULTS: Multiple logistic regression analysis showed that birth weight of < 1606 g (adjusted OR, 13.16; 95% CI, 1.16-352.75), and complex cardiac anomalies (adjusted OR 22.39; 95% CI 2.45-569.14) were significant risk factors for death. We have created a new classification close to Spitz classification using the presence of complex cardiac anomalies and birth weight. The mortality rates were 0% for class I (n = 0/40), 7.1% for class II (n = 1/14), 33.3% for class III (n = 3/9), and 100% for class IV (n = 2/2). The AUC of the new classification was better than that of the Spitz classification (0.939 vs 0.812, respectively; p = 0.04). CONCLUSION: New prognostic classification can improve the stratification of EA patients and be a useful predictor of survival.

Mortality and morbidity in oesophageal atresia.

BACKGROUND: Several classification systems exist to predict mortality in oesophageal atresia, the most widely quoted of these being over 20 years old. No classification system exists to predict morbidity. We sought to test whether these classification systems remain relevant and to determine whether they can be useful to predict morbidity. In addition, we aimed to identify independent risk factors for predicting mortality and morbidity. METHODS: Neonates presenting with oesophageal atresia over a 20-year period (1990-2010) were retrospectively reviewed. Discriminative statistical analysis compared the performance of current classification systems. Stepwise logistic regression analysis of the influence of perioperative risk factors on mortality and duration of ventilatory support and intensive care unit stay were performed. RESULTS: All classification systems predicted mortality in this series of 248 neonates. Birth weight, cardiac anomalies and pre-operative pneumonia were independent risk factors for predicting mortality in oesophageal atresia. The Waterston classification is the most useful classification for predicting post-operative morbidity in terms of length of hospital stay and time spent ventilated. CONCLUSION: Despite advances in the neonatal care of the very low birth weight infant and those with congenital cardiac disease, these conditions remain relevant in predicting mortality and morbidity in oesophageal atresia.

Prevalence, characteristics, and survival of children with esophageal atresia: A 32-year population-based study including 1,417,724 consecutive newborns.

BACKGROUND: Esophageal atresia (EA) is a congenital malformation of the upper gastrointestinal tract with an estimated prevalence varying from 1 in 2500 to 1 in 4500 births. The aim of this study was to describe the epidemiology of EA between 1981 and 2012 and evaluate patients' survival. METHODS: This study used data from a population-based Italian Congenital Malformation Registry. The survival status was ascertained by linking the registry records, vital records and the regional registries of patients. Kaplan-Meier methods were used to estimate survival probabilities up to 25 years and Cox proportional hazards regression was used to evaluate factors that affected survival. RESULTS: A total of 407 cases of EA were identified among 1,417,724 total births. After the exclusion of cases with chromosomal anomalies, 49.9% of the patients presented with at least one associated congenital anomaly. The 25-year survival probability was 85.1% (95% confidence interval [CI], 80.8-89.4), with most deaths occurring during the first months of life. Patients' characteristics associated with decreased survival probability were low birth weight (hazard ratio, 3.7; 95% CI, 1.7-8.3) and presence of additional major defects (hazard ratio, 2.8; 95% CI, 1.3-6.0). A significant improvement in survival over the decades was observed for patients with nonisolated EA. CONCLUSION: This study detected a significant improvement in survival of individuals with EA over the past decades and identified the strongest predictors of mortality. These results will be important for the planning of the clinical management and formulation of prognosis when EA is diagnosed in a newborn. Birth Defects Research (Part A) 106:542-548, 2016. © 2016 Wiley Periodicals, Inc.

Delayed diagnosis: An important prognostic factor for oesophageal atresia in developing countries.

AIM: The aim of this study is to analyse the effect of delayed diagnosis on mortality rates, and evaluate the role of delayed diagnosis as a new prognostic factor in patients with oesophageal atresia (OA), especially in developing countries. METHODS: The records of 80 consecutive patients with OA (2008-2013) were reviewed. Patients were divided into two groups according to the time of diagnosis. As we demonstrated the effect of delayed diagnosis on mortality, we decided to develop a new classification that will be utilised to predict the prognosis of OA. The discrimination ability of the new prognostic classification was compared with those of the Waterston, Montreal and Spitz classifications using the area under the curve. RESULTS: The parameters of the new prognostic classification were birth weight less than 2000 g, the presence of major cardiac/life-threatening anomalies and delay in diagnosis. Class I consisted of patients with none or one of these parameters. Class II consisted of patients with two or three of these parameters. The area under the curve of the new classification was better than those of the other classifications in determining the prognosis of patients with OA. CONCLUSIONS: Delayed diagnosis of OA significantly led to morbidity and mortality. Although delayed diagnosis is not a characteristic of newborn or a marker of severity for OA and is a health care system issue in developing countries, we here point out that it is a prognostic factor in its own right. Our new classification has a superior discriminatory ability compared to the above-mentioned classifications.

Oesophageal atresia is correctable and survivable in infants less than 1 kg.

INTRODUCTION: Management of oesophageal atresia (OA) and trachea-oesophageal fistula (TOF) in babies of low birth weight is challenging especially when associated with other anomalies. Birth weight of <1500 g has previously formed part of a classification system designed to predict outcome, alongside the cardiac status of the patient. Improvements in neonatal care have led to increasing numbers of premature low birth weight infants surviving. The aim of this study was to look at the experience of our institution in the extremely low birth weight (ELBW) patients. METHODS: A retrospective review of our institutions OA database was performed from 1993 to June 2015. Patients of birth weight less than 1000 g were included. A review of our OA/TOF clinical database and notes review established the following; gestation, birth weight, associated anomalies, operative procedures, morbidity and mortality. RESULTS: Of 349 patients with OA across the 22-year period, 9 ELBW patients were identified (<1000 g). Six males and three females. Gestational age ranged from 23 to 34 weeks and median birth weight was 815 g ranging from 630 to 950 g. Overall survival was 56 % (5/9). There were double the numbers of ELBW OA/TOF patients seen in the second half of the study period presumably the result of improving neonatal care. Seven patients had type C OA with TOF and underwent emergency TOF ligation, two had concomitant oesophageal repair. One of these patients died from NEC; the other survived. Of the five who had isolated TOF ligation three died-two from cardiac disease and one from prematurity. Both type A patients survived and after initial gastrostomy placement one had a primary delayed repair, the other a gastric transposition. All three babies under 800 g died-one from cardiac disease the others from conditions indicative of their prematurity-necrotising enterocolitis and intraventricular haemorrhage. CONCLUSIONS: 50 % survival is achievable in OA/TOF under 1 kg and the Spitz classification is still applicable in this group as a whole. However, none of the current classification systems are applicable in infants <800 g who in our study all had poor outcomes. We suggest these should be considered as separate group when predicting outcomes.

Does prenatal diagnosis modify neonatal treatment and early outcome of children with esophageal atresia?

OBJECTIVE: Our study aimed at (1) evaluating neonatal treatment and outcome of neonates with either a prenatal or a postnatal diagnosis of esophageal atresia (EA) and (2) analyzing the impact of prenatal diagnosis on outcome based on the type of EA. STUDY DESIGN: We conducted a population-based study using data from the French National Register for infants with EA born from 2008-2010. We compared prenatal, maternal, and neonatal characteristics among children with prenatal vs postnatal diagnosis and EA types I and III. We defined a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and death at 1 year. RESULTS: Four hundred sixty-nine live births with EA were recorded with a prenatal diagnosis rate of 24.3%; 82.2% of EA type I were diagnosed prenatally compared with 17.9% of EA type III (P < .001). Transfer after birth was lower in case of prenatal diagnosis (25.6% vs 82.5%; P < .001). The delay between birth and first intervention did not differ significantly among groups. The defect size was longer among the prenatal diagnosis group (2.61 vs 1.48 cm; P < .001). The composite variables were higher in prenatal diagnosis subset (44% vs 27.6%; P = .003) and in EA type I than in type III (58.1% vs 28.3%; P < .001). CONCLUSION: Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity rate related to the EA type (type I and/or long gap). Even though it does not modify neonatal treatment and the 1-year outcome, prenatal diagnosis allows antenatal parental counselling and avoids postnatal transfers.

Health-related quality of life among children, young people and adults with esophageal atresia: a review of the literature and recommendations for future research.

PURPOSE: Esophageal atresia (EA) is a rare congenital malformation requisite of surgical treatment. Survival rates have significantly increased in recent decades, but treated children are at risk of chronic morbidity. This study aims to review the literature on health-related quality of life (HRQOL) in patients with EA and describe the questionnaires used. METHODS: A literature search was conducted in Pubmed, Cinahl and PsycINFO, from inception to January 2015. A meta-analysis of studies investigating HRQOL in patients with EA compared to healthy references was performed. The effect size was calculated as Cohen's d. RESULTS: Twelve articles (published 1995-2014) describing HRQOL among children, young people and adults were identified. Only European studies were found, and these had variable sample sizes (8-128). Overall HRQOL was reported to be reduced in five of the seven studies that compared overall HRQOL with a healthy reference population. Impaired physical or general health was described in eight articles. In the meta-analysis, eight eligible studies provided 16 estimates of the effect of EA, six of which reached statistical significance for worse HRQOL (p < 0.05). Using Cohen's criteria, the pooled estimate of the effect of EA was small for overall and physical HRQOL, and <0.2 for the mental and social HRQOL components. Altogether, 15 different questionnaires were used, and none were condition specific for EA. CONCLUSIONS: Different findings are reported; however, this study suggests that patients with EA may have a reduced HRQOL. Moreover, HRQOL is not adequately measured in this group. Additional research is required.

Performance of prenatal diagnosis in esophageal atresia.

OBJECTIVE: The aim of this study was to evaluate the performance of prenatal diagnosis of esophageal atresia (EA) and its associated abnormalities. METHODS: We conducted a retrospective study from a pediatric database of EA managed postnatally in a single center. Prenatal data included ultrasound and magnetic resonance imaging parameters including amniotic fluid (AF) volume, stomach visualization, AF biochemistry, and associated malformations. Postnatal data included type of EA, mortality, and postnatal diagnosis of associated malformations. RESULTS: One hundred twenty-two cases were included. The diagnosis was suspected prenatally in 39/122 (32%) cases. Polyhydramnios was noted in 64/122 (52.4%), and the stomach was not visualized or small in 39 (32%). There was 14 (11.5%), 2 (1.6%), 101 (82.8%), 5 (4.1%), and 0 (0%) types I, II, III, IV, and V, respectively. EA was suspected prenatally in 12/14 (85.7%) in type I and in 27/108 (25%) in cases with tracheoesophageal fistula (II + III + IV + V). Magnetic resonance imaging was performed in 28 cases, which confirmed EA in 19/28 (sensitivity 67.8%). AF biochemistry was performed in 17 cases, which confirmed EA in 15/17 (sensitivity 88.2%) cases. Of the 69 syndromic associations, 41/69 (59.4%) cases were detected prenatally. Associated malformation was a strong predictor of postnatal death [19/69 vs 3/53, odds ratio 6.33 (1.76; 22.75), p < 0.01]. CONCLUSION: Prenatal diagnosis of EA remains challenging. MRI and AF biochemistry may prove useful in the diagnosis of EA. Prenatal ultrasound and MRI examination should also focus on associated anomalies. © 2015 John Wiley & Sons, Ltd.

Esophageal atresia: a critical review of management at a single center in Algeria.

The purpose was to study the outcomes and factors affecting the survival of esophageal atresia in our center. A retrospective analysis of 86 cases of esophageal atresia (EA) over a 10-year period was performed with 46 boys and 42 girls. Demographic data, birth weight, gestational age, consanguinity, incidence of associated anomalies, place of delivery, history of feeding, and outcomes were studied. EA with distal tracheoesophageal fistula (TEF) was the commonest type with 58/86 (67%). The percentage of patients with at least one associated anomaly was 52/86 (60%), with 7/86 (8%) who are from consanguineous parents; most commonly associated anomalies were cardiac 13/86 (15%). The average gestational age and birth weight were 36 ± 2 weeks and 2300 ± 570 g, respectively. Survival rates for the patients according to the Waterston classification was 80% in group A, 58% in group B, and 25% in group C (three patients died before surgery). Prematurity, the gap between the two ends of the esophagus, and preoperative respiratory status were the most significant factors affecting the survival. Late complication of EA/TEF include respiratory symptoms, especially in the first year, associating tracheomalacia and bronchopulmonary infections in about 24/45 (53%), recurrence of TEF 3/45 (7%), esophageal stricture 26/45 (58%), and gastroesophageal reflux 22/45 (49%). The high incidence of delayed diagnosis, low birth weight, and lack of advanced neonatological management are important contributory factors to the poor outcome. The frequency of late complications highlights the need for multidisciplinary clinics to follow these children's.

Surgical intervention for esophageal atresia in patients with trisomy 18.

Trisomy 18 is a common chromosomal aberration syndrome involving growth impairment, various malformations, poor prognosis, and severe developmental delay in survivors. Although esophageal atresia (EA) with tracheoesophageal fistula (TEF) is a potentially fatal complication that can only be rescued through surgical correction, no reports have addressed the efficacy of surgical intervention for EA in patients with trisomy 18. We reviewed detailed clinical information of 24 patients with trisomy 18 and EA who were admitted to two neonatal intensive care units in Japan and underwent intensive treatment including surgical interventions from 1982 to 2009. Nine patients underwent only palliative surgery, including six who underwent only gastrostomy or both gastrostomy and jejunostomy (Group 1) and three who underwent gastrostomy and TEF division (Group 2). The other 15 patients underwent radical surgery, including 10 who underwent single-stage esophago-esophagostomy with TEF division (Group 3) and five who underwent two-stage operation (gastrostomy followed by esophago-esophagostomy with TEF division) (Group 4). No intraoperative death or anesthetic complications were noted. Enteral feeding was accomplished in 17 patients, three of whom were fed orally. Three patients could be discharged home. The 1-year survival rate was 17%: 27% in those receiving radical surgery (Groups 3 and 4); 0% in those receiving palliative surgery (Groups 1 and 2). Most causes of death were related to cardiac complications. EA is not an absolute poor prognostic factor in patients with trisomy 18 undergoing radical surgery for EA and intensive cardiac management.

A nationwide analysis of clinical outcomes among newborns with esophageal atresia and tracheoesophageal fistulas in the United States.

BACKGROUND: The aim of this study was to examine national outcomes in newborn patients with esophageal atresia and tracheoesophageal fistula (EA/TEF) in the United Sates. METHODS: Kids' Inpatient Database (KID) is designed to identify, track, and analyze national outcomes for hospitalized children in the United States. Inpatient admissions for pediatric patients with EA/TEF for kids' Inpatient Database years 2000, 2003, 2006, and 2009 were analyzed. Patient demographics, socioeconomic measures, disposition, survival and surgical procedures performed were analyzed using standard statistical methods. RESULTS: A total of 4168 cases were identified with diagnosis of EA/TEF. The overall in-hospital mortality was 9%. Univariate analysis revealed lower survival in patients with associated acute respiratory distress syndrome, ventricular septal defect (VSD), birth weight (BW) < 1500 g, gestational age (GA), time of operation within 24 h of admission, coexisting renal anomaly, imperforate anus, African American race, and lowest economic status. Multivariate logistic regression identified BW < 1500 g (odds ratio [OR] = 4.5, P < 0.001), operation within 24 h (OR = 6.9, P < 0.001), GA <28 wk (OR = 2.2, P < 0.030), and presence of VSD (OR = 3.8, P < 0.001) as independent predictors of in-hospital mortality. Children's general hospital and children's unit in a general hospital were found to have a lower mortality rate compared with not identified as a children's hospital after excluding immediate transfers (P = 0.008). CONCLUSIONS: BW < 1500 g, operation within 24 h, GA < 28 wk, and presence of VSD are the factors that predict higher mortality in EA/TEF population. Despite dealing with more complicated cases, children's general hospital and children's unit in a general hospital were able to achieve a lower mortality rate than not identified as a children's hospital.

Retrospective cohort study of long-gap oesophageal atresia.

AIM: Long-gap oesophageal atresia (LGOA) remains a rare variant of a relatively common congenital malformation. Objectives of this study were to review the short-term results including survival, length of stay and post-operative complications for infants with LGOA managed at a single centre in addition to their growth and neurodevelopmental assessment. METHODS: Retrospective review of the case notes of all infants admitted with oesophageal atresia to our institution from January 2001 to May 2011. Infants with LGOA were selected based on pre-defined criteria. Demographic and clinical variables and details of follow-up visits including developmental assessments were extracted from their case notes. RESULTS: Of 101 infants with oesophageal atresia, 15 fulfilled the criteria for LGOA. Overall survival was 80%. Median length of stay was 83 days. Additional congenital anomalies were present in nine (60%). A fall in weight centile during hospitalisation or outpatient follow-up signifying growth failure was seen in a majority with 11 of 13 patients showing this phenomenon. Follow-up at our institution ranged from 6 months to 9 years. Developmental assessments (Bayley-III) commenced in August 2006 were available in four patients (age 5-13 months) and were abnormal in all, with particular delay in the gross motor domain. CONCLUSIONS: Infants with LGOA spend a long time in hospital. They remain at significant risk of growth failure during hospitalisation and following discharge. There appears to be a risk of developmental delay that warrants close monitoring.