Esophageal atresia with distal tracheoesophageal fistula: Associated anomalies and prognosis in the 1980s

doi:10.1016/S0022-3468(89)80214-3

Journal of Pediatric Surgery

Volume 24, Issue 10, October 1989, Pages 1055-1059

https://doi.org/10.1016/S0022-3468(89)80214-3 Get rights and content

Ninety-seven newborns with esophageal atresia and distal tracheoesophageal fistula (EA-TEF) were treated between 1979 and 1985 inclusive; there were 54 boys and 43 girls. Their weights ranged from 800 to 4,000 g (average, 2.5 kg). They included: 28 neonates with cardiac defects (most common: patent ductus arteriosus [PDA], ventricular septal defect and atrial septal defect [VSD-ASD]), of whom 18 survived (64%); 17 babies with other gastrointestinal anomalies (imperforate anus, duodenal atresia), of whom 12 survived (70%); 12 patients with skeletal malformations (digital, vertebral), of whom 11 survived (91%); 8 newborns with genitourinary abnormalities (hypospadias, undescended testis), of whom 6 survived (75%); and 16 infants with other congenital lesions (trisomy 18, lung agenesis-hypoplasia), of whom 3 survived (18%). Forty-six infants (average, 2.7 kg) had no other anomalies and all survived. As the number of systems with defects increased, both the weight of the baby and survival rate decreased. From this entire series of 97 newborns with EA-TEF, 81 (83%) survived (average, 2.3 kg). Sixteen babies died (average, 1.9 kg); 11 had defects incompatible with life. Eleven of the 16 were never operated on (seven patients with trisomy died within 5 days, and four patients with complex cardiac defects died within 3 weeks). Four of the 16 who were operated on died between 3 months and 2 years from chest problems and one newborn died in the operating room. We concluded that (1) newborns who have the common type of EA-TEF will almost certainly survive if there are no other anomalies; (2) the most frequently associated congenital defects are cardiovascular (28%), gastrointestinal (17%), skeletal (12%), and genitourinary (8%); (3) as the number of systems with defects increases, the weight of the baby and its survival rate fall; and (4) 11% will have trisomy and/or complex cardiac defects with no survival.

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Esophageal atresia and congenital stenosis

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Should all babies with oesophageal atresia have routine screening for midgut malrotation anomalies? A systematic review in search of evidence
2022, Journal of Pediatric Surgery
Background/Purpose Oesophageal Atresia (OA) is associated with co-existent anomalies. There is a controversy of literature pertaining to the risk (s) of intestinal malrotation. In order to guide management we critically evaluate the incidence of IM anomalies in OA newborns.
Design MEDLINE and EMBASE databases were searched using keywords “(O)Esophageal Atresia and Malrotation/Associated Abnormalities/Associated Anomalies”. Full texts of articles were screened if manuscripts exclusively reported patients with OA malrotation and/or associated anomalies. Larger case series (> 10patients) were included if abstract (s) showed that associated anomalies were systematically assessed. Full eligibility criteria required at least one case of malrotation in an OA index case. Data were collected on article type, number of patients and method (s) of diagnosis.
Results 632 abstracts were screened of which 158 papers were analysed based on inclusion criteria-30 manuscripts documented the incidence (%) of malrotation. Incidence rate (s) were 0.5–13%. Malrotation was observed to have a higher incidence (10–44%) in OA babies with other gastrointestinal anomalies (VACTERL).
Conclusion Newborns with OA appear to be at a higher risk (%) of having intestinal malrotation anomalies than healthy babies. Prospective studies are required to accurately quantify and define the ‘ true incidence ‘ of this association. Given the potential lethal consequences of midgut volvulus screening may be justified in OA babies. Consensus guidelines (DELPHI) exploring surgeons attitudes with regards management of ‘ asymptomatic malrotation ‘ disorders in OA newborns may further guide best practice.
Newborn with annular pancreas and H-Type tracheoesophageal fistula
2021, Journal of Pediatric Surgery Case Reports
Esophageal atresia with tracheoesophageal fistula (EA/TEF) and annular pancreas are both rare congenital anomalies, with very few reports of neonates presenting with both. More specifically, there are no reported cases of neonates with both annular pancreas and the rarest form of TEF, the H-type fistula.
Here, we present the case of a neonate with annular pancreas diagnosed at birth requiring duodenoduodenostomy with delayed diagnosis of concomitant H-type TEF requiring subsequent fistula ligation with favorable outcomes.
When patients with known congenital anomalies have persistent symptoms of unidentified etiology, heightened suspicion for other concomitant congenital anomalies can lead to prompt diagnosis, timely surgical management and good outcomes.
Flexible endoscopic diagnosis and treatment of esophageal stenosis in children with noninvasive ventilation support
2018, Pediatrics and Neonatology
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Patients underwent an NIV technique of “nasopharyngeal oxygen with intermittent nose closure and abdominal compression, PhO2–NC–AC” for airway, oxygenation and ventilation support throughout all these FE procedures. A detailed description of using this NIV technique during FE was reported previously.3 Briefly, a pharyngeal pure oxygen flow of 0.5 L/kg/min was delivered via a nasopharyngeal catheter, for continuous support.
Esophageal stenosis (ES) is characterized by dysphagia, failure to thrive, and long-term disability. It requires repeated management because it is refractory. Traditionally, these procedures are performed under general anesthesia with airway intubation.
This study investigated the safety and efficacy of the management of interventional flexible endoscopy (IFE) performed with a novel noninvasive ventilation (NIV) support.
Use of a short-length flexible endoscope with NIV of pharyngeal oxygen, nose closure, and abdominal compression during IFE was investigated. Medical charts of patients aged ≤10 years with a diagnosis of ES in our hospital between 1990 and 2014 were reviewed and analyzed. The outcome measurement included the number of IFE with balloon dilatation (BD), laser therapy (LT), stent placement, procedural complications, and the success rate.
Ten patients were enrolled. The most common etiologies were esophageal atresia with/without tracheoesophageal fistula (n = 6), followed by caustic injury (n = 2), and unknown etiology (n = 2). Nine patients who were considered successfully managed received an average of 2.8 BD sessions and 1.6 LT sessions. The complication rate of IFE in this study was 1.08% (1/93). One esophageal perforation developed after BD (1/63) and none after LT (0/30).
In this study, IFE with this NIV support is a safe, feasible and valuable modality which could rapidly examine and manage ES.
Effect of Gastrointestinal Malformations on the Outcomes of Patients With Congenital Heart Disease
2017, Annals of Thoracic Surgery
Citation Excerpt :
Interestingly, patients with Down syndrome tended to have more abdominal GI malformations. These associations might contribute to some extent to the survival differences in both groups, because patients with thoracic GI malformations and VACTERL syndrome may have had additional comorbidities related to the syndrome [3, 6, 22]. This observation might suggest that survival might be influenced by the patients’ associated anatomic or functional abnormalities rather than only related to the GI malformation or the cardiac diagnosis.
The goal of this study was to assess the effect of associated gastrointestinal malformations (GI) on the outcomes of patients undergoing congenital heart operations.
Neonates and infants with thoracic (esophageal atresia, tracheoesophageal fistula) and abdominal (duodenal stenosis/atresia, imperforate anus, Hirschsprung disease) GI malformations undergoing congenital heart operations between 1995 and 2015 were included. Two control groups were created, one for each group. Patients were matched by diagnosis, procedure, history of prematurity, presence of genetic syndrome, and a propensity score including weight and year of operation.
The cohort included 383 patients: 52 (14%) with thoracic GI malformations and 98 (25%) thoracic GI controls, 80 (21%) with abdominal GI malformations and 153 (40%) abdominal GI controls. Median follow-up was 6 years (range, 16 days to 20 years). Patients with thoracic GI malformations had longer length of stay (p < 0.001), longer intubation times (p = 0.002), and higher perioperative death (p = 0.015) than controls. There was a tendency for worse overall survival than controls, mainly explained by the higher risk of early death (p = 0.06). No difference was found in outcomes between patients with abdominal GI malformations and controls.
Patients with thoracic GI malformations have worse perioperative outcomes than controls, but their long-term survival does not seem to be significantly different. Abdominal GI malformations do not have a significant effect on outcomes. The presence of GI malformations should likely not preclude patients from undergoing congenital heart operations, but careful family counseling is necessary, especially for thoracic GI malformations.
Pediatric surgical capacity in Africa: Current status and future needs
2017, Journal of Pediatric Surgery
Citation Excerpt :
In our survey, the mortality rate for common pediatric cancers was 50–60%, compared to less than 20% in high-income settings [31]. Similarly for congenital anomalies such as intestinal and oesophageal atresia and abdominal wall defects, developed countries report high survival rates (> 90%) [32–36], while late presentation and lack of access to care play a major role in the low survival rates (50–65%) we showed in Africa for such congenital conditions. For example, neonates with bowel obstruction in Africa often do not reach care for several days [37,38], resulting in malnutrition, sepsis, and high mortality.
African pediatric surgery (PS) faces multiple challenges. Information regarding existing resources is limited. We surveyed African pediatric surgeons to determine available resources and clinical, educational, and collaborative needs.
Members of the Pan-African Pediatric Surgical Association (PAPSA) and the Global Pediatric Surgery Network (GPSN) completed a structured email survey covering PS providers, facilities, resources, workload, education/training, disease patterns, and collaboration priorities.
Of 288 deployed surveys, 96 were completed (33%) from 26 countries (45% of African countries). Median PS providers/million included 1 general surgeon and 0.26 pediatric surgeons. Median pediatric facilities/million included 0.03 hospitals, 0.06 ICUs, and 0.17 surgical wards. Neonatal ventilation was available in 90% of countries, fluoroscopy in 70%, TPN in 50%, and frozen section pathology in 35%. Median surgical procedures/institution/year was 852. Median waiting time was 40 days for elective procedures and 7 days? for emergencies. Weighted average percent mortality for key surgical conditions varied between 1% (Sierra Leone) and 54% (Burkina Faso). Providers ranked collaborative professional development highest and direct clinical care lowest priority in projects with high-income partners.
The broad deficits identified in PS human and material resources in Africa suggest the need for a global collaborative effort to address the PS gaps.
Level 5, expert opinion without explicit critical appraisal.
Difficult esophageal atresia: Trick and treat
2014, Seminars in Pediatric Surgery
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Other authors27,28 also achieved satisfactory results via a right thoracotomy in all patients with a RAA, and they support this approach in all EA patients. Duodenal obstruction (DO) in the form of duodenal atresia or stenosis may be associated with EA/TEF in up to 6% of patients.29 Several treatment options have been proposed for this association.
Although most patients with esophageal atresia (EA) and tracheo-esophageal fistula (TEF) may benefit from “standard” management, which is deferred emergency surgery, some may present unexpected elements that change this paradigm. Birth weight, associated anomalies, and long gap can influence the therapeutic schedule of the patients with EA/TEF and can make their treatment tricky. As a consequence, detailed information on these aspects gives the power to develop a decision-making process as correct as possible. In this article, we will review the most important factors influencing the treatment of patients with EA/TEF and will share our experience on the diagnostic and therapeutic tips that may provide pivotal help in the management of such patients.

View all citing articles on Scopus

^*: Presented at the 20th Meeting of the Canadian Association of Paediatric Surgeons, Ottawa, Ontario, Canada, September 21–24, 1988.

¹: From the Division of General Surgery, Hospital for Sick Children, Toronto, Ontario, Canada.

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Esophageal atresia with distal tracheoesophageal fistula: Associated anomalies and prognosis in the 1980s*

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr

Lancet

J Pediatr

J Thorac Cardiovasc Surg

Esophageal atresia and tracheoesophageal fistula

The influence of cardiovascular malformations on the prognosis of esophageal atresia and distal tracheoesophageal fistula—A 15-year experience

Pediatr Surg Int

Esophageal atresia and congenital stenosis