Esophageal atresia: Prognostic classification revisited

doi:10.1016/j.surg.2009.01.017

Surgery

Volume 145, Issue 6, June 2009, Pages 675-681

https://doi.org/10.1016/j.surg.2009.01.017 Get rights and content

Background

Although the Spitz classification is the most widely used prognostic classification for esophageal atresia and/or tracheoesophageal fistula (EA), its discrimination ability remains unclear. We sought to develop a more accurate prognostic classification for EA.

Methods

The records of 121 consecutive infants with EA (1980–2005) were reviewed. The independent variables included 6 clinical characteristics, and the dependent variables were survival and mortality. Stepwise logistic regression analysis was used to construct models predicting mortality and create a revised prognostic classification. The discrimination abilities of the revised classification and the Spitz classification were compared using receiver-operating characteristic (ROC) curves.

Results

Birth weight and the presence of major cardiac anomalies were significant prognostic factors for mortality, and major cardiac anomalies affected mortality more than birth weight. The ROC curve for birth weight suggested that 2,000 g was an appropriate cutoff point. The Spitz classification was revised as follows: the revised class I (low-risk group) consisted of patients without major cardiac anomalies and birth weight >2,000 g; class II (moderate-risk group) consisted of patients without major cardiac abnormalities and birth weight <2,000 g; class III (relatively high-risk group) consisted of patients with major cardiac anomalies and birth weight >2,000 g; and class IV (high-risk group) consisted of patients with major cardiac anomalies and birth weight <2,000 g. The ROC comparisons showed that the revised classification provided a significant improvement (P = .049).

Conclusion

This revised classification can improve the stratification of EA patients and be a useful predictor of survival.

Section snippets

Patients and methods

The study involved 121 consecutive EA patients treated at Kobe Children's Hospital between January 1980 and December 2005. This study was done with the approval of the institutional review board (registered no. R20-3). The data collected included the anatomic type of EA, operative interventions, gestational week, weight at birth, presence of major cardiac anomalies, presence of associated anomalies other than cardiac anomalies, presence of chromosomal abnormalities, and patient outcomes. Major

Results

A total of 121 infants with EA were admitted to our institute over the 26-year period. Anatomic variations of total cases were as follows: EA + distal tracheoesophageal atresia (TEF), 101 (84%); EA + proximal TEF, 1 (1%); EA + distal and proximal TEF, 1 (1%); pure EA, 15 (12%); and H-type EA, 3 (3%). Among the 121 infants, there were 67 males and 54 were females. Average of birth weight of all cases was 2,330 ± 660 g, and the average gestational week was 37 ± 3 weeks. Spitz classification were

Discussion

In 1962, Waterston et al¹ proposed a prognostic classification of EA, which was based on birth weight, pneumonia, and associated congenital anomalies. One of the important points of its classification was respiratory state of EA neonate. In response to advances in neonatal care, Poenaru et al¹² suggested that only severe pulmonary dysfunction requiring mechanical ventilation remained a prognostic risk factor in their series, and proposed a new classification based on preoperative ventilator

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Cited by (79)

Optimal Surgical Method and Timing for Low-birth-weight Esophageal Atresia Babies: Multi-institutional Observational Study
2024, Journal of Pediatric Surgery
Previous research has shown that low birth weight is one of the risk factors for esophageal atresia. However, there remains a paucity of evidence on the timing and the treatment method.
Data were collected using a multi-institutional observational study in 11 hospitals that performed surgeries on esophageal atresia babies whose birth weights were ≤1500 g from 2001 to 2020.
Of the 46 patients analyzed, median birth weight was 1233 (IQR 1042–1412) g. Within 46 cases, 19 (41%) underwent definitive esophageal anastomosis at the median of age in 8 (IQR 2–101) days. Thirteen out of 19 experienced either closure of tracheoesophageal fistula, gastrostomy, or esophageal banding at the first operation, followed by esophageal anastomosis. Seven infants, including four cases of <1000 g, underwent anastomosis after one month of age to wait for weight gain (variously 2–3000 g). Twenty-one out of 27 infants (78%) who did not receive anastomosis died within one year of age, including 21 (78 %) with major cardiac anomalies and 24 (89%) with severe chromosomal anomalies (trisomy 18). Six survivors in this group, all with trisomy 18, lived with palliative surgical treatments.
In our study, the definitive esophageal anastomosis was effective either at the first operation or as a later treatment after gaining weight. Although having severe anomalies, some infants receive palliative surgical treatments, and the next surgery was considered depending on their condition.
II.
Morbidity and mortality among neonates with esophageal atresia and/or tracheoesophageal fistula in the United States
2023, International Journal of Pediatric Otorhinolaryngology
Although rare, esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) can lead to severe morbidity and mortality. A known complication of EA and/or TEF repair is vocal fold motion impairment (VFMI).
To characterize the prevalence of VFMI among repaired EA and/or TEF at a national level and to identify factors associated with in-patient mortality and common clinical endpoints in this population (tracheostomy and gastrostomy tube dependence).
This is a retrospective cohort study of inpatient neonate admissions with EA and/or TEF in the 2016 Kids’ Inpatient Database (KID). Patients with a history of EA and/or TEF were identified with ICD-10 codes. VFMI prevalence was assessed by observed frequencies and percentages. Clinical endpoints included gastrostomy (G-)tube placement, tracheostomy status, and inpatient mortality. Potential predictors of these three primary outcomes included cardiac anomalies, airway disorders, chromosomal anomalies, surgery within 24 h of admission, prematurity, and low birthweight (LBW). Chi-squared analysis was performed between each parameter and the primary outcomes. Significant associations (p-value<0.05) were further assessed with multivariate logistic regression.
Of 1062 neonates with history of EA and/or TEF, 24 (2.3%) had VFMI. Among this population, 27 patients underwent tracheostomies and 63 required G-tubes. There was a significantly higher likelihood of tracheostomy in patients with VFMI compared to patients without (16.7% versus 2.2%; p < 0.01). After multivariate analysis, birthweight less than 1.5 kg (BW < 1.5 kg; OR = 2.5, 95% CI: 1.03–6.10), extreme prematurity (OR = 7.5, 95% CI: 2.00–28.07), cardiac anomalies (OR = 1.8, 95% CI: 1.01–3.04) and chromosomal anomalies (OR = 4.2, 95% CI: 2.39–7.44) were significantly associated with in-hospital mortality. Bronchopulmonary dysplasia (BPD; OR = 4.7, 95% CI: 1.73–12.78) and BW < 1.5 kg (OR = 2.4, 95% CI: 1.05–5.49) were significant predictors for G-tube status. For tracheostomy, BPD (OR = 15.8, 95% CI: 4.70–52.83) and subglottic stenosis (SS, OR = 11.2, 95% CI: 1.85–68.02) remained significant predictors after multivariate regression.
The national prevalence of VFMI among neonates following EA and/or TEF repair was 2.3% and was associated with an increased likelihood of tracheostomy in the KID 2016. Significant predictors of in-hospital mortality were cardiac anomalies, chromosomal anomalies, extreme prematurity, and BW < 1.5 kg. BPD and SS were significant predictors of tracheostomy, and BPD and BW < 1.5 kg were significant predictors for G-tube requirements.
Esophageal Surgery in Neonates: Esophageal Atresia, Gastroesophageal Reflux, and Other Congenital Anomalies
2023, Principles of Neonatology
Esophageal pathologies in the newborn period include esophageal atresia (EA), gastroesophageal reflux, and other congenital anomalies such as stenosis, duplication cysts, and vascular rings. The presentation of esophageal pathology in the newborn includes both upper gastrointestinal symptoms and respiratory symptoms, and the diagnosis can be challenging at times. EA with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus and requires surgical correction in all cases. Although the management of short-gap EA has become quite successful during the past several decades, there continues to be significant morbidity as infants grow into childhood. To date, the surgical management of EA has been based largely on individual surgeon experience, preference, and training, with wide variation occurring nationally in certain aspects of care. However, recent multicentered registries have begun to elucidate the optimal aspects of EA/TEF management, and prospective studies will continue to move the field forward to improve outcomes. Similarly, the surgical management of other esophageal pathologies in the neonatal period is benefiting from increasing evidence and understanding of those disorders.
Management of neonates with oesophageal atresia and tracheoesophageal fistula
2022, Early Human Development
Oesophageal atresia with very low birth weight: Clinical characteristics and long-term outcome
2022, Journal of Pediatric Surgery
An assessment of the clinical data and outcome of patients with oesophageal atresia (OA) with very low birth weight (VLBW) was completed.
With ethical consent, we reviewed the records of 327 successive patients with OA from 1980 to 2020. Main outcome measures survival and oesophageal repair were compared between patients with VLBW(≤1500 g) and with BW>1500 g.
Thirty-four (10%) patients had VLBW. Gross types of OA in VLBW were similar as in other patients: A (15%/7%), B (3%/3%), C (78%/82%), D (3%/4%), E (0%/7%), F (0%/1%) (p = 0.16–0.99). In VLBW the incidence of congenital heart disease (CHD) (47%) and trisomy 13/18 and Cri du Chat (15%) were higher than in BW>1500 g (23% and 1%), (p = 0.001 both). In VLBW one-month mortality was 35% vs 4% in patients with BW>1500 g (p < 0.001), overall mortality 56% and 8% (p < 0.001), respectively. Cause of one-month mortality in VLBW (12 patients) were CHD w/wo chromosomal abnormality (n = 7), cerebral hemorrhage (n = 2), gastric perforation (n = 1), anastomotic leakage (n = 1) and pulmonary hemorrhage (n = 1). Of VLBW patients 79% and of other patients 99% underwent oesophageal repair (p < 0.001). Repair in VLBW patients included early (n = 18) or delayed (n = 5) end-to-end anastomosis and reconstruction (n = 4). Anastomotic complications occurred in 24% of patients with VLBW and in 17% with BW>1500 g, (p = 0.31). From 1980–2000 to 2001–2020 survival in VLBW changed from 11% to 81% (p = 0.002). During 2001 – 2020 all VLBW patients underwent repair.
OA with VLBW had high incidence of life-threatening associated anomalies and decreased survival. Recently survival and rate of oesophageal repair have improved significantly.
Risk Factors of Early Mortality and Morbidity in Esophageal Atresia with Distal Tracheoesophageal Fistula: A Population-Based Cohort Study
2021, Journal of Pediatrics
To identify the risk factors for early mortality and morbidity in a population with distal esophageal atresia (EA)-tracheoesophageal fistula.
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.
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).
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.

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Original CommunicationEsophageal atresia: Prognostic classification revisited

Background

Methods

Results

Conclusion

Section snippets

Patients and methods

Results

Discussion

Lancet

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

Original Communication
Esophageal atresia: Prognostic classification revisited