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. 2026 Jul 13;42(1):302. doi: 10.1007/s00383-026-06538-9

Long-term medical support after definitive surgery for esophageal atresia: analysis using a national inpatient database in Japan

Yoshitsugu Yanagida 1,2, Shotaro Aso 3, Michimasa Fujiogi 1, Kosuke Kirino 4, Tetsuya Ishimaru 5, Kenji Santo 6, Shuichiro Uehara 6, Tomoaki Taguchi 7, Kensuke Yoshimura 2,8, Hideo Yasunaga 2, Jun Fujishiro 1,✉
PMCID: PMC13364871  PMID: 42439942

Abstract

Purpose

Long-term medical needs after definitive surgery for esophageal atresia (EA) are incompletely understood. The real-world patterns of home medical care and medication use after hospital discharge remain underexplored.

Methods

Using the Japanese national inpatient Diagnosis Procedure Combination database, we identified infants who underwent definitive surgery for EA between July 2010 and March 2023. We described patients’ background characteristics, perioperative information, long-term home medical care (home tracheostomy care, home mechanical ventilation, home oxygen therapy, and home enteral nutrition), and medication use (antacids, prokinetic agents, and anti-asthmatics) at 1, 3, 5, and 7 years post-definitive surgery. Subgroup analysis based on congenital heart disease (CHD) was conducted.

Results

Overall, 806 patients met the eligibility criteria. Home enteral nutrition was the most frequently performed home medical care (22.0% of patients at 1 year post-surgery), whose proportion decreased gradually over time. Antacid and prokinetic agent use also declined over time. Subgroup analysis revealed that patients with CHD required home oxygen therapy and home enteral nutrition more frequently than those without CHD.

Conclusions

A substantial proportion of patients required home longitudinal nutritional and respiratory support post-definitive surgery for EA. Our findings provide important information for long-term healthcare needs of patients receiving definitive surgery for EA.

Level of evidence

III (retrospective comparative study).

Supplementary Information

The online version contains supplementary material available at 10.1007/s00383-026-06538-9.

Keywords: Esophageal atresia, Neonatal surgery, Long-term home medical care

Introduction

Short-term survival after definitive surgery for esophageal atresia (EA) has improved remarkably in recent decades [1, 2]. With advancements in perioperative management and neonatal intensive care, even high-risk infants now survive into childhood. Consequently, an increasing number of patients have to live with chronic postoperative complications, making long-term outcomes an important clinical concern [3, 4].

Severe tracheomalacia associated with EA and vocal cord paralysis following definitive surgery can lead to respiratory dysfunction, necessitating tracheostomy and long-term mechanical ventilation [5–7]. In addition, enteral nutrition is frequently required because of dysphagia and impaired oral intake related to esophageal dysfunction [8, 9]. Furthermore, patients with gastroesophageal reflux or anastomotic stricture often require long-term pharmacotherapy, including proton pump inhibitors [8, 10]. Furthermore, several of these conditions require continuous management in the home setting.

The prevalence, duration, and longitudinal trends in home medical support among patients with EA at the population level are unclear because only a few longitudinal studies have investigated long-term medical support in patients receiving surgery for EA and were limited by their single-center setting, small sample sizes, and short observation periods [10, 11].

Therefore, this study aimed to describe the current status of long-term home medical support following definitive surgery for EA in a real-world clinical setting, using a national inpatient database in Japan.

Materials and methods

Data source

This retrospective cohort study was conducted using the Diagnosis Procedure Combination database, which contains discharge summaries and administrative claims data of approximately 8,000,000 inpatient admissions annually; more than 1,200 hospitals across Japan voluntarily contribute to this database. The database includes data from all 82 academic hospitals in Japan [12], and approximately 90% of hospitals equipped with neonatal intensive care units [12, 13]. The database includes the following information: unique hospital identifiers; sex, age, body weight and height; diagnoses, comorbidities at admission, and post-admission complications recorded using the International Classification of Diseases, Tenth Revision (ICD-10) codes [14]; and procedures performed. Academic hospitals and other tertiary care centers submit outpatient claims data. Previous validation studies have demonstrated a high level of accuracy for diagnoses, procedural coding, and operative information within the database [15–17].

This study was conducted in accordance with the STROCSS guidelines [18]. The requirement for informed consent was waived because all patient data were anonymized. This study protocol was approved by the institutional review board [approval number: 3501-(5), May 19, 2021].

Patient selection

We identified patients who underwent definitive surgery for EA within the first year of life between July 2010 and March 2023. Patients were excluded if they met any of the following criteria: (i) definitive surgery was recorded more than once during the study period because it was not possible to distinguish whether these records represented palliative procedures (e.g., tracheoesophageal fistula ligation) from reoperation; or (ii) the postoperative observation period was less than 1 year. For all eligible patients, rehospitalizations and outpatient visits were tracked for up to 12 years after the definitive surgery.

Patient background and perioperative information

The patients’ background variables included sex, birth weight, gestational age (in weeks), and congenital abnormalities categorized as follows based on the components of VACTERL syndrome: vertebral malformations (ICD-10 codes: Q76), anorectal malformations (Q42.3, Q43.6, Q43.7), congenital heart disease (Q20–26), renal malformations (Q60–64), and limb malformations (Q69–74). Additional variables included mild or severe birth asphyxia, hospital volume, teaching hospital status, and fiscal year. Mild and severe birth asphyxia were defined as neonatal resuscitation for first-degree birth asphyxia (Apgar scores: 4–6) and second-degree birth asphyxia (Apgar scores: 0–3), respectively [19]. Hospital volume was defined as the mean annual number of radical operations for EA performed at each hospital and was categorized into three groups: low- (< 1.5 cases/year), medium- (≥ 1.5 to < 2 cases/year), and high-volume (≥ 2 cases/year). Perioperative information included age at surgery (in days), duration of anesthesia (in minutes), postoperative anastomotic leakage, transfusion and vasopressor use within 30 days after definitive surgery, and postoperative hospital stay (in days). Anastomotic leakage was defined as either a recorded diagnosis of anastomotic leak after admission or the requirement for continuous suction drainage for ≥ 3 weeks following definitive surgery [20].

Outcomes

The study outcomes were the use of home medical care and medication at 1 year (infancy), 3 years (toddler), 5 years (preschool age), and 7 years (school age) after definitive surgery. Home medical care encompassed tracheostomy care, home mechanical ventilation, home oxygen therapy, and home enteral nutrition. Medications included antacids, prokinetic agents, and anti-asthmatic agents. These outcomes were identified based on the Japanese procedure codes, medical device utilization, and medication prescriptions recorded at hospital discharge or outpatient visits (Table S1). We defined the duration of each medical care or medication use as the period from the initial to last recorded claims for these medical care modalities or medications. When the duration encompassed a given follow-up time point (1, 3, 5, or 7 years after definitive surgery), the patient was considered to be receiving corresponding home medical care or medication at that time point.

Statistical analysis

Categorical variables were presented as numbers and percentages, and continuous variables as medians with interquartile ranges (IQR). The Cochran–Armitage trend test was employed to investigate serial changes in the proportion of each home-medical care modality and medication use. The chi-squared test was used to compare the proportions of categorical variables.

We calculated the proportion of patients receiving each type of home medical care or medication at each follow-up time point.

We conducted a subgroup analysis based on the presence of congenital heart disease (CHD) because CHD is a major comorbidity of EA and may influence long-term postoperative management. We further performed a subgroup analysis among patients with CHD according to the presence of severe CHD, as defined in a previous study [21], because the severity of CHD may affect long-term outcomes. Details of the definition of severe CHD are provided in Table S3. We also conducted a subgroup analysis based on hospital volume because hospital volume may influence the outcomes. We set a significance level of P < 0.05 for all statistical tests; all reported P values were two-sided. All analyses were performed using Stata/SE 18.0 (Stata Corp., College Station, TX, USA).

Results

We identified 1276 patients who underwent definitive surgery for EA within the first year of life between July 2010 and March 2023. We excluded 58 patients for whom definitive surgery was recorded more than once during the study period and 412 patients with a postoperative observation period of less than 1 year. The final study cohort comprised 806 patients (Fig. 1).

Fig. 1.

Fig. 1

Flow diagram of the patients for the patients included in this study

The patients’ background characteristics and perioperative information are summarized in Table 1. The median (IQR) birth weight was 2384 g (1928–2724), and the median (IQR) gestational age was 37 weeks (35–39). CHD was the most common congenital malformation (30.3%). The median (IQR) age at surgery was 2 days (1–6), and the median (IQR) duration of anesthesia was 312 min (244–401). Anastomotic leakage after the definitive surgery occurred in 67 patients (8.3%). The median (IQR) postoperative hospital stay was 55 days (35–114). The proportions of home medical care and medication use, and trend analyses in the entire patient cohort are described in Table 2; Fig. 2. The observational periods exceeded 3, 5, and 7 years for 459 (56.9%), 277 (34.4%), and 152 patients (18.9%), respectively. Enteral nutrition was the most frequent home medical care, and its proportion decreased over time (P = 0.04). Less than 10% of patients received other home medical care modalities, including home tracheostomy care, home mechanical ventilation, and home oxygen therapy nutrition, at each time point. The proportion of medication use including antacids and prokinetic agents decreased over time (both P < 0.01). The proportion of utilization of anti-asthmatic agents was the highest at 3 or 5 years among the follow-up time points (Fig. 3).

Table 1.

Patients’ background characteristics and perioperative information

Patient backgrounds N = 806
Sex, male, n (%) 450 (55.8)
Birth weight, g (IQR) 2384 (1928–2724)
Gestational age, week (IQR) 37 (35–39)
Congenital malformations, n (%)
Vertebral malformation 26 (3.2)
Anorectal malformation 155 (19.2)
Cardiac malformation 244 (30.3)
Renal malformation 57 (7.1)
Limb malformation 71 (8.8)
Birth asphyxia, n (%)
Mild 84 (10.4)
Severe 72 (8.9)
Teaching hospital, n (%) 804 (99.8)
Annual hospital volume, n (%)
< 1.5 425 (52.7)
≥ 1.5, < 2 206 (25.6)
≥ 2 175 (21.7)
Fiscal year, n (%)
2010 44 (5.5)
2011 67 (8.3)
2012 85 (10.5)
2013 76 (9.4)
2014 85 (10.5)
2015 65 (8.1)
2016 68 (8.4)
2017 88 (10.9)
2018 63 (7.8)
2019 62 (7.7)
2020 59 (7.3)
2021 44 (5.5)
Perioperative information
Age at definitive surgery, days (IQR) 2 (1–6)
Duration of anesthesia, min (IQR) 312 (244–401)
Anastomotic leakage, n (%) 67 (8.3)
Blood transfusion within 30 days after surgery, n (%) 325 (40.3)
Vasopressor use within 30 days after surgery, n (%) 453 (56.2)
Postoperative hospital stay, days (IQR) 55 (35–114)

IQR Interquartile range; EA Esophageal atresia

Table 2.

Trend analyses of the proportion of use of each home medical care modality and medication after definitive surgery for EA

Outcomes 1 year 3 years 5 years 7 years
(n=806) (n=459) (n=277) (n=152) P value
Home medical care, n (%)
Home tracheostomy care 70 (8.7) 32 (7.0) 19 (6.9) 9 (5.9) 0.12
Home mechanical ventilation 25 (3.1) 15 (3.3) 8 (2.9) 3 (2.0) 0.54
Home oxygen therapy 43 (5.3) 40 (8.7) 26 (9.4) 8 (5.3) 0.15
Home enteral nutrition 177 (22.0) 96 (20.9) 48 (17.3) 25 (16.4) 0.04
Medication use, n (%)
Antacids 304 (37.7) 157 (34.2) 70 (25.3) 36 (23.7) <0.01
Prokinetic agents 237 (29.4) 125 (27.2) 58 (20.9) 28 (18.4) <0.01
Anti-asthmatic agents 164 (20.3) 160 (34.9) 97 (35.0) 39 (25.7) <0.01

EA Esophageal atresia

Fig. 2.

Fig. 2

Trends in the proportion of home medical care at each follow-up time point among 806 patients

Fig. 3.

Fig. 3

Trends in the proportion of medication use at each follow-up time point among 806 patients

Subgroup analysis demonstrated a higher frequency of home oxygen therapy and home enteral nutrition in patients with CHD than in those without CHD (Table 3; Figs. 4 and 5). Trend analyses in the subgroups showed a declining trend in the proportion of enteral nutrition and digestive medication among patients without CHD. In contrast, although the proportion of antacid use decreased, a significant trend in the proportion of enteral nutrition and prokinetic agent use was not observed among patients with CHD (Tables S3 and S4). The subgroup analysis stratified by the presence of severe CHD among patients with CHD demonstrated no significant differences in the proportions of patients requiring home tracheostomy care, home mechanical ventilation, home enteral nutrition, or medication use. The frequency of home oxygen therapy was significantly higher in patients with severe CHD than in those with non-severe CHD (Table S5, Figures S1 and S2). The subgroup analysis stratified by hospital volume found no consistent differences in outcomes among the three hospital-volume groups (Table S6, Figures S3 and S4).

Table 3.

Comparison of the proportion of use of each home medical care modality and medication between patients with or without CHD

Outcomes 1 year 3 years 5 years 7 years
with CHD without CHD with CHD without CHD with CHD without CHD with CHD without CHD
(n=244) (n=562) P value (n=140 (n=319) P value (n=90) (n=187) P value (n=55) (n=97) P value
Medical care , n (%)
Home tracheostomy care 31 (12.7) 39 (6.9) 0.01 17 (12.1) 15 (4.7) <0.01 9 (10.0) 10 (5.3) 0.15 5 (9.1) 4 (4.1) 0.21
Home mechanical ventilation 14 (5.7) 11 (2.0) <0.01 7 (5.0) 8 (2.5) 0.17 5 (5.6) 3 (1.6) 0.07 2 (3.6) 1 (1.0) 0.27
Home oxygen therapy 30 (12.3) 13 (2.3) <0.01 26 (18.6) 14 (4.4) <0.01 17 (18.9) 9 (4.8) <0.01 6 (10.9) 2 (2.1) 0.02
Home enteral nutrition 71 (29.1) 106 (18.9) <0.01 44 (31.4) 52 (16.3) <0.01 26 (28.9) 22 (11.8) <0.01 15 (27.3) 10 (10.3) 0.01

Medication use

, n (%)

Antacids 99 (40.6) 205 (36.5) 0.27 56 (40.0) 101 (31.7) 0.08 28 (31.1) 42 (22.5) 0.12 15 (27.3) 21 (21.6) 0.43
Prokinetic agents 68 (27.9) 169 (30.1) 0.53 47 (33.6) 78 (24.5) 0.04 23 (25.6) 35 (18.7) 0.19 12 (21.8) 16 (16.5) 0.42
Anti-asthmatic agents 57 (23.4) 107 (19.0) 0.16 59 (42.1) 101 (31.7) 0.03 39 (43.3) 58 (31.0) 0.04 16 (29.1) 23 (23.7) 0.47

CHD: Congenital heart disease

Fig. 4.

Fig. 4

Trends in the proportion of home medical care at each follow-up time point among patients with and without CHD a home tracheostomy care; b home mechanical ventilation; c home oxygen therapy; d home enteral nutrition

Fig. 5.

Fig. 5

Trends in the proportion of related medication use at each follow-up time point among patients with and without CHD a antacids; b prokinetic agents; c anti-asthmatic agents

Discussion

This study investigated the long-term home medical care modalities and mediation use after definitive surgery for EA using a nationwide claims database in Japan. Enteral nutrition was the most frequently performed home medical care modality. Antacids and prokinetic agents were used often, although the proportion of their use decreased over time. Subgroup analysis demonstrated a higher proportion of home oxygen therapy and home enteral nutrition in patients with CHD than in those without CHD.

Some patients required enteral nutrition, antacids, and prokinetic agents after definitive surgery for EA, with peak proportions of approximately 20–40%. These findings indicate that some patients with EA experience continuous digestive dysfunction after definitive surgery. Gastroesophageal reflux and dysphagia are reported to be major digestive complications of EA, requiring continuous tube feeding and medication support [22, 23]. However, the proportion of enteral nutrition and these digestive medications decreased gradually over time. Because gastrointestinal dysfunction may improve with growth, some patients receiving definitive surgery for EA did not need enteral nutritional support.

Anti-asthmatic agents were also prescribed frequently, consistent with previous studies reporting a high prevalence of respiratory problems and asthma in patients with EA [24, 25]. In our study, the proportion of anti-asthmatic use was the highest at 3 to 5 years after definitive surgery. This pattern may be partly explained by the natural epidemiology of asthma in childhood because the prevalence of asthma in Japan generally increases around 3 years of age [26].

Our subgroup analysis showed that patients with CHD required a higher proportion of home oxygen therapy and home enteral nutrition than those without CHD. Patients without CHD exhibited a declining trend in the frequency of enteral nutrition and digestive medication, whereas no such consistent declining trend was observed in patients with CHD. Additionally, patients with severe CHD required home oxygen therapy more frequently than those with non-severe CHD. The coexistence of CHD may partly contribute to a complicated postoperative course and increased nutritional and respiratory problems [27]. Children with CHD, particularly those with severe CHD, may require prolonged oxygen administration due to cardiopulmonary instability, including pulmonary hypertension [28, 29], which may partly explain the higher proportion of home oxygen therapy among patients with CHD than in those without CHD. Furthermore, children with CHD face a higher risk of feeding and swallowing difficulties and malnutrition compared with healthy children [30, 31], which may explain the increased need for nutritional support and absence of a clear declining trend in the proportions of digestive support.

Our study demonstrates the nationwide long-term patterns in medical care and medication use in home settings among patients with EA after definitive surgery, which were not adequately reported by previous studies. Describing the actual patterns of medical care and medication use required in daily life, as well as subgroup differences according to CHD status, may provide valuable information regarding long-term medical support required for patients with EA, and may also help patients and caregivers better understand the potential medical support required after hospital discharge.

Our study has some limitations. First, we were unable to track patients transferred across different hospitals in this database. Thus, the proportions of home medical care and medication use may have been underestimated. Additionally, the DPC database does not contain information on the reasons for discontinuation of follow-up and does not track patients after transfer to another hospital. Therefore, we were unable to distinguish between transfer to another hospital, discontinuation of outpatient follow-up because of a favorable clinical course, and true loss to follow-up. Second, we excluded patients with an observation period of less than 1 year. Because these patients may have included those who died within the first year after surgery, our results may be subject to survival bias and may therefore underestimate the true burden of home medical care and medication use. Conversely, patients with an observation period of less than 1 year may also have had favorable clinical courses, thereby discontinuing follow-up on their own, which may have led to overestimation. Third, the DPC database does not contain information on the subtype of EA (Gross classification) or gap length. Fourth, the definition of a high-volume hospital in our study (≥ 2 cases per year) was lower than the thresholds commonly used in international settings, such as ≥ 5 cases per year. We excluded patients with multiple records of definitive surgery and those with an observation period of < 1 year, which may have reduced the calculated annual hospital volume.

In conclusion, we described the real-world status and longitudinal trends in home medical support after definitive surgery for EA using a nationwide claims database in Japan. Although a certain proportion of patients required ongoing home medical support after surgery, the need for enteral nutrition and gastrointestinal medications generally decreased over time. In contrast, such declining trends were not consistently observed among patients with comorbid CHD, who required more intensive and prolonged medical support.

Supplementary Information

Below is the link to the electronic supplementary material.

Acknowledgements

This work was supproted by MHLW Research on Rare and Intractable Diseases Program Grant Number JPMH26FC1027.

Abbreviations

EA

Esophageal atresia

ICD-10

International classification of diseases, tenth revision

CHD

Congenital heart disease

Author contributions

Conceptualization: Yoshitsugu Yanagida; Formal analysis and investigation: Yoshitsugu Yanagida; Writing – original draft preparation: Yoshitsugu Yanagida; Writing – review and editing; Shotaro Aso, Michimasa Fujiogi, Kosuke Kirino, Tetsuya Ishimaru, Kenji Santo, Shuichiro Uehara, Tomoaki Taguchi, Kensuke Yoshimura, Hideo Yasunaga, Jun Fujishiro; Funding acquisition: Tomoaki Taguchi, Kensuke Yoshimura, Hideo Yasunaga; Resources: Tomoaki Taguchi, Kensuke Yoshimura, Hideo Yasunaga; Supervision: Hideo Yasunaga.

Funding

Open Access funding provided by The University of Tokyo. This work was supported by grants from the Ministry of Health, Labour and Welfare, Japan (26AA2006, 26AA2001, and 26FC1027).

Data availability

No datasets were generated or analysed during the current study.

Declarations

Conflict of interest

The authors declare no competing interests.

Ethical approval

This study was conducted in accordance with the STROCSS criteria. This study was approved by our institutional review board [approval number: 3501-(5), May 19, 2021].

Consent to participate

The requirement for informed consent was waived because the patient data were anonymized.

Footnotes

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

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Associated Data

This section collects any data citations, data availability statements, or supplementary materials included in this article.

Supplementary Materials

Data Availability Statement

No datasets were generated or analysed during the current study.


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