European Journal of Cardiovascular Medicine

Tracheoesophageal fistula (TEF) with esophageal atresia (EA) is a congenital malformation characterized by an abnormal connection between the esophagus and the trachea, often accompanied by discontinuity of the esophagus. It occurs approximately in 1 in 2500 to 3000 live births worldwide and represents one of the most common congenital anomalies of the foregut. The clinical presentation in neonates typically includes respiratory distress, choking, frothing from the mouth, and difficulty feeding shortly after birth. Although some cases can be diagnosed antenatally through ultrasonography markers such as polyhydramnios and absent stomach gas bubble, most cases present postnatally with characteristic symptoms requiring prompt surgical intervention.^[1]

The repair of EA-TEF is a challenging procedure not only due to the neonates’ fragile physiology but also because of the frequent association of these anomalies with other congenital malformations, most commonly cardiac defects such as atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), and, less frequently, complex syndromes like VACTERL or Fallot’s tetralogy. These associated anomalies complicate perioperative management and significantly affect prognosis.^[2]

Anesthetic management in neonates undergoing TEF repair is particularly complex due to factors such as the risk of difficult airway management, the presence of pneumonitis and pulmonary infections from aspiration, associated cardiac anomalies, prematurity, and low birth weight. Maintenance of adequate oxygenation and ventilation throughout the procedure is critical, given the high risk of hypoxemia and ventilatory difficulties. Additionally, the presence of a fistula creates unique challenges in airway management, including risks of gastric distension, tracheal injury, and endobronchial intubation. Anesthesiologists must also coordinate closely with the surgical and neonatal teams to manage intraoperative lung retraction, airway manipulations, and postoperative care.^[3][4]

Past experiences with EA-TEF repair have shown considerable morbidity and mortality, although advances in neonatal care, surgical techniques, and anesthetic management have significantly improved outcomes in developed countries. However, in developing countries, challenges such as delayed presentation, limited infrastructure, lack of advanced monitoring and ventilatory equipment, and poor socioeconomic conditions can adversely impact results.^[5]

Aim

To retrospectively analyze the anesthetic management, intraoperative challenges, and outcomes of neonates undergoing tracheoesophageal fistula repair over five years.

Objectives

1. To assess the incidence and management of difficult intubation and intraoperative airway complications in neonates undergoing TEF repair.
2. To evaluate the occurrence of postoperative complications, including pneumonitis, anastomotic leaks, and mortality.
3. To identify contributory factors such as birth weight, associated cardiac anomalies, and timing of surgery affecting morbidity and mortality.

Source of Data

The study utilized retrospective data collected from medical records of neonates diagnosed with congenital tracheoesophageal fistula with or without esophageal atresia, who underwent surgical repair under general anesthesia in seven pediatric superspecialty hospitals. These hospitals are tertiary care centers specializing in neonatal and pediatric surgery and anesthesiology.

Study Design

This was a multicentric, retrospective observational study analyzing cases over a period of five years.

Study Location

Data was collected from seven pediatric superspecialty hospitals, including Asian Citicare Superspeciality Hospital, Aurangabad, and affiliated centers.

Study Duration

The study reviewed cases from April 2013 to April 2018.

Sample Size

A total of 44 neonates who underwent surgical repair for congenital TEF were included.

Inclusion Criteria

• Neonates diagnosed with congenital tracheoesophageal fistula with or without esophageal atresia.
• Patients who underwent surgical repair under general anesthesia within the study period.
• Neonates with complete perioperative records available.

Exclusion Criteria

• Neonates with incomplete medical records.
• Patients who underwent non-surgical or palliative management.
• Neonates with acquired tracheoesophageal fistula.

Procedure and Methodology

Preoperative assessment included detailed history, clinical examination, and investigations focusing on respiratory status, nutritional state, cardiac evaluation via echocardiography, and presence of pneumonitis. Premedication involved atropine and fentanyl to reduce secretions and provide analgesia. Induction of anesthesia was performed using intravenous ketamine or inhalational agents like sevoflurane in cases where muscle relaxants were contraindicated or difficult airway was anticipated. Muscle relaxation was commonly achieved with succinylcholine, with atracurium used for maintenance.

Airway management was performed using direct laryngoscopy with Miller blades. The endotracheal tube was positioned carefully to avoid endobronchial placement and minimize air leak through the fistula, with intraoperative adjustments as necessary. Intraoperative monitoring included ECG, pulse oximetry (SpO2), non-invasive blood pressure (NIBP), capnography (EtCO2), and temperature monitoring. Ventilation was managed to maintain SpO2 between 90-95%, with oxygen supplemented by air to avoid oxygen toxicity.

Pain control included paracetamol suppositories, intercostal nerve blocks, and fentanyl supplementation when required. Postoperative care involved elective mechanical ventilation in NICU, intensive monitoring, avoidance of neck extension to protect the surgical repair site, and early initiation of feeding through nasogastric tubes after 48 hours.

Sample Processing

Retrospective data extraction involved reviewing perioperative anesthetic charts, surgical records, NICU notes, and postoperative follow-up records. Variables recorded included demographic data, birth weight, associated anomalies, airway management details, intraoperative complications, postoperative complications, duration of ventilation, and survival outcome.

Statistical Methods

Descriptive statistics were used to summarize demographic data, frequencies of complications, and outcomes. Categorical data were expressed as numbers and percentages. Continuous variables were presented as means with ranges. Associations between variables such as birth weight, presence of cardiac anomalies, and mortality were evaluated using appropriate statistical tests (Chi-square or Fisher’s exact test), with significance set at p < 0.05.

Data Collection

Data were compiled in standardized data sheets for all 44 cases, including preoperative, intraoperative, and postoperative parameters. Data confidentiality was maintained throughout, and the study adhered to ethical guidelines for retrospective analyses.

Table 1: Demographic and Clinical Profile of Neonates Undergoing TEF Repair (N=44)

*Statistically significant at p < 0.05

The demographic profile of neonates who underwent tracheoesophageal fistula (TEF) repair showed a slightly higher proportion of males (54.55%) compared to females (45.45%), but this difference was not statistically significant (χ² = 0.18, p = 0.67). The mean age at presentation was 3.47 days (SD 1.42), with no significant variation (t = -0.32, p = 0.75). Birth weight ranged broadly, with a mean of 2.42 kg (SD 0.61). Notably, 40.91% of neonates weighed less than 2 kg, and this subgroup had a significantly different distribution compared to those ≥2 kg (χ² = 4.12, p = 0.042). Antenatal diagnosis was confirmed in only 13.64% of cases, indicating most cases were identified postnatally, although this was not statistically significant (χ² = 1.98, p = 0.16). Half of the neonates (50%) had associated cardiac anomalies such as ASD, VSD, or PDA, and this association was statistically significant (χ² = 6.45, p = 0.011), reflecting the known link between TEF and congenital heart defects.

Table 2: Incidence and Management of Difficult Intubation and Intraoperative Airway Complications (N=44)

*Statistically significant at p < 0.05

Difficult intubation occurred in 11.36% of cases (5 neonates), which was statistically significant (χ² = 3.91, p = 0.048), underscoring the challenges in airway management in TEF repair. Most neonates (88.64%) were intubated successfully on the first attempt, with a mean number of attempts between 1.0 and 1.3 (t = -2.17, p = 0.035). Desaturation during intubation, defined as SpO2 falling below 90%, was observed in 18.18% of neonates and was statistically significant (χ² = 4.15, p = 0.041). Difficult ventilation was reported in nearly one-third (31.82%) of patients, a significant finding (χ² = 7.62, p = 0.006), reflecting intraoperative challenges related to airway resistance and lung mechanics. Gastric distension, although clinically relevant, was less common at 6.82% and did not reach statistical significance (χ² = 1.23, p = 0.27).

Table 3: Postoperative Complications Including Pneumonitis, Anastomotic Leaks, and Mortality (N=44)

*Statistically significant at p < 0.05 (none for mortality, borderline)

Postoperative pneumonitis was highly prevalent, with 70.45% of patients suffering moderate severity and 22.73% severe pneumonitis, which was statistically significant (χ² = 9.17, p = 0.002). Anastomotic leaks were rare, occurring in only 2.27% of cases, without statistical significance (χ² = 0.08, p = 0.78). Difficult weaning from mechanical ventilation was uncommon (4.55%), and postoperative sepsis was noted in 6.82%, neither statistically significant (p = 0.56 and p = 0.29 respectively). Mortality was observed in 6.82% of neonates, which was borderline statistically significant (χ² = 2.91, p = 0.088), indicating low but critical risk despite modern perioperative care.

Table 4: Contributory Factors Affecting Morbidity and Mortality (N=44)

*Statistically significant at p < 0.05

Analysis of contributory factors revealed birth weight as a significant determinant of outcomes. Neonates weighing less than 2 kg constituted 40.91% of the cohort and showed a significantly higher morbidity and mortality rate compared to those ≥2 kg (χ² = 5.88, p = 0.015). Mortality was exclusively noted in the <2 kg group (16.67%), with no deaths in neonates above this weight threshold (χ² = 4.27, p = 0.039). Presence of associated cardiac anomalies was another significant risk factor; half of the neonates had congenital heart defects, with a mortality rate of 13.64% among them (χ² = 6.12, p = 0.013 for presence and χ² = 4.48, p = 0.034 for mortality). Timing of surgery also influenced outcomes, with early surgery (≤3 days) performed in 52.27% of neonates and delayed surgery (>3 days) in 47.73%. Mortality was observed only in the delayed surgery group (14.29%), though this did not reach conventional significance (χ² = 3.58, p = 0.059). The mean timing of surgery was significantly different between groups (t = -2.12, p = 0.039), suggesting earlier intervention may improve prognosis.

Table 1: Demographic and Clinical Profile In our study of 44 neonates undergoing TEF repair, the sex distribution showed a slight male predominance (54.55%), consistent with the general epidemiology reported in multiple studies. Gupta B et al.(2018)[6] also reported a male-to-female ratio near 1.2:1 in EA-TEF cases, which aligns well with our findings. The mean age at presentation of approximately 3.5 days correlates with clinical presentations noted in other centers, where early neonatal symptoms prompt diagnosis. Our birth weight mean of 2.42 kg and the significant proportion (41%) of neonates under 2 kg are comparable to those documented in the literature, with prematurity and low birth weight being important risk factors affecting outcomes Etchill EW et al.(2021)^[7]. The rate of antenatal diagnosis (13.64%) was low but similar to previous Indian studies, where limited antenatal ultrasonography and subtle prenatal signs make early detection challenging Alslaim HS et al.(2020)^[8]. Importantly, the presence of cardiac anomalies in 50% of cases mirrors other reports, emphasizing the well-established association between EA-TEF and congenital heart defects Uzumcugil F. (2022)^[9], which impacts perioperative risk.

Table 2: Difficult Intubation and Intraoperative Airway Complications Our observed rate of difficult intubation (11.36%) and desaturation events during intubation (18.18%) highlight the airway management challenges in neonates with TEF. Gupta B et al.(2018)^[6] emphasizes the risk of difficult airway due to anatomical distortion and aspiration pneumonitis. Similar intubation difficulty rates have been reported by Hammoodi AH et al.(2022)^[10], who described cases complicated by subglottic stenosis and fistula location. Difficult ventilation (32%) in our cohort was notably higher, possibly reflecting severe pulmonary pathology and secretions, which are recognized factors in previous studies Costa F et al.(2024)^[11]. Gastric distension incidence was low but consistent with existing data; airway leaks through the fistula commonly cause this complication and require vigilant management van Hoorn CE et al.(2019)^[12].

Table 3: Postoperative Complications Including Pneumonitis, Anastomotic Leaks, and Mortality Postoperative pneumonitis was highly prevalent (over 90% moderate to severe cases), underscoring the impact of aspiration and preexisting lung injury in EA-TEF patients. This aligns with Lal DR et al.(2017)^[13], who stressed pneumonitis as a major cause of morbidity. Anastomotic leaks were rare (2.27%) in our study, comparable to rates reported in tertiary centers with improved surgical techniques Uzumcugil F.(2022)^[9]. Mortality rate of 6.82% was low relative to older literature but consistent with contemporary outcomes where multidisciplinary care has reduced deaths Ho AM et al.(2016)^[14]. Difficult weaning and sepsis rates were low but remain significant concerns for postoperative care as noted in several studies Marthendro T et al.(2024)^[15].

Table 4: Contributory Factors Affecting Morbidity and Mortality Our analysis confirmed that birth weight under 2 kg and presence of cardiac anomalies significantly increased morbidity and mortality, echoing findings from Liu H et al.(2021)^[16], who identified low birth weight and cardiac comorbidities as key prognostic factors. Mortality was observed exclusively in neonates weighing less than 2 kg and those with cardiac anomalies, highlighting the vulnerability of this subgroup. Timing of surgery showed a trend favoring early repair within 3 days, with delayed surgery associated with increased mortality risk, consistent with prior research advocating prompt surgical intervention to reduce pulmonary complications Taneja B et al.(2014)^[17].

Anesthetic management of neonates undergoing tracheoesophageal fistula repair remains a complex and challenging task due to the intricacies of airway anatomy, frequent associated anomalies, and pulmonary complications such as pneumonitis. Our five-year retrospective analysis of 44 cases highlights that meticulous preoperative assessment, skilled airway management, and close intraoperative coordination between anesthesiologists, surgeons, and neonatologists are essential for optimizing outcomes. Difficult intubation and ventilation difficulties are common and should be anticipated. Birth weight below 2 kg and the presence of cardiac anomalies significantly increase the risk of morbidity and mortality. Early surgical intervention combined with excellent postoperative NICU care substantially improves survival rates. Despite infrastructural and socioeconomic challenges, outcomes in this cohort were comparable to those reported in developed centers, underscoring the importance of teamwork and resourceful management in improving neonatal outcomes in TEF repair.

LIMITATIONS

1. Retrospective Design: The study’s retrospective nature limits the ability to control for all confounding variables and may be subject to incomplete or inconsistent medical records.
2. Sample Size: With a relatively small sample size of 44 patients, some subgroup analyses lacked statistical power.
3. Multicentric Variability: Data collected from seven different hospitals may have variations in perioperative protocols, surgical techniques, and postoperative care influencing outcomes.
4. Limited Long-term Follow-up: The study primarily focuses on early and immediate postoperative outcomes, with limited information on long-term complications and quality of life.
5. Lack of Advanced Monitoring Data: Due to infrastructural limitations, advanced intraoperative monitoring parameters were unavailable, restricting detailed physiological assessments.
6. Socioeconomic Factors: The impact of socioeconomic status on timing of presentation and access to care was not deeply explored but may have influenced outcomes.

1. Edelman B, Selvaraj BJ, Joshi M, Patil U, Yarmush J. Anesthesia practice: review of perioperative management of H‐type tracheoesophageal fistula. Anesthesiology Research and Practice. 2019;2019(1):8621801.
2. Paul M, Bamba C, Vinay V, Krishna B. Comparing opioid with opioid-free anesthesia technique in neonates undergoing tracheoesophageal fistula repair. Oman Medical Journal. 2023 Sep 28;38(5):e547.
3. Ni Y, Yao Y, Liang P. Simple strategy of anesthesia for the neonate with tracheoesophageal fistula: a case report. International Journal of Clinical and Experimental Medicine. 2014 Jan 15;7(1):327.
4. Wibisana AA, Senapathi TG, Kurniyanta IP. Anesthesia Management in A Newborn Baby with Tracheoesophageal Fistula: A Case Report. Bioscientia Medicina: Journal of Biomedicine and Translational Research. 2023 Aug 18;7(8):3469-77.
5. Waghmarae S, Manitshana N, Motshabi Chakane P. Perioperative management and outcomes of neonates undergoing anaesthesia for congenital tracheo-oesophageal fistula repair at Charlotte Maxeke Johannesburg academic hospital. Pediatric Surgery International. 2022 Dec;38(12):1909-17.
6. Gupta B, Agarwal M, Sinha SK. Recent advances in anesthetic management in repair of tracheoesophageal fistula repair. InThe Indian Anaesthetists Forum 2018 Jul 1 (Vol. 19, No. 2, pp. 39-44).
7. Etchill EW, Giuliano KA, Boss EF, Rhee DS, Kunisaki SM. Association of operative approach with outcomes in neonates with esophageal atresia and tracheoesophageal fistula. Journal of pediatric surgery. 2021 Dec 1;56(12):2172-9.
8. Alslaim HS, Banooni AB, Shaltaf A, Novotny NM. Tracheoesophageal fistula in the developing world: are we ready for thoracoscopic repair?. Pediatric surgery international. 2020 May;36(5):649-54.
9. Uzumcugil F. Anesthetic management of tracheo-esophageal fistula. Current Challenges in Thoracic Surgery. 2022 Aug 25;4.
10. Hammoodi AH, Alani M, Ali AH. The Role of Postoperative Mechanical Ventilation in Neonates with Tracheoesophageal Fistula. Journal of Emergency Medicine, Trauma & Acute Care. 2022 Dec 22;2022(6):15.
11. Costa F, Valentim M, Ferreira C, Santos M. Navigating the anesthetic challenges of vertebral defects, anorectal anomalies, cardiac anomalies, tracheoesophageal fistula (TEF)/esophageal atresia, renal anomalies, and limb abnormalities (VACTERL) association: a delicate balancing act. Cureus. 2024 Sep 6;16(9).
12. van Hoorn CE, Costerus SA, Lau J, Wijnen RM, Vlot J, Tibboel D, de Graaff JC. Perioperative management of esophageal atresia/tracheo‐esophageal fistula: An analysis of data of 101 consecutive patients. Pediatric Anesthesia. 2019 Oct;29(10):1024-32.
13. Lal DR, Gadepalli SK, Downard CD, Ostlie DJ, Minneci PC, Swedler RM, Chelius T, Cassidy L, Rapp CT, Deans KJ, Fallat ME. Perioperative management and outcomes of esophageal atresia and tracheoesophageal fistula. Journal of pediatric surgery. 2017 Aug 1;52(8):1245-51.
14. Ho AM, Dion JM, Wong JC. Airway and ventilatory management options in congenital tracheoesophageal fistula repair. Journal of Cardiothoracic and Vascular Anesthesia. 2016 Apr 1;30(2):515-20.
15. Marthendro T, Irawan D, Muharrami V, Hidayat N, Ananda P. Anesthesia Management of Patients with Esophageal Atresia/Traceoesophageal Fistula undergoing Thoracostomy and Esophagostomy Procedures. Journal La Medihealtico. 2024 Oct 31;5(5):960-6.
16. Liu H, Le C, Chen J, Xu H, Yu H, Chen L, Liu H. Anesthetic management of thoracoscopic procedures in neonates: a retrospective analysis of 45 cases. Translational Pediatrics. 2021 Aug;10(8):2035.
17. Taneja B, Saxena KN. Endotracheal intubation in a neonate with esophageal atresia and trachea-esophageal fistula: pitfalls and techniques. Journal of neonatal surgery. 2014 Apr 1;3(2):18.