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 Table of Contents  
ORIGINAL ARTICLE
Year : 2022  |  Volume : 7  |  Issue : 1  |  Page : 94-98

Factors affecting outcome in neonates with esophageal atresia with or without tracheesophageal fistula


Department of Pediatric Surgery, Faculty of Medical Sciences, KhajaBandaNawaz University, Kalaburagi, Karnataka, India

Date of Submission05-Jul-2021
Date of Acceptance26-Aug-2021
Date of Web Publication27-Jun-2022

Correspondence Address:
Dr. Nandkishor Dhanvantrao Shinde
Block C, F-1, Asian Gardenia, Kalaburagi - 585 104, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/bjhs.bjhs_68_21

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  Abstract 


BACKGROUND: Esophageal atresia (EA) with or without tracheesophageal fistula (TEF) is a common congenital anomaly requiring urgent surgical intervention after birth. Due to improvement in antenatal diagnosis and postnatal management in neonates with EA, there are better overall survival rates in the developed countries. However, the outcome is still poor in developing countries where multiple factors contribute to higher morbidity and mortality.
AIM: To evaluate various factors affecting the perioperative management and their outcome in neonates with EA with or without TEF.
MATERIALS AND METHODS: This descriptive observational study was conducted for 4 years. Neonates who underwent surgery for EA with or without TEF at our institute were included. Age of the neonate at presentation, gender, birth weight, period of gestation, antenatal diagnosis records, clinical presentation, associated congenital anomalies, surgical interventions, gap between upper and lower esophageal ends, postoperative complications, and outcome was recorded.
RESULTS: Total, 44 neonates underwent surgery for esophageal atresia with or without TEF in our institute. Male-to-female ratio was 2.4:1. The mean birth weight of neonates was 2500 ± 900 g. The mean gestation age was 35 ± 5 weeks. Sepsis was the leading cause of postoperative morbidity seen in 45.5%, followed by anastomotic leak in 22.7%. Postoperative mortality was 59% with the survival rate of 41%.
CONCLUSION: Sepsis, hypothermia, delayed diagnosis, delayed referral, aspiration pneumonitis, anastomotic leaks were the preventable factors affecting the outcome.

Keywords: Complications, esophageal atresia, sepsis, survival, tracheesophageal fistula


How to cite this article:
Shinde ND, Mankar K, Adarsh Gowda M R, Tousif M. Factors affecting outcome in neonates with esophageal atresia with or without tracheesophageal fistula. BLDE Univ J Health Sci 2022;7:94-8

How to cite this URL:
Shinde ND, Mankar K, Adarsh Gowda M R, Tousif M. Factors affecting outcome in neonates with esophageal atresia with or without tracheesophageal fistula. BLDE Univ J Health Sci [serial online] 2022 [cited 2022 Aug 16];7:94-8. Available from: https://www.bldeujournalhs.in/text.asp?2022/7/1/94/348276



Esophageal atresia (EA) with or without tracheesophageal fistula (TEF) is a congenital anomaly that occurs in one in 3000 births and requires surgical intervention immediately after birth.[1],[2]

These neonates present immediately after birth with the inability to pass a nasogastric tube, regurgitation of feed, or respiratory distress. The condition may be an isolated anomaly or associated with other major congenital anomalies such as vertebral, anorectal malformations, cardiac defects, TEF with or without EA, renal malformations, and limb defects (VACTERL/VATER syndrome) and rarely, Di-George syndrome, polysplenia, and Pierre Robin syndrome.[3]

EA with or without TEF requires early diagnosis, resuscitation, and urgent surgical intervention.[4]

EA repair is a complex surgery even in full-term neonates and has a negative impact on blood circulation and respiration, causing extra perioperative stress.[5],[6] Open or thoracoscopic approaches for surgical repair are the treatment modalities of choice.[7] With the advancement in surgical technique, pediatric anesthesia, ventilatory support, and neonatal perioperative care, there is a significant improvement in survival, with survival rates of >90% being reported; recent studies with thoracoscopic repair have reported mortality rates as low as 3%, with case fatalities related to associated congenital anomalies.[8],[9],[10]

The aim of this study is to evaluate the factors affecting surgical and perioperative management and its outcome in neonates with EA with or without a TEF at a tertiary care institute in resource-constrained developing country.


  Materials and Methods Top


This descriptive observational study was conducted for 4 years from April 2017 to March 2021. Neonates who underwent surgery for EA with or without TEF at our institute were included in the study. Neonates who refused surgery or died without surgery were excluded from the study. Preoperatively, stiff NG tube of 8 French was inserted perorally in each patient and an X-ray was taken to confirm the diagnosis, isolated esophageal atresia ruled out by gas shadows in the abdomen on X-ray. Two-dimensional-echocardiogram, ultrasound abdomen and pelvis was done to rule out associated anomalies.

After confirming the diagnosis, neonates were resuscitated. A continuous pharyngeal suction was kept, intravenous fluids, broad-spectrum antibiotics, and oxygen therapy with or without assisted ventilation were started.

After stabilization, right thoracotomy through the 4th intercostal space was performed with an extrapleural approach. The azygos vein was ligated and divided. The TEF was transfixed and divided, the upper esophageal pouch was identified and dissected, an attempt was done to do a primary esophago esophageal anastomosis with interrupted single layer 6/0 Vicryl sutures over 6 French feeding tubes. A chest drain was kept in the extrapleural space. Those neonates with long gap, or only fistula, were transfixed without dividing it and cervical esophagostomy and feeding gastrostomy were performed. Those neonates with pure esophageal atresia, cervical esophagostomy and feeding gastrostomy were performed.

Postoperatively, all the neonates were kept in the neonatal intensive care unit. NG tube feeding was started after 48 h. Chest tube was removed on the 5th postoperative day if there were no signs of leak. A contrast oesophagogram was performed on the 8th postoperative day and if there was no leak then oral feeding was established. Follow-up was done weekly for 1 month, then every 3 monthly for 1 year.

Age of neonate at presentation, gender, birth weight, period of gestation, antenatal diagnosis records, clinical presentation, type of EA/TEF (as per Gross's classification), associated congenital anomalies, surgical interventions, gap between upper and lower esophageal ends, postoperative complications, and final outcome were recorded. Qualitative data were arranged in proportions. Quantitative data were arranged in Mean and Standard deviation. All the data were analyzed using software SPSS 24.0 version (IBM, Amonk, New York, USA).


  Results Top


During our study period, 44 neonates underwent surgery for esophageal atresia with or without TEF in our institute. Out of 44 neonates, 31 (70.5%) were males and 13 (29.5%) were female with a male-to-female ratio of 2.4:1. Mean birth weight of neonates was 2500 ± 900 g. The mean gestation age was 35 ± 5 weeks with 17 (38.6%) neonates being born full term (≥37 weeks). Fifteen (34%) patients were suspected of having EA/TEF on antenatal USG screening. However, a history of polyhydramnios was seen in 35 (79.5%) pregnancies. Forty-one (93.2%) neonates were delivered in the hospital.

The mean age of presentation to the institute was 2.8 ± 2.5 days, with the most delayed presentation on the 7th day with respiratory symptoms. The most common presentation was excessive salivation from the mouth in 42 neonates (95.5%), followed by regurgitation of feeds in 38 (86.4%), followed by respiratory distress in 20 (45.5%). Eight (18.2%) neonates presented with shock and 12 (27.5%) were hypothermic at the time of presentation to the institute.

All neonates underwent chest radiograph with the stiff 8 Fr NG tube in situ with confirmed diagnosis in 100% neonates. In our study, due to missed diagnosis at birth, 20 (45.5%) neonates were fed, which had led to aspiration pneumonia at presentation. Associated congenital anomalies were seen in 47.7% of neonates [Table 1], congenital heart diseases (CHDs) were the most common associated congenital anomaly seen in 12 (27.5%) neonates with Ventricular septal defect in 58%neonates, followed by renal anomaly in 4 (9.1%) neonates [Table 1].
Table 1: Associated congenital anomalies with esophageal atresia with or without tracheoesophageal fistula

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According to Gross classification[11] in our study, Type C (III) fistula was most common to be seen in 41 (93.2%) of neonates followed by Type A (I) in 2 (4.5%) and Type E (H type) fistula observed in 1 (2.2%) neonates.

Primary repair was performed in 41 (93.2%) neonates and staged repair in 3 (6.8%) cases; out of which in 1 neonate with long gap, (more than 3 cm) only fistula was transfixed without dividing it and cervical esophagostomy and feeding gastrostomy was performed; in two neonates with pure esophageal atresia cervical oesophagostomy and feeding gastrostomy was performed in the first stage and subsequent esophageal replacement with the gastric tube was done in the second stage after 6 months.

Gap between the upper and lower esophageal pouch was <2 cm in 38 (86.4%) neonates, intermediate (2–3 cm) in 3 (6.8%) neonates, and long gap more than 3 cm in 3 (6.8%) neonates.

The average duration of surgery was 90–120 min in our study. The average duration of postoperative hospital stay was 15 ± 5.3 days.

Sepsis was the leading cause of postoperative morbidity seen in 20 (45.5%), followed by anastomotic leak in 10 (22.7%) with major anastomotic leak in 1 (2.2%) neonate [Table 2]. All of them were managed conservatively with intravenous antibiotics and mediastinal drainage; only 1 with a major anastomosis leak required the second surgery. Postoperative mechanical ventilation was required in 32 (72.7%) neonates. Neonates were discharged once oral feeds were established.
Table 2: Postoperative complication

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Postoperative mortality in our study was 26 (59%) with the survival rate of 41%. Various factors associated with postoperative mortality are described in [Table 3]. Survival was 80% in neonates with birth weight more than 2500 g. Postoperative survival rate was 75% in term infants as compared to only 22% in preterm infants. 37.5% of the neonates with shock at presentation survived to discharge. Similarly, 58% of neonates with hypothermia survived. The neonates operated within the initial 48 h of life, had postoperative survival of 70%. Sepsis (80%) was the most common cause of postoperative mortality [Table 3].
Table 3: Factors associated with postoperative mortality

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  Discussion Top


In the recent decade, overall survival rates for EA with or without a TEF in the developed countries are significantly better due to improvement in antenatal diagnosis and postnatal management in neonates. Survival is still poor in developing countries where multiple factors contribute to higher morbidity and mortality. Compared to developed western countries, most of the tertiary care centers are poorly equipped with machines and human resources and located in metro cities. Prematurity is still a major problem for developing countries which increases susceptibility to sepsis and mortality.[12]

In our study, male-to-female ratio is 2.4:1, in other studies also EA is reported more common in boys than girls.[13]

The mean birth weight of neonates was 2500 ± 900 g in our study, other studies also show similar data.[14],[15] The mean gestational age was 35 ± 5 weeks, which is comparable to other similar studies.[16]

In most of the neonates with EA with or without a TEF, antenatal polyhydramnios has been reported to be 55%, but in our study, polyhydramnios was seen in 79.5% of pregnancies which is little higher.[17],[18]

In our setup, average age of presentation to the institute was 2.8 ± 2.5 days, 45.5% of neonates had respiratory distress at presentation, 18.2% neonates had shock and 27.5% were hypothermic at the time of presentation, hence delayed presentations established aspiration pneumonitis which further led to poor outcome in our study.

Long gap EA with gap longer than 3 cm, approximately greater than the height of two vertebral bodies led to tension anastomosis which had higher chances of anastomotic leak and sepsis which decreased survival.[19],[20] In our study, long gap more than 3 cm was seen in 6.8% of neonates.

The average duration of surgery was 90–120 min in our study. It is observed that the long duration of surgery (>120 min) had poor outcomes due to exposure of the neonate to side effects of anesthetic drugs, intraoperative hypothermia, and increased risk of sepsis.

According to Gross classification,[11] 84% of our patients had EA type C (distal fistula), 7% had type A (isolated atresia), 6% had type E (isolated fistula), 2% had type B (proximal fistula), and 1% had type D (proximal and distal fistulas).[11] In our study, we found Type C (III) fistula which was most commonly seen in 93.2% of neonates followed by Type A (I) in 4.5%, and Type E (H type) fistula were observed in 2.2% neonates.

Various prognostic classifications were proposed to stratify neonates who have EA and/or TEF. Waterston's et al., risk classification[21] being the oldest (1962) and commonly used. This classification system is further revised by Spitz et al.[22] and later by Poenaru et al.[23] In the Waterston et al., classification, prognostic categories based on birth weight, the presence of pneumonia, and other congenital anomalies are used.[21] Spitz et al., classification is based on the birth weight and the presence of a major cardiac anomaly.[22]

Associated congenital anomalies were seen in 47.7% of neonates in our study and CHDs were the most common associated congenital anomaly seen in 27.5% of neonates, other similar studies also observed approximately 30%–60% of neonates with EA with or without a TEF had associated life-threatening congenital anomalies.[18],[20],[24],[25] Associated CHD and multiple congenital anomalies increases the risk of mortality.[24],[25],[26]

Anastomotic complications are common after primary repair. Many maneuvers attempted to decrease these complications. Postoperative elective ventilation with neck flexion after primary repair of esophageal atresia has reduced anastomotic leakage effectively in many studies.[27] The 22.7% postoperative anastomotic leakage rate in our series was comparable with the incidence rate ranging from 3% to 25% in similar studies.[27],[28],[29]

Sepsis was the main cause of mortality besides major or life-threatening anomalies, long gaps and major leaks in most of the other similar studies.[15],[18],[28],[30] In our study also 45.5% of neonates had postoperative sepsis and among them 80% of neonates had mortality postoperatively.

The overall postoperative mortality was 59% in our study, with the survival rate of 41%. This survival rate was significantly lower, as compared to developed countries where survival rates of >90% were reported, and recent studies with thoracoscopic repair have reported mortality rates as low as 3%.[4],[7],[10] However, few recent studies from Third World countries had reported survival rates as low as 39.7% depicting the poor status in developing countries.[31]

Among various risk factors studied, low birth weight (LBW) had an inverse relationship with survival, survival improved significantly to 80% in infants with birth weight >2500 g, as most of the LBW neonates are also preterm and prematurity increases the mortality. Aspiration pneumonia is also one of the important factors affecting survival. Most of the infants were delivered at peripheral hospitals where due to lack of awareness about congenital anomalies, these neonates are often diagnosed late and referred late. Delayed diagnosis of EA/TEF had also reported the poor outcome in neonates.[32],[33]

Sepsis, shock, hypothermia, delayed diagnosis, delayed referral, aspiration pneumonitis, anastomotic leaks were the preventable factors affecting outcomes in neonates with EA with or without fistula. Hence, early diagnosis of neonates with EA/TEF within few hours of birth and immediate transfer to tertiary care centers equipped with pediatric surgeons and neonatal care is required to improve the survival rate in developing countries. Delay in diagnosis and transfer increases chances of aspiration pneumonia and sepsis which may decrease chances of survival.


  Conclusion Top


Sepsis, hypothermia, delayed diagnosis, delayed referral, aspiration pneumonitis, anastomotic leaks were the preventable factors. Improving the transport facilities, early diagnosis, pediatric surgical facilities and neonatal care at peripheral health services avoids most of the preventable factors and improves survival in developing countries.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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