Keerthidarshini, Sandeep R, and Shanbag: Clinical profile of new borns with trachea-esophageal fistula and esophageal atresia and factors associated with outcome at a tertiary care centre


Introduction

In 1697, esophageal atresia (EA) was first described that was associated with trachea-esophageal fistula (TEF) by Thomas Gibson. First surgical repair was performed successfully by Cameron Haight in 1941. EA with or without TEF is one of the most common congenital anomaly. It is a rare condition but it presents challenge for pediatric surgery. In developing countries, this condition is characterized by pneumonitis as the patients report late to the hospitals.1, 2

It has been estimated that esophageal atresia-tracheoesophageal (EA-TEF) incidence is one in 2500-4000 live births.3 It is sporadic in nature. The incidence is more in twin births of about 2.56 times more compared to single births.3

During 4-5 weeks of embryonic development, the there is abnormal septation of caudal foregut leads to TEF. In case the tracheoesophageal septum is positioned abnormally posteriorly, then the trachea and esophagus get connected with each other resulting in EA with fistula. If the esophagus cannot recanalize during eighth week of embryonic development, then EA occurs without TEF.4

TEF can be suspected in cases of polyhydramnios and also if the stomach bubble is absent in the fetus. Prenatal scans for the diagnosis of EA has low sensitivity. Karyotyping can help in suspected cases.5 EA can be diagnosed if it is not possible to pass the nasogastric catheter beyond 10-15 cm in the stomach. X-ray chest will show curled nasogastric catheter in upper esophageal pouch.6

TEF is curable and with surgery more than 90% cases survive and hence now a days there is more emphasis on improving quality of life of those who respond to surgery.7

There is good prognosis for isolated TEF but cases with both EA and TEF have guarded prognosis and it depends upon the associated abnormalities.8

In one study it was found that the esophageal stricture was the most common complication in 35% of cases followed by anastomotic leak in 16% and recurrent fistulae was seen in 3% of the cases.9

Although EA-TEF is a rare condition, it is one of the most common congenital anomaly. It is treatable. There is paucity of data on this subject matter due to rarity of the condition. More and more data on clinical profile, complications, and outcome are needed to guide the future research. Hence present study was carried out to study the clinical profile of newborns with trachea-esophageal fistula and esophageal atresia and factors associated with outcome at a tertiary care centre.

Materials and Methods

Study design

Hospital based prospective study.

Study period

From July 2018 to December 2019.

Sample size

During the study period, it was possible to include 21 cases of newborns with trachea-esophageal fistula and esophageal atresia as the condition is rare.

Ethical considerations

Institution Ethics Committee permission was obtained. Child assent was obtained from parents. All cases were managed as per the standard guidelines.

Settings

Present study was carried out at neonatal intensive care unit, Indira Gandhi institute of child health.

Inclusion criteria

  1. Newborns with tracheoesophageal fistula and esophageal atresia of either gender.

  2. Getting admitted and operated at the study site.

Exclusion criteria

  1. Parents not willing to include newborn data with anonymity.

  2. Lost to follow up.

Methodology

Newborns presenting to the Department of Pediatrics, Indira Gandhi institute of child health suspected of having TEF or EA and parents willing to share the data in the present study were included. The diagnosis was confirmed by taking detailed history and investigations like X-ray chest after passing nasogastric catheter.

Baseline data like age, sex, whether pre-term or term baby, gestational age was recorded in the pre designed, pre tested, and semi structured study questionnaire. They were admitted in the Pediatrics wards. Depending upon the requirement, the newborns were kept on either ventilator or were given the mechanical ventilation prior to the surgery to stabilize them. 2-D echo was carried out to find out the associated cardiac abnormalities. All babies underwent investigations like complete blood count, C reactive protein, X-ray, serum electrolytes, arterial blood gases, and blood culture.

Once the baseline pre anesthetic requirements were met with, the newborn was taken for surgery. Depending upon the final diagnosis the type of surgery required was determined and the procedure was carried out as per the standard guidelines and operating techniques. Various surgical procedures performed were End to end anastomosis, Esophageal anastomosis, Esophagostomy, Esophagostomy + gastrostomy, Feeding gastrostomy, Fistula ligation, Gastrostomy, Tracheoesophageal ligation, Tracheoesophageal repair, Thoracotomy + gastrostomy + esophagostomy.

Adequate post-operative care was taken. The child was examined thoroughly. All cases were followed till the complete post-operative period and the complications and outcome was assessed. Outcome was classified as death or discharge.

Statistical analysis

The data was entered in the Microsoft Excel worksheet and analyzed using proportions. Yates corrected chi square was used and two tailed p value <0.05 was taken as statistically significant.

Results

Table 1

Distribution of study subjects as per baseline parameters

Variable

Number

Percentage

Sex

Male

15

71.4

Female

06

18.6

Term status

Term baby

18

85.7

Pre-term baby

03

14.3

Gestational age

Appropriate for gestational age

16

76.2

Small for gestational age

03

14.3

Intra uterine growth retardation

02

9.5

Diagnosis

Tracheoesophageal fistula

19

90.5

Esophageal atresia

02

9.5

Males outnumbered females giving a male to female ratio of 2.5:1. There were only 3 (14.3%) pre-term babies. Majority i.e. 16 (76.2%) were appropriate for gestational age. Only two cases (9.5%) were found to have esophageal atresia. (Table 1)

Figure 1

Distribution of study subjects as per requirement of pre-operative ventilator.

https://typeset-prod-media-server.s3.amazonaws.com/article_uploads/004d4ab2-cd4e-44b1-94ea-e24f22ba0508/image/34b7a2be-acbd-49a5-a5ec-e327efd9bc9a-uimage.png

Majority (57.1%) did not require ventilator pre-operatively. Remaining nine cases required it and the most common requirement was mechanical ventilation in 33.3% of the cases. (Figure 1)

Figure 2

Distribution of study subjects as per associated abnormalities.

https://typeset-prod-media-server.s3.amazonaws.com/article_uploads/004d4ab2-cd4e-44b1-94ea-e24f22ba0508/image/6ef38610-26cb-4011-b0be-bb0048b8e04c-uimage.png

Majority (61.9%) of the cases had no associated abnormality. Two cases had associated atrial septal defect (9.5%). Vacterl was seen in only two cases (Figure 2)

Figure 3

Distribution of study subjects as per surgery performed

https://typeset-prod-media-server.s3.amazonaws.com/article_uploads/004d4ab2-cd4e-44b1-94ea-e24f22ba0508/image/fa1e2588-9b85-49e5-bfa0-abb6d595f285-uimage.png

The most common type of surgery required and performed was tracheoesophageal repair in 52.4% of the cases. (Figure 3 )

Figure 4

Distribution of study subjects as per the outcome

https://typeset-prod-media-server.s3.amazonaws.com/article_uploads/004d4ab2-cd4e-44b1-94ea-e24f22ba0508/image/192f28f8-d8d8-4ed7-b59d-87b14baf46b8-uimage.png

Five cases (23.8%) got Discharge against medical advice while two cases (9.5%) died during the post-operative period. 14 cases (66.7%) recovered and were discharged (Figure 4)

Table 2

Distribution of study subjects as per post-operative complications

Post-operative complications

Number

Percentage

Nil

02

9.5

Anastomotic leak

12

57.1

Gastro esophageal reflux disease

03

14.3

Respiratory morbidity

01

4.8

Stricture

02

9.5

Tracheomalacia

01

4.8

Total

21

100

Most of the cases (57.1%) had Anastomotic leak. Among others was Gastro esophageal reflux disease in three cases (14.3%) followed by stricture in two cases and two cases had no complications (Table 2)

Table 3

Association between term status and outcome   

Term status

Outcome

Total

Chi square

P value

Death

Discharge

DAMA

Pre term

0

2 (9.5%)

1 (4.8%)

3 (14.3%)

0.4667

0.792

Term

2 (9.5%)

12 (57.1%)

4 (16.1%)

18 (85.7%)

Total

2 (9.5%)

14 (66.7%)

5 (23.8%)

21 (100%)

We examined an association between term status and outcome. There were two deaths in term babies compared to zero in pre term babies but this difference was not found to be statistically significant (p>0.05). (Table 3)

Table 4

Association between associated abnormalities with outcome

Associated abnormalities

Outcome

Total

Chi square

P value

Death

Discharge

DAMA

No

2 (9.5%)

8 (38.1%)

3 (14.3%)

13 (61.9%)

1.988

0.369

Yes

0

7 (33.3%)

1 (4.8%)

8 (38.1%)

Total

2 (9.5%)

15 (71.4%)

4 (19.1%)

21 (100%)

Among Babies with no associated abnormalities two deaths were encountered compared to nil among those with associated abnormalities. But this difference was not found to be statistically significant (p>0.05). (Table 4)

Discussion

Males outnumbered females giving a male to female ratio of 2.5:1 in the present study. Al-Salem AH et al10 in their review of 94 cases also observed that males were than females (55 vs. 39). Tsai JY et al11 in their analysis of 81 cases over two decades also noted that males outnumbered over females (46 vs. 35). Engum SA et al12 also reported male preponderance from their study (127 vs. 100). Acher CW et al13 also noted that 56% in their study subjects were males.

There were only 3 (14.3%) pre-term babies. Majority i.e. 16 (76.2%) were appropriate for gestational age. Only two cases (9.5%) were found to have esophageal atresia.

Majority (57.1%) did not require ventilator pre-operatively. Remaining nine cases required it and the most common requirement was mechanical ventilation in 33.3% of the cases. There were two cases who had aspiration pneumonia while Al-Salem AH et al10 in their study found that the incidence of aspiration pneumonia was very high i.e. 39.4% at the time of admission.

Majority (61.9%) of the cases had no associated abnormality. Two cases had associated atrial septal defect (9.5%). Vacterl was seen in only two cases. AH et al10 in their study found that 49% had associated abnormalities which are higher than the present study of 38.1%. Engum SA et al 12 had reported from their study that 64% of the babies had associated abnormalities. Friedmacher F et al 14 reported from their study that 64.2% had associated abnormalities.

The most common type of surgery required and performed was tracheoesophageal repair in 52.4% of the cases. Five cases (23.8%) got Discharge against medical advice while two cases (9.5%) died during the post-operative period. 14 cases (66.7%) recovered and was discharged. The mortality rate was high (30.8%) in the study carried out by AH et al.10 In the study by Tsai JY et al11 the most common surgery performed was primary esophageal anastomosis in 44 cases out of total 81 cases included. Tsai JY et al11 found that the mortality rate was 22% which is higher than the present study of 9.5%. Engum SA et al12 had performed a single-layer anastomosis 81% of the cases. In their study the mortality rate was only 5% which is lower than the present study. Friedmacher F et al14 also carried out primary surgical repair in majority of their study subjects and the death rate was only 4.2% during the post operative period. Acher CW et al 13 also observed in their study that standard open repair was most commonly performed surgery.

Most of the cases (57.1%) had Anastomotic leak. Among others was Gastro esophageal reflux disease in three cases (14.3%) followed by stricture in two cases and two cases had no complications. In the study by Tsai JY et al11 the most common complication was stricture in 40% of the cases followed by anastomotic leak in 19% of the cases among those who underwent primary esophageal anastomosis. Engum SA et al12 had reported from their study that 16% had anastomotic leak, 35% had stricture, 3% had recurrent fistula. Incidence of post operative leak was 26% in the study by Acher CW et al.13

We examined an association between term status and outcome. There were two deaths in term babies compared to zero in pre term babies but this difference was not found to be statistically significant (p>0.05). Among Babies with no associated abnormalities two deaths were encountered compared to nil among those with associated abnormalities. But this difference was not found to be statistically significant (p>0.05). But Tsai JY et al11 found that the death rate was more in those with the associated abnormalities.

Conclusion

Trachea-esophageal fistula and esophageal atresia was more common in males. Tracheoesophageal repair was commonly required surgery. Recovery rate was good. Anastomotic leak was most common complication. Outcome like death was not associated with term status and associated abnormalities.

Conflict of Interest

The authors declare that there are no conflicts of interest in this paper.

Source of Funding

None.

References

1 

L Spitz Oesophageal atresiaOrphanet J Rare Dis200722410.1186/1750-1172-2-24

2 

A E Mortell R G Azizkhan Esophageal atresia repair with thoracotomy: the Cincinnati contemporary experienceSemin Pediatr Surg200918112910.1053/j.sempedsurg.2008.10.003

3 

L Spitz KW Ashcraft GW Holcomb JP Murphy Esophageal atresia and tracheoesophageal malformationsPediatrics SurgeryPhiladelphia: Saunders200535270

4 

A Goyal M O Jones J M Couriel P D Losty Oesophageal atresia and tracheo-oesophageal fistulaArch Dis Child Fetal Neonatal Ed20069153814

5 

C Sparey G Jawaheer A M Barrett S C Robson Esophageal atresia in the Northern Region Congenital Anomaly Survey, 1985-1997: prenatal diagnosis and outcomeAm J Obstet Gynecol2000182242731

6 

E E Laffan A Daneman S H Ein D Kerrigan D E Manson Tracheoesophageal fistula without esophageal atresia: are pull-back tube esophagograms needed for diagnosis? Pediatr Radiol2006361111417

7 

R Babu A Pierro L Spitz D P Drake E M Kiely The management of esophageal atresia in neonates with right-sided aortic archJ Pediatr Surg2000351568

8 

S Teich D P Barton M E Ginn-Pease D R King Prognostic classification for esophageal atresia and tracheoesophageal fistula: Waterston versus MontrealJ Pediatr Surg199732710759

9 

S A Engum J L Grosfeld K W West F J Rescorla L R Scherer Analysis of morbidity and mortality in 227 cases of esophageal atresia and/or tracheoesophageal fistula over two decadesArch Surg199513055028

10 

A H Al-Salem M Tayeb S Khogair A Roy N Al-Jishi K Alsenan Esophageal atresia with or without tracheoesophageal fistula: success and failure in 94 casesAnn Saudi Med20062621169

11 

J Y Tsai L Berkery D E Wesson S F Redo N A Spigland Esophageal atresia and tracheoesophageal fistula: surgical experience over two decadesAnn Thorac Surg199764377883

12 

S A Engum J L Grosfeld K W West F J Rescorla Scherer 3rd LR. Analysis of morbidity and mortality in 227 cases of esophageal atresia and/or tracheoesophageal fistula over two decadesArch Surg199513055028

13 

C W Acher D J Ostlie C M Leys S Struckmeyer M Parker P F Nichol Long-Term Outcomes of Patients with Tracheoesophageal Fistula/Esophageal Atresia: Survey Results from Tracheoesophageal Fistula/Esophageal Atresia Online CommunitiesEur J Pediatr Surg201626647680

14 

F Friedmacher B Kroneis A Huber-Zeyinger P Schober H Till H Saur Postoperative Complications and Functional Outcome after Esophageal Atresia Repair: Results from Longitudinal Single-Center Follow-UpJ Gastrointest Surg201721692735



jats-html.xsl

© This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.


  • Article highlights
  • Article tables
  • Article images

Article History

Received : 28-06-2021

Accepted : 07-09-2021

Available online : 14-10-2021


View Article

PDF File   Full Text Article


Copyright permission

Get article permission for commercial use

Downlaod

PDF File   XML File   ePub File


Digital Object Identifier (DOI)

Article DOI

https://doi.org/10.18231/j.ijmpo.2021.028


Article Metrics






Article Access statistics

Viewed: 148

PDF Downloaded: 56



Wiki in hindi