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Year : 2018  |  Volume : 7  |  Issue : 1  |  Page : 14-22

Esophageal atresia: Early outcome analysis from a high-volume tertiary care institute in India

Department of Paediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India

Date of Web Publication16-Apr-2018

Correspondence Address:
Rahul Gupta
Department of Paediatric Surgery, SMS Medical College, Jaipur, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/sjhs.sjhs_116_17

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Context: Despite the progress made in the field of surgical techniques and neonatal care, conditions such as prematurity, very low birth weight, and associated anomalies compounded with delayed diagnosis may negatively influence the successful outcome of esophageal atresia (EA). Aims: The aim is to study the early outcome analysis of patients with EA and tracheoesophageal fistula (TEF) at a high-volume tertiary care institute. Settings and Design: A retrospective study performed from January 2016 to December 2016. Subjects and Methods: The study included all patients of EA and TEF admitted in the Neonatal Intensive Care Unit of our department. Results: There were 430 new cases of EA/TEF. Type C was the most common (90.23%), followed by Type A (8.37%), Type E (0.69%), and one case each of Type D, esophageal web, and esophageal stenosis. Two hundred and twenty-four (52.10%) remained undiagnosed on the 1st day of life. The average birth weight was 2200 g; 64.65% of patients were low birth weight. Associated major anomalies were present in 18.84% patients with gastrointestinal tract anomalies in 13.49% cases (anorectal malformation – 11.86%, duodenal atresia [DA] – 2.79%, and triple atresia – 0.93%) and VACTERL association in 11.63%. Surgical procedures were performed in 413 (96.05%) patients. Survival was seen in 29.77% cases, mortality in 68.84% and 1.39% patients left against the medical advice. The survival was better in Type C (29.89%) than Type A (27.78%) and also those without associated anomalies. VACTERL association had a very high (80%)-mortality rate. Septicemia (63.85%), severe pneumonia (53.71%), and congestive heart failure (24.32%) were main causes of mortality. Conclusions: Although there has been a marked improvement in the antenatal diagnosis of EA, most cases in our study remain undiagnosed in first 24 h of birth. Associated major anomalies were documented in approximately 1/5th patients. Survival was present in less than 1/3rd patients; survival outcomes were better in EA without associated anomalies. Early diagnosis with the help of red rubber catheter shortly after birth, measures to prevent pneumonia, strict infection control measures for prevention of septicemia, deployment of sufficient resources, and investigations for other associated anomalies are recommended to improve the outcomes of EA.

Keywords: Associated major anomalies, delayed diagnosis, esophageal atresia, outcome, red rubber catheter, tracheoesophageal fistula

How to cite this article:
Gupta R. Esophageal atresia: Early outcome analysis from a high-volume tertiary care institute in India. Saudi J Health Sci 2018;7:14-22

How to cite this URL:
Gupta R. Esophageal atresia: Early outcome analysis from a high-volume tertiary care institute in India. Saudi J Health Sci [serial online] 2018 [cited 2022 Oct 3];7:14-22. Available from: https://www.saudijhealthsci.org/text.asp?2018/7/1/14/230228

  Introduction Top

Esophageal atresia (EA, one in 2500 live births) encompasses a group of congenital anomalies comprising an interruption in the continuity of the esophagus combined with or without a persistent communication with the trachea.[1],[2] It requires prompt surgical treatment for best results. The outcomes in addition to meticulous surgical technique are also dependent on the perioperative neonatal intensive care. EA is managed regularly in most tertiary neonatal units. The mortality rate associated with EA in West was reported to be <10%.[1] Furthermore, the prognosis of high-risk neonates in Europe has improved because of advanced neonatal intensive care.[3] Conditions such as prematurity, low birth weight, associated major anomalies compounded with delayed diagnosis of this entity, and scarcity of resources may negatively influence the successful outcome. Herein, we aim to study the early outcome analysis of patients with EA and tracheoesophageal fistula (TEF) at a high-volume tertiary care institute. We also describe our experience, evaluate the risk factors, and suggest measures for improving the outcomes in the future.

  Subjects and Methods Top

All neonates with EA and TEF admitted in the Neonatal Intensive Care Unit (NICU) of the department of pediatric surgery over 1 year from January 2016 to December 2016 were retrospectively studied. Patients transferred from pediatric medicine department and other hospitals with suspected EA/TEF and whose diagnosis was later confirmed were also included. Patients transferred to the unit after corrective surgery performed outside were excluded.

The clinical, operative records and other demographic details of patients of EA were analyzed along with comorbidities for next 3 months. Charts were reviewed according to age at presentation, sex, antenatal diagnosis, chief complaints, and preoperative investigations undertaken for the establishment of diagnosis, associated anomalies, operative procedure, intraoperative findings, postoperative complications, and treatment outcomes. Classification of EA was done according to Gross's anatomic classification.[1],[4] Various parameters were reviewed to analyze the risk factors associated with the morbidity and mortality. Associated anomalies that were either obvious or life-threatening requiring urgent intervention were identified and recorded. As the resources were restricted (nonavailability of bedside echocardiography [echo]), all the patients were not subjected to echo for complete cardiac evaluation. The patients who were clinically stable and having either (a) clinical features suggestive of cardiac anomalies (cyanosis/cardiac murmur/overload) or (b) radiographic features, were subjected with echo.

VACTERL association was defined as (a) if the patient has 3 or more anomalies of the vertebral, anorectal, cardiac (excluding patent ductus arteriosus and patent foramen ovale), renal/genitourinary, and limb systems including EA and (b) If the patient had both the “core” features, that is, anorectal malformation (ARM) and TEF.[5] There should be the absence of any clear evidence for an alternate, overlapping diagnosis.

The diagnosis of EA was confirmed by radiographs (both anteroposterior [AP] and lateral views) with red rubber catheter in situ. Chest drain was inserted in all patients with EA undergoing thoracotomy, taking into consideration, delayed presentation in most cases with development of wet lungs or pneumonia. Intravenous fluids, antibiotics, and parenteral nutrition were continued postoperatively. Before starting feeds, a contrast study was undertaken on the 7th postoperative day. Chest drain was removed after a 7th postoperative day when there were no signs of anastomotic leak. Thereafter, gradual feeds were initiated. Routine placement of nasogastric (NG) tube was not performed for feeding purposes (as per institutional protocol). NG tube placement was performed in only those cases with long gap with anastomosis under tension.

Long-term follow-up was not included in the study. Three cases of Type E (TEF) were diagnosed by contrast esophagography. Long gap was defined by the presence of >3 cm or 3 vertebral bodies; measurement of the gap was performed intraoperatively.

  Results Top

There were 430 new cases of EA/TEF. There were 244 (56.74%) males and 186 (43.26%) females with male: female ratio of 1.31:1. Type C was the most common (90.23%), followed by Type A (8.37%) and Type E (0.69%) as shown in [Table 1]. No case of EA with proximal TEF (Type B) could be identified.
Table 1: Types of esophageal atresia and associated anomalies

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Out of 430 admissions, only 50% cases were admitted in our institution on the 1st day of life [Table 2]. Out of 430 patients, 394 (91.63%) were admitted directly in NICU after being referred from peripheral centers or other institutions, while 36 (8.37%) neonates were transferred from the pediatric medicine department of our institution. Twenty-five of these were transferred (after diagnoses) on the same day, but there was delayed diagnoses in 11 cases, ranging from 1 to 10 days. The total delay in days was 27 days for these 11 neonates. Out of these 36 neonates, only 8 (22.22%) were survived.
Table 2: Outcomes of esophageal atresia as per the day of admission in institution and day of diagnoses

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Antenatal diagnosis of EA was present in only two cases. Only 47.90% cases were diagnosed on the 1st day of life [Table 2]. Two hundred and twenty-four (52.10%) remained undiagnosed on the 1st day of life. In 77.67% neonates, symptoms of excessive salivation and frothing from the mouth (chief presenting) made the mandatory checking with infant feeding tube to diagnose EA in the first 48 h of life. In 22.33% patients, routine checking with IFT was not performed, and the diagnosis was delayed beyond the first 2 days of life. The survival was best (36.59%) seen with neonates diagnosed on the 3rd day of life.

The diagnoses in all the cases in the study were confirmed by radiographs (both AP and lateral views) with red rubber catheter in situ. Three cases of Type E (TEF) were diagnosed by contrast esophagography. Contrast CT could be performed in only one case of Type E.

Of the 16 cases diagnosed on or later than 7th day of life [Table 2], 13 patients were Type C, one neonate with Type A, and two were Type E (diagnosed beyond the neonatal period).

Maximum cases, that is, 144 (33.49%) were admitted between July and September, with peak in July with 50 (11.63%) patients. Minimum number of cases, that is, 24 (5.58%) and 28 (6.51%) were seen in February and March, respectively.

The weight of the neonates ranged from 900 to 3500 g; 64.65% patients were low birth weight. The average birth weight was 2200 g, mode 2500 g, and median 2300 g. Weight-wise distribution of patients and their outcomes is summarized in [Table 3]. Outcome (survival) was better among females 34.95% (65) than males 25.82% (63) cases. Neonate with lowest birth weight who survived in our series weighed 1400 g.
Table 3: Outcome of esophageal atresia as per birth weight

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Associated major anomalies were present in 18.84% (81) cases with Type A (22.22%) more than Type C (18.56%) as shown in [Table 1]. The affected organ system most commonly associated with EA was gastrointestinal (GI) tract with 13.49% (58) cases. ARM was seen in 11.86% (51) cases. Low-type anomalies were present in 1.63% (7) cases, while high type in 10.23% (44) patients. Among the high-type anomalies, cloacal anomaly was seen in 0.69% (Type C-3) patients, and vestibular fistula was seen in 1.63% (Esophageal web-1, Type C-6) patients. ARM was followed by DA which was present in 2.79% (Type C-9, Type A-3) cases. In one of the case, DA was associated with multiple intestinal atresias. Triple stresia was present in 0.93% (Type C-3, Type A-1) neonates.

There were 11.63% (Esophageal web-1, Type A-6, and Type C-43) patients with anomalies that were within the constellation of defects of VACTERL association. Out of these 50 patients, mortality was seen in 80% (40) patients, while only 18% (9) patients survived and 2% (1) patient left against medical advice (LAMA). The outcomes were inferior to the overall outcome. EA Type C, DA, and low ARM along with hydrocephalus suggestive of “VACTERL-H” association was seen in one of our female patient.

Echo could be performed in 33 patients in the study. Cardiac anomalies could be identified preoperatively in only 2.33% (10) patients only, out of which cardiac septal defects were confirmed in 0.93% (4) cases, while other cardiac defects were seen in six neonates. Radiographs suggested Dextrocardia in (0.12%) 2 patients, which was confirmed intraoperatively [Figure 1]. Furthermore, right aortic arch (RAA) was recognized intraoperatively in 0.93% (4) neonates as shown in [Figure 2]. Thus, cardiac anomalies were documented in 3.72% (16) neonates only.
Figure 1: Radiographs (a and b) suggestive of dextrocardia with red rubber catheter in situ (b); intraoperative photographs (c and d) with dextrocardia along with anastomosis of both ends (d); postoperative esophagogram (e)

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Figure 2: Intraoperative photographs (a and b) with right posterolateral thoracotomy approach showing right aortic arch before and after its mobilization, respectively; radiograph (c) suggestive of right aortic arch with red rubber catheter in situ in the upper pouch near the thoracic inlet

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Cleft lip/palate anomaly and absent radius were present in 0.69% (3) patients each. Other malformations such as severe vertebral abnormalities were seen in 0.47% (2) cases. One case each of Pierre–Robin Syndrome, laryngeal stenosis, hydrocephalus, and posterior urethral valves (PUVs) was present in the study. Spontaneous pneumoperitoneum was seen in 0.69% (3) patients.

Surgical procedures were performed in 96.05% (413) patients. Six (1.39%) patients did not undergo definite surgery because the parent's attendants declined further treatment early in the study (LAMA) and 11 (2.56%) out of 430 patients died before any intervention (preoperatively) could be contemplated due to the preexisting cardiac malformations and/or severe pneumonia. All procedures were performed between 2 and 48 h following diagnoses and under the supervision of the consultants. In Type C patients with associated anomalies (72) and those without (316), the surgical procedure was done in 66 and 307 cases, respectively. The surgical procedures of all the cases have been summarized in [Table 4]. A transanastomotic tube was left in 15 patients with Type C because of wide (>3 cm) gap length between the two esophageal ends. However, circular myotomy was not performed in any of the patients with long gap. Among all the cases under study, primary gastric pull up was performed in 3 patients.
Table 4: Summary of procedures undertaken for various types of esophageal atresia

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All 4 neonates with RAA [Figure 2] were managed with right posterolateral thoracotomy (RPLT). Two patients with dextrocardia [Figure 1] were also managed with RPLT; out of the two, one patient had low birth weight, and gap length of >3 cm died, while the other survived.

One patient was transferred to our department with the diagnosis of PUVs with bilateral vesicoureteral reflux on the 11th day of life. The neonate underwent vesicostomy because of persistently elevated creatinine levels in spite of bladder catheterization. The diagnosis of EA was established after feeds were initiated postoperatively; he aspirated and died [Table 4].

Kimura's duodenoduodenostomy was performed in 11 out of 12 patients. Repair of gastric perforation was done in addition to Kimura's DD in one case of DA. Colostomy for high-type ARM was performed in 36 cases, left transverse loop colostomy in 34, and sigmoid loop colostomy in 2 patients; in one case colostomy could not be contemplated due to associated comorbidities. In all cases with vestibular fistula (7), the colostomy was deferred as primary repair is being performed for these at 3 months of age. Low-type anomalies were managed by anoplasty in 4 out of 7 cases; in rest of the patients, the surgical procedure was not performed and only anal dilatation was performed in view of unstable general condition.

Tracheostomy in addition to primary repair of EA Type C was performed in one neonate with associated Pierre–Robin syndrome. In three cases with spontaneous pneumoperitoneum, in addition to primary repair, abdominal drain placement was done in 2 cases due to tension/marked pneumoperitoneum.

Both patients with esophageal stenosis and esophageal web were managed by RPLT [Table 4]. Fistula ligation from cervical approach was performed in one patient with Type E. Type D was diagnosed after the dye study was performed postoperatively (RPLT, distal fistula ligation, and end-to-end esophageal anastomosis).

Three patients were reoperated in the same admission for major anastomotic leak. Reanastomosis (1), gastrostomy with feeding jejunostomy (1), and gastrostomy (1) were performed in these patients; only one survived.

Survival was seen in 29.77% cases, mortality in 68.84% and LAMA in 1.39% [Table 5]. The outcome (survival) was better in Type C (29.89%) than Type A (27.78%) and also those without than with associated malformations in both Type C (31.96% vs. 20.83%) and Type A (28.57% vs. 25%).
Table 5: Outcomes as per the type of esophageal atresia

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More than one reason could be attributed to the cause of death in 296 patients in the present study. Eleven (3.72%) patients out of 296, died before operative intervention could be contemplated. One (0.34%) patient of EA with PUV died after vesicostomy was performed, as mentioned previously. Twelve (4.05%) patients died within 24-h postoperatively. Mortality was highest, that is, 147 (49.67%) between 1 and 3 days postoperatively, followed by 84 (28.38%) patients who died between 4 and 7 days, while 41 succumbed beyond 1-week postoperatively. The most common etiology was septicemia, which was in 189 (63.85%) patients followed by respiratory failure (severe pneumonia) in 159 (53.71%) patients and congestive heart failure 72 (24.32%). Sclerema was confirmed in 5 (1.69%), while major anastomotic leak was seen in 7 (2.36%) patients.

Complications among the patients with favorable outcome (128) were: wound infection (11), pneumonia (11), and wound dehiscence (9), septicemia with thrombocytopenia (7), minor anastomotic leak (3), major anastomotic leak (1), and gastroesophageal reflux (GER, 3). The mean hospital stay for patients with favorable outcome was 12 days (range 7–22 days).

Follow-up was poor with only 59 (46.09%), out of 128 patients reporting during 3 months duration. Recurrence of TEF was reported in none of the case during follow-up of 3 months.

There were 20 readmissions (M12, F8), out of which 18 patients were Type C and 2 were Type A. Pneumonia (11) was the indication for readmission followed by anastomotic stricture (4), septicemia (2), tracheomalacia (1), minor leak (1), and major anastomotic leak (1).

Out of four patients who demonstrated stricture within 3 months of follow-up postsurgery, 2 responded to esophageal dilatation, while the other two underwent redo surgeries for severe anastomotic stricture (impassable, endoscopically) with stricture excision and reanastomosis (1) and gastrostomy (1) in other. Latter cases were associated with long-gap (>3 cm) EA. One patient with major anastomotic leak underwent esophagostomy with gastrostomy. Patients with esophagostomy and/or gastrostomy are under follow-up for definitive surgery.

  Discussion Top

The concept of ligation of the TEF and anastomosis of the two ends of the esophagus in EA was proposed by Richter in 1913.[1] First survivors following staged repair with later esophageal replacement were reported by Leven and Ladd in 1939.[1] Cameron Haight is credited with first (1941) successful primary anastomosis of the esophageal segments.[6] With advancement in early diagnoses and neonatal care, there has been a marked improvement in survival.

EA is diagnosed in the antenatal period by “absent fetal stomach bubble” associated with polyhydramnios and presence of blind-ending upper pouch on fetal swallowing on ultrasound examination. Antenatal detection rate of EA is low and was reported to be 9.2% in one study,[7] while in our series, it was seen in only two isolated cases. In our setup, the resources for antenatal diagnosis were scarce and diagnosis was made in the postnatal period (when the neonate became symptomatic).

In our patients, the presentation was similar to that described in the previous literature, that is, drooling of saliva from the mouth (excessive salivation/frothing), choking or transient cyanosis shortly after birth, vomiting or regurgitation after attempted feeds, and difficulty in breathing and respiratory distress.[6] Without the early diagnosis of EA, pulmonary symptoms usually develop by the 2nd day; this may be diagnosed as pneumonia (attributed to the respiratory infection). Pneumonia was the second most common (53.71%), but the primary preventable cause of death in our study. Pneumonia occurs due to the aspiration of the salivary secretions from the upper esophageal pouch and reflux of the gastric secretions through the distal fistula.

EA is confirmed by passing no. 10 sterile, blunt-tipped red rubber catheter into the esophagus. Failure to pass beyond 10 cm (usually) or failure to negotiate into the stomach (occasionally) is considered diagnostic of EA.[6],[8],[9] Early establishment of diagnosis in preventing the undesired complications like pneumonia has been stressed by various authors.[1],[4],[6] The author recommends the routine use of no. 10 sterile, blunt-tipped soft red rubber catheter to rule out EA shortly after the birth in the delivery (resuscitation) room. This procedure would prevent any patient with EA being missed, and thereby preventing delayed diagnosis. In our setup, antenatal diagnosis of EA is seldom present, thus the use of red rubber catheter is important for increasing the catch. In any neonate with respiratory distress, EA should be ruled out as it might have been missed earlier. In addition, it is used to evaluate for the level of upper pouch in radiograph, facilitating identification and dissection of the upper pouch during primary repair. Use of infant feeding tube must be discouraged as it may be associated with false-negative results due to accidental transtracheal gastric intubation through the TEF [8] or passage up to a significant length in the elongated upper esophageal pouch.[9]

In our series, the diagnosis was not made at the time of birth or in the 1st day of life in more than 50% cases which led to pneumonia and high mortality rate. However, 77.67% neonates were diagnosed in the first 48 h of life; this included most of the neonates with associated malformations particularly with severe malformations (VACTERL association). They were diagnosed on the 1st or 2nd days of life either because of the conspicuous physical findings (e.g. absence of anus) or bilious vomiting in case of DA. Low favorable outcome (27.18% and 32.81%) among the early diagnosed cases (day 1 and day 2, respectively) was because of the associated malformations. This explains the cause of overall high mortality in patients diagnosed within 2 days of life.

Birth weight has tremendous impact on the outcome and has been substantiated by authors from Asia.[10],[11] Low birth weight was reported as 36.1% by Chang et al.,[10] while it was high (64.65%) in our patients. Patients weighing <2000 g at birth had a significantly lower survival rate compared with patients weighing at least 2500 g at birth. The favorable outcome was best with neonates weighing ≥3000 g in our series. Thus, low birth weight was associated with poor prognosis in our study.

The incidence of Type A was 8.37% in our series, which is similar to reports presented by other authors. Incidence is described as 7% according to one study and may range from 2% to 15%.[10],[11] Type C was more than 90% in our study and the values corroborate with other Asian study.[1] Incidence of Type E (0.69%) was lower than described (4%) in the previous literature.

Associated major anomalies were present in 18.84% cases, which are lower than the other series, as detailed assessment of cardiac, urological and other minor anomalies could not be completed. The incidence of anomalies associated with EA/TEF range between 30% and 60%.[1],[11],[12],[13] In one recent study from Korea, the incidence was as high as 69.4%.[10] Approximately 5% anomalies are incompatible with life.[13]

Among all the associations, most common are the cardiovascular anomalies (1/3rd cases), ranging from 30% to 59.7%.[11],[12],[13] The most common anomalies in this group are Tetralogy of Fallot and ventricular septal defect. Others are septal defects, pulmonary atresia/pulmonary stenosis, aortic stenosis, and dextrocardia.[1],[10],[11],[12],[13] According to Myers et al., the routine investigation should include renal ultrasound and echo,[14] but a detailed cardiovascular assessment was not part of study in our resource-limited (including shortage of workforce) high-volume setup, although thorough search is vital as its treatment may take priority overcorrection of EA. There is nonavailability of pediatric cardiac surgery facilities in our setup.

VACTERL association refers to the nonrandom co-occurrence of vertebral anomalies (V), anal atresia (A), congenital heart defects (C), TEF, renal anomalies (R), and limb defects (L).[5] VACTERL association was present in 19.4% patients in one recent study,[10] lower yield (11.63%) in our study was due to under evaluation in a recourse-limited setup. If two or more than two anomalies are considered for the diagnostic criteria of VACTERL association, it may be as high as 41%.[11] Furthermore, VACTERL-H association was seen in one of our female patient. This patient was a female sex which is in contrast to previous reports in the literature.[5]

After the cardiovascular anomalies, the other organ system involved is GI (24%), followed by genitourinary (20%), orthopedic, and other miscellaneous anomalies.[13] GI anomalies were seen in 26.67% cases in one large series,[15] but in our series, the total percentage was less (13.49%). ARM was also the most common anomaly (11.86%) among the GI group, as seen in other studies also.[11],[13],[15] High-type anomalies predominate and more with Type A.[1],[13]

None of the newborn underwent chromosomal analysis, but there was one neonate with DA along with features consistent with Down's syndrome in our study. There is 5% incidence of chromosomal anomalies in EA.[13] Rarely, EA may be associated with the Holt–Oram syndrome, DiGeorge syndrome, polysplenia, and Pierre–Robin syndrome (seen in our series).[16]

Surgery in EA should ideally be performed within the first 24 h as delay in surgical correction increases the risk of aspiration pneumonia, as discussed earlier. Operative approach for EA with TEF is classical RPLT with the extrapleural approach.[1],[6]

About 5% patients with EA have RAA,[13] but in our case, it was documented in 0.93% neonates only. Preoperative detection rate is low (8%–10%).[13],[17] If the diagnosis is suggested preoperatively with either echo or radiographs, thoracotomy is usually performed opposite to the side of the aortic arch.[10],[11] We performed RPLT in all the 4 cases as preoperative echo could not be undertaken. In two of these cases, gap length of more than 3 cm was encountered with insufficient length to perform the tension-free anastomosis outside the RAA. The aorta was mobilized by dividing few highest intercostal arteries. The distal esophageal pouch was then brought behind the mobilized aortic arch. The anastomosis was performed under the arch.[17]

The gap length of more than 3 cm was associated with high mortality rate. It has been more frequently observed in patients with aberrant vessels and is associated with high morbidity.[10],[11],[18] In centers with advanced neonatal care, delayed primary anastomosis with gastrostomy formation without esophagostomy is performed at 3 months of age for EA Type A.[19]

Among the postoperative complications mentioned in the literature, anastomotic stricture is the most common and may be present in 30%–40% cases.[1] Other major complications are anastomotic leak (15%–20%), GER (40%), recurrence of TEF (5%–14%), tracheomalacia (10%), recurrent pneumonia, and hiatal hernia.[1],[10],[11],[12],[13] Wound-related complications and pneumonia were the major causes of morbidity in our study. GER was seen in 3 patients among 59 cases which were in follow-up; all were treated conservatively. In one study, approximately 50% patients with GER required surgery.[20]

Factors influencing the outcome were birth weight, presence of pneumonia, associated malformations/anomalies, type of atresia, and long gap.[11] Montreal classification (prognostic grouping) is based on the use of a ventilator and major combined anomalies.[21] Although the delayed presentation is associated with poor outcome, there are reports of survival with diagnosis as late as 17th day of life.[22] In our series, there was one survival (Type C) with diagnosis as late as 15th day of life.

In West, the survival of full-term infants with EA without associated anomaly has increased above 95% and birth weight >1500 g with no major cardiac anomaly was 98.5%.[23] The overall mortality rates range from 10% to 30%.[24] Mortality in our series was very high (68.84%) as compared to Western literature and also more than other centers in Asia.[1],[7],[10],[11] Outcome (survival) with EA was low (29.77%) in comparison to infants with other neonatal surgical conditions in the NICU.[25]

The most common cause of mortality in our patients was septicemia, which was also seen in one large study performed.[11] Among the major congenital anomalies responsible for mortality in EA, almost 6%–11% of have trisomy or complex cardiac defects which are incompatible with life, thereby precluding any active management.[13] Furthermore, infants with major and complex cardiac anomalies have 30% and 70% risk of mortality, respectively.[13],[26] VACTERL association has been associated with mortality in 1/5th cases mainly due to complex cardiac anomalies;[13] in our study, it was 4/5th patients. Respiration-related complications were most common etiology of mortality in one study.[7] In our series, 2.56% patients died before any intervention could be contemplated owing to complex cardiac anomalies and/or pneumonia. Furthermore, congestive heart failure was the cause of mortality in 1/5th patients.

Survival in developed countries is not directly related to surgical techniques, but for the timely primary health care and early referral, proper transportation, availability of timely pediatric surgical facility, parallel growth of advance neonatal anesthesia and prevention of hypothermia, development and availability of sophisticated medical devices, availability of neonatal ventilators and respiratory support system, neonatal ICU with trained NICU staffs, and infection control measures.[25],[27]

Our figures are not satisfactory and contradictory to other large series owing to: (a) patient characteristics: low birth weight, late presentation to tertiary centers', delayed diagnoses, presence of pneumonia, undiagnosed associated malformations/anomalies, and VACTERL association and (b) resources: poor primary health-care infrastructure and transportation system, lack of equipped neonatal ICU with trained personnel, poor patient staff ratio, high volume of neonates with other congenital malformations, overcrowding leading to cross infection and septicemia.

Early diagnosis, prevention of pneumonia, adequate preoperative resuscitation, in neonates undergoing major surgery is invaluable for the outcome of surgery.

Recommendations for improving the favorable outcomes are as follow:

  1. Improvement of ultrasonography facilities for antenatal diagnoses of EA and associated malformations for planned institutional delivery and timely diagnosis. EA must be ruled out in all newborns' just after the birth by putting no. 10 sterile, blunt-tipped soft red rubber catheter into the esophagus to prevent the undesired complications associated with delayed diagnosis
  2. Prevention of aspiration of salivary secretions which may lead to wet lungs and thereby pneumonia is paramount. As the workforce is short, Replogle tube [28] or continuous low-pressure mucus suction apparatus preoperatively and during transport is mandatory
  3. Evaluation of associated malformations, particularly cardiac anomalies with the help of bedside echo facilities. Urological assessment with bedside ultrasonography facilities for increasing the yield of VACTERL association
  4. Round the clock neonatal care under neonatologist (not available in our setup). Thorough improvement in the nursing care delivery system and staffing pattern by imparting advanced training and their supervision. Patient-to-nurse ratio must be 1:1 from the present situation of 10:1. Modification in the present “open care system” in the NICU, which could be responsible for cross infection among our neonates. Strict infection control measures and involvement of dedicated microbiologist for periodic assessment of the cultures from NICU
  5. Provisions for genetic studies of patients, especially those with VACTERL association.

Our institution is managing a very large number of cases of EA each year. Furthermore, the total cases managed per year are probably one of the largest in the world, to the best of our knowledge. Here is a descriptive analysis of all the patients of EA with associated anomalies and the procedures performed along with true mortality and morbidity data. The weakness of our research is (a) retrospective evaluation as compared to prospective study, (b) detailed evaluation of cardiac anomalies, urogenital anomalies, and minor could not be evaluated in our study due to resource limitations, and (c) long follow-up was not completed.

Furthermore, the findings of the present study would have important implications for future research, as it may serve as reference point figures (baseline data) for guiding the researchers for taking the precise measures to improve the outcomes. It may serve to initiate further epidemiological research.

  Conclusion Top

In our study, Type C was the most common and approximately 1/3rd cases were LBW. Associated major anomalies were documented in approximately 1/5th patients with GI anomalies in 13.49% cases and VACTERL association in 11.63%. Surgical procedures were performed in 96.05% patients. Survival was present in <1/3rd patients; it was better in EA without associated anomalies and also with Type C than Type A. VACTERL association had a very high (1/5th) mortality rate. Overall survival of neonates with EA is still poor in our high-volume tertiary care center. Delayed diagnosis, late presentation, septicemia, preceding pneumonia, congestive heart failure, and overcrowding in ICU were the main causes of poor outcome in our series. Wound-related complications and pneumonia were the major causes of morbidity.

Although there has been a marked improvement in the antenatal diagnosis of EA in the West, in our setup, due to resource limitations, antenatal detection of EA is low. Furthermore, most cases in our study remain undiagnosed in first 24 h of birth. Therefore, it is recommended that EA must be ruled out in all newborns' (especially in areas with high incidence) with the help of red rubber catheter shortly after birth. Measures to prevent pneumonia, strict infection control measures for prevention of septicemia, deployment of sufficient resources, and investigations for other associated anomalies are recommended to improve the outcomes of EA.


I am sincerely thankful to faculty, residents, and nursing staff of Department of Paediatric Surgery, SMS Medical College, Jaipur, for helping me in this endeavor. Special thanks to Dr. Sunil Kumar Mehra for his support.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

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  [Figure 1], [Figure 2]

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5]


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