The site of the Media Sphera Publishers contains materials intended solely for healthcare professionals.
By closing this message, you confirm that you are a certified medical professional or a student of a medical educational institution.

R.A. Nafikova

Bashkir State Medical University;
Republic Children’s Hospital

E.V. Karaninskii

Bashkir State Medical University

A.E. Neudachin

Republican Children’s Hospital

I.I. Galimov

Bashkir State Medical University

Combination of esophageal atresia with proximal tracheoesophageal fistula and meconium peritonitis: a case report


R.A. Nafikova, E.V. Karaninskii, A.E. Neudachin, I.I. Galimov

More about the authors

Views: 876

Downloaded: 24

To cite this article:

Nafikova RA, Karaninskii EV, Neudachin AE, Galimov II. Combination of esophageal atresia with proximal tracheoesophageal fistula and meconium peritonitis: a case report. Pirogov Russian Journal of Surgery = Khirurgiya. Zurnal im. N.I. Pirogova. 2023;(5):101‑104. (In Russ., In Engl.)


Esophageal atresia with proximal tracheoesophageal fistula is a rare variant of this malformation. According to different authors, the incidence ranges from 0.8% to 5.7%. Meconium peritonitis is also a rare disease following intestinal perforation and leading to aseptic peritonitis. Prevalence of meconium peritonitis is 1 per 30000 newborns. According to the literature, atresia of the esophagus, small or large intestine, anorectal malformation and bowel duplication are associated with meconium peritonitis in 5-6% of cases. However, we found only one report of combination of esophageal atresia with proximal fistula and meconium peritonitis in the literature.

The female neonate was born at 38 weeks of gestation. Birth weight was 3870 g, height was 53 cm. Apgar scores were 7 and 8 after 1 and 5 min, respectively. The child was breathing spontaneously and did not need for oxygen support. The abdomen was soft, painless and not swollen. Physical examination revealed no any other abnormalities. Antenatal ultrasound at 22 weeks of gestation revealed signs of esophageal atresia. Thus, there was an attempt to insert a feeding tube into esophagus after birth. However, orogastric tube was not passing to the stomach after 7 cm. A definitive diagnosis was made after contrast-enhanced X-ray examination with water-soluble contrast 1.0 mL injected into orogastric tube. There was no evidence of contrast agent passage to the stomach. Proximal esophagus was dilated and ended at the C8-Th1. There was no contrast agent leakage in the tracheobronchial tree, and stomach or intestines were not filled with gas. Additionally, numerous calcifications in abdominal cavity could indicate on meconium peritonitis (Fig. 1). Abdominal ultrasound revealed moderate amount of fluid with inclusions (fibrin?) and calcifications.

Fig. 1. Chest and abdominal X-ray in direct projection.

Contrast agent is localized in proximal esophagus at the level of C8-Th1. No gas filling of the stomach and intestines. There is a large amount of calcifications in abdominal cavity.

Bronchoscopy and esophagoscopy were performed to rule out proximal tracheoesophageal fistula. There was an oval-shaped 3-mm defect on the border of the upper and middle thirds of the trachea in membranous part (proximal tracheoesophageal fistula) (Fig. 2). The lumen was not visualized. Esophagoscopy found no fistula.

Fig. 2. Bronchoscopy. Proximal tracheoesophageal fistula.

Diagnostic laparoscopy was performed after appropriate preoperative preparation. We found a conglomerate of intestinal loops and adhesive process. Bowel loops were covered with fibrin and calcifications. Meconium peritonitis (MP) was confirmed that required conversion to laparotomy. When dissecting conglomerate, we found perforation 2.0 x 2.0 mm of the transverse colon wall covered with calcifications and fibrin (Fig. 3). Resection of this segment (2 cm long) was followed by a single-barrel colostomy. The efferent colon was sutured. Adhesiolysis, irrigation and drainage of abdominal cavity (in the right iliac region) were performed. Further revision revealed a small-sized stomach. Conventional gastrostomy (Kader procedure) was performed. To assess the distal esophagus, we applied intraoperative contrast-enhanced examination of the stomach. A water-soluble contrast agent was injected through gastrostomy. Distal esophagus was visualized is at the level of diaphragm, TEF was not detected (Fig. 4). End-to-end esophageal anastomosis was impossible. Proximal TEF was sutured, and cervical esophagostomy was performed. As soon as clinical state became stable, the child was discharged. Retrosternal colonic esophagoplasty is scheduled.

Fig. 3. Calcifications and fibrin on bowel walls. Intraoperative image.

Fig. 4. X-ray of the stomach and esophagus: microgastria, occlusion (atresia) of distal esophagus at the level of diaphragm.


There are cases of combination of esophageal atresia and meconium peritonitis. In 1981, Chattergee S.K. et al. reported combination of esophageal atresia with meconium peritonitis. The diagnosis was based on native X-ray data: no intestinal gas filling, abdominal calcifications. In 2003, Hibi M., Shimotake T., Iwai N. reported esophageal atresia combined with distal TEF, duodenal atresia and meconium peritonitis. The diagnosis was also established considering X-ray findings. In 2012, Theron A. et al. described a similar case of esophageal atresia combined with proximal TEF and meconium peritonitis. Proximal fistula was found during bronchoscopy. Esophageal anastomosis was impossible in all these cases due to large diastasis between proximal and distal parts of the esophagus [1-3].

Thus, radiography is the primary method of diagnosis confirming both diseases. However, the absence of gas-filled bowel loops on X-ray images in pediatric patients with esophageal atresia can be regarded as fistula-free form of atresia. Importantly, esophageal atresia can be with proximal TEF in rare cases [4]. There are currently no clear guidelines for tracheobronchoscopy in children with esophageal atresia, and this procedure is not performed routinely. According to our experience, tracheobronchoscopy is valuable for preoperative diagnosis and avoiding possible errors in treatment. Exploration of the intestine is a significant part of surgery because perforation hole is usually very small and covered with calcifications. Perforation of the ileum is more common in patients with meconium peritonitis. In our case, perforation was located in the transverse colon [5-8].


Thus, esophageal atresia with proximal TEF is a rare malformation like meconium peritonitis. However, combination of these diseases is extremely rare. Considering small incidence and no guidelines for the diagnosis and treatment of this disease, the following diagnostic procedures should be performed prior to surgery:

1) Chest and abdomen X-ray.

2) Contrast-enhanced examination of proximal esophagus with water-soluble contrast agent 0.5-1.0 mL through a feeding tube.

3) Abdominal ultrasound to assess abdominal organs, effusion and concomitant malformations.

4) Bronchoscopy and esophagoscopy are valuable to rule out proximal fistula.

5) Contrast-enhanced examination of the stomach through a gastrostomy to assess distal esophagus and possibility of esophageal anastomosis.

The authors declare no conflicts of interest.

Email Confirmation

An email was sent to with a confirmation link. Follow the link from the letter to complete the registration on the site.

Email Confirmation

We use cооkies to improve the performance of the site. By staying on our site, you agree to the terms of use of cооkies. To view our Privacy and Cookie Policy, please. click here.