A successful repair of esophageal atresia (EA) with distal tracheoesophageal fistula (TEF) to a 6-day-old child in a low-resource setting. Case report

Abstract

Introduction

Congenital tracheoesophageal fistula (TEF) is a rare and life-threatening anomaly that requires prompt surgical intervention. The case report highlights a successful TEF repair in setting with significant observed cases and low survival rate.

Case presentation

We present a Type C Tracheoesophageal fistula successfully repaired by a conventional Open Surgical approach. An infant of 3 days born at term with Birth weight of 3 kg, presenting with postprandial regurgitation, choking and abdominal distension followed by early features of pneumonia. Initial chest X-ray with nasogastric (NS) tube catheter revealed features of esophageal atresia (EA) with distal TEF. An echocardiogram (ECHO) was performed, which revealed moderate patent ductus arteriosus (PDA) and small left to right shunt. A successful delayed primary repair was performed on the sixth day of life in the division of pediatric surgery center.

Conclusion

The successful outcome in this case serves as a testament to the dedication and resourcefulness of the healthcare team. It underscores the importance of high suspicious index, collaboration, adaptability, and skill in providing quality care in low-resource settings, reaffirming the possibility of saving lives even in challenging circumstances.

Highlights

• •We present a Type C Tracheoesophageal fistula successfully repaired by a conventional Open Surgical approach.
• •In many advanced centers, the thoracoscopic approach and VAT are modalities of choice over Conventional Open Thoracotomy, however due to limited resources in our setting, Conventional Open Thoracotomy was done with remarkable success results.
• •Postoperatively, it is recommended to do a water-soluble contrast-enhanced evaluation of the esophagus for the fifth to seventh day to ascertain any anastomotic leakage before initial enteral feeding. To our patients, since there was an NG-tube, enteral feeding started earlier at 3rd day after surgery and subsequently small feeds were tolerated even after NG-Tube removal after radiological assessment of the esophagus

1. Introduction

The most prevalent congenital condition affecting the upper GI tract is esophageal atresia/tracheoesophageal fistula (TEF) with an estimate of 70 %. According to the VACTREL spectrum, infants with EA/TEF also have other congenital defects including the vertebrae/ribs and cardiovascular system accounting for 68.9 % and 65.6 % of patients, respectively. In addition to that, among the variants of EA/TEF, type C is the most common variant [1,2].

Early postnatal diagnosis and proper initial treatment are crucial for a better outcome. Commonly infants present with choking, respiratory distress and unsuccessful insertion of nasogastric tube. This can be confirmed by a plain chest X-ray showing coiling of the tube in the mediastinum gas-filled GI tract on lateral view in case of distal TEF [3].

In order to establish the feasibility of surgical treatment, as well as its safety for the patient, it is necessary to take into account the presence or absence of congenital heart anomalies (CHD). The presence of CHD necessitates special anesthesia consideration for good outcome from EA/TEF surgical repair [4].

The surgical approach is centered on the TEF's location. The best way to access the cervical and cervical-mediastinal FTE is through a cervicotomy, either with or without a median sternotomy. A right thoracotomy, a video-assisted thoracoscopic surgery (VATS) technique, is typically required for the mediastinal or intrathoracic TEF [5]. The case report has been reported in line with the SCARE criteria [6].

2. Case report

A 3-day old female infant born at term with 3 kg birth weight who was referred from primary health facility to a tertiary health facility, presented with postprandial regurgitation of undigested breast milk since birth associated with choking and abdominal distension followed by dry cough and difficulty in breathing from the 2nd day of life.

Upon assessment, she was alert, acyanotic, afebrile, with normal posture, normal primitive reflexes and normal vital signs. The systemic exam revealed bilateral fine crepitations on respiratory examination and a distended hyper tympanic abdomen.

2.1. Clinical findings

After initial resuscitation with suctioning, oxygen therapy and IV antibiotics and fluids; a plain chest X-ray was done with NG-tube and revealed bilateral lung opacity more on the left lung, normal mediastinum, normal cardiac silhouette, and failure to the distal tip of NG-Tube to advance, coiled just above the carina and presence of a gastric bubble (Fig. 1). Thus a final diagnosis of Esophageal Atresia with Distal Tracheoesophageal fistula was made.

Fig. 1.

Pre-operative plain anteroposterior (AP) chest X-ray.

An ECHO was done and revealed a moderate PDA and 3.5 cm left – right shunt with good biventricular function of 91 % thus surgical repair for EA/TEF was planned.

2.2. Surgical intervention

On the sixth day of life; surgical primary repair was done. In the left lateral position, right thoracotomy was done through the 4th intercoastal space, ribs were retracted to access the posterior mediastinum. Gross esophageal atresia with proximal blind-ended esophagus and distal TEF was visualized. The azygous vein was identified, ligated and divided. The fistula was closed with prolene suture and divided, the tracheal opening was closed. NG-Tube was inserted in the proximal esophagus to identify the blinded end of esophagus followed by a water tight-end to end esophageal-esophageal anastomosis. The NG-Tube was advanced to stomach, hemostasis was ensured, right chest tube was inserted followed by wound closure.

2.3. Post-operative care

The child was admitted to Neonatal ICU for ventilator support and close monitoring, nutritionally kept on total parental nutrition (TPN), antibiotics and proton pump inhibitors. Day three postoperatively there was neither significant output from the chest tube nor air bubbles. The findings were complemented with a control chest X-ray hence the chest tube was removed and started feeding on expressed breast milk via an NG tube (Fig. 2).

Fig. 2.

Post-operative plain chest X-ray.

On day seven postoperatively the patient was extubated and on the tenth day, the NG tube was removed. A contrasted chest X-ray was done and did not reveal any anastomotic leakage or stenosis (Fig. 3) and started oral feeding, on twenty-eighth day she was discharged home. The patient was seen at the clinic monthly with remarkable weight gain, remission of chest symptoms with normal feeding patterns.

Fig. 3.

Post-operative gastrographin contrasted X-rays.

3. Discussion

The overall survival for type C TEF is generally good for infants with no other serious malformations, it is over 90 % [7], and however in our setting we are still experiencing a significant number of morbidity and mortality among patients where 10 up to 15 patients diagnosed annually. This may be due to delayed diagnosis and inadequate initial treatment such as frequent suctioning to prevent aspiration pneumonia which contributes to high mortality even before surgical intervention.

Regarding surgical management of Esophageal Atresia with Distal TEF, it is preferably recommended to perform delayed or staged repair with esophagostomy and gastrostomy, especially for patients with nutritional derangements, respiratory infection or other serious congenital malformations [8]. In primary repair complications such as anastomotic leakage, fistula recurrence and recurrent laryngeal nerve injury are highly anticipated however in our setting primary anastomosis are normally done as well as in this case delayed primary repair was performed and had no such complication.

In many advanced centers, the thoracoscopic approach and VAT are modalities of choice over Conventional Open Thoracotomy. Minimally invasive approaches have advantages in terms of operative time, time to extubation and time for first feeding and hospital stay however there is a significant difference in terms of serious early complications like anastomotic leakage and strictures [9] however due to limited resources in our setting, that has one consultant pediatric surgeon, limited imaging modalities and unavailability of instruments for thoracoscopic approach, Conventional Open Thoracotomy was done with remarkable success results.

Postoperatively, it is recommended to do a water-soluble contrast-enhanced evaluation of the esophagus for the fifth to seventh day to ascertain any anastomotic leakage before initial enteral feeding. To our patients, since there was an NG-tube, and unavailability of a water-soluble contrast-enhanced evaluation, enteral feeding started earlier at 3rd day after surgery and subsequently small feeds were tolerated even after NG-Tube removal and radiological assessment of the esophagus was done to another facility with a water-soluble contrast. Furthermore, the Enhanced Recovery After Surgery (ERAS) protocol recommends initiating enteral feeding as soon as possible to accelerate recovery and minimize nutrition-related complications [10].

Early diagnosis of congenital TEF is essential to safeguard the health and well-being of the affected infant, prevent life-threatening complications, and improve the long-term outcomes and quality of life for the child.

4. Conclusion

The aforementioned instance highlights the significance of maintaining a high index of suspicion for diagnosis of EA/TEF when confronted with a newborn who exhibits regurgitation, choking and acute respiratory distress. This case demonstrates that with the right approach, adequate training, and a commitment to patient welfare, congenital TEF repair can be achieved effectively in low-resource centers, ultimately contributing to improved healthcare accessibility and outcomes for underserved populations.

Consent

Written informed consent was obtained from the patient's parents for publication and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Ethical approval

Ethical approval for this study was provided by the Ethical Committee MUHAS, Dar es Salaam Tanzania on 15 August 2023.

Funding

This work was not funded.

Author contribution

Dr. Novath Ngowi: participated in production of initial manuscript.

Dr. Sylvery Mwesige: participated in production of initial manuscript, collection of data.

Dr. Mohammed Salim: participated in revision of manuscript, proofreading.

Dr. Victor Ngotta: surgeon who operated the patient, study conception and production of manuscript.

Guarantor

Dr Victor Ngotta

Declaration of competing interest

The author declares neither financial nor non-financial interests that may be relevant to writing and publication.