Diagnostic Difficulties Reveal Rare form of Membranous Esophageal Atresia

Case Report

An esophageal atresia (EA) was suspected in a term newborn that, shortly after birth, presented with poor feeding, swallowing difficulties, excessive salivation and rattling breathing. Initial blood gas analysis was normal. A nasogastric tube (NGT) was introduced. The X-ray showed the NGT tip in the stomach with gas in the abdomen. Enteral feeding was done via NGT which was tolerated well and the newborn stabilised. At 1.5 days of life, drooling of saliva with progressive respiratory distress was observed and supplemental oxygen (FiO₂ 25%) was given. The NGT was removed on the suspicion that its location had shifted. However, upon attempts to reintroduce the NGT, difficulty was experienced when it was approximately 10 cm past the mouth. Finally the tube was able to pass and an X-ray confirmed that the tip was correctly placed in the stomach. At that time the intestinal air distribution was normal, with no apparent esophageal pouch after the addition of 5 ml of air, but an infiltrate was observed in the right upper lobe. Therefore, esophageal stenosis and secondary aspiration or a tracheoesophageal fistula (TF) were suspected. Feeding was stopped and empiric antibiotherapy started.

An endoscopic exploration showed a membrane with a small orifice in the middle third of the esophagus. An esophagogram at 9 days of age with water-soluble contrast revealed a blind pouch and contrast in the bronchial tree ([Fig. 1a–c]). A proximal TF was confirmed by bronchoscopy ([Fig. 2]).

Fig. 1 Esophagography with water-soluble contrast at 9 days of age: Initially a the NGT was removed several centimeters and contrast was injected at the middle third of the esophagus displaying a normal distal esophagus b, removing it further to the upper portion of the esophagus, contrast was injected in a very small amount, confirming the presence of a blind pouch and also contrast in the bronchial tree c. In this occasion it was impossible to re-pass the NGT showing a resistance at the level of the blind pouch.

Fig. 2 Membranous atresia with a tiny opening and proximal fistula, an uncategorized anomaly.

The patient was diagnosed with a membranous EA with a small hole and associated proximal TF. At 11 days of age, right posterolateral thoracotomy was performed, showing a wide TF at the level of T2–T3. The esophageal membrane showed an eccentric orifice of 6 mm in diameter. The TF was sectioned and ligated. A cylindrical portion of the esophagus was sectioned, including the membrane, and end-to-end anastomosis was performed.

The newborns condition in the immediate postoperative period was favorable. After 4 days, feeding was started and was well tolerated. After 8 days, the general state deteriorated and an esophagogram confirmed a partial anastomotic dehiscence. Enteral feeding was interrupted and empiric antibiotics were administrated. A new esophagogram carried out 6 days later showed closure of the defect.

Feeding was reinitiated through the NGT which was now being poorly tolerated, with abundant gastric retentions. To accelerate gastric emptying, erythromycin as a prokinetic drug was added, which improved digestive tolerance. However, gastric retentions reappeared 7 days later, as well as vomiting and abdominal distension. Suspecting an obstruction, an ultrasound was performed confirming the existence of a hypertrophic pyloric stenosis (HPS).

A pyloromyotomy was performed at 34 days of age without complications. Esophageal dilatation was required at 3 months of age.