A Rare Variant Of Esophageal Atresia

To the Editor,

Pure esophageal atresia (PEA) is characterized by a long segment between the esophageal pouches without tracheoesophageal fistula (TEF). Here, we present a very rare case of esophageal atresia (EA) in which the two blind pouches were connected by an atretic segment and proximal TEF was observed.

A girl baby was born with a birth weight of 1970 g. Radiologic diagnosis of PEA was made by detecting a blind pouch of the proximal esophagus and the gasless abdomen. A gastrostomy was made. At 4 weeks of age, the patient underwent thoracotomy. It was observed that both ends of the esophagus were connected by an atretic segment (Figure 1). This segment was resected, and both ends were anastomosed. Mediastinal exploration did not show TEF. Histopathological examination of the specimen showed that the atretic segment consisted of smooth muscle tissue without lumen (Figure 1). The patient was discharged on the 14th day. She was hospitalized twice due to right upper lobe pneumonia within the next 2 months. Contrast esophagogram showed a missed proximal TEF. The diagnosis was confirmed by bronchoscopy. Therefore, she underwent rethoracotomy. The upper TEF was identified at the level of thoracic inlet and was repaired.

This type EA is very rare and only a few cases ha- ve been reported in the literature. Membranous obstruction with normal outer appearance of the esophagus and an atretic esophageal midportion in which a cyst located in this segment have been described (1,2). Sanal et al. (3) described a new subtype in which the proximal and the distal segments of the esophagus are connected by an atretic segment as observed in our case. The diagnosis of upper TEF is more difficult and requires a high index of suspicion based on clinical symptoms. It is associated with choke while attempting to feed, unexplained cyanotic spells or bouts of pneumonia. It may be missed on routine esophageal contrast studies. Even though a confident radiologic diagnosis, bronchoscopy is often required to confirm the diagnosis. Preoperative bronchoscopy could not be performed in our case because of technical difficulties. The classic location of an upper TEF within the upper pouch is near its distal end, but it was at the most proximal location in our case. All of these might be the reason for the missed TEF.

EA with muscular continuity is a rare entity, and may be associated with upper pouch TEF.  Therefore, great care must be taken to exclude a proximal TEF in these cases.

REFERENCES

1. van Loosbroek AF, Baeten CG, Kootstra G. A rare case of congenital esophageal obstruction by double membranes: a case report. Eur J Pediatr Surg 1991; 1:366-8.
2. Matsumoto Y, Ogawa K, Yamamoto T, et al. Extremely ra- re types of esophageal atresia: two case reports of membra- nous atresia and multiple atresia of the esophagus. Sur- gery 1972; 71:795-800.
3. Sanal M, Haeussler B, Tabarelli W, et al. Pure esophageal atresia with normal outer appearance: case report. J Pedi- atr Surg 2007; 42:E1-3