Tubular duplication of the esophagus

A 6-year-old boy presented to the emergency department with an impacted foreign body in the esophagus. His medical history was marked by a mild dysphagia for solid food, present since the age of 2 years. His physical examination was unremarkable.

An upper endoscopy was performed. After a coin in the esophagus was removed, an esophageal stenosis and an orifice in the esophageal wall were noticed (Fig. 1). Biopsy of the double lumen showed chronic esophagitis. A barium esophagram (Fig. 2) was also carried out and contributed to a diagnosis of tubular duplication of the esophagus.

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FIG. 1

Upper endoscopy of the esophagus reveals an esophageal stenosis and an orifice in the esophageal wall (arrow).

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FIG. 2

Barium esophagram contributed to a diagnosis of tubular duplication of the esophagus: diverticulum (arrow), double lumen (arrowheads), septum (curved arrows).

Esophageal duplication is a rare congenital anomaly with an estimated incidence of 1 in 8200, representing about 10% of all foregut duplications.1^–3 The cystic form is the most usual presentation of the duplication, whereas the tubular type accounts for only 5%–10% of cases.4 Esophageal duplication cysts are commonly found in the lower third (60%–95%) and on the right side,1^,5 whereas the tubular form is frequently reported in the middle and lower esophagus1^,4^,6^,7

The duplication may be discovered incidentally in adulthood1^,3^,4^,6^,8; however, from 70%–90% of the cases present before 2 years of age.7^,8

The most frequent symptoms are dysphagia, digestive hemorrhage, retrosternal pain and, especially, respiratory symptoms (recurrent pneumonia, stridor, respiratory distress) that may be present in more than 80% of the cases.7 Vertebral anomaly may be associated with the disease.4^,7^,9 This type of association may be explained in terms of notochordodysraphy (split notochord syndrome), the result of an abnormal division of the notochord in the third week of embryogenesis.10 However, Bremer11 suggests an error associated with the vacuolization of the esophagus in the fifth and seventh weeks of embryogenesis.

The diagnosis may be carried out by means of an esophagram,4^–7^,9 upper endoscopy and computed tomography scan.1^–3^,8 An endoscopy is used to perform a biopsy in the double lumen of the esophagus and may reveal an ectopic mucosa. 6 In our case, the esophageal duplication was diagnosed by endoscopy and esophagram, which are the most commonly performed examinations in the diagnosis of the tubular form of the duplication. Magnetic resonance imaging contributes by following the double lumen throughout its length and by revealing the communication with the esophageal lumen, clarifying the anomalous anatomy.2^,4

Surgical treatment has been recommended by some authors because of the risk of malignization.3^,7^,12^,13 Other authors suggest surgical treatment only in the presence of symptoms.1^,2^,7 Extensive surgery such as esophagectomy is usually required for the tubular forms. Recently, a laparoscopic approach for resection of cystic forms has been reported.14