Esophageal atresia is a condition in which the proximal and distal portions of the esophagus do not communicate. The reported incidence is 1 in 3590 pregnancies.Prenatal diagnosis is particularly interesting allowing search for associated malformations related to worse prognosis forms (reference ultrasound, MRI and amniocentesis) and planning the birth in an adapted medico-surgical center. Diagnosis of EA is usually suspected because of indirect and non-specific signs: association of polyhydramnios and absent or small stomach bubble. The visualization in ultrasound or MRI of cervical or thoracic fluid image corresponding to the expansion of the bottom of upper esophageal ("pouch sign") increases the specificity of diagnosis. If EA is confirmed at birth, surgical management consists in a primary end-to-end anastomosis in first days of life, or in two-steps surgery if the defect is too large. Although current prognosis of EA is good, frequency of surgical complications and esophageal lesions secondary to gastroesophageal reflux justify a systematic and multidisciplinary extended follow-up.
To conclude, the presence of the upper neck pouch sign on USG is an additional sign that helps in the diagnosis of esophageal atresia; this sign is seen regardless of the presence or absence of a tracheoesophageal fistula.When seen, the pouch is specific for this diagnosis, but is not appreciated in up to 57% of proved cases of esophageal atresia.Therefore, in the presence of polyhydramnios, the radiologist should look for the pouch sign in the neck, irrespective of whether the stomach is present or absent.
1. Branthberg. Esophageal obstruction-prenatal detection rate and outcome. Ultrasound Obstet Gynecol 2007;30:180-7.
2. Farrant P. The antenatal diagnosis of esophageal atresia by ultrasound. Br J Radiol 1980;53:1202-3
3. Kalache KD, Chaoui R, Mau H. The upper neck pouch sign: A prenatal sonographic marker for esophageal atresia. Ultrasound Obstet Gynecol 1998;11:138-40.
4. Kalache KD, Wauer R, Mau H. Prognostic significance of the pouch sign in fetuses with prenatally diagnosed esophageal atresia. Am J Obstet Gynecol 2000;182:978-81
5. Rossi C, Domini M, Aquino A. A simple and safe method to visualize the inferior pouch in esophageal atresia without fistula. Pediatr Surg Int 1998;13:535-6