Table 1 Ultrasound and magnetic resonance imaging associated findings, and fetal karyotype in reported cases with prenatally diagnosed esophageal atresia with duodenal atresia

Duenhoelter et al. [31] 1976

Near term

Ultrasound showed two communicating upper abdominal masses. It also failed to ingest contrast media after injection of radiopaque material into the amniotic sac

Normal

Hayden et al. [8] 1983

34

Ultrasound showed two large fluid-filled cystic structures within the fetal abdomen

T21

Estroff et al. [32] 1994

16.5

Ultrasound showed an abdominal C-shaped fluid collection suggesting a dilated stomach which extended into the chest behind the heart. A follow-up ultrasound showed continuity between the fluid collection in the fetal chest and the dilated stomach. Later, fetal ascites and skin edema developed

Normal

16.7

Ultrasound showed an abdominal C-shaped fluid collection suggesting a dilated stomach with progressive polyhydramnios

Normal

22.5

Ultrasound showed an abnormal triangular cranial shape in addition to dumbbell-shaped stomach. Mild to moderate polyhydramnios was appreciated

Normal

Pameijer et al. [33] 2000

18

Level II ultrasound showed a cystic mass, which was assumed to be the stomach, with a posterior mediastinal, intrathoracic portion compressing the left atrium. No evidence of dilated proximal esophageal pouch. On follow-up polyhydramnios was appreciated with the presence of the classic “double bubble” sign. Ultrafast fetal magnetic resonance imaging showed a cystic mass extending through the esophageal hiatus. Postnatal findings: pure esophageal atresia, duodenal atresia, biliary atresia, and pancreatic ductal atresia. Subsequently, the neonate was diagnosed with a type I choledochal cyst

T21

Marquette et al. [34] 2004

12

Ultrasound showed a single large cystic structure in the anterior upper abdomen

T21

Mitani et al. [11] 2009

25

Ultrasound showed a double cystic structure with a dilated stomach and duodenum and an intrathoracic cyst. Magnetic resonance imaging and ultrasound at 26 weeks showed shrinkage of the stomach and duodenum and appearance of massive ascites, suggesting rupture of either structures. Distention of the proximal esophagus was noted as an “upper pouch” sign. Polyhydramnios was noted at 30 weeks

Normal

Kanasugi et al. [24] 2013

20

Ultrasound showed multicystic dysplastic left kidney, normal-sized stomach, no abdominal cysts, no cardiac anomalies, and no limb abnormalities. At 31 weeks of gestation, fetus A developed a left hydroureter with dilatation of the colon

Normal

Kadohira et al. [25] 2014

17

Ultrasound showed intra-abdominal cystic mass with typical “double bubble” sign with no other structural abnormalities. Follow-up ultrasound at 26 weeks demonstrated marked dilatation of the stomach and duodenum. Similar to our findings, a peristalsis-like movement was appreciated in the mediastinum of the fetus and subsequently confirmed by magnetic resonance imaging to be a dilated distal esophageal pouch

Normal