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Findings

CXR: There is a nasogastric tube terminating in the patient's mid-esophagus; repeated attempts failed to pass the tube. The abdomen is distended and gasless.

U/S: There is significant distension of a fluid-filled stomach.

MRI: There is dilatation of the stomach and proximal duodenum, producing a "double bubble" appearance. The distal esophagus also appears distended.

Diagnosis

Combined duodenal and esophageal atresia without tracheoesophageal fistula.

Discussion

The combination of esophageal and duodenal atresia is a rare but well-known association. This combination is most often associated with a tracheoesophageal fistula. Combined esophageal and duodenal atresia with absence of tracheoesophageal fistula, as demonstrated in this case, is even more rare; yet this entity exhibits a constellation of radiographic features considered to be virtually pathognomonic.

Clinically, the diagnosis of esophageal atresia is established by the failure to pass a nasogastric tube. The chest x-ray confirms that the tube tip is in the mid-esophagus, and the gasless abdomen reflects the absence of tracheoesophageal fistula. However, duodenal atresia, which is commonly a plain film diagnosis on the basis of a gas-distended stomach and proximal duodenum (the so-called "double bubble"), cannot be diagnosed on this study because of the lack of communication of the air-filled esophagus with the stomach and duodenum.

Thus, the ultrasound study establishes the additional diagnosis of duodenal atresia, with massive accumulation of secretions within the closed loop involving the distal esophagus to the duodenum. This distension is far greater than would be seen in duodenal atresia alone.

This case is of special interest because of the availability of a prenatal MRI study. Could the diagnosis have been made prenatally? The MRI clearly demonstrates duodenal atresia with "double bubble" distension of the stomach and proximal duodenum. However, the diagnosis of esophageal fistula is difficult to make prospectively on MRI. In this case the proximal esophagus is not dilated, as one might expect in esophageal atresia, although a review of fetal thoracic MRI by Levine et al. suggests that distension of the proximal esophagus is not a reliable finding for esophageal atresia. Perhaps more useful in this case is the observation of distal esophageal distension, reflecting reflux of secretions into the blind-ending lower segment of esophagus. However, the specificity of this finding is uncertain. To our knowledge, no case of prenatal MR imaging of combined esophageal and duodenal atresia has been reported in the literature.

In summary, the combination of duodenal and esophageal atresia without tracheoesophageal fistula is a rare but known clinical entity. The diagnosis is important to make because of significant associated morbidity and mortality; this patient had no associated congenital abnormalities and underwent successful staged surgical repair. From an imaging standpoint, the case is of particular interest because of the need for multimodality imaging to establish the complete diagnosis.

References

Crowe JE, Sumner TE. Combined esophageal and duodenal atresia without tracheoesophageal fistula: characteristic radiographic changes. Am J Roentgenol. 1978 Jan;130(1):167-8.

Hayden CK Jr, Schwartz MZ, Davis M, Swischuk LE. Combined esophageal and duodenal atresia: sonographic findings. Am J Roentgenol. 1983 Feb;140(2):225-6.

Levine D, Barnewolt CE, Mehta TS, Trop I, Estroff J, Wong G. Fetal thoracic abnormalities: MR imaging. Radiology. 2003 Aug;228(2):379-88.

McCook TA, Felman AH. Esophageal atresia, duodenal atresia, and gastric distention: report of two cases. Am J Roentgenol. 1978 Jul;131(1):167-8.


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