From the Guidelines
The radiographic findings of a "double bubble" sign with no air seen in the distal intestine are highly suggestive of duodenal atresia, a congenital obstruction of the duodenum, which requires prompt surgical intervention. The classic "double bubble" sign, as described, shows two distinct, round gas-filled structures representing the dilated stomach and proximal duodenum, with no air visible in the distal intestine, indicating a complete obstruction at the level of the duodenum 1. This pattern is characteristic of duodenal atresia, which is the most common etiology of this finding, as supported by a study of 50 neonates with duodenal obstruction, where 32 had duodenal atresia 1.
Key Considerations
- The absence of distal bowel gas confirms a complete obstruction at the duodenal level.
- Duodenal atresia requires prompt surgical intervention to create a bypass between the proximal duodenum and distal small bowel.
- Preoperative management includes nasogastric decompression, intravenous fluids, and electrolyte management.
- Additional imaging and genetic testing should be considered to identify potential associated conditions, such as Down syndrome, which is present in approximately 30% of cases.
Diagnostic Approach
- The "double bubble" sign on radiographs is a key diagnostic finding, as noted in the ACR Appropriateness Criteria for vomiting in infants 1.
- Other etiologies, such as duodenal web, congenital midgut volvulus, internal hernia, and obstructing duplication cyst, are rare and should be considered in the differential diagnosis 1.
From the Research
Key Radiographic Findings
- The "double bubble" sign is a characteristic radiographic finding seen on both lateral and frontal views, indicating a complete obstruction at the level of the duodenum 2, 3.
- This sign is represented by two distinct, round gas-filled structures: a dilated stomach and a dilated proximal duodenum, with no air seen in the distal intestine 2, 3.
Duodenal Atresia
- Duodenal atresia is a rare congenital gastrointestinal obstruction, usually recognized by the "double bubble" sign on prenatal imaging 2, 3.
- The condition is associated with trisomy 21 and other genetic abnormalities, such as heterotaxy syndrome and microdeletion of 4q22.3 2.
- Duodenal atresia can be treated surgically, with minimally invasive surgery being a safe and feasible option 4, 5.
Surgical Management
- Laparoscopic management of congenital duodenal and small bowel obstruction is a viable treatment option, with a low rate of complications such as anastomotic leaks and stricture 4.
- The surgical approach can be either open or minimally invasive, with the latter being associated with shorter hospitalization and faster advancement to full feeding 5.
- Parenteral nutrition may be necessary for some patients following surgery, but it is not always required and can be associated with a higher risk of sepsis 6.
Diagnostic Evaluation
- Prenatal double bubble sign is a reliable predictor of duodenal atresia, and additional anatomic anomalies and/or genetic abnormalities are identified in a significant proportion of cases 2.
- Postnatal diagnosis is confirmed by review of operative reports and additional postnatal evaluation, including cytogenetic analysis, molecular analysis, and/or clinical genetic evaluation 2.