Signs in Imaging

Jeffrey Traubici, MD

The Double Bubble Sign1

APPEARANCE
The double bubble sign is a nding observed on radiographs of newborns and infants (Fig 1). It is reproducible with a variety of other imaging modalities, including upper gastrointestinal studies and sonography (Fig 2). The nding depicts two airlled structures in the upper abdomen, with little or no air distally. Horizontal-beam radiographs will demonstrate air and uid levels in both bubbles. obstruction. In infants in whom the radiograph demonstrates a double bubble, one should consider both intrinsic and extrinsic causes of obstruction. The intrinsic causes are duodenal atresia, duodenal stenosis, and duodenal webs; the extrinsic causes include annular pancreas, malrotation of the gut with obstruction produced by midgut volvulus or by Ladd bands, and preduodenal position of the portal vein (2). Although most often observed on abdominal radiographs, a double bubble can be demonstrated with prenatal sonography, in which case the bubbles are uid lled. A fetal double bubble should be interpreted with caution, as transient double bubbles have been noted in fetuses subsequently found to be healthy (3). This observation may result from transient duodenal uid accumulation and slow peristalsis. The sonographic variant of the double bubble sign is useful in assessing the duodenum of neonates with esophageal atresia without tracheoesophageal stula for possible duodenal obstruction. Duodenal atresia serves as a clinical and radiologic illustration of this sign, since it is the entity most often identied with a double bubble. Infants with duodenal atresia present with bilious vomiting early in the neonatal period. The atretic segment is most often just beyond the ampulla of Vater (4) at the junction of foregut and midgut. If the atresia is proximal to the ampulla, the vomiting is nonbilious. The prevalence of duodenal atresia is one in 10,000 newborns (5), with no sex-associated difference in prevalence. Although more distal small-bowel atresia is believed to be secondary to an ischemic episode, duodenal atresia is thought to represent a failure of recanalization (6) of the bowel lumen that is a solid tube early in fetal life. Duodenal atresia is associated with many congenital syndromes, the foremost being Down syndrome. Approximately 30% of children with duodenal atresia have trisomy 21 (5). There is also an association with anomalies of the VACTERL (vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb anomalies) spectrum. By differentiating intrinsic obstructive causes such as duodenal atresia, stenosis, and web from potentially catastrophic midgut volvulus is important, particularly if surgical treatment will be delayed. While an upper gastrointestinal series will resolve the dilemma, specic ndings at clinical examination and with radiography are useful. Most infants with malrotation and obstruction from Ladd bands have bile-stained vomiting starting within

EXPLANATION
The proximal left-sided bubble is the air- and uid-lled stomach. The proximal duodenum represents the second bubble to the right of the midline. The specic duodenal structural anomaly causing the obstruction determines the appearance, prominence, and presence of air distal to the obstruction. Duodenal atresia produces complete obstruction, and, unless an alternative route exists for proximal air to reach the remainder of the small bowel, there is no distal bowel gas. Such an alternative route has been described if air bypasses the site of atresia via a bid common bile duct with an insertion both proximal and distal to the atretic segment (1). Partially obstructing anomalies, such as duodenal web or duodenal stenosis, allow passage of some air distal to the obstruction and a less dramatic double bubble.

DISCUSSION
The detection of the double bubble sign leads to suspicion of a number of structural anomalies, all of which cause duodenal

Index terms: Duodenum, stenosis or obstruction, 73.1435 Infants, newborn, gastrointestinal tract, 73.11, 73.12981 Signs in Imaging Published online: July 19, 2001 10.1148/radiol.2202980350 Radiology 2001; 220:463 464
1

From the Department of Diagnostic Radiology, Yale University School of Medicine, New Haven, Conn. Received January 16, 1998; revision requested February 27; nal revision received November 2, 1999; accepted December 7. Address correspondence to the author, Department of Diagnostic Radiology, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada M5G 1X8 (email: jeff.traubici@utoronto.ca). RSNA, 2001

A trainee (resident or fellow) wishing to submit a manuscript for Signs in Imaging should rst write to the Editor for approval of the sign to be prepared, to avoid duplicate preparation of the same sign.

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Figure 1. Supine frontal radiograph of the abdomen demonstrates the double bubble sign: an enlarged stomach (S) and proximal duodenum (D) in a neonate with duodenal atresia. Note the absence of distal gas.

Figure 2. Prenatal sonogram in a sagittal oblique plane to the fetus demonstrates the double bubble sign in a fetus with duodenal atresia. In utero, the stomach (S) and duodenum (D) are lled with uid.

24 hours of birth, and the abdomen is usually not distended unless volvulus is superimposed. In an infant with midgut volvulus, an abdominal physical examination may reveal tenderness and peritoneal signs, and the child appears more ill. Intrinsic causes of obstruction result in a chronically distended duodenal bulb that is larger than the duodenal bulb in the midgut volvulus. Indeed, if the stomach is distended and the duodenum is nearly normal or only slightly distended, then the diagnosis of malrotation and midgut volvulus must be considered, and an upper gastrointestinal series is the study of choice. There may or may not be distal gas, depending on whether the volvulus has caused complete or partial obstruction and the duration of the volvulus. If the infant has recently vomited, then a repeat radiograph may be necessary after the child has swallowed more air or after more air has been instilled into the stomach. For the neonate with the classic appearance of a double bubble, additional radiologic investigation is unnecessary, and the surgeon is alerted to plan for surgery, since all congenital causes of duodenal obstruction require surgery. If a delay is contemplated, then the clinical and radiologic team should consider an upper gastrointestinal series to indicate that the infant does not have a malrotation. Surgical treatment for duodenal obstruction includes a num-

ber of different techniques for the intrinsic causes, including duodenoduodenostomy and duodenojejunostomy (7).
Acknowledgment: iting. I thank Marc S. Keller, MD, for assistance in ed-

References 1. Kassner EG, Sulton A, DeGroot T. Bile duct anomalies associated with duodenal atresia: paradoxical presence of small bowel gas. AJR Am J Roentgenol 1972; 116:577583. 2. Leonidas JC, Berdon W. The neonate and young infant: the gastrointestinal tract. In: Silverman FN, Kuhn JP, eds. Caffeys pediatric x-ray diagnosis. 9th ed. St Louis, Mo: Mosby, 1993; 2048 2055. 3. Zimmer EZ, Bronshtein M. Early diagnosis of duodenal atresia and possible sonographic pitfalls. Prenat Diagn 1996; 16:564 566. 4. Fonkalsrud EW, deLorimier AA, Hays MD. Congenital atresia and stenosis of the duodenum: a review compiled from the member of the surgical section of the American Academy of Pediatrics. Pediatrics 1969; 43:79 83. 5. Rudolph C. Congenital atresias, stenosis and webs. In: Rudolph AM, Hoffman JIE, Rudolph C, eds. Rudolphs pediatrics. 20th ed. Stamford, Conn: Appleton & Lange, 1996; 1069. 6. ORahilly R, Muller F. Human embryology and teratology. New York, NY: WileyLiss, 1996; 229. 7. Magnuson DK, Schwartz MZ. Stomach and duodenum. In: Oldham KT, Colombani PM, Foglia RP, eds. Surgery of infants and children: scientic principles and practice. Philadelphia, Pa: Lippincott Raven, 1997; 11331162.

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