Congenital absence or complete closure of part of the duodenal lumen; causes neonatal intestinal obstruction with a classic double bubble appearance on imaging.
Most common congenital intestinal obstruction in newborns; if not recognized promptly it leads to dehydration and electrolyte imbalances in the neonate. Strongly associated with Down syndrome and a favorite exam topic due to its hallmark imaging and presentation.
Prenatal: often detected via polyhydramnios on ultrasound (fetus can't swallow amniotic fluid); may even show a double bubble sign in utero. Neonatal: onset of vomiting (usually bilious, green) within 24–48 hours after birth, typically after first feeding.
Exam/Imaging: Abdomen is often scaphoid (sunken) due to empty distal bowel; X-ray reveals the double bubble sign – two air-filled bubbles (distended stomach and duodenum) with little or no gas beyond. Newborn continues to pass meconium normally (since obstruction is proximal).
Associations: ~30% of cases occur in infants with trisomy 21 (Down syndrome). About half of babies have other anomalies (especially congenital heart defects or other GI malformations like an annular pancreas causing the obstruction).
Neonate with bilious emesis → assume a surgical emergency (rule out malrotation/volvulus first, as it can also cause proximal obstruction and requires immediate intervention).
If abdominal X-ray shows a clear double bubble with no distal gas in a stable infant → likely duodenal atresia; begin stabilization and plan for surgery (no emergent overnight surgery needed unless volvulus is suspected).
Stabilize with NG tube decompression (to prevent aspiration) and IV fluids to correct dehydration and electrolyte imbalances. Evaluate for other anomalies (e.g. get an echocardiogram for cardiac defects, especially if Down syndrome is present) before surgery.
If some gas is seen beyond a double bubble, consider a partial obstruction (duodenal stenosis/web or a less complete blockage) or malrotation – an upper GI contrast study can help differentiate these.
Remember: pyloric stenosis presents later (weeks old) and distal atresias (jejunal/ileal) show multiple dilated loops, so use timing and imaging to distinguish.
Condition
Distinguishing Feature
Hypertrophic pyloric stenosis
nonbilious projectile vomiting at 3–6 weeks, palpable "olive" mass in epigastrium
Malrotation with midgut volvulus
neonatal bilious vomiting plus acute abdominal pain/distension; can also produce double bubble but with rapid clinical deterioration (ischemia)
Jejunal or ileal atresia
multiple air-fluid levels on X-ray ("triple bubble" if proximal jejunum); often due to in utero vascular accident (risk factors like maternal cocaine)
Stabilize first: NPO (no oral feeding), NG tube suction to decompress the stomach, and IV fluid/electrolyte resuscitation. Correct any dehydration or metabolic alkalosis before surgery.
Surgical repair: perform a duodenoduodenostomy (surgical bypass of the atretic segment by connecting proximal and distal duodenum) once the infant is stabilized. Postoperatively, gradually advance feedings. Prognosis is excellent (>90% survival) in the absence of severe other anomalies.
Mnemonic: Double Bubble = Down syndrome + Duodenal Atresia (the two D's go together for this classic neonatal x-ray finding).
Bilious vomiting in any neonate = emergency until proven otherwise. Always involve pediatric surgery early and rule out midgut volvulus, which can mimic duodenal atresia but is life-threatening if not treated immediately.
Unrecognized congenital heart disease or other anomalies in a baby with duodenal atresia can complicate anesthesia and surgery. Always screen for associated defects (e.g. cardiac echo for Down syndrome patients) before operative repair.
Neonate develops bilious vomiting → immediately stop feeds, place NG tube, and obtain abdominal X-ray.
If X-ray shows double bubble with little/no distal gas → duodenal atresia is likely. (If distal gas is present, consider partial obstruction or malrotation and perform contrast study.)
Consult pediatric surgery; initiate IV fluids and electrolyte correction, and screen for any other anomalies (e.g. cardiac evaluation).
Proceed with surgical duodenoduodenostomy in the first days of life once stabilized.
Mother with polyhydramnios gives birth to a baby with Down syndrome features who vomits green fluid on Day 1 → duodenal atresia (confirmed by double bubble on X-ray).
Newborn with immediate bilious emesis and a radiograph showing two large air bubbles in the upper abdomen (stomach and duodenum) with no gas beyond → classic double bubble sign indicating duodenal atresia.
Case 1
A full-term newborn has been vomiting green fluid since birth. The pregnancy was complicated by polyhydramnios, and the infant has facial features consistent with Down syndrome.
Case 2
A 1-day-old neonate has recurrent episodes of projectile vomiting that are green in color. On exam, the lower abdomen is scaphoid (sunken) and not distended. An orogastric tube returns a large amount of fluid.
X-ray of an infant abdomen showing the classic "double bubble" sign of duodenal atresia (two air-filled shadows representing the stomach and duodenum, with no gas in the intestines beyond).
