Duodenal Atresia and the Double-Bubble Sign
Ansley Splinter, MD, MAcM • Rakhi Gupta Basuray, MD
Department of Pediatrics, The Ohio State University College of Medicine, Columbus, Ohio
Nationwide Children’s Hospital, Columbus, Ohio
Splinter A, Gupta Basuray R. Duodenal atresia and the double-bubble sign. Consultant. 2020;60(7):e9. doi:10.25270/con.2020.04.00007
Received November 7, 2019. Accepted March 20, 2020.
The authors report no relevant financial relationships.
We thank Devon Swick, DO, for her participation in the care of this patient and for providing the radiographic image.
Ansley Splinter, MD, MAcM, Division of Hospital Medicine, Nationwide Children’s Hospital, 700 Children’s Dr, Columbus, OH 43205 (email@example.com)
A boy was born via normal spontaneous vaginal delivery at 36 weeks and 1 day of gestation to a 20-year-old, gravida 1, para 1 woman. Prenatal care had been established in the first trimester, and the pregnancy had been uncomplicated. Routine second-trimester ultrasonographic anatomy scan findings and serologic test results had been normal, although group B β-hemolytic streptococcus results were unknown due to spontaneous rupture of membranes and preterm labor. The mother was treated empirically with ampicillin over the next 13 hours, and the delivery was uncomplicated, with Apgar scores of 7 at 1 minute and 9 at 5 minutes of life. The neonate’s initial physical examination findings were within normal limits.
The neonate’s glucose level was checked within the first hour of life and was found to be low at 33 mg/dL. The asymptomatic hypoglycemia was treated with a dose of oral dextrose gel applied to the buccal mucosa. Within the following hour, an episode of mucoid emesis with apparent choking and dusky color change occurred. Oxygen saturation values dropped to as low as 64% on room air. Resuscitation with bulb suction and several minutes of continuous positive air pressure (CPAP) via facemask was required for recovery.
At the following glucose check, another low value of 39 mg/dL was identified. The neonate was once again treated with oral dextrose gel, which again resulted in oxygen desaturation and CPAP resuscitation. Per hospital protocol on the management of neonatal hypoglycemia, supplementation with formula was advised for the second occurrence of low glucose levels. Due to recurrent desaturations with feeding however, a total of only 6 mL of formula was able to be provided. The neonate was subsequently placed on cardiorespiratory monitors until his next feed. During this time, the neonate’s respiratory effort and glucose levels normalized. Prior to the next feed, however, the neonate once more presented with choking, a large emesis, and hypoxia, requiring bulb suctioning and CPAP resuscitation.
After consultation with the neonatology team, an orogastric (OG) tube for decompression was placed, and approximately 15 mL of amniotic fluid and milk was evacuated from the stomach, in addition to approximately 10 mL of air. The OG tube was subsequently removed, and the neonate demonstrated toleration of 20-mL feeds without event.
At 9 hours of life, the neonate once more presented with a large emesis, this time with palpable loops of bowel, and no passage of meconium. Chest and abdominal radiographs were obtained and revealed the classic double-bubble finding indicative of congenital duodenal atresia (DA) (Figure).
Figure. An abdominal radiograph showing the classic double-bubble finding indicative of congenital duodenal atresia.
The double-bubble radiographic finding, reflecting gaseous distension of the stomach left of midline, and proximal small intestine right of midline, with an absence of distal bowel gas, promptly brought into consideration a diagnosis of DA.
This congenital condition is most often a consequence of defective recanalization of the duodenal lumen taking place in the first trimester of fetal development.1 It is not until the third trimester, however, that the condition becomes more readily detected with ultrasonography. In contrast to a newborn, in whom the double-bubble is secondary to trapped air, fetal stomach and duodenal distension is secondary to retained amniotic fluid.2 As a result, polyhydramnios commonly occurs. Additional conditions such as trisomy 21 and VACTERL association (vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb abnormalities) are also frequently associated with duodenal atresia and are identifiable prenatally.3 The prenatal ultrasonography for our patient was obtained at 18 weeks of gestation and demonstrated no anatomic abnormalities or polyhydramnios.
Overall, DA has a low incidence of approximately 1 in 10,000 live births, but it is the most common of all intestinal atresias.4,5 Boys are affected slightly more than girls at a rate of 1.4 to 1.4 Of the 4 types of DA, type 1 is the most common and involves a continuous segment of bowel with a membranous luminal obstruction, as opposed to type 4, in which there are multiple atretic regions of the small bowel.4
Symptoms of DA manifest within the first 2 days of life as oral intake increases. Bilious emesis or less commonly nonbilious emesis occurs if the region of obstruction is proximal to the hepatopancreatic ampulla.1 As such, the clinical presentation may not be associated with abdominal distension, even without passage of meconium.1,5
Due to the emergent need of surgery for malrotation with midgut volvulus, distinguishing this condition from DA is critical.4 However, an upper gastrointestinal tract radiographic series, the standard for diagnosing malrotation, must be done cautiously, because the required barium contrast poses a risk of aspiration with DA.1 More recently, ultrasonography has been emerging as a reliable alternative for diagnosing malrotation.6 And the administration of contrast via enema has the ability to reveal microcolon, secondary to disuse, in patients with duodenal atresia.5 The difference in time to presentation further aids in distinguishing the conditions, since midgut volvulus more commonly presents after the first 2 days of life.1
Treatment of DA involves gastric decompression and intravenous hydration while awaiting definitive surgical repair. The high sensitivity and negative predictive value of normal prenatal cardiac screening test results can negate the need for further postnatal cardiac imaging and thereby avoid delays in surgical correction.7 Duodenoduodenostomy or duodenojejunostomy may be performed laparoscopically or with an open approach, in which the proximal and distal segments of the intestine are anastomosed after removing the atretic region.4,5
The prognosis is generally favorable for patients with DA, with morbidity and mortality related to the type of DA and associated abnormalities in other organ systems.
OUTCOME OF THE CASE
Immediately after the diagnosis of DA was made, an OG tube was repositioned, and 20 mL of gastric fluid was evacuated from the stomach. The pediatric surgery team was consulted, and arrangements were made to transfer the neonate to a higher level of care for surgical intervention.
- Sigmon DF, Eovaldi BJ, Cohen HL. Duodenal atresia and stenosis. StatPearls. Updated February 25, 2020. Accessed March 23, 2020. https://www.ncbi.nlm.nih.gov/books/NBK470548
- Pariente G, Landau D, Aviram M, Hershkovitz R. Prenatal diagnosis of a rare sonographic appearance of duodenal atresia: report of 2 cases and literature review. J Ultrasound Med. 2012;31(11):1829-1833. doi:10.7863/jum.2012.31.11.1829
- Best KE, Tennant PWG, Addor M-C, et al. Epidemiology of small intestinal atresia in Europe: a register-based study. Arch Dis Child Fetal Neonatal Ed. 2012;97(5):F353-F358. doi:10.1136/fetalneonatal-2011-300631
- Adams SD, Stanton MP. Malrotation and intestinal atresias. Early Hum Dev. 2014;90(12):921-925. doi:10.1016/j.earlhumdev.2014.09.017
- Morris G, Kennedy A Jr, Cochran W. Small bowel congenital anomalies: a review and update. Curr Gastroenterol Rep. 2016;18(4):16. doi:10.1007/s11894-016-0490-4
- Zhang W, Sun H, Luo F. The efficiency of sonography in diagnosing volvulus in neonates with suspected intestinal malrotation. Medicine (Baltimore). 2017;96(42):e8287. doi:10.1097/MD.0000000000008287
- Stephens CQ, Dukhovny S, Rowland KJ, Hamilton NA. Neonatal echocardiogram in duodenal obstruction is unnecessary after normal fetal cardiac imaging. J Pediatr Surg. 2018;53(11):2145-2149. doi:10.1016/j.jpedsurg.2018.04.039