Duodenal atresia

Duodenal atresia is the congenital absence or complete closure of a portion of the lumen of the duodenum. It causes increased levels of amniotic fluid during pregnancy (polyhydramnios) and intestinal obstruction in newborn babies. Newborns present with bilious or non-bilous vomiting (depending on where in the duodenum the obstruction is) within the first 24 to 48 hours after birth, typically after their first oral feeding. Radiography shows a distended stomach and distended duodenum, which are separated by the pyloric valve, a finding described as the double-bubble sign.

Duodenal atresia
Radiograph of abdomen with double-bubble sign from duodenal atresia
SpecialtyPediatric gastroenterology, Pediatric surgery
SymptomsAntenatal period: polyhydramnios Neonatal period: bilious or non-bilious vomiting within first 36 hours of life, abdominal distension, lack of stooling
Usual onsetDuring embryological development
CausesUnknown
Risk factorsDown syndrome
Differential diagnosisannular pancreas, duodenal stenosis
Treatmentnasogastric suction, surgery (duodenoduodenostomy)

Treatment includes suctioning out any fluid that is trapped in the stomach, providing fluids intravenously, and surgical repair of the intestinal closure.

Signs and symptoms

History and physical examination

During pregnancy, duodenal atresia is associated with increased amniotic fluid in the uterus, which is called polyhydramnios.[1] This increase in amniotic fluid is caused by the inability of the fetus to swallow the amniotic fluid and absorb it in their digestive tract.

After birth, duodenal atresia may cause abdominal distension, especially of the upper abdomen. Bilious or non-bilious vomiting, depending on the position of the atresia in relation to the Ampulla of Vater, commonly occurs within the first day of life.

Associated conditions

Approximately 20–40 percent of all infants with duodenal atresia have Down syndrome and 50% have a congenital cardiac anomaly.[2] Approximately 8% of infants with Down syndrome have duodenal atresia.[1]

Diagnosis

Antenatal

Antenatal ultrasonography allows for earlier detection and diagnosis of duodenal atresia. The duodenum is not typically filled with fluid on imaging, however if fluid is visualized on ultrasound, this may suggest duodenal atresia as it causes obstruction and fluid may not pass distal to the atretic area.[3] Early diagnosis of duodenal atresia provides time for clinicians to provide prenatal counseling and prepare for postnatal management. [4]

Postnatal

The diagnosis of duodenal atresia is usually confirmed by radiography. An X-ray of the abdomen is the first step in evaluation. The x-ray should be obtained after placement of a nasogastric tube (feeding tube), evacuating the stomach and filling 40-50 ml of air [5] to demonstrate two large air filled spaces, the so-called "double bubble" sign.[6][7] The air is trapped in the stomach and proximal duodenum, which are separated by the pyloric sphincter, creating the appearance of two bubbles visible on x-ray. Since the closure of the duodenum is complete in duodenal atresia, no air is seen in the distal duodenum. Note that the double bubble sign is typically pathologic however it is not specific for duodenal atresia and may indicate other pathologies such as annular pancreas or midgut volvulus.[8] A limited upper gastrointestinal series using barium contrast can be used to differentiate between duodenal atresia and midgut volvulus. [9]

Causes

The cause of duodenal atresia is not known. Dr. Julius Tandler hypothesized in 1900 that the etiology of duodenal atresia was due to occlusion of the duodenal lumen and failure to re-canalize during embryological development. Research surrounding duodenal atresia in recent years has switched focus to the fibroblast growth factor pathway in mouse models as Tandler’s original hypothesis was unable to account for the different types of duodenal atresia.[10]

Diagnosis

Classification

Duodenal atresia is classified into 3 types:[5]

  • Type 1: the most common form of duodenal atresia, making up 92% of cases. A web made of mucosa and submucosa obstructs the duodenal lumen however there are no defects in the muscle coat.[5]
  • Type 2: rarest form of duodenal atresia, making up 1% of cases. The proximal and distal ends of the duodenum are atretic and separated but are connected by a cord (mesentery still intact).[5]
  • Type 3: makes up 7% of duodenal atresia cases. The ends of the duodenum are atretic without any tissue attachment (mesentery defect).[5]

Management

Early treatment includes removing fluids from the stomach via a nasogastric tube, and providing fluids intravenously.[11] The definitive treatment for duodenal atresia is surgery (duodenoduodenostomy or duodenojejunostomy), which may be performed openly or laparoscopically.[12] The surgery is required but not immediately urgent - a 24 to 48-hour delay is permissible to facilitate transport, further evaluation and fluid resuscitation.[11] The initial repair has a 5 percent morbidity and mortality rate.[13] Nasogastric suction is typically continued post-operatively and patients can be transitioned to small oral feeds once nasogastric tube output decreases significantly or stops completely.[5]

Complications

Prognosis is usually very good, although complications are more likely to occur when there are serious congenital anomalies.[11] Complications from surgical repair include Peptic ulcer disease, Gastroesophageal reflux disease, Cholecystitis, Esophagitis, Megaduodenum, Blind loop syndrome, and anastomotic leak.[9] Late complications may occur in about 12 percent of patients with duodenal atresia, and the mortality rate for these complications is 6 percent.[13]

Epidemiology

Duodenal atresia occurs in 1 in every 10,000 live births and is the most common intestinal atresia, constituting up to 60% of intestinal atresias.[14]

References
  1. "Duodenal Atresia". Archived from the original on 2012-08-05. Retrieved 2007-12-03.
  2. Bethell, George Stephen; Long, Anna-May; Knight, Marian; Hall, Nigel J.; BAPS-CASS (2019-06-22). "Congenital duodenal obstruction in the UK: a population-based study". Archives of Disease in Childhood. Fetal and Neonatal Edition. 105 (2): fetalneonatal-2019-317085. doi:10.1136/archdischild-2019-317085. ISSN 1468-2052. PMC 7063389. PMID 31229958.
  3. Blumer, Steven L.; Zucconi, William B.; Cohen, Harris L.; Scriven, Richard J.; Lee, Thomas K. (2004). "The vomiting neonate: a review of the ACR appropriateness criteria and ultrasound's role in the workup of such patients". Ultrasound Quarterly. 20 (3): 79–89. doi:10.1097/00013644-200409000-00002. ISSN 0894-8771. PMID 15322385. S2CID 40208130.
  4. Shawis, Rang; Antao, Brice (2006). "Prenatal bowel dilatation and the subsequent postnatal management". Early Human Development. 82 (5): 297–303. doi:10.1016/j.earlhumdev.2006.02.005. ISSN 0378-3782. PMID 16626900.
  5. Gharpure, Vivek (January 2014). "Duodenal atresia". Journal of Neonatal Surgery. 3 (1): 14. doi:10.47338/jns.v3.78. ISSN 2226-0439. PMC 4420424. PMID 26023485.
  6. Poki HO, Holland AJ, Pitkin J (2005). "Double bubble, double trouble". Pediatr. Surg. Int. 21 (6): 428–31. doi:10.1007/s00383-005-1448-z. PMID 15912365. S2CID 21589667.
  7. Traubici J (August 2001). "The double bubble sign". Radiology. 220 (2): 463–4. doi:10.1148/radiology.220.2.r01au11463. PMID 11477252.
  8. Papandreou, E.; Baltogiannis, N.; Cigliano, B.; Savanelli, A.; Settimi, A.; Keramidas, D. (2004). "Annular pancreas combined with distal stenosis. A report of four cases and review of the literature". La Pediatria Medica e Chirurgica: Medical and Surgical Pediatrics. 26 (4): 256–259. ISSN 0391-5387. PMID 16366413.
  9. Sigmon, David F.; Eovaldi, Benjamin J.; Cohen, Harris L. (2021), "Duodenal Atresia And Stenosis", StatPearls, Treasure Island (FL): StatPearls Publishing, PMID 29261981, retrieved 2021-11-07
  10. Jones, Matthew L. M.; Sarila, Gulcan; Chapuis, Pierre; Hutson, John M.; King, Sebastian K.; Teague, Warwick J. (2020). "The Role of Fibroblast Growth Factor 10 Signaling in Duodenal Atresia". Frontiers in Pharmacology. 11: 250. doi:10.3389/fphar.2020.00250. ISSN 1663-9812. PMC 7076179. PMID 32210824.
  11. Kimura, Kim; Loening-Baucke, V (May 1, 2000). "Bilious vomiting in the newborn: rapid diagnosis of intestinal obstruction". American Family Physician. 61 (9): 2791–8. PMID 10821158. Retrieved 16 September 2012.
  12. Spilde, Troy L; St Peter, SD; Keckler, SJ; Holcomb GW, 3rd; Snyder, CL; Ostlie, DJ (June 2008). "Open vs laparoscopic repair of congenital duodenal obstructions: a concurrent series". Journal of Pediatric Surgery. 43 (6): 1002–5. doi:10.1016/j.jpedsurg.2008.02.021. PMID 18558173.
  13. Escobar, Mauricio A; Ladd, AP; Grosfeld, JL; West, KW; Rescorla, FJ; Scherer LR, 3rd; Engum, SA; Rouse, TM; Billmire, DF (June 2004). "Duodenal atresia and stenosis: long-term follow-up over 30 years". Journal of Pediatric Surgery. 39 (6): 867–71, discussion 867–71. doi:10.1016/j.jpedsurg.2004.02.025. PMID 15185215. Retrieved 16 September 2012.
  14. Best, Kate E.; Tennant, Peter W. G.; Addor, Marie-Claude; Bianchi, Fabrizio; Boyd, Patricia; Calzolari, Elisa; Dias, Carlos Matias; Doray, Berenice; Draper, Elizabeth; Garne, Ester; Gatt, Miriam (September 2012). "Epidemiology of small intestinal atresia in Europe: a register-based study". Archives of Disease in Childhood. Fetal and Neonatal Edition. 97 (5): F353–358. doi:10.1136/fetalneonatal-2011-300631. ISSN 1468-2052. PMID 22933095. S2CID 20852328.
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