Laboratory Studies

The following studies are indicated in duodenal atresia. Histologic examination is rarely performed or necessary, because repair does not involve removal of the obstruction.

Serum electrolytes

If duodenal atresia is diagnosed early, electrolyte and fluid balance should be normal. If the diagnosis is delayed at all, laboratory assessment of electrolyte and fluid status is imperative for an infant with duodenal atresia.

Prolonged vomiting can result in a hypokalemic/hypochloremic metabolic alkalosis with paradoxical aciduria.

Chromosome analysis

When trisomy 21 is suspected, a full genetic analysis should be performed. However, it is not necessary to obtain such an analysis prior to operative repair of the duodenal anomaly.

Prenatal ultrasonography

Perform prenatal ultrasonography during any pregnancy with associated polyhydramnios. Examination of a fetus with duodenal atresia may reveal a dilated fluid-filled stomach and duodenum in addition to other (eg, cardiac) abnormalities. Note that prenatal ultrasonography does not reliably detect duodenal stenosis.

The absence of the above findings does not rule out duodenal obstruction. Fetuses with duodenal atresia may have normal ultrasonographic findings in the presence of fetal vomiting. Monitor mothers with amniotic fluid abnormalities with repeat scans.

Diagnosis prior to birth enables prenatal consultation with a pediatric surgeon as well as provides parents an opportunity to discuss plans for postnatal care and management.

Erect and recumbent plain radiography of the abdomen

When duodenal atresia is suspected, erect and recumbent plain radiography of the abdomen should be the first imaging study obtained after birth.

A characteristic finding of duodenal obstruction is the double-bubble image of an air-filled stomach proximal to an air-filled first portion of the duodenum. The absence of gas in the remaining small and large bowel suggests atresia, whereas scattered amounts of gas distal to the obstruction suggest stenosis or malrotation/volvulus.

Cardiac and/or renal ultrasonography

More than 50% patients with duodenal atresia have other congenital anomalies.^{[1, 12]}

Ultrasonography of the heart and kidneys may be warranted to identify potentially life-threatening abnormalities prior to definitive repair of the duodenal obstruction.

Upper gastrointestinal (GI) contrast evaluation

An upper GI contrast study may be useful to rule out the presence of malrotation with midgut volvulus or to confirm the presence of duodenal obstruction (see below), particularly if distal air is present.

Pediatric duodenal atresia. Upper gastrointestinal series demonstrating duodenal atresia.

View Media Gallery

Treatment & Management

Piper HG, Alesbury J, Waterford SD, Zurakowski D, Jaksic T. Intestinal atresias: factors affecting clinical outcomes. J Pediatr Surg. 2008 Jul. 43 (7):1244-8. [QxMD MEDLINE Link].
Freeman SB, Torfs CP, Romitti PA, et al. Congenital gastrointestinal defects in Down syndrome: a report from the Atlanta and National Down Syndrome Projects. Clin Genet. 2009 Feb. 75 (2):180-4. [QxMD MEDLINE Link].
Al-Salem AH. Congenital duodenal obstruction. Atlas of Pediatric Surgery. Cham, Switzerland: Springer; 2020.
Piper HG, Alesbury J, Waterford SD, Zurakowski D, Jaksic T. Intestinal atresias: factors affecting clinical outcomes. J Pediatr Surg. 2008 Jul. 43 (7):1244-8. [QxMD MEDLINE Link].
Applebaum H, Lee SL, Puapong DP. Duodenal atresia and stenosis - annular pancreas. In: Grosfeld JL, O’Neill JA, Fonkalsrud EW, Coran A, eds. Pediatric Surgery. Philadelphia, PA: Mosby Elsevier; 2006. 6th ed: 1260-8.
Kay S, Yoder S, Rothenberg S. Laparoscopic duodenoduodenostomy in the neonate. J Pediatr Surg. 2009 May. 44 (5):906-8. [QxMD MEDLINE Link].
Chiarenza SF, Bucci V, Conighi ML, et al. Duodenal atresia: open versus MIS repair-analysis of our experience over the last 12 years. Biomed Res Int. 2017. 2017:4585360. [QxMD MEDLINE Link].
Reid IS. Biliary tract abnormalities associated with duodenal atresia. Arch Dis Child. 1973 Dec. 48 (12):952-7. [QxMD MEDLINE Link].
Mali V, Wagener S, Sharif K, Millar AJ. Foregut atresias and bile duct anomalies: rare, infrequent or common?!. Pediatr Surg Int. 2007 Sep. 23 (9):889-95. [QxMD MEDLINE Link].
Waever E, Neilsen OH, Arnbjornsson E, Kullendorff CM. Operative management of duodenal atresia. Pediatr Surg Int. 1995. 10:322-4.
Alatas FS, Masumoto K, Esumi G, Nagata K, Taguchi T. Significance of abnormalities in systems proximal and distal to the obstructed site of duodenal atresia. J Pediatr Gastroenterol Nutr. 2012 Feb. 54 (2):242-7. [QxMD MEDLINE Link].
Mustafawi AR, Hassan ME. Congenital duodenal obstruction in children: a decade's experience. Eur J Pediatr Surg. 2008 Apr. 18 (2):93-7. [QxMD MEDLINE Link].
Elliott GB, Kliman MR, Elliott KA. Pancreatic annulus: a sign or a cause of duodenal obstruction?. Can J Surg. 1968 Jul. 11 (3):357-64. [QxMD MEDLINE Link].
Morris JK, Springett AL, Greenlees R, et al. Trends in congenital anomalies in Europe from 1980 to 2012. PLoS One. 2018. 13 (4):e0194986. [QxMD MEDLINE Link]. [Full Text].
Akinloye OW, Truong W, Giacomantonio M, Mateos D, El-Naggar W. Coexistence of meconium ileus with duodenal atresia and trisomy 21 in a newborn: a case report. J Perinatol. 2014 Nov. 34 (11):875-6. [QxMD MEDLINE Link].
Kucinska-Chahwan A, Posiewka A, Bijok J, Jakiel G, Roszkowski T. Clinical significance of the prenatal double bubble sign - single institution experience. Prenat Diagn. 2015 Nov. 35 (11):1093-6. [QxMD MEDLINE Link].
Haeusler MC, Berghold A, Stoll C, Barisic I, Clementi M, for the EUROSCAN Study Group. Prenatal ultrasonographic detection of gastrointestinal obstruction: results from 18 European congenital anomaly registries. Prenat Diagn. 2002 Jul. 22 (7):616-23. [QxMD MEDLINE Link].
Shawis R, Antao B. Prenatal bowel dilatation and the subsequent postnatal management. Early Hum Dev. 2006 May. 82 (5):297-303. [QxMD MEDLINE Link].
Britton JR, Britton HL. Gastric aspirate volume at birth as an indicator of congenital intestinal obstruction. Acta Paediatr. 1995 Aug. 84 (8):945-6. [QxMD MEDLINE Link].
Gray SW, Skandalakis JE. Embryology for Surgeons: The Embryological Basis for the Treatment of Congenital Defects. Philadelphia, PA: WB Saunders; 1972. 147-8.
van der Zee DC. Laparoscopic repair of duodenal atresia: revisited. World J Surg. 2011 Aug. 35 (8):1781-4. [QxMD MEDLINE Link].
Rosales-Velderrain A, Betancourt A, Alkhoury F. Laparoscopic repair of duodenal atresia in a low birth weight neonate. Am Surg. 2014 Sep. 80 (9):834-5. [QxMD MEDLINE Link].
Li B, Chen WB, Zhou WY. Laparoscopic methods in the treatment of congenital duodenal obstruction for neonates. J Laparoendosc Adv Surg Tech A. 2013 Oct. 23 (10):881-4. [QxMD MEDLINE Link].
Ghaffarpour N, Svensson PJ, Svenningsson A, Wester T, Mesas Burgos C. Supraumbilical incision with U-u umbilicoplasty for congenital duodenal atresia: the Stockholm experience. J Pediatr Surg. 2013 Sep. 48 (9):1981-5. [QxMD MEDLINE Link].
Balakumar V, DeRoss AL, Kouretas PC, Boulanger SC, Barksdale EM. Pyloric exclusion for treatment of complicated duodenal atresia. Neonatology. 2014. 105 (4):263-6. [QxMD MEDLINE Link].
Brantberg A, Blaas HG, Salvesen KA, Haugen SE, Mollerlokken G, Eik-Nes SH. Fetal duodenal obstructions: increased risk of prenatal sudden death. Ultrasound Obstet Gynecol. 2002 Nov. 20 (5):439-46. [QxMD MEDLINE Link].
Brockel MA, Polaner DM, Vemulakonda VM. Anesthesia in the pediatric patient. Urol Clin North Am. 2018 Nov. 45 (4):551-60. [QxMD MEDLINE Link].
Miscia ME, Lauriti G, Lelli Chiesa P, Zani A. Duodenal atresia and associated intestinal atresia: a cohort study and review of the literature. Pediatr Surg Int. 2019 Jan. 35 (1):151-7. [QxMD MEDLINE Link].
Rothenberg SS. Laparoscopic duodenoduodenostomy for duodenal obstruction in infants and children. J Pediatr Surg. 2002 Jul. 37 (7):1088-9. [QxMD MEDLINE Link].
Spilde TL, St Peter SD, Keckler SJ, Holcomb GW 3rd, Snyder CL, Ostlie DJ. Open vs laparoscopic repair of congenital duodenal obstructions: a concurrent series. J Pediatr Surg. 2008 Jun. 43 (6):1002-5. [QxMD MEDLINE Link].
St Peter SD, Little DC, Barsness KA, et al. Should we be concerned about jejunoileal atresia during repair of duodenal atresia?. J Laparoendosc Adv Surg Tech A. 2010 Nov. 20 (9):773-5. [QxMD MEDLINE Link].
Adzick NS, Harrison MR, deLorimier AA. Tapering duodenoplasty for megaduodenum associated with duodenal atresia. J Pediatr Surg. 1986 Apr. 21 (4):311-2. [QxMD MEDLINE Link].
Ein SH, Kim PC, Miller HA. The late nonfunctioning duodenal atresia repair--a second look. J Pediatr Surg. 2000 May. 35 (5):690-1. [QxMD MEDLINE Link].
Dewberry LC, Hilton SA, Vuille-Dit-Bille RN, Liechty KW. Tapering duodenoplasty: a beneficial adjunct in the treatment of congenital duodenal obstruction. J Int Med Res. 2020 Jan. 48 (1):300060519862109. [QxMD MEDLINE Link].
Bax NM, Ure BM, van der Zee DC, van Tuijl I. Laparoscopic duodenoduodenostomy for duodenal atresia. Surg Endosc. 2001 Feb. 15 (2):217. [QxMD MEDLINE Link].
Mentessidou A, Saxena AK. Laparoscopic repair of duodenal atresia: systematic review and meta-analysis. World J Surg. 2017 Aug. 41 (8):2178-84. [QxMD MEDLINE Link].
Son TN, Kien HH. Laparoscopic versus open surgery in management of congenital duodenal obstruction in neonates: a single-center experience with 112 cases. J Pediatr Surg. 2017 Dec. 52 (12):1949-51. [QxMD MEDLINE Link].
Sidler M, Djendov F, Curry JI, et al. Potential benefits of laparoscopic repair of duodenal atresia: insights from a retrospective comparative study. Eur J Pediatr Surg. 2020 Feb. 30 (1):33-8. [QxMD MEDLINE Link].
Parmentier B, Peycelon M, Muller CO, El Ghoneimi A, Bonnard A. Laparoscopic management of congenital duodenal atresia or stenosis: a single-center early experience. J Pediatr Surg. 2015 Nov. 50 (11):1833-6. [QxMD MEDLINE Link].
Dewberry LC, Vuille-Dit-Bille RN, Kulungowski AM, Somme S. A single surgeon laparoscopic duodenoduodenostomy case series for congenital duodenal obstruction in an academic setting. J Laparoendosc Adv Surg Tech A. 2018 Dec. 28 (12):1517-9. [QxMD MEDLINE Link].
Holler AS, Muensterer OJ, Martynov I, Gianicolo EA, Lacher M, Zimmermann P. Duodenal atresia repair using a miniature stapler compared to laparoscopic hand-sewn and open technique. J Laparoendosc Adv Surg Tech A. 2019 Oct. 29 (10):1216-22. [QxMD MEDLINE Link].
Valusek PA, Spilde TL, Tsao K, St Peter SD, Holcomb GW 3rd, Ostlie DJ. Laparoscopic duodenal atresia repair using surgical U-clips: a novel technique. Surg Endosc. 2007 Jun. 21 (6):1023-4. [QxMD MEDLINE Link].
Arnbjornsson E, Larsson M, Finkel Y, Karpe B. Transanastomotic feeding tube after an operation for duodenal atresia. Eur J Pediatr Surg. 2002 Jun. 12 (3):159-62. [QxMD MEDLINE Link].
Bairdain S, Yu DC, Lien C, et al. A modern cohort of duodenal obstruction patients: predictors of delayed transition to full enteral nutrition. J Nutr Metab. 2014. 2014:850820. [QxMD MEDLINE Link].
Goring J, Isoldi S, Sharma S, et al. Natural orifice endoluminal technique (NOEL) for the management of congenital duodenal membranes. J Pediatr Surg. 2020 Feb. 55 (2):282-5. [QxMD MEDLINE Link].
Barabino A, Arrigo S, Gandullia P, Vignola S. Duodenal web: complications and failure of endoscopic treatment. Gastrointest Endosc. 2012 May. 75 (5):1123-4. [QxMD MEDLINE Link].
Barabino A, Gandullia P, Arrigo S, Vignola S, Mattioli G, Grattarola C. Successful endoscopic treatment of a double duodenal web in an infant. Gastrointest Endosc. 2011 Feb. 73 (2):401-3. [QxMD MEDLINE Link].
Chung PH, Wong CW, Ip DK, Tam PK, Wong KK. Is laparoscopic surgery better than open surgery for the repair of congenital duodenal obstruction? A review of the current evidences. J Pediatr Surg. 2017 Mar. 52 (3):498-503. [QxMD MEDLINE Link].

Media Gallery

Pediatric duodenal atresia. Incision for duodenal exposure.
Pediatric duodenal atresia. Side-to-side duodenoduodenostomy.
Pediatric duodenal atresia. Diamond-shaped duodenoduodenostomy.
Pediatric duodenal atresia. Three anatomic types of duodenal atresia are recognized. In type 1 atresia, a membrane traverses the internal diameter of the duodenum. This membrane may be elongated, giving rise to the windsock type 1 duodenal atresia. In type 2 atresia, the atretic ends of the duodenum are connected by a fibrous cord. In type 3 atresia, the atretic segments are completely separated.
Pediatric duodenal atresia. Upper gastrointestinal series demonstrating duodenal atresia.

of 5

Author

Frederick Merrill Karrer, MD, FACS Professor of Surgery and Pediatrics, Head, Division of Pediatric Surgery, University of Colorado School of Medicine; The Dr David R and Kiku Akers Chair in Pediatric Surgery, Surgical Director, Pediatric Transplantation, The Children’s Hospital of Colorado

Frederick Merrill Karrer, MD, FACS is a member of the following medical societies: American Academy of Pediatrics, American Association for the Study of Liver Diseases, American College of Surgeons, American Pediatric Surgical Association, American Society of Transplant Surgeons, Denver Academy of Surgery, International Pediatric Transplant Association, Pacific Association of Pediatric Surgery, Transplantation Society, Western Surgical Association, Western Trauma Association

Disclosure: Nothing to disclose.

Coauthor(s)

Sarah Ogle, DO, MS Fellow in Pediatric Minimally Invasive Bariatric Surgery, Department of Surgery, Children’s Hospital of Colorado, University of Colorado School of Medicine, Anschutz Medical Campus

Sarah Ogle, DO, MS is a member of the following medical societies: American College of Surgeons, Association for Academic Surgery, National Neurotrauma Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David A Piccoli, MD Chief of Pediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine

David A Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Chief Editor

Carmen Cuffari, MD Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching. for: Abbott Nutritional, Abbvie, speakers' bureau.

Additional Contributors

Casey M Calkins, MD Associate Professor of Surgery, Division of Pediatric Surgery, Medical College of Wisconsin; Consulting Staff, Department of Pediatric Surgery, Children's Hospital of Wisconsin

Casey M Calkins, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association

Disclosure: Nothing to disclose.

Jayant Deodhar, MD Associate Professor in Pediatrics, BJ Medical College, India; Honorary Consultant, Departments of Pediatrics and Neonatology, King Edward Memorial Hospital, India

Disclosure: Nothing to disclose.

D Dean Potter, MD Fellow in Pediatric Surgery, The Children's Hospital

D Dean Potter, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, Minnesota Medical Association, International Pediatric Endosurgery Group

Disclosure: Nothing to disclose.