eMedicine Specialties > Pediatrics: Surgery > General Surgery

Duodenal Atresia and Stenosis: Surgical Perspective

Author: Nicola Lewis, MBBS, FRCS, Specialist Registrar, Department of Surgery, Birmingham Children's Hospital, UK
Coauthor(s): Philip Glick, MD, MBA, Professor, Departments of Surgery, Pediatrics, and Gynecology and Obstetrics, Vice-Chairperson for Research and Development, Department of Surgery, State University of New York at Buffalo
Contributor Information and Disclosures

Updated: Oct 1, 2008

Introduction

Fonkalsrud reviewed 503 cases of congenital duodenal obstruction treated between 1957 and 1967.1 Of patients who were surgically treated, 64% survived. Deaths were attributed to associated malformations, respiratory complications, prematurity, and anastomotic complications.

More recent survival rates for infants born with duodenal atresia or stenosis are approximately 90-95%.2,3 Increased survival rates can be attributed to advances in respiratory care, hyperalimentation, improved pediatric anesthesia, improvements in the recognition and management of associated anomalies, and more refined surgical techniques (eg, the diamond-shaped anastomosis4 ).

History of the Procedure

In 1733, Calder described the first 2 recorded cases of duodenal atresia. The first successfully treated case was reported by Vidal in 1905; a gastrojejunostomy was performed. In 1914, Ernest performed the first successful duodenojejunostomy in an infant with duodenal atresia. Current surgical management more commonly includes duodenoduodenostomy and duodenoplasty.

Problem

See Pathophysiology.

Frequency

The incidence of duodenal atresia is 1 case per 5,000-10,000 live births.

Etiology

Most cases of duodenal atresia are sporadic. Investigations of familial cases of duodenal atresia suggest an autosomal recessive inheritance in these individuals.1,5

Pathophysiology

In 1900, Tandler described the traditionally accepted theory on the normal development of the duodenum.6 The duodenum develops from the caudal part of the foregut and the cranial part of the midgut. At 4 weeks' gestation, it consists of an epithelial tube surrounded by mesenchyme. At 5-6 weeks' gestation, the epithelium proliferates while the surrounding mesenchymal walls are still narrow; the epithelial cells fill the lumen, completely obliterating it. Subsequent epithelial apoptosis at 8-10 weeks' gestation leads to vacuolation and recanalization of the duodenum. Failure of vacuolation may lead to intrinsic duodenal obstruction.

Presentation

In 38-55% of patients, intrinsic duodenal obstruction is associated with another significant congenital anomaly.1,7,8,9 Approximately 30% of cases are associated with Down syndrome, and 23-34% of cases are associated with isolated cardiac defects. Esophageal atresia may be present in 7-12% of patients.10 Other GI anomalies include malrotation, anterior portal vein, second distal web, anorectal anomalies, intestinal atresias, cloacal anomalies, and renal tract anomalies. Duodenal atresia is associated with prematurity and low birth weight.11,8 Rarely, duodenal atresia is seen as a part of Feingold syndrome.12

Duodenal atresia

Duodenal atresia is prenatally detected in 32-57% of patients.7,13 Sonographic features of high intestinal obstruction (ie, duodenal obstruction with a dilated stomach [double-bubble sign]) become apparent in the third trimester. Polyhydramnios develops in 32-59% of cases; in the presence of polyhydramnios, normal findings on ultrasonography of the fetus do not exclude duodenal atresia.14,7,15 A similar appearance can be observed in fetuses with a choledochal cyst, external duodenal compression, and a normal stomach with a sharp incisura. Approximately 80% of cases are prenatally diagnosed with confirmation following delivery.13

Prenatal diagnosis of duodenal atresia should lead to a search for other associated anomalies and amniocentesis for karyotype analysis.

Following delivery, a thorough physical examination should be performed, including careful examination of the anus.

Healthy newborn infants have gastric aspirates that measure less than 5 mL. Congenital intestinal obstruction is associated with gastric aspirates that measure greater than 30 mL.16 An infant with a gastric aspirate that measures greater than 30 mL in the delivery room or newborn nursery should be evaluated for duodenal atresia and other causes of upper intestinal obstruction.

Symptoms of upper intestinal obstruction commence within the first 24 hours after birth. However, patients may present hours or days after delivery. Sustained vomiting (bilious or nonbilious) is the most common symptom, occurring in approximately 85% of cases.2,1,3 Nonbilious vomiting occurs when atresia is present above the papilla of Vater. Vomiting is associated with variable dehydration, changes in serum electrolytes, and weight loss.

Normal meconium may be observed in the early stages.14,3 The high level of the obstruction makes global abdominal distension an infrequent finding, but fullness in the epigastrium, caused by the dilated duodenum and stomach, may be noted.

Differentials include malrotation and volvulus, intestinal atresia or stenosis in other locations, and extrinsic duodenal obstruction, duodenal duplication, or congenital bands.

Duodenal stenosis

The incomplete nature of the obstruction in duodenal stenosis results in a variable and often delayed presentation. It usually results in recurrent episodes of vomiting, aspiration, or failure to thrive. Some patients present in adulthood with gastroesophageal reflux, peptic ulceration, or obstruction of the duodenum proximal to the stenosis by a bezoar.

Indications

The definitive management of patients with intrinsic duodenal obstruction is surgical correction.

Relevant Anatomy

Duodenal atresia or stenosis usually occurs in the first or second part of the duodenum, most often near the papilla of Vater. The common bile duct may open into an intraluminal mucosal web.

The 3 anatomic types of duodenal atresia as described by Gray and Skandalakis (see Media file 1) are as follows:

  • Type 1: The most common type is formed by a membrane composed of mucosa and submucosa. This membrane traverses the internal diameter of the duodenum. The duodenum and stomach proximal to the obstruction are dilated and hypertrophied. The duodenum distal to the obstruction is narrowed. A variation of this occurs when the membrane is elongated in the shape of a windsock, and the site of origin of the membrane is proximal to the level of obstruction.
  • Type 2: The atretic ends of the duodenum are connected by a fibrous cord.
  • Type 3: Complete separation of the atretic segments occurs. Most of the biliary duct anomalies associated with duodenal atresia are observed in type 3 defects.15

Various biliary tract and pancreatic anomalies have been demonstrated in patients with duodenal atresia or stenosis. These include stenosis and duplication of the distal common bile duct, choledochal cysts, and annular pancreas. Of note, air in the distal duodenum and gallbladder on plain radiography is suggestive of a bifid common bile duct. Double duodenal atresia or stenosis is less frequently reported.17

Contraindications

In the patient with associated tracheoesophageal fistula, ligation of the fistula should precede correction of the duodenal atresia. This can be performed on 2 occasions or simultaneously. Repair of the atresia prior to ligation of the tracheoesophageal fistula could lead to duodenal rupture.

More on Duodenal Atresia and Stenosis: Surgical Perspective

Overview: Duodenal Atresia and Stenosis: Surgical Perspective
Workup: Duodenal Atresia and Stenosis: Surgical Perspective
Treatment: Duodenal Atresia and Stenosis: Surgical Perspective
Follow-up: Duodenal Atresia and Stenosis: Surgical Perspective
Multimedia: Duodenal Atresia and Stenosis: Surgical Perspective
References
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References

  1. Fonkalsrud EW, DeLorimier AA, Hays DM. Congenital atresia and stenosis of the duodenum. A review compiled from the members of the Surgical Section of the American Academy of Pediatrics. Pediatrics. Jan 1969;43(1):79-83. [Medline].

  2. Bailey PV, Tracy TF Jr, Connors RH, et al. Congenital duodenal obstruction: a 32-year review. J Pediatr Surg. Jan 1993;28(1):92-5. [Medline].

  3. Mooney D, Lewis JE, Connors RH, Weber TR. Newborn duodenal atresia: an improving outlook. Am J Surg. Apr 1987;153(4):347-9. [Medline].

  4. Kimura K, Mukohara N, Nishijima E, et al. Diamond-shaped anastomosis for duodenal atresia: an experience with 44 patients over 15 years. J Pediatr Surg. Sep 1990;25(9):977-9. [Medline].

  5. Mishalany HG, Der Kaloustian VM, Ghandour MH. Familial congenital duodenal atresia. Pediatrics. Mar 1971;47(3):633-4. [Medline].

  6. Boyden EA, Cope JG, Bill AH Jr. Anatomy and embryology of congenital intrinsic obstruction of the duodenum. Am J Surg. Aug 1967;114(2):190-202. [Medline].

  7. Grosfeld JL, Rescorla FJ. Duodenal atresia and stenosis: reassessment of treatment and outcome based on antenatal diagnosis, pathologic variance, and long-term follow-up. World J Surg. May-Jun 1993;17(3):301-9. [Medline].

  8. Harberg FJ, Pokorny WJ, Hahn H. Congenital duodenal obstruction. A review of 65 cases. Am J Surg. Dec 1979;138(6):825-8. [Medline].

  9. Kimble RM, Harding J, Kolbe A. Additional congenital anomalies in babies with gut atresia or stenosis: when to investigate, and which investigation. Pediatr Surg Int. 1997;12(8):565-70. [Medline].

  10. Spitz L, Ali M, Brereton RJ. Combined esophageal and duodenal atresia: experience of 18 patients. J Pediatr Surg. Feb 1981;16(1):4-7. [Medline].

  11. Hancock BJ, Wiseman NE. Congenital duodenal obstruction: the impact of an antenatal diagnosis. J Pediatr Surg. Oct 1989;24(10):1027-31. [Medline].

  12. Feingold M, Hall BP, Lacassie Y, Martinez-Frias ML. Syndrome of microcephaly, facial and hand abnormalities, tracheoesophageal fistula, duodenal atresia, and developmental delay. American Journal of medical genetics. 1997;69(3):[Medline].

  13. Phelps S, Fisher R, Partington A, et al. Prenatal ultrasound diagnosis of gastrointestinal malformations. J Pediatr Surg. Mar 1997;32(3):438-40. [Medline].

  14. al-Salem AH, Khwaja S, Grant C, Dawodu A. Congenital intrinsic duodenal obstruction: problems in the diagnosis and management. J Pediatr Surg. Dec 1989;24(12):1247-9. [Medline].

  15. Reid IS. Biliary tract abnormalities associated with duodenal atresia. Arch Dis Child. Dec 1973;48(12):952-7. [Medline].

  16. Britton JR, Britton HL. Gastric aspirate volume at birth as an indicator of congenital intestinal obstruction. Acta Paediatr. Aug 1995;84(8):945-6. [Medline].

  17. Stringer MD, Brereton RJ, Drake DP, Wright VM. Double duodenal atresia/stenosis: a report of four cases. J Pediatr Surg. May 1992;27(5):576-80. [Medline].

  18. Keckler SJ, St Peter SD, Spilde TL, Ostlie DJ, Snyder CL. The influence of trisomy 21 on the incidence and severity of congenital heart defects in patients with duodenal atresia. Pediatr Surg Int. Aug 2008;24(8):921-3. [Medline].

  19. Tashjian DB, Moriarty KP. Duodenal atresia with anomalous bile duct masquerading as midgut volvulus. Journal of Pediatric Surgery. 2001;36(6):956-957. [Medline].

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  27. Cragan JD, Martin ML, Moore CA, Khoury M. Descriptive epidemiology of small intestinal atresia, Atlanta, Georgia. Teratology. Nov 1993;48(5):441-50. [Medline].

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Further Reading

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Keywords

duodenal atresia, stenosis, congenital duodenal obstruction, gastrojejunostomy, duodenojejunostomy, duodenoduodenostomy, duodenoplasty, intrinsic duodenal obstruction, high intestinal obstruction, upper intestinal obstruction, sustained vomiting, bilious vomiting, nonbilious vomiting, loss of fluid, loss of electrolytes, hyperalimentation, Down syndrome, esophageal atresia, malrotation, anterior portal vein, second distal web, anorectal anomalies, intestinal atresias, cloacal anomalies, renal tract anomalies, polyhydramnios, choledochal cyst, failure to thrive

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Contributor Information and Disclosures

Author

Nicola Lewis, MBBS, FRCS, Specialist Registrar, Department of Surgery, Birmingham Children's Hospital, UK
Disclosure: Nothing to disclose.

Coauthor(s)

Philip Glick, MD, MBA, Professor, Departments of Surgery, Pediatrics, and Gynecology and Obstetrics, Vice-Chairperson for Research and Development, Department of Surgery, State University of New York at Buffalo
Philip Glick, MD, MBA is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Thoracic Society, Association for Academic Surgery, Association for Surgical Education, Central Surgical Association, Federation of American Societies for Experimental Biology, Medical Society of the State of New York, Phi Beta Kappa, Physicians for Social Responsibility, Royal College of Surgeons of England, Sigma Xi, Society for Pediatric Research, Society for Surgery of the Alimentary Tract, Society of Critical Care Medicine, and Society of University Surgeons
Disclosure: Nothing to disclose.

Medical Editor

Robert K Minkes, MD, PhD, Professor of Surgery, University of Texas Southwestern; Chief of Surgical Services, Children's Medical Center of Dallas-Legacy
Robert K Minkes, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Andre Hebra, MD, Chief, Division of Pediatric Surgery, Medical University of South Carolina; Professor of Surgery and Pediatrics, Medical University of South Carolina
Andre Hebra, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, Association for Academic Surgery, Society of Laparoendoscopic Surgeons, South Carolina Medical Association, Southeastern Surgical Congress, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

H Biemann Othersen Jr, MD, Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina
H Biemann Othersen Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Surgery of Trauma, American Burn Association, American Cancer Society, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society for Parenteral and Enteral Nutrition, American Surgical Association, American Thoracic Society, British Association of Paediatric Surgeons, Society for Surgery of the Alimentary Tract, Society of Critical Care Medicine, South Carolina Medical Association, Southeastern Surgical Congress, Southern Medical Association, Southern Society for Pediatric Research, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Chief Editor

Marleta Reynolds, MD, Professor of Surgery, Feinberg School of Medicine, Northwestern University; Interim Head, Division of Pediatric Surgery, Department of Surgery, Children's Memorial Hospital of Chicago
Marleta Reynolds, MD is a member of the following medical societies: American Pediatric Surgical Association
Disclosure: Nothing to disclose.

 
 
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