Duodenal atresia

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

In 1900, J. Tandler reported duodenal stenosis due to failure of recanalization of duodenum during fetal development in pregnancy. In 1961, Thomas Santulli and William Blanc described the figure 8 formation of small bowel, which is described as apple-peel intestinal atresia. In 1936, William Ladd developed a surgical procedure to correct the duodenal malrotation.

Duodenal atresia discovery is as follows:^[1][2]

• In 1900, J. Tandler was the first to discover the association of duodenal stenosis due to failure of recanalization of the embryonic duodenum and the development of duodenal atresia.
• In 1961, Thomas V. Santulli and William A. Blanc described apple-peel intestinal atresia, which is the figure 8 formation of the small bowel.

• In 1936, a surgical procedure was developed by William Ladd to to correct the malrotation of duodenal atresia.^[3]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Intestinal atresia, including duodenal atresia, may be classified into four subtypes: Type I, Type II, Type III, and Type IV. Type I is the most common subtype which involves the complete mucosal membrane, with muscularis and serosa remaining intact.

• Intestinal atresia may be classified into several subtypes based on the location of the intestinal atresia:^[1][2]
  • Duodenum
    • Type I
      • Involves complete mucosal membrane or diaphragm
      • Muscularis and serosa remaining intact
      • No discontinuation of the bowel
    • Type II
      • Fibrous cord connects two discontinued sections of bowel
    • Type III
      • No fibrous cord connecting the proximal and distal duodenum
    • Type IV
      • Several absent areas of bowel
      • String of sausage appearance
  • Jejunum
  • Ileum

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

It is thought that duodenal atresia is the result of failure of neural cell migration during the 8th to 10th week of duodenal re-canalization. It is associated with down syndrome, vertebral defects, anal anomalies, esophageal atresia, annular pancreas, malrotation, renal abnormalities, cardiac causes, and mandibulofacial anomalies.

• Duodenum starts developing during the 6th and 7th week of gestation.^[1][2]
  • Re-canalization occurs during the 8th to 10th week of gestation.
• It is thought that duodenal atresia is the result of failure of re-canalization of the duodenum in 8 to 10 weeks of fetal development.
  • This is due to failure of neural cell migration

• Duodenal atresia is not transmitted genetically.

Duodenal atresia is commonly associated with the following:^[3][4]

• Down syndrome in 25 %to 40% of cases
• VATER
  • Vertebral defects
  • Anal anomalies
  • Esophageal atresia
  • Renal abnormalities
• Malrotation
• Annular pancreas
• Biliary tract abnormalities
• Cardiac anomalies
• Mandibulofacial anomalies

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

The cause of duodenal atresia has not been identified. To review risk factors for the development of duodenal atresia, click here.

• There are no life-threatening causes of duodenal atresia, however complications resulting from untreated duodenal atresia is common.

• There are no known genetic causes of duodenal atresia.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

Duodenal atresia must be differentiated from other diseases that cause persistent vomiting, and feeding difficulties, such as volvulus, jejuno-ileal atresia, malrotation, and meconium ileus. It is differentiated based on imaging.

• Duodenal atresia must be differentiated from other diseases that cause persistent vomiting, and feeding difficulties, such as volvulus, jejuno-ileal atresia, and malrotation.^[1][2][3]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

The prevalance of duodenal atresia is 0.1 to 0.4 per 100000. It commonly affects neonates and has a male to female ratio of 2 to 1.

The epidemiology and demographics of duodenal atresia are as follows:^[1][2]

• The incidence of intestinal atresia is:
  • 0.1 to 0.4 per 100000 for duodenum
  • 0.015 per 100000 for jejunum
  • 0.12 per 100000 for ileum

• The prevalence of duodenal atresia is estimated to be 0.1 to 0.4 per 100000 of cases annually.
  • About 60% of intestinal atresias
  • 30% associated with down syndrome

• Duodenal atresia commonly affects individuals in infancy.

• There is no racial predilection to duodenal atresia.

• Duodenal atresia affects males more commonly than females.
  • The male to female ratio is approximately 2 to 1.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

The most potent risk factor in the development of duodenal atresia is down syndrome. Other risk factors include annular pancreas, and VACTERL syndrome.

The risk factors are as follows:^[1][2][3]

• The most potent risk factor in the development of duodenal atresia is down syndrome.

• Other risk factors include annular pancreas, and VACTERL anomalies.
  • VACTERL
    • Vertebral defects
    • Anal anomalies
    • Cardiac anomalies
    • Tracheoesophageal atresia
    • Renal abnormalities
    • Limb abnormalities
• Malrotation
• Annular pancreas
• Biliary tract abnormalities
• Mandibulofacial anomalies

• Common risk factors in the development of duodenal atresia include:
  • Down syndrome in 30% of the cases.

• VACTERL
  • Vertebral defects
  • Anal anomalies
  • Cardiac anomalies
  • Tracheoesophageal atresia
  • Renal abnormalities
  • Limb abnormalities

• Malrotation
• Biliary tract abnormalities

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

There is insufficient evidence to recommend routine screening for duodenal atresia. However, screening for down syndrome may show duodenal atresia during pregnancy.

• There is insufficient evidence to recommend routine screening for duodenal atresia.

Screening for down syndrome is as follows:^{[1][2][3][4][5][6]}

• Genetic counseling is usually offered to families who may have an increased chance of having a child with Down syndrome along with:
  • genetic testing
  • amniocentesis
    • Involves inserting instruments into the uterus which carry a small risk of causing fetal injury.
  • chorionic villus sampling (CVS)
    • Involves inserting instruments into the uterus which carry a small risk of causing fetal injury.
  • Percutaneous umbilical blood sampling (PUBS) are usually offered

• Common screening procedures for Down syndrome are given in Table 1.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]

The symptoms of duodenal atresia usually develop in the first 48 hours of life, and start with symptoms such as bilious vomiting in 80% of the cases. Prognosis is generally very good, and the survival rate of patients with duodenal atresia is approximately 90%.

• The symptoms of duodenal atresia usually develop in the first 48 hours of life, and start with symptoms such as vomiting.^[1]
  • Bilious vomiting in 80% of the cases.
  • Non-bilious vomiting in 20% of the cases.
  • Dehydration
• If duodenal atresia is left untreated it will result in death.

The complications of duodenal atresia are as follows:^[2][3]

• There are no complications associated with duodenal atresia, however post surgical complications may occur.

• Post surgical complications include:
  • Adhesions
  • Duodenal dysmotility
  • Megaduodenum with blind loop syndrome
  • Duodenogastric reflux and gastritis
  • Peptic ulcer disease
  • Gastroesophageal reflux
  • Cholelithiasis
  • Cholecystitis

• Prognosis is generally good, and the survival rate of patients with duodenal atresia is approximately 90% after surgery.^[4]

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