• Clinical science

Duodenal atresia and stenosis

Abstract

Duodenal atresia is a complete occlusion or absence of the duodenal lumen, whereas duodenal stenosis refers to an incomplete obstruction caused by narrowing. Both conditions present with signs of obstruction, including a distended upper abdomen, delayed meconium passage, and vomiting. If the defect is located proximal to the major duodenal papilla, nonbilious vomiting occurs, whereas a defect occurring distal to the papilla causes bilious vomiting. Evidence of polyhydramnios on prenatal ultrasonography may lead to an early diagnosis of duodenal obstruction. An abdominal x-ray typically shows the double bubble sign. Duodenal atresia and stenosis generally have a favorable prognosis and may be surgically treated through duodenoduodenostomy.

Epidemiology

References:[1][2]

Epidemiological data refers to the US, unless otherwise specified.

Pathophysiology

Duodenal atresia occurs when recanalization of the closed duodenum fails to occur or occurs only partially during the embryonic period (usually between the 8th–10th week of gestation). Because the development of the duodenum is connected to the growth of the pancreas and the hepatobiliary system, duodenal atresia is commonly associated with anomalies of these organs as well.

References:[1]

Clinical features

Intrauterine

Postpartum

  • Vomiting, that is typically bilious if the stenosis is distal to the major duodenal papilla
    • Atresia or high-grade stenosis: vomiting few hours after birth
    • Mild stenosis: vomiting after a few days
  • Distended upper abdomen and scaphoid lower abdomen
  • Delayed meconium passage

References:[3][1]

Diagnostics

Prenatal

Postnatal

  • X-ray of the abdomen
  • Associated anomalies may also be investigated with the following methods:
    • Physical examination
    • Ultrasound of the abdomen and kidneys
    • Echocardiography
    • X-ray of the thorax
    • X-ray, ultrasound, and/or MRI of the spine

References:[4][3]

Differential diagnoses

Jejunal atresia

  • Definition: absence of the jejunal lumen
  • Epidemiology: less common than duodenal atresia
  • Pathophysiology
    • Vascular accident in utero necrosis and reabsorption of the jejunum
    • Risk factors: vasoconstrictive drugs (e.g., cocaine, MDMA, or cigarettes) during pregnancy
  • Clinical features: similar to duodenal atresia
    • Polyhydramnios (intrauterine)
    • Bilious vomiting and upper abdominal distension (postpartum)
  • Diagnosis: abdominal x-ray shows a triple bubble sign and gasless colon
  • Treatment: surgical correction with bypass of the occluded part of bowel is always required

The differential diagnoses listed here are not exhaustive.

Treatment

  • Preoperative management
    • Parenteral nutrition via a central catheter shortly after birth
    • Fluid replacement and restoration of the electrolyte balance
    • Gastric decompression
  • Surgery: bypass the atresia or stenosis
    • The exact procedure depends on the anatomic findings and associated anomalies
    • Common procedure: duodenoduodenostomy or duodenojejunostomy with a proximal transverse-to-distal longitudinal (diamond-shaped) anastomosis

References:[5]

Prognosis

  • If diagnosis and treatment occur quickly, the survival rate of isolated duodenal atresia (without further anomalies) is 95%.
  • The presence of other anomalies worsens the prognosis.

References:[2]

  • 1. Kimura K, Loening-Baucke V. Bilious Vomiting in the Newborn: Rapid Diagnosis of Intestinal Obstruction. Am Fam Physician. 2000; 61(9): pp. 2791–2798. url: http://www.aafp.org/afp/2000/0501/p2791.html.
  • 2. Choudhry MS, Rahman N, Boyd P, Lakhoo K. Duodenal atresia: associated anomalies, prenatal diagnosis and outcome. Pediatr Surg Int. 2009. doi: 10.1007/s00383-009-2406-y.
  • 3. Editors: Coppola CP, Kennedy Jr. AP, Scorpio RJ. Pediatric Surgery: Diagnosis and Treatment. Cham, CH: Springer International Publishing; 2014.
  • 4. Brantberg A, Blaas H-G K, Salvesen KA, Haugen SE, Mollerlokken G, Eik-Nes SH. Fetal duodenal obstructions: increased risk of prenatal sudden death. Ultrasound Obstet Gynecol. 2002; 20: pp. 439–446. doi: 10.1046/j.1469-0705.2002.00831.x.
  • 5. Nicola Lewis, FRCS(Paed Surg). Pediatric Duodenal Atresia and Stenosis Surgery. In: Kim ES. Pediatric Duodenal Atresia and Stenosis Surgery. New York, NY: WebMD. http://emedicine.medscape.com/article/935748-overview#a4. Updated February 8, 2016. Accessed December 22, 2016.
last updated 10/19/2018
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