Congenital Duodenal Obstruction

Congenital duodenal obstructions are a group of inborn conditions that cause obstruction of the lumen of the duodenal segment of the small intestine, impeding the passage of food and gastric juices. The condition may have intrinsic etiologies, such as duodenal atresia (most common) and/or extrinsic etiologies, such as an annular pancreas. The hallmark of duodenal obstruction is bilious vomiting. The condition can be diagnosed through prenatal or postnatal ultrasound. Associated anomalies, including congenital heart disease, are common. Treatment includes fluid resuscitation and surgical duodenoduodenostomy.

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Epidemiology and Etiology

Epidemiology

  • Overall incidence: 2.5–10 per 100,000 live births
  • Duodenal atresia: 
    • Incidence: 1 in 10,000 live births
    • 50% of infants with duodenal atresia are premature.
    • Common concomitant anomalies:
      • Down’s syndrome (25%–40%)
      • Congenital heart disease (30%): associated with increased mortality
      • Annular pancreas (30%): associated with late pancreatitis, gastroesophageal reflux disease, peptic ulcer disease, and gastric cancer
      • Malrotation (20%–30%)
      • Renal anomalies (5%–15%)
      • Esophageal atresia (5%–10%)
      • Skeletal malformations (5%)
      • Anorectal anomalies (5%)
  • Annular pancreas:
    • Incidence: 5–15 per 100,000 adults
    • Common concomitant abnormalities:
      • Maternal polyhydramnios 
      • Meckel’s diverticulum
      • Down’s syndrome
      • Imperforate anus
      • Duodenal atresia
      • Esophageal atresia
  • Duplication cysts:
    • Incidence: 1 in 45,000 births
    • Duodenal duplication cysts make up only 5% of reported cases.
  • Congenital bands: Incidence of duodenal obstruction due to Ladd’s bands is not reported. 

Etiology

  • Intrinsic (most common):
    • Duodenal atresia (most common): failed recanalization of the duodenum during embryonal development
    • Duodenal stenosis: incomplete recanalization of the duodenum during embryonal development
  • Extrinsic: 
    • Annular pancreas: band of pancreatic tissue surrounding the duodenum arising from incorrect rotation of the pancreatic bud during embryonal development 
    • Congenital bands (Ladd’s bands): fibrous bands of peritoneum that persist due to malrotation of the small intestines
    • Duplication cyst: cystic malformations of the small intestines that can deform the lumen of the duodenum
  • Intrinsic and extrinsic causes may occur concomitantly.

Clinical Presentation

Signs and symptoms of intestinal obstruction:

  • Polyhydramnios and bile-stained amniotic fluid may be present at birth.
  • Bilious vomiting without abdominal distension on 1st day of life (hallmark)
  • Delayed symptoms or prolonged vomiting and failure to thrive based on severity of occlusion
  • Abdominal peristaltic waves may be present, especially early in the course.

Associated abnormalities:

  • Biliary atresia
  • Agenesis of gallbladder
  • Renal, limb, and vertebral malformations

Diagnosis and Management

Diagnosis

History

  • Prenatal: 
    • Duodenal obstruction in other pregnancy
    • Bowel dilatation or polyhydramnios on prenatal screening
    • Maternal diabetes mellitus
  • Perinatal:
    • Gestational age
    • Hours from birth to onset of vomiting
    • Timing of meconium passing
  • Family history:
    • Siblings with intestinal malformations
    • Cystic fibrosis

Physical exam

  • General:
    • Signs pointing to dehydration
    • Dysmorphic features suggestive of associated abnormalities seen with atresia (e.g., Down’s syndrome)
  • Skin: jaundice
  • Abdomen: Distension suggests that obstruction is not duodenal but more distal.
  • Rectal exam: anal atresia associated with duodenal atresia

Imaging

  • Prenatal ultrasound: 
    • Shows “double bubble” sign (air-filled stomach and duodenum) 
    • May reveal polyhydramnios 
  • Radiography:
    • Double bubble sign confirms diagnosis of duodenal obstruction. 
    • Contrast studies may occasionally be needed to rule out malrotation and volvulus.
  • Computed tomography (CT) or magnetic resonance imaging (MRI) may be used for better visualization.

Management

Medical therapy

  • Nasogastric decompression 
  • Fluid resuscitation

Surgical management

  • Duodenal atresia and/or annular pancreas are corrected with duodenoduodenostomy. 
  • Long-term prognosis is excellent. 

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References

  1. Kliegman RB, ST Geme JW, Blum MJ, Shah SS, Tasker RC, Wilson KM, Behrman RE. (2016). In Nelson’s Textbook of Pediatrics. (20th ed.) Elsevier.
  2. Ravitch MM. (1975). The pancreas in infants and children. Surg Clin North Am, 55(2):377–85. https://doi.org/10.1016/s0039-6109(16)40587-6
  3. Sangüesa Nebot C, Llorens Salvador R, Carazo Palacios E, Picó Aliaga S, Ibañez Pradas V. (2018). Enteric duplication cysts in children: varied presentations, varied imaging findings. Insights Imaging, 9(6):1097–1106. https://doi.org/10.1007/s13244-018-0660-z
  4. Sadler TW, Langman J. (2012). Langman’s medical embryology (12th ed.) Wolters Kluwer Health/Lippincott Williams & Wilkins.

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