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  • • Other etiologies:

    • -Intestinal atresia

      -Intestinal duplication

      -Mesenteric or omental cyst

      -Meckel diverticulum

      -Foreign body

      -Meconium ileus

      -Annular pancreas

    • 10-20% of patients with abdominal wall defects have intestinal atresia

    • 90-95% of duodenal atresia is distal to ampulla

    • 90% of jejunoileal atresias have complete atresia

    • Distal ileum most common site of atresia

    • 3.6-20% of patients have multiple areas of intestinal atresia

    • Patent accessory pancreatic duct common with annular pancreas

    • 50% of duodenal atresia with complete atresia and 50% with webs or diaphragm

    • Trisomy 21 associated with duodenal atresia

    • 10-20% of patients with cystic fibrosis develop meconium ileus (concretions of meconium usually found just proximal to ileocecalvalve secondary to decreased pancreatic exocrine activity)

    • 33-50% of patients with meconium ileus undergo proximal volvulus, perforation, or atresia that occurs in utero

    • Cardiac anomalies associated with duodenal atresia

    • 5-10% of patients with Meckel diverticulum will present with obstruction secondary to volvulus or intussusception

    • 95% of foreign bodies that pass beyond the gastroesophageal junction pass through remainder of GI tract uneventfully

    • Mesenteric cysts 2-fold more common than omental cysts

    • Omental and mesenteric cysts diagnosed before 10 years of age

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Epidemiology

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  • • Intestinal atresia: 3.5/10,000 births

    • All congenital duodenal obstructions: 1/6000-1/10,000 births

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Symptoms and Signs

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  • • Bilious emesis

    • Abdominal distention

    • Irritability

    • Maternal polyhydramnios

    • Failure to pass meconium

    • Umbilical cord ulceration (rarely with intestinal atresia)

    • Abdominal mass if mesenteric/omental cyst or duplication

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Laboratory Findings

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  • • Test for CFTR mutations or sweat chloride analysis in patients with meconium ileus to diagnose cystic fibrosis

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Imaging Findings

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  • Abdominal x-ray: Shows transition point of gas (soap bubble appearance intraluminal in meconium ileus)

    Upper or lower GI series: Demonstrates transition point of obstruction (concretions in meconium ileus)

    CT scan or US: May demonstrate cystic mass with mesenteric or omental cysts

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Rule Out

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  • • Malrotation

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  • • History and physical exam

    • Abdominal x-ray

    • Upper and/or lower GI series

    • CFTR or sweat chloride (to document cystic fibrosis in patients with meconium ileus)

    • Echocardiogram in patients with duodenal atresia

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Surgery

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  • • Primary anastomosis following short segmental resection (if web associated) after careful exam for other sites of obstruction or atresia

    • Duodenoduodenostomy for annular pancreas and duodenal atresia (possible excision of web for atresia)

    • Operative retrieval of foreign body if symptomatic, or an alkaline battery, or if persists in 1 location (no transition) for 1 week or more or several weeks in stomach

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Medications

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  • • Nonoperative management possible in 65% cases of meconium ileus, enemas using gastrograffin, tween-80, or N-acetylcysteine are beneficial

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Complications

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  • • Short bowel syndrome if left with < 40 cm

    • Damage to ampulla ...

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