Intestinal atresia

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Intestinal atresia
ICD-10 Q41., Q42.
ICD-9 751.1 751.2
OMIM 223400 243600
DiseasesDB 31514 33000

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]


Overview

Intestinal atresia is a malformation where the there is a narrowing or absence of a portion of the intestine. This defect can either occur in the small or large intestine.

Types of intestinal atresia

The different types of intestinal atresia are named after their location:

  • Jejunal atresia - malformation of the jejunum, the second part of the intestine extending from the duodenum to the ileum
  • Duodenal atresia - malformation of the duodenum, part of the intestine that empties from the stomach
  • Ileal atresia - malformation of the ileum, the lower part of the small intestine
  • Colon atresia - malformation of the colon

Diagnosis

Intestinal atresias are often discovered before birth: either during a routine sonogram which shows a dilated intestinal segment due to the blockage, or by the development of polyhydramnios (the build up of too much amniotic fluid in the uterus). These abnormalities are indications that the fetus may have a bowel obstruction which a more detailed ultrasound study can confirm.

Some fetuses with bowel obstruction have abnormal chromosomes. An amniocentesis is recommended because it can determine not only the sex of the baby, but whether or not there is a problem with the chromosomes.

Intestinal atresia must be differentiated from other diseases that cause a failure to pass meconium or abdominal distension in infants, including meconium plug syndrome, small left colon syndrome, and congenital hypothyroidism.

Disease Prominent clinical features Radiological findings
Meconium plug syndrome
Abdominal x-ray with contrast showing inspissated meconium in the intestine, proximal to the colon - Case courtesy of Radswiki, Radiopaedia.org, rID 11606
Small left colon syndrome
Abdominal x-ray with contrast, shows decreased caliber of the descending and sigmoid colon, loss of haustration along with filling defects corresponding to retained feces - Case courtesy of Dr Eric F Greif, Radiopaedia.org, rID 30024
Distal small intestine/colon atresia
  • Failure to pass meconium due to failure of intestine recanalization.
  • Proximal lesions have an earlier onset of symptoms than distal lesions.
  • Colonic atresia may affect normal children or may be associated with other abnormalities as Hirschsprung's disease or gastroschisis.[3]
Normal appearing colon that is small and unused. Contrast fills the whole colon and passes to the ileum - Case courtesy of A.Prof Frank Gaillard, Radiopaedia.org, rID 5959
Meconium ileus
Contrast enema shows inspissated meconium starting from the mid-sigmoid colon and going up till the splenic flexure. The colon is normal in diameter, ruling out microcolon - Case courtesy of Dr Michael Sargent, Radiopaedia.org, rID 6009
Congenital hypothyroidism

Treatment

Fetal and neonatal intestinal atresia treated with using laparotomy after birth. If the area affected is small, the surgeon may be able to remove the damaged portion and join the intestine back together. In instances where the narrowing is longer, or the area is damaged and cannot be used for period of time, a temporary stoma may be placed.

See also

External links


  1. Keckler SJ, St Peter SD, Spilde TL, Tsao K, Ostlie DJ, Holcomb GW, Snyder CL (2008). "Current significance of meconium plug syndrome". J. Pediatr. Surg. 43 (5): 896–8. PMC 3086204Freely accessible. PMID 18485962. doi:10.1016/j.jpedsurg.2007.12.035. 
  2. Berdon WE, Slovis TL, Campbell JB, Baker DH, Haller JO (1977). "Neonatal small left colon syndrome: its relationship with aganglionosis and meconium plug syndrome". Radiology. 125 (2): 457–62. PMID 910057. doi:10.1148/125.2.457. 
  3. Spitz L (2006). "Observations on the origin of congenital intestinal atresia". S. Afr. Med. J. 96 (9 Pt 2): 864. PMID 17077911. 
  4. HOLSCLAW DS, ECKSTEIN HB, NIXON HH (1965). "MECONIUM ILEUS. A 20-YEAR REVIEW OF 109 CASES". Am. J. Dis. Child. 109: 101–13. PMID 14237408. 
  5. "Elementary school performance of children with congenital hypothyroidism. New England Congenital Hypothyroidism Collaborative". J. Pediatr. 116 (1): 27–32. 1990. PMID 2295961. 

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