Intestinal atresia

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Intestinal atresia
ICD-10	Q41, Q42
ICD-9	751.1 751.2
OMIM	223400 243600
DiseasesDB	31514 Template:DiseasesDB2

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	Transient intestinal obstruction for 1-2 days after birth. Obstruction is functional due to dilated colon and delayed passage of feces. Usually seen in premature infants.	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	Reduced caliber of the colon starting from splenic flexure and going down, causing intestinal obstruction. Characterized by a sudden change of the colon diameter. Usually associated with gestational diabetes.	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.	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	Intestinal obstruction due to inspissation of meconium in the distal ileum. Most cases of meconium ileus are secondary to cystic fibrosis. Typically presents with failure to pass meconium and abdominal distension with or without vomiting. Meconium ileus may first present with complications, including perforation and volvulus.	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	Most infants are born asymptomatic. Symptoms, if present, may include, but not limited to: Macroglossia Hoarse cry Umbilical hernia Puffy facies Increased head circumference Hypothermia

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

• Bowel obstruction
• Ileus
• Atresia
• Pediatric surgery

External links

• UCSF Fetal Treatment Center: Bowel Obstructions
• Information about congenital malformations of the gastrointestinal tract in premature babies - The Hospital for Sick Children

Template:Congenital malformations and deformations of digestive system

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