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Jejunal Atresia

 Jejunal Atresia



Overview

jejunal atresia is one of several types of intestinal atresia. The rare birth defect causes a portion of the membrane (mesentery) that connects the intestines to the abdominal wall to be malformed or missing. The abnormality leads to intestinal blockages (atresia).

This article covers the symptoms and causes of jejunal atresia. It also looks at how ultrasounds and other tests can detect jejunal atresia and how it's treated.

Symptoms 

Atresia can often be diagnosed before a baby is born. Routine prenatal screenings may detect the condition because the signs of an intestinal blockage are usually visible on an ultrasound.

Babies with atresia are sometimes born before their due date (preterm) and the symptoms of the condition usually appear within the first day or two after birth.

The symptoms of jejunal atresia in newborns can include:1

Problems with feeding

Throwing up a yellow-green fluid (bile)

Failure to thrive

Swollen belly

No bowel movements

CAUSES

Jejunal atresia occurs sporadically for unknown reasons in fetuses that are generally normal in all other respects. The best theory to explain what happens is that the blood flow to a segment of the bowel is cut off with the result that the affected segment disappears leaving a gap in the bowel.

There is some evidence to suggest a possible genetic cause, especially in cases of multiple jejunal atresia (type 4). This theory is based on reports of families with more than one affected child. An autosomal recessive pattern of inheritance has been proposed, but no specific genes have been found to be associated with the condition.

Recessive genetic disorders occur when an individual inherits a non-working gene from each parent. If an individual receives one working gene and one non-working gene for the disease, the person will be a carrier for the disease, but usually will not show symptoms. The risk for two carrier parents to both pass the non-working gene and, therefore, have an affected child is 25% with each pregnancy. The risk to have a child who is a carrier, like the parents, is 50% with each pregnancy. The chance for a child to receive working genes from both parents is 25%. The risk is the same for males and females.

Although most babies with jejunal atresia are otherwise healthy, patients with the following diagnoses are believed to be predisposed to jejunal atresia: cystic fibrosis, gastroschisis and antenatal midgut volvulus. Approximately 10% of those with jejunal atresia will also be diagnosed with cystic fibrosis. Maternal vasoconstrictive medications, maternal cigarette smoking and maternal cocaine use may contribute to developing the disease.

AFFECTED POPULATIONS

Jejunal atresia affects male and female newborns in equal numbers.

DISORDERS WITH SIMILAR SYMPTOMS

Symptoms of the following disorder can be similar to those of jejunal atresia. Comparisons may be useful for a differential diagnosis:

Duodenal atresia is a rare disorder in which there is a blockage of the normal opening in the first division of the small intestine, the duodenum. This malformation causes excessive accumulation of amniotic fluid before birth and vomiting of the neonate immediately after birth. Other symptoms of this disorder may be: a swollen upper abdomen; a yellowish coloring of the skin (jaundice). The obstruction can be repaired with surgery.

DIAGNOSIS

The atresia may be detected by ultrasound before birth but the diagnosis must be confirmed after birth. Plain x-rays of the abdomen in patients with jejunal atresia show dilated segments of bowel filled with gas and liquid. A barium swallow, which is an x-ray test after the baby drinks liquid barium, may be used to assess the upper digestive tract and confirm the obstruction. An additional finding in some patients with jejunal atresia is a smaller than normal large bowel called a microcolon beyond the point of blockage. A barium enema can confirm the bowel obstruction and unused colon.

STANDARD THERAPIES

Surgery to repair the intestinal obstruction is performed as soon as possible after delivery. A section of the intestine including the atresia will be removed and the two ends will be sewn together. Food is given by a vein but not by mouth (parenteral nutrition) for a period of time before and after surgery until proper function of the intestine is established.

Genetic counseling is recommended for patients and their families. Other treatment is symptomatic and supportive.

Type of Doctor Department : Pediatric surgeon

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