Jejunoileal Atresia: Symptoms, Causes, Treatment, Types, Prognosis

The word ‘Atresia’ comes with meaning of Greek components where “a” means no or without and “tresis” mean orifice. So, atresia means no orifice or hole to channel out. Jejunoileal comes from the name of one of our intestinal parts, and that is jejunum. So totally, it is a congenital obstruction of the intestinal lumen or jejunum.

Our small intestine is segmented into three parts. They are named duodenum, jejunum and ileum. In case of jejunoileal atresia, there is an obstruction found mostly in jejunum and even in ileum as well. It is found 1 out of 1000 newborns till now have a chance of having jejunoileal atresia, and is common than the duodenal atresia.

There are signs by which a new born can be identified with this rare condition of jejunoileal atresia. It is normally found that unlike other newborns, a newborn with jejunoileal atresia vomits green bile within a day of its birth.

• The bile and nutrients in intestine can’t be used up or absorbed due to the obstruction, so it finds an alternative way to come out with vomit.
• This vomiting may occur after two or three days for the cases where obstructions are farther down in the intestine.
• A baby with swollen belly can also be a sign of jejunoileal atresia due to no bowel movement on its first day, where most of the babies do have normal bowel movement after their birth.
• Hyperbilirubinemia is also very common for this kind of case with jejunoileal atresia or intestinal obstruction. The old haemoglobins are transformed into bilirubin. This bilirubin’s need to be excreted with stool. The stool colour is even formed because of the presence of bilirubin. So, due to bowel movement disorder because of intestinal obstruction enterohepatic circulation of bilirubin is increased, which may result in jaundice.

There are four types atresia found for this disease of jejunoileal atresia. Each type of atresia occurs due to different causes.

• Type-1 Intestinal Atresia: In this case, a membrane gradually forms inside of the intestine when the baby is in its mother’s womb. This membrane generally blocks the intestine, but the intestine itself gets its normal length.
• Type-2 Intestinal Atresia: The intestine develops and gets its length, but there is an involvement of scar tissue. A blind end formation occurs in the intestine, and scar tissue connects it with a smaller segment of that intestine.
• Type-3 Intestinal Atresia: The intestinal villi play a major role in nutritional absorption, but type-3 intestinal atresia results in nutritional deficiency and gut syndrome. In this case two blind ends of the intestine are separated which reduces the length of the intestine.
• Type-4 Intestinal Atresia: This type of atresia comes with multiple intestinal obstructions, where parts or the sections of intestine are blocked. Like type-3, type-4 intestinal atresia also results in short intestinal length.

The intestinal obstruction, especially the rare jejunoileal atresia occurs because of the membrane which attaches the small intestine to the abdominal wall of a new born. Jejunum, a portion of the small intestine twists around an artery that leads to atresia or intestinal blockage.

If a clinician or doctor wants to check the family history, then it must be noted that jejunoileal atresia may occur without a family history. So, it is a consequence and according to scientists theory it happens due to disruption of blood flow to the developing foetus’s jejunum.

Types of intestinal obstructions are found by using the technique of prenatal ultrasounds. This ultrasound imaging technique indicates the level of amniotic fluid. If there is an excess of amniotic fluid found in ultrasound, then there is a chance of intestinal atresia. An intestinal failure of absorbing amniotic fluid results in excess of this fluid and is found by the ultrasound imaging technique.

If a new born is under suspect of intestinal obstruction or intestinal atresia, then the new born must go through the following diagnostic processes:

• Abdominal X-ray for Diagnosing Jejunoileal Atresia: In most of the cases, it is found as a useful way to diagnose the atresia.
• Abdominal Ultrasound for identifying Jejunoileal Atresia: It is a diagnostic technique using ultrasonography. In this technique, the clinician can view or get a clear image of the gastrointestinal (GI) organs and their functions. A gel is applied on the abdominal skin and a transducer is placed on the skin to send the ultrasound. The ultrasound then bounces off the internal organs and return back to the ultrasound machine. This gives an image on the monitor, the image of GI tracks and the image of intestine, which is then evaluated for jejunoileal atresia.
• Lower Gastrointestinal (GI) Series for Jejunoileal Atresia: This is a diagnostic process to examine the lower part of small intestine, large intestine and the rectum. The X-ray contrast agent is used to coat the inside of intestines for this diagnosis. This contrast agent is normally given into the rectum of a new-born. It allows the internal intestinal organs to be seen on an X-ray. This X-ray can show the intestinal obstructions, width of the bowel and other problems associated with the bowel movement.
• Upper GI series for Diagnosing Jejunoileal Atresia: This helps to understand the problems with upper gastrointestinal tracks, especially if there is any associated duodenal obstruction because of duodenal atresia. In this diagnostic process, liquid named barium is administered orally. When it is successfully reached into the stomach, X-rays are then able to show or find out problems with upper GI series.

Surgery is performed in most of the cases of jejunoileal atresia, but it depends upon the types of the intestinal atresia. The operations are mostly focused on removal of the intestinal blind end sections. The remaining ends are then joined or sutured. The surgery is performed in the warmed room or operating room with proper care under general anaesthesia.

Types of intestinal atresia determine the types of surgery which are as follows:

• For type 1 intestinal atresia as the web or membrane blocks the intestine, the web wall is needed to be removed by the surgery. So, the incision is done in the bowel wall to remove the web, and then the bowel wall is sutured.
• Both the location and goal of the surgery is different for the other types. As for type-2 to type-4 intestinal atresia, the atresic bowel segment is removed or resected. The anastomosis is done by joining the cut ends.

Prognosis or the long term outlook for patients with jejunoileal atresia solely depends upon how the functional bowel remains after the surgery. A patient with an inadequate length of bowel or lower GI track may develop short bowel syndrome at a later stage.

As it is a rare disease and found in 1 out of 1000 new-borns, so you may not worry about your new-born. However, it is better to be aware of it, especially about the signs and symptoms of the jejunoileal atresia as that one single case could be for your new-born or of any near and dear ones. Knowing the symptoms will help to make an early diagnosis and start the treatment or surgical procedures as soon as possible.

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