What is Intestinal Atresia?

It is the failure of the intestines to develop continuity during the baby's development in the womb. Duodenal (duodenum) atresia, jejuno-ileal atresia (2nd and 3rd parts of the small intestine) and colonic (large intestine) atresia are evaluated under this heading. The causes of jejuno-ileal obstruction will be evaluated in this article.

What is Jejuno-ileal Obstruction in Newborns and Infants?

It is the failure of the jejunum and ileum to develop continuity during the baby's development in the womb.

What are the Theories of the Formation of Jejuno-ileal Atresia?

There are a number of theories explaining the formation of jejuno-ileal atresia, but the exact cause is unknown. The theory that is still accepted today is the recanalization theory. According to this theory, all intestines are lumenless during the development phase in the womb, but later become hollow lumens. Any problem at this stage causes jejuno-ileal atresia.

Other theories on this subject are:  the intestines turning around themselves in the womb (volvulus), intestinal knotting, internal hernia, injury to the vessels feeding the intestines for any reason in the womb.

Who is jejuno-ileal atresia more common in and how often is it seen?

Jejuno-ileal atresia is seen equally in boys and girls. It is seen in approximately 1 in 5000 live births.

What are the additional anomalies that can be seen in jejuno-ileal atresia?

The rate of additional anomalies in jejuno-ileal atresia is low. Babies are generally normal except for atresia. However, around 10% may be accompanied by extra-abdominal anomalies, which are rarely; may be esophageal, gastric, duodenal, colonic, rectal atresia, biliary atresia, meningomyelocele, cystic fibrosis and Hirschsprung disease.

Are There Types of Jejuno-ileal Atresia?

Yes, there are. Although different classifications have been made, the most commonly used classification is as follows.

Type 1; There is a diaphragm or membrane in the diaphragmatic lumen, when viewed from the outside, the intestinal integrity is not disrupted. This structure is called a web and is also called Windosock anomaly in appearance. There is no problem with the length of the intestine.

Windsock anomaly - It is a type of Type 1 atresia. In this anomaly, the web that obstructs the intestinal lumen prolapses more distal to the intestinal lumen. When viewed from the outside, atresia appears to be more distal to the intestine because it prolapses more distal to the place where it is attached to the intestinal wall that obstructs the intestinal lumen. However, when looked at carefully, the area where the web attaches to the intestinal wall can be noticed in some cases.

Type 2; Atresic blind ends are connected to each other with a fibrous band, there is no defect in the intestinal meso. The lumen integrity is also impaired.

Type 3 a-; Atresic ends are separate from each other, intestinal meso is defective. The proximal part ends blindly without connection to the distal part, there is a V-shaped defect in the mesentery, there is excessive dilatation in the proximal, there may be impaired circulation, perforation. The total intestinal length is usually short.

Type 3 b- “apple peel”, “Christmas tree”-; Proximal jejunal atresia close to Treitz, absence of dorsal mesentery, significant shortening in total intestinal length, large mesenteric defect, spiral decompressed distal intestine around a single feeding vessel. The distal segment is retrogradely fed by the ileocolic, right colic artery. The recurrence rate in siblings is 15-18%.

Type 4-; Multiple atresias and a combination of Type 1 to Type 3 atresias. They constitute 25-35% of jejunoileal atresias.

 

Can Jejunoileal Atresia Be Diagnosed Antenatally (In the Womb)?

A definitive diagnosis cannot be made, but there may be suspicious findings, which are; polyhydramnios (excessive uterine fluid in the mother), dilation of the baby's intestinal lumen and hyperechoic intestinal appearance.

How is Jejunoileal Atresia Definitively Diagnosed, What Are Its Clinical Findings?

A definitive diagnosis is made after the baby is born with the history and physical examination. The diagnosis is confirmed with imaging methods. In the history, ultrasound findings performed in the womb should be questioned, when vomiting and abdominal distension started in the baby after birth, whether there are any additional problems, and the type of vomiting should be questioned.

In the physical examination, the abdomen may be swollen from medium to severe depending on the level of atresia.

Normally, the amount of stomach content aspirate in the newborn is around 5 mm, and in these patients, aspiration of more than 20 cc of stomach content when a nasogastric tube is placed is a warning for intestinal atresia.

Imaging Methods; In a plain abdominal radiograph taken in an upright position, there are several to many air-fluid levels related to the level of atresia. The proximal (above) part of the atresia is dilated. No gas is seen distally (below) the atresia.

 

Images: X-ray images of intestinal atresia

However, sometimes there may be gas in the distal area, in which case incompletely obstructed atresia or other diseases in the differential diagnosis should be considered. For this, barium gastric duodenum radiography can be taken.

What is Included in the Differential Diagnosis of Jejunoileal Atresia?

Jejuno-ileal atresia can be confused with many diseases that require and do not require surgery. For this, the following diseases should definitely be considered; intrinsic intestinal obstruction (meconium ileus), extrinsic intestinal obstruction (midgut volvulus, internal hernia), colonic atresia, total colonic aganglionosis, non-mechanical ileus (sepsis, maternal drugs, prematurity, hypothyroidism)

What are the risks of Jejunoileal Atresia when it is not treated?

It can result in fluid electrolyte loss, intestinal perforation, sepsis and death.

How is Jejuno-ileal Atresia Treated?

The definitive treatment for jejuno-ileal atresia is surgical correction. However, there are certain treatment steps that need to be taken into consideration and performed before surgery to reduce the risk of mortality and morbidity. These are; to prevent the baby from vomiting and the stomach contents from entering the lungs (aspiration), for this reason the patient is not fed and a nasogastric tube is inserted. Serum treatment is started to replace the patient's fluid and electrolyte losses. Antibiotics are started, necessary precautions are taken to prevent the baby from hypothermia, and a urinary catheter is inserted if there is excessive fluid loss.

Operative approach to jejuno-ileal atresia:

The definitive treatment for jejuno-ileal atresia is surgery, as mentioned above. The method may differ depending on the type of atresia. Multiple atresias and Apple Peel may require multiple surgeries.

How is Jejunoileal Atresia Follow-up Done After Surgery?

The treatment process can take 5-10 days, sometimes longer. Fluid electrolyte support is continued, sometimes total parenteral nutrition may be required, and after the color of the content coming from the nasogastric tube has lightened and its amount has decreased, patients can be started to be fed orally. It can sometimes take up to 3 weeks to start oral feeding. The patient who is fed completely orally is discharged if there are no additional complaints. In this case, the presence of additional anomalies or failure of the surgery may be considered and medicated radiographs may be taken.

What are the Risks and Complications of Jejuno-ileal Atresia Surgery?

Since the patient will receive anesthesia, there may be risks and complications (such as respiratory distress, heart problems), and in the surgical procedure, a number of problems such as anastomosis leakage, stenosis, bleeding, intra-abdominal infection, wound infection may be encountered. In addition, anastomosis stenosis may also be seen in the late period.

In jejuno-ileal atresia, the prognosis depends on the accompanying additional anomalies and the type of atresia. If there is no additional anomaly and it is isolated atresia, the survival rate is around 95%. However, if there is an additional anomaly and Jejuno-ileal and/or multiple atresias and Apple Peel atresias, survival may be much lower because the length of the intestine is also short in these cases.

Are There Factors Determining Postoperative Prognosis in Jejuno-ileal Atresia?

Yes, there are.

• It depends on the localization of atresia (ileum adapts better than jejunum),
• The maturity of the intestine (time is needed for the intestine to mature in premature babies),
• The length of the small intestine,
• The presence of the ileocecal valve.

Are There Late-Term Problems When Surgery is Successful?

When surgery is successful, late-term problems are generally not encountered. However, as in every patient who has abdominal surgery, intestinal adhesion may occur. In such a case, bilious (yellow) vomiting, abdominal distension, and abdominal pain occur. Intestinal adhesion (Birid Ileus) should be considered when these findings are encountered in every patient who has abdominal surgery. In such cases, surgery may sometimes be required.

*** The information provided here and the content of the website are arranged for the purpose of informing visitors, especially families. No information should be considered as advice by visitors and should not lead to any decision or action. The patient should definitely be examined by a pediatric surgeon, and a decision should be made by consulting him/her and consulting his/her personal knowledge.