Jejunoileal Atresia- Types, Symptoms, Diagnosis and Treatment

Introduction

Jejunoileal atresia is the stenosis or obstruction of the intestines causing narrowing or closure of the structure in newborns. The condition is life-threatening as it interferes with the baby's normal intestinal function and feeding. Intestinal atresia usually affects the intestines but can cause obstruction or atresia in any part of the gastrointestinal tract. Therefore, the symptoms, occurrence, diagnosis, and treatment of jejunoileal atresia mainly depend upon the infected site. The condition is prevalent in females and males but can occur only in one in every 1500 live births.

What Are the Symptoms of Jejunoileal Atresia?

The symptoms are jejunoileal atresia are as follows:

• Abdominal swelling.

• Difficulty in feeding.

• Reduced bowel movement.

• Vomiting bile fluid.

What Are the Types of Jejunoileal Atresia?

The different types of jejunoileal atresia are as follows:

• Intestinal Atresia Type I - In this type of atresia, a web-like membrane is formed inside the intestine when the baby is developing in the uterus. The condition causes blockage in the intestine by the membrane formation, but the intestines' length remains unaffected.

• Intestinal Atresia Type II - In this case, the length of the intestine is regular. Still, the dilated section develops a blind end connected to the smaller segment of the intestine via scar tissue.

• Intestinal Atresia Type III - In this type, a flaw or defect in the intestinal blood supply separates the two bling ends. The condition significantly reduces the length of the small intestine, which further causes short-gut syndrome or long-term nutritional deficiencies.

• Intestinal Atresia Type IV - In this type, the intestine length is significantly reduced due to multiple blockages in the intestinal sections.

What Are the Causes of Jejunoileal Atresia?

Jejunooileal atresia occurs due to the following reasons:

• An inherited genetic defect such as familial multiple intestinal atresias.

• Vascular accidents cause the insufficient blood supply to the developing fetus’s intestine. This defect causes loss of functionality due to ischemia in the different segments of the small intestine.

• Some researchers have also linked cocaine and cigarette smoking during pregnancy to jejunoileal atresia.

How Is Jejunoileal Atresia Diagnosed?

Jejunoileal Artesia is diagnosed in the following ways:

1. Prenatal Diagnosis - This type of diagnosis is done mainly during the third trimester of the pregnancy with the help of prenatal ultrasound. Prenatal diagnosis of a mother is performed due to the high risk of developing congenital disabilities in newborns with intestinal atresia to eliminate the following conditions:

• Esophageal atresia.

• Congenital heart diseases.

• Down syndrome.

• Annular pancreas (pancreas does not form properly due to a congenital disability).

• Genitourinary malformations.

• Bowel rotational abnormalities.

2. Postnatal Diagnosis - This is the diagnosis done after the birth of a baby. Newborns with jejunoileal atresia vomit frequently; therefore, some specific diagnostic procedures rule out congenital disabilities. These include:

• Lower Gastrointestinal (GI) X-Ray - It helps to investigate the small intestine, large intestine, and rectum. In the procedure, a radio-opaque contrast media is administered through the rectum to form a thin lining around the intestine. This helps in the clear view of the internal body structures with minute details of obstruction and strictures.

• Upper Gastrointestinal (GI) X-Ray - The diagnosis helps to investigate the duodenum and pylorus by using barium as a contrast medium, which is given through the mouth for a clear view of the structures inside the body.

• Abdominal X-Ray - This helps to take images of the internal structure of the newborn’s abdomen for diagnosis.

• Echocardiogram (ECG) - It helps to detect any congenital heart defects in the newborn. In babies, jejunoileal or intestinal atresia conditions can be fatal, leading to complications such as congenital heart defects.

• Abdominal Ultrasound - This non-invasion diagnostic procedure uses sound waves to view the functionality of the internal organs. Abdominal ultrasound or USG detects the abnormalities in the abdomen related to jejunoileal atresia symptoms.

How Is Jejunoileal Atresia Treated?

Jejunoileal atresia does not have any prenatal treatment options available. The doctor can treat the condition in newborns only with surgical intervention after birth. The surgery depends upon the location and nature of the jejunoileal atresia or blockage. Thus, the treatment is carried out in the following ways:

1. Stabilization of the Baby

• A nasogastric tube is inserted into the stomach through the nose to empty the accumulated gases and stomach contents under negative pressure to prevent aspiration and vomiting in the baby.

• Intravenous nutritional supply is provided to the newborn.

• Also, some blood tests and X-ray procedures are done during this pre-surgical phase of the treatment procedure.

2. Surgery

• The surgery is mainly laparoscopic or open surgery. In a laparoscopic procedure, multiple small incisions are given along the intestine to insert instruments and a camera. While in open surgery, a single large incision is made in the abdomen, and the affected part of the intestine is removed. Then, the ends are sutured again to maintain continuity.

• The surgery is performed under general anesthesia to avoid discomfort or pain for the baby.

• Sometimes, a doctor creates an opening or a stoma in the abdomen if the intestines take longer to heal. The stoma helps to collect the feces temporarily in the colostomy bag until it passes through the anus.

3. Post-Surgical Care - The baby’s stay can be up to one to three weeks post-surgery to monitor the vitals continuously. Also, the nutritional supply is continued with a nasogastric tube unless the baby starts taking food through the oral cavity or mouth. Post-surgical follow-ups are scheduled after discharge to assess a baby's general well-being and growth parameters.

What Is the Differential Diagnosis for Jejunoileal Atresia?

• Internal hernia.

• Colonic atresia.

• Meconium ileus.

• Congenital small left colon syndrome.

• Intestinal malrotation with midgut volvulus.

• Hirschsprung disease.

What Are the Complications Associated With Jejunoileal Atresia?

• Postoperative sepsis can occur in 5 % to 8 % of cases.

• An anatomic leak occurs in 5 % to 7 % of cases.

• Short bowel syndrome (SBS) occurs due to feeding abnormalities, prolonged hospital stays, and increased infection rates.

• Adhesive bowel syndrome.

Conclusion

Jejunoileal atresia is a rare condition of intestinal obstruction in newborns, which can be fatal in some cases if not detected initially. However, advanced surgical techniques, nutritional support, pediatric anesthesia, intensive care units, and post-operative care for neonates have increased the survival rates in newborns with intestinal atresia.