Definition

Duodenal atresia is a congenital anomaly characterized by incomplete development of the duodenum. This anomaly inhibits the passage of food into the subsequent segment of the small intestine. This condition has a prevalence rate of 1 in every 5,000–10,000 live births. Approximately 25–40% of cases develop among infants diagnosed with Down syndrome.

Causes

Complications during fetal gastrointestinal development are the cause of duodenal atresia. The morphology of the small intestine will transform from a solid tube to a tube with channels between the eighth and tenth weeks of pregnancy. However, cases of duodenal atresia result in the complete closure of the duodenum due to incomplete protrusion of the small intestine. Duodenal stenosis is when food may pass through a minor space in the duodenum despite its constriction. Nevertheless, the specific cause of duodenal atresia remains unknown.

Risk Factor

Duodenal atresia is not hereditary. Typically, duodenal atresia is correlated with other medical conditions, such as:

• Down Syndrome
  Approximately 25–40% of duodenal atresia cases occur in infants with Down syndrome. Conversely, duodenal atresia is observed in 3% of infants diagnosed with Down syndrome per 100.

• VACTERL
  If you were born with VACTERL, you would have many structural problems with your body, such as problems with your spine, your anus not forming correctly, your trachea and esophagus not connecting, your kidneys not working properly, and your limbs not working properly.

• Malformations of the pancreas
  A manifestation of pancreatic malformations that may impact the duodenum is the development of a pancreatic ring, which encircles the small intestine and is identical to a finger ring.

• Intestinal obstruction
  Multiple intestinal organs that could develop abnormalities are the rectum, the absorptive intestine (ileum atresia), and the large intestine (jejunal atresia).

Symptoms

Duodenal atresia can be identified immediately after birth, particularly within 24-38 hours. Common manifestations include:

• Vomiting
  Vomiting typically manifests during the initial administration of breast milk and intensifies with consecutive breast feedings. Vomiting can manifest as the forceful expulsion of stomach contents, which may appear as a milky white or greenish color due to the presence of bile. The combination of bile and vomit may lead to electrolyte disturbances if prompt fluid administration is not administered.

• Distended and tense abdomen

• Absence of bowel movement

Diagnosis

Duodenal atresia can be diagnosed prenatally or postnatally.

Prenatal
Generally, an obstetrician-gynecologist may perform an ultrasound (USG) as a pregnancy test. An ultrasound can detect two amniotic fluid-filled cavities in the uterus. Preoperative identification of duodenal atresia promotes planning for the surgical intervention.

Postnatal

1. Ultrasonography, also known as USG.
   A postnatal ultrasound can also be performed to assess the baby's condition.
   
   
2. X-ray imaging
   X-ray imaging is recommended for the first diagnosis of duodenal atresia. X-rays can reveal the presence of two air-filled cavities within the infant's gastric region. Additionally, contrast fluid administered via a feeding tube can assist in X-ray examination to distinguish duodenal atresia from other intestinal disorders.
   
   
3. Laboratory examinations
   Laboratory examination. Early detection of the condition eliminates the need for laboratory testing. Nevertheless, monitoring the baby's electrolyte levels and blood fluid is necessary if he or she vomits green, seems pale, and is very thirsty.
   
   
4. Genomic analysis
   A chromosomal analysis may also be performed if Down syndrome in the infant is suspected. In infants with Down syndrome, chromosome examination reveals an extra chromosome on chromosome 21.

Management

Preoperative management of infants with duodenal atresia consists of the following:

1. Insertion of a gastrostomy tube or nasogastric tube. The insertion of a nasogastric tube will be performed to alleviate gastric pressure, mainly if the infant appears satiated.
   
   
2. Fluids and electrolytes will also be administered. Due to the child's impaired digestion, intravenous feeding will be administered as necessary
   

Surgery is the primary therapeutic approach for duodenal atresia. The surgical operation in question is referred to as a duodenoduodenostomy, which involves the connection of the obstructed portion of the duodenum to the adjacent open section of the small intestine. There are multiple methods available for doing this procedure, specifically:

• A laparotomy is a type of surgical procedure that involves making an incision in the abdominal wall
• Minor incision surgery (laparoscopy). This procedure is conducted with a laparoscope, an instrument equipped with a camera at its tip, enabling surgery without needing a big incision.

This treatment is obligatory; however, it is not an urgent procedure. If any congenital conditions may pose a risk to the baby's health, such as congenital heart disease, these conditions can be prioritized for treatment or carefully monitored.

The baby will be warm throughout the surgery. IV feeding will be provided until the infant can defecate after surgery. After that, oral breast milk can take the place of IV feeding. After surgery, infants may stay in the hospital for several days to observe their condition.

After two weeks, wound healing, nutritional sufficiency, and digestive function are evaluated. Following that, the baby will be monitored annually to prevent further complications.

Complications

The initial complications associated with duodenal atresia include:

• Milk-induced dehydration results from the body's inability to digest the milk
• Gall bladder inflammation, often known as cholecystitis
• Esophagitis refers to the inflammation of the esophagus
• Gastric ulcer disease refers to a medical condition characterized by the presence of ulcers in the stomach
• Gastroesophageal reflux disease (GERD) refers to regurgitating food from the stomach back into the esophagus
• Pancreatitis is the inflammation of the pancreas, the gland responsible for producing digesting enzymes
• Mortality. The mortality rate associated with duodenal atresia has significantly declined over the past five decades, with fatalities primarily attributed to concurrent life-threatening conditions, such as cardiac abnormalities

The surgical procedure used for attaching the small intestine might also cause complications. Possible complications may consist of:

• Megaduodenum
  Megaduodenum refers to an abnormally enlarged duodenum, which is the first part of the small intestine. A hypertrophic duodenum characterizes this syndrome. Treatment for abnormalities like megaduodenum involves the surgical removal of excess duodenal tissue using clips.

• Blind loop syndrome
  This syndrome disrupts the normal digestive pathway for food.

• Intestinal junction leakage

• Gastrointestinal motility disorders
  Food cannot be digested effectively, which leads to adverse outcomes.

Hence, the implementation of post-operative control is critical. This is performed in anticipation of potential complications.

Prevention

The etiology of duodenal atresia remains uncertain. However, anticipatory precautions can be taken throughout pregnancy. Regular prenatal monitoring, along with an ultrasound scan, will enhance the understanding of both parents and doctors in cases where there is a suspicion of a fetal congenital anomaly. By obtaining early knowledge, one can effectively strategize and prepare for both birth preparation and newborn care before the actual birth.

When to see a doctor?

If your one-day-old won't or can't swallow breast milk, it may have problems with digestion. Vomiting breast milk or green vomit in the first 24-38 hours after birth increases the risk of duodenal atresia. Immediately take your baby to the doctor if concerns arise. Another warning indicator is if the infant does not urinate or defecate in the first 24 hours.

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