Atresia and duodenal stenosis (2023)

continuing education activity

Duodenal atresia is a congenital intestinal obstruction that can cause bilious or nonbilious vomiting within the first 24 to 38 hours of neonatal life, usually after the first oral feeding. It is associated with intrauterine polyhydramnios and is one of the most common causes of fetal intestinal obstruction. If duodenal atresia is not diagnosed prenatally, the diagnosis can be made radiographically with an abdominal radiograph as the first step in evaluation. If necessary, a controlled examination with contrast medium can be performed. This activity reviews the pathophysiology and presentation of duodenal atresia and highlights the role of the interprofessional team in management.


  • Review the presentation of a child with duodenal atresia.

  • Description of the assessment of a patient with suspected duodenal atresia.

  • Outline treatment options for duodenal atresia.

  • Explain the importance of improving coordination of care among members of the interprofessional team to improve outcomes for patients with duodenal atresia.

Access free multiple-choice questions on this topic.


Duodenal atresia is a congenital intestinal obstruction that can cause bilious or nonbilious vomiting within the first 24 to 38 hours of neonatal life, usually after the first oral feeding. It is associated with intrauterine polyhydramnios and is one of the most common causes of fetal intestinal obstruction. Prenatal ultrasound can make the diagnosis. If duodenal atresia is not diagnosed prenatally, the diagnosis can be made radiographically with an abdominal radiograph as the first step in evaluation. If necessary, a controlled examination with contrast medium can be performed. Barium can be used for a limited upper gastrointestinal (UGI) series or water/Pedialyte for an ultrasound to confirm the diagnosis. CT plays a limited role, if any, in the evaluation of duodenal atresia.[1]


Obstruction of the duodenum causes duodenal atresia, usually distal to the ampulla of Vater in the second segment of the duodenum. During the eighth to tenth week of embryonic development, duodenal recanalization defects are the main cause of duodenal atresia. In duodenal atresia, there is complete obstruction of the duodenal lumen. Duodenal stenosis is a narrowing that leads to incomplete closure of the duodenal lumen. Duodenal tissue is a less common cause of duodenal obstruction that tends to cause a windsock deformity of the duodenal lumen.[2]


Duodenal atresia occurs in 1 in 5,000 to 10,000 live births. It is often associated with other abnormalities, such as trisomy 21/Down syndrome and cardiac malformations. About 30% to 40% of children with duodenal atresia have Down syndrome. In patients with trisomy 21/Down syndrome, the prevalence of congenital duodenal atresia is 3%. There is no difference in prevalence between genders. There is an association with VACTERL, annular pancreas, and other intestinal atresias including jejunal atresia, ileal atresia, and rectal atresia.[3]


Duodenal atresia is thought to occur due to failure of the solid epithelial cord to recanalize or excessive endodermal proliferation. However, the exact etiology is so far unknown.[4]

history and body

Duodenal atresia presents early as vomiting, which usually occurs within the first 24 to 38 hours of life after the first feeding and gets progressively worse if left untreated. Vomiting can sometimes be projectile, which, like pylorospasm and gastroesophageal reflux, can mimic hypertrophic pyloric stenosis.[5][6]The clinical appearance of bilious vomiting indicates a congenital intestinal obstruction distal to the parental bleb. There are cases of atresia proximal to the ampulla of Vater that occur without bilious vomiting. The ampulla of vater is located in the second or descending part of the duodenum. Excessive bilious vomiting can cause hypokalemic hypochloremic metabolic alkalosis with paradoxical aciduria, particularly when fluid intake is delayed. Patients present with symptoms of abdominal distension and lack of bowel movement.


The prenatal image shows a double bladder, a diseased stomach filled with amniotic fluid, and a second nearby but more distal fluid-filled, often circular (but obtuse) structure (the second bladder), which is the occluded part of the duodenum. The use of prenatal ultrasound has allowed earlier diagnosis of duodenal atresia. One advantage of neonatal abdominal ultrasound is that it can be performed in the neonatal intensive care unit or nursery. When antenatal ultrasound is performed, the duodenum is usually not fluid-filled, and the presence of a fluid-filled duodenum suggests duodenal atresia. If a double bladder sign is seen on prenatal ultrasound, the sonographer must demonstrate a connection between the two fluid-filled structures because the duplicated foregut cyst, like other abdominal cysts, can mimic the appearance of a double bladder sign. bladder.[7][8]

The initial postnatal radiograph to diagnose duodenal atresia is an abdominal radiograph. In duodenal atresia, there is gas in the stomach and proximal duodenum, but no gas distally in the small or large intestine. A plain abdominal radiograph may show the double bladder sign, which is seen after birth as a large radiolucent (air-filled) stomach, usually in the normal position to the left of the midline, and a smaller bladder more distal to the midline. to the right of the midline, representing an enlarged duodenum. A double bleb sign on an abdominal radiograph is a reliable indicator of duodenal atresia. Other causes of intestinal obstruction can mimic a double bladder sign. Annular pancreas is the second most common cause of duodenal atresia. A jejunal or more distal obstruction may extend more distally or there may be more than two vesicles.[8]

In patients with Down syndrome, double bubble radiographs have a higher positive predictive value. The prevalence of duodenal atresia in Down syndrome is much higher than in the general population. Further evaluation with additional modalities is not always necessary before proceeding with surgery. Duodenal stenosis can also cause the double bladder sign; however, if there is a stricture, at least a small amount of gas may be present distal to the obstruction. The absence of gas in the stomach on the abdominal radiograph indicates esophageal atresia without tracheoesophageal fistula. Sometimes distal to a segment of duodenal atresia there may be air that has entered the distal intestine through an abnormal biliary tree. The radiographic appearance of the double blister sign should prompt immediate surgical consultation. If duodenal atresia is suspected and not detected prenatally, barium fluoroscopy can be used to evaluate the gastrointestinal tract. If necessary, additional examination with ultrasound or an upper gastrointestinal series (UGI) may be performed. More recently, neonatal ultrasonography has been very helpful in clarifying the anatomy, especially when fluid is used as a contrast medium.[9]A possible mimic of the double bladder sign is the pseudo-double bladder sign created by the curved configuration of the stomach, where the two bladders represent normal fluid in the proximal and distal stomachs.

Barium contrast is sometimes administered through an orogastric or nasogastric tube under fluoroscopy to evaluate the esophagus, stomach, and duodenum. Only a controlled amount of barium is introduced to confirm obstruction. It is then removed through a nasogastric tube to prevent reflux and possible aspiration. The main purpose of the IGU is to distinguish between duodenal atresia and midgut volvulus, an important distinction since midgut volvulus requires emergency surgery. By contrast, duodenal atresia can be treated electively.[10]

Pediatric CT plays a very limited role, if any, in the diagnosis and evaluation of duodenal atresia. However, a CT reconstruction can provide a more detailed assessment of bowel design in unclear cases. Neonatal CT is more technically challenging, as it may require sedation, bandaging, and initiation of a neonatal IV line for contrast administration. In addition, CT involves ionizing radiation and may be more difficult to interpret because there is less abdominal fat separating the abdominal viscera than in older patients.

Treatment / Management

Treatment includes nasogastric suction to decompress the stomach and surgery to correct the obstructing lesion. Duodenoduodenostomy is the typical surgery performed. A duodenodoudenostomy can be performed open or laparoscopically. Doudenodoudenostomy is a type of bypass procedure that is considered technically challenging for a laparoscopic approach. A diamond-shaped shunt is constructed with a proximal transverse duodenal shunt to the distal longitudinal duodenal shunt. The bypass procedure prevents damage to the pancreas, main pancreatic duct, pancreatic duct, and common bile duct. Before surgery, the stomach and proximal duodenum are decompressed with a nasogastric tube, and intravenous fluid resuscitation is performed. Possible complications of duodenoduodenostomy include gastroesophageal reflux, megaduodenum, and impaired duodenal motility. A complete surgical evaluation includes looking for additional areas of intestinal obstruction.[11]

differential diagnosis

Knowing whether the vomit is bilious or not is helpful in developing a differential diagnosis. The primary considerations for cases of bilious vomiting in newborns are duodenal atresia, duodenal stenosis, annular pancreas, but especially intestinal malrotation caused by midgut volvulus, in which flow from the superior mesenteric artery to the distal bowel is compromised. Midgut volvulus usually occurs days later in life than duodenal atresia. It is important to look for other gastrointestinal abnormalities associated with Down syndrome, such as: B. Anal atresia, Hirschsprung disease, diaphragmatic hernia, and omphalocele.[8]


The prognosis after successful surgical treatment of duodenal atresia is excellent. A study that followed infants from 1972 to 2001 showed late complications in up to 12% of patients and late mortality of 6%.[12]


Complications are most often associated with surgery and include:

  • megaduodeno

  • blind loop syndrome

  • cholecystitis

  • esophagitis

  • stomach ulcers

  • Gerd

  • pancreatitis

  • Anastomoseninsuffizienz

Postoperative and rehabilitation care.

Postoperatively, patients often require continuous nasogastric drainage, which may need to be accompanied by parenteral nutrition. Once the nasogastric tube has been significantly decreased or stopped, feedings can be started with a small amount and continued as tolerated. The exact time to start enteral nutrition depends on the patient. If there is question about the duodenoduodenostomy site or another anastomotic site, such as a duodenojejunal anastomosis, an upper GI series with water-soluble contrast medium can be performed.[13][14]


Because duodenal atresia requires surgical repair, a pediatric surgeon should be consulted if this condition is diagnosed.

Warning and information for the patient

Although there is no way to prevent the development of duodenal atresia, parents should be informed that this condition is related to problems in embryonic development and should not blame themselves or actions taken before or during the pregnancy for contributing to the development of duodenal atresia. to development of duodenal atresia.

Pearls and other themes

In utero, duodenal atresia, an obstruction of the proximal gastrointestinal tract, causes polyhydramnios by interfering with the gastrointestinal absorption of amniotic fluid swallowed by the fetus distal to the level of intestinal obstruction. Polyhydramnios is defined as abnormally large amounts of amniotic fluid in the amniotic sac during pregnancy. Prenatal ultrasonography is diagnostic of polyhydramnios when the amniotic fluid index is greater than 25 cm or when the total amniotic fluid is estimated to be greater than 1,500 to 2,000 mL. In duodenal atresia, amniotic fluid swallowed by the fetus is prevented from traveling distally to be absorbed by the fetal gastrointestinal tract and transported across the placenta into the maternal circulation. Instead, it is returned to the amniotic fluid. Eighty percent of polyhydramnios cases are due to maternal or idiopathic causes. Maternal causes include congestive heart failure and gestational diabetes. Gestational diabetes can cause fetal hyperglycemia and fetal polyuria. Fetal causes include congenital abnormalities affecting swallowing and intestinal obstruction, including duodenal atresia. Other abnormalities that cause polyhydramnios include esophageal atresia, head and neck masses, chest masses, and supratentorial disorders that disrupt swallowing. In addition, polyhydramnios increases the risk of umbilical cord prolapse, placental abruption, and preterm delivery.

Improve the results of the health team

Duodenal atresia is a relatively common intestinal disorder that often occurs shortly after birth; As such, it is best managed by an interprofessional team that includes a pediatric surgeon, obstetrician, neonatal nurse, nutritionist, pediatrician, intensive care physician, and geneticist. The definitive treatment for this condition is surgery, but the procedure often presents complications that lengthen hospital stays. Mortality rates from duodenal atresia have decreased significantly in the last three decades, averaging 2% to 5%. Mortality rates are not directly related to surgery but to other associated organ anomalies, such as: B. complex congenital heart defects. However, survival continues to improve with better NICU care, nutritional support, and better pediatric anesthesia. Long-term survival is currently the norm for most babies (greater than 80%) with duodenal atresia. The biggest controversy going forward is the use of endoscopy to remove the duodenal mesh. This technique is still experimental. The other controversy refers to the moment of feeding the patient after surgery. Experts recommend a fluoroscopic examination to check for leaks before starting feeding.



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What is atresia and stenosis of duodenum? ›

Duodenal atresia and duodenal stenosis are abnormalities in which there is an absence or complete closure (atresia) in the first part of the small intestines (duodenum) or narrowing (stenosis) of the duodenum. These obstructions in the digestive tract of infants prevent proper absorption of food.

What is the difference between duodenal atresia and duodenal stenosis? ›

What is the difference between duodenal atresia and duodenal stenosis? Duodenal atresia and duodenal stenosis are both congenital blockages of the small intestine. Babies with duodenal atresia have a complete closure in the first part of their duodenum. Babies with duodenal stenosis have an unusually narrow duodenum.

Can normal babies have duodenal atresia? ›

During pregnancy, your baby's duodenum normally changes from a solid form to a tube (recanalization). Duodenal atresia occurs when recanalization does not completely happen. Duodenal atresia is present in more than one in 5,000 live births.

What is the success rate of duodenal atresia surgery? ›

The survival rate of patients who undergo surgical treatment for duodenal atresia and stenosis has remarkably improved from 45% to 96% over the last 50 years due to improvements in diagnosis, neonatal intensive care, and surgical management [1].

How do you fix duodenal stenosis? ›

What is the treatment for duodenal atresia or stenosis in infants? Duodenal atresia or stenosis is treated surgically. A new connection between the upper duodenum and distal duodenum must be created to bypass the blockage.

What are the signs and symptoms of duodenal stenosis? ›

Duodenal Stenosis

Presenting symptoms include nausea and vomiting resulting from gastric outlet obstruction. Duodenal stenosis is usually a late event and occurs in patients in poor general condition who have already received a biliary endoprosthesis.

Does duodenal atresia mean Down syndrome? ›

Duodenal atresia can be an isolated condition (which means no other birth defect or condition occurs with it), but is also more common in infants with Down syndrome (trisomy 21). About one in three babies born with duodenal atresia has Down syndrome.

Can atresia be treated with surgery? ›

Surgery is an option, and in certain situations necessary, for treatment of atresia in these situations: to create an ear canal allowing for hearing restoration. replacement or alteration of one, two, or all three of the middle ear bones if inadequately formed for sound conduction.

What is the cause of duodenal atresia? ›

The cause of duodenal atresia is not known. It is thought to result from problems during an embryo's development. The duodenum does not change from a solid to a tube-like structure, as it normally would. Many infants with duodenal atresia also have Down syndrome.

At what age does duodenal atresia present? ›

Duodenal atresia presents early in life as vomiting, usually occurring within the first 24 to 38 hours of life after the first feeding, and progressively worsens if not treated.

What are the long term effects of duodenal atresia? ›

The most frequent long-term complications, after the first month, are small bowel obstructions, stenoses and incisional hernias. In duodenal atresia, adhesive bowel obstruction, incisional hernias and gastro-oesophageal reflux disease are described.

Can duodenal atresia be misdiagnosed? ›

Duodenal atresia was suspected on routine prenatal ultrasonography at 20-week gestation in 33 cases and confirmed in 29 (48%) cases with 4 false-positive diagnoses (1 normal bowel and 3 high jejunal atresias). No prenatal diagnosis was made in 32 (52%) babies.

How long does a duodenal switch surgery last? ›

The Duodenal Switch is an inpatient surgical procedure takes approximately 2 to 3 hours to complete. It begins with a Gastric Sleeve procedure in order to remove a large portion of the stomach. The stomach is divided vertically with the portion intact, shaped like a banana.

How long is the hospital stay for duodenal switch? ›

A hospital stay after a duodenal switch procedure is typically 24 to 48 hours. Many patients return to normal activity within one to two weeks.

What are the complications of duodenal atresia surgery? ›

Late complications include blind-loop syndrome, megaduodenum with altered duodenal motility, gastritis with duodenal-gastric reflux, peptic ulcer, esophagitis and gastroesophageal reflux, pancreatitis, and cholecystitis.

Can duodenal atresia be cured? ›

Duodenal atresia can only be treated with surgery to repair the connection between the stomach and the intestines. Surgery is usually done 1-3 days after birth. It is important to create a plan with doctors to make sure you and your baby get the care you need before and after birth.

How rare is duodenal atresia? ›

Duodenal atresia occurs in 1 in 5,000 live births and is the most common cause of fetal bowel obstruction.

What is the recurrence risk for duodenal atresia? ›

Recurrence: Isolated: no increased risk of recurrence. Part of trisomy 21: 1%.

What is the most common type of duodenal atresia? ›

Type 1: the most common form of duodenal atresia, making up 92% of cases. A web made of mucosa and submucosa obstructs the duodenal lumen however there are no defects in the muscle coat. Type 2: rarest form of duodenal atresia, making up 1% of cases.

Where do you feel duodenal pain? ›

The most common ulcer symptom is a dull or burning pain in your belly between your breastbone and your belly button (navel). This pain often occurs around meal times and may wake you up at night.

What diseases are associated with duodenal atresia? ›

Duodenal atresia is associated with Down's syndrome (30%), annular pancreas (23%), congenital heart disease (22%), malrotation (20%), oesophageal atresia (8%), others (20%) [5]. The mainstay of treatment is surgical intervention. Duodenoduodenostomy is most frequently performed operation.

What is the most common genetic abnormality in a Down syndrome baby? ›

Trisomy 21: About 95% of people with Down syndrome have Trisomy 21. With this type of Down syndrome, each cell in the body has 3 separate copies of chromosome 21 instead of the usual 2 copies.

What happens if Kasai procedure fails? ›

The only effective treatment for 'failing' Kasai portoenterostomy is liver transplantation (LT). However, to maximise a patient's chances to achieve the proclaimed >95% survival with sequential surgical management, medical follow-up and treatment must be planned carefully.

Which atresia is most common? ›

Jejunoileal atresia: The most common type of neonatal intestinal obstruction, jejunoileal atresia occurs in 1 in 1,000 to 1 in 3,000 live births.

How do you fix an atresia? ›

Small bowel atresia is repaired in an operation under general anaesthetic which lasts around two hours. The operation to repair the atresia can is usually carried out using open surgery. Sometimes a laparoscopic (keyhole) procedure may help diagnose the problem so that it can be repaired using open surgery.

What is the mortality in duodenal atresia? ›

Background: Duodenal atresia and stenosis is a frequent cause of congenital, intestinal obstruction. Current operative techniques and contemporary neonatal critical care result in a 5% morbidity and mortality rate, with late complications not uncommon, but unknown to short-term follow-up.

Can babies with duodenal atresia pass meconium? ›

Some infants with duodenal atresia may pass meconium. Patients can also have poor feeding, failure-to-thrive, be small for gestational age, or premature. Excessive vomiting can cause hypokalemic hypochloremic metabolic alkalosis. Patients can have symptoms of abdominal distension and absent bowel movements.

Which is better duodenal switch or bypass? ›

Statistically, the duodenal switch has the best overall weight loss results compared to the gastric bypass and the gastric sleeve. On average, patients who have the duodenal switch can expect to lose 80 to 100% of their excess weight.

Can you eat normally after a duodenal switch? ›

For 5-7 days after your surgery, you will need to follow a clear liquid diet. The purpose of this phase is to allow for healing time and to stay hydrated. Fluids will not stretch your stomach. Fluids may cause discomfort or pain in the first couple of days, so it is important to go very slowly.

Does insurance cover duodenal switch? ›

Will my medical insurance cover the cost of duodenal switch surgery? A. Generally speaking, most insurance policies who offer bariatric surgery benefit will also cover duodenal switch procedure.

How much of the stomach is removed for a duodenal switch? ›

In duodenal switch, the surgeon removes 80% of the stomach, similar to the gastric sleeve procedure. It bypasses a portion of the small intestine, like the gastric bypass.

Why do I poop so much after duodenal switch surgery? ›

It can take time for your intestines to adapt, so bowel movements can be very liquid and frequent after the procedure. This condition may lessen over time, but may be permanent.

Is duodenal switch surgery painful? ›

Immediately after this weight loss surgery, there will be a normal amount of swelling and pain around the incision site. Some less common duodenal switch risks are infection and obstructions. Long term complications can vary from patient to patient.

What is atresia and stenosis? ›

What are atresia and stenosis? Atresia and stenosis are birth defects in which the esophagus, stomach or intestines do not develop properly. The defects cause blockages in the digestive tract. An atresia leads to a complete blockage, while a stenosis causes a partial blockage.

What is gastrointestinal atresia or stenosis? ›

What is Intestinal Atresia and Stenosis? Intestinal atresia is a broad term used to describe a complete blockage or obstruction anywhere in the intestine. Stenosis refers to a partial obstruction that results in a narrowing of the opening (lumen) of the intestine.

What is the treatment for atresia? ›

Treatment. Doctors treat biliary atresia with a surgery called the Kasai procedure and eventually, in most cases, a liver transplant. Thanks to advances in treatment, more than 80 to 90 percent of infants with biliary atresia survive to adulthood.

What age does duodenal atresia present? ›

Duodenal atresia presents early in life as vomiting, usually occurring within the first 24 to 38 hours of life after the first feeding, and progressively worsens if not treated.

What are complications of duodenal atresia? ›

Late complications include blind-loop syndrome, megaduodenum with altered duodenal motility, gastritis with duodenal-gastric reflux, peptic ulcer, esophagitis and gastroesophageal reflux, pancreatitis, and cholecystitis.

What heart defect is associated with duodenal atresia? ›

The association between duodenal atresia, intestinal malrotation, cardiac anomalies and Down syndrome is infrequently reported. We present a prenatally suspected case of duodenal atresia which was associated with malrotation and atrial septal defect in a patient of Down syndrome.

What are the symptoms of duodenal atresia? ›

Symptoms of duodenal atresia include:
  • Upper abdominal swelling (sometimes)
  • Early vomiting of large amounts, which may be greenish (containing bile)
  • Continued vomiting even when infant has not been fed for several hours.
  • No bowel movements after first few meconium stools.
Apr 14, 2021

What causes stenosis of the bowel? ›

Experts believe that intestinal atresia and stenosis are caused by an inadequate supply of blood to your baby's intestines during fetal development. They appear to run in families, although a specific genetic cause has yet to be discovered.


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