Duodenal atresia is one of the most common inborn defects of the digestive system namely of the intestine that is often associated with other congenital malformations and genetic pathologies (21 trisomy). The frequency of occurrence of this disorder is affected by the hypoxia of fetus in the first trimester of pregnancy, caused by severe chronic respiratory and cardiovascular diseases, stress, diabetes, drug and alcohol abuse during pregnancy. Nowadays, duodenal atresia is one of the most common congenital defects in the neonates, moreover, well-diagnosed and curable in most of the cases if not neglected and timely detected.

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radiograph with double bubble sign

Image: “Frontal view of the abdomen with double bubble sign, patient was found to have duodenal atresia.” by JasonRobertYoungMD – Own work. License: CC BY-SA 4.0


Definition and Background

Duodenal atresia is a congenital defect of the digestive system in newborn babies stipulated by the partial narrowing (stenosis) or absence of the lumen of the duodenum (the first part of the intestine), which makes absorption and the passage of food through the digestive canal impossible causing duodenal obstruction.

Duodenal stenosis is associated with variable and delayed manifestation followed by concealed symptomatic at the beginning and lasts up to adulthood. The disorder is associated in most of the cases with other inborn defects (52-78%). It is usually diagnosed within the first hours of life.

Etiology of Duodenal Atresia

There is no precise theory about the causative factor for the occurrence of congenital duodenal obstruction as the cases are mostly sporadic. It is suggested that there is an autosomal recessive trait regarding the inheritance of the defect.

28% of cases of duodenal atresia were diagnosed while ultrasound examination as early as 20 weeks gestation, later the defect may become more evident. There is often an excess of amniotic fluid (polyhydramnios).

The malformation in the form of duodenal atresia may develop due to the failure of intrauterine recanalization of the bowel lumen, which is initially a solid tube (11-12 weeks gestation). The reason for this defect is hypoxia of the fetus at the early stages of embryogenesis (ischemic episode).

Epidemiology of Duodenal Atresia

Duodenal atresia is regarded a rare disease; the frequency of its occurrence is from 1 of 7,500 live births to 1 of 40,000 live birth.

One-half of the newborn babies with duodenal atresia are delivered prematurely.

There is a pattern for intrinsic inborn duodenal obstructions:

  • Duodenal atresia, 40-60%;
  • Duodenal web, 35-45%;
  • Annular pancreas, 10-30%;
  • Duodenal stenosis, 7-20%)

Internationally

Finland occupies a leading place in the rate of incidence, which makes up 1 to 3,400 cases. This defect is equally common between the two genders similarly affecting boys and girls worldwide.

The US statistics for the cases of duodenal atresia states that the occurrence of this malformation is 1 per 6,000 births.

There is no any racial propensity.

Presentation of Patients with Duodenal Atresia

History

duodenal atresia

Image: “Duodenal atresia in neonate.” by Kinderradiologie Olgahospital Klinikum Stuttgart – Own work. License: CC BY-SA 3.0

Complete duodenal atresia:

  • Bilious vomiting occurs during the first hours of life (85%). Congenital defects of the intestine associated with bilious vomiting and the time of manifestation:
    • Duodenal atresia, a few hours after birth, no distention;
  • Malrotation with volvulus, at 3 to 7 days, rapid deterioration with volvulus;
    • Jejunoileal atresia, during the first hours of birth, abdominal distention;
  • Age: newborn babies
  • The color of the vomits is yellow-green or clear; sometimes there is brown granular matter
  • Absence of bowel movements
  • Absence of urination after several voiding
  • Swollen upper abdomen (Scaphoid abdomen)
  • Jaundice of the skin
  • Unresponsive to treatment constipations
  • Meconium passage is not affected during the first 24 hours after birth.

Partial duodenal atresia (stenosis) symptoms depend on the severity of the defect and the illness may not show up any symptoms for months and years and usually manifests with long-last vomiting and dehydration if the electrolyte balance is not maintained appropriately and timely.

  • Nonbilious vomiting occurs in those cases when the defect allocates above the papilla of Vater
  • Recurrent episodes of vomiting
  • Food aspiration
  • Failure to thrive

Associated with duodenal atresia congenital defects:

  • Down syndrome
  • VACTERL association
  • Annular pancreas
  • Anal atresia
  • Ileal atresia
  • Jejunoileal atresia

Physical examination

In the case of duodenal atresia, careful examination of anus has to be conducted in order to exclude the possibility of atresia.

Measurement of gastric aspiration: normally it does not exceed 5 ml, duodenal atresia is suspected when an infant’s gastric aspiration is larger than 30 ml. Fullness of epigastrium may be detected manually and visually, when the food gets stacked above the narrowed lumen.

In adults: peptic ulceration, gastroesophageal reflux, stenosis by bezoar.

Differential Diagnosis of Duodenal Atresia

Atresia Anal

Esophageal

Small bowel

Plain radiograph appearance Duodenal stenosis

Duodenal web

Intestinal malrotation

Midgut volvulus

Ultrasound appearance of a “double bubble type” (distal gas) Fetal choledochal cyst

Fetal omental cyst

Fetal duplicated cyst

Diagnosis of Duodenal Atresia

Laboratory studies

  • Serum electrolytes have to be determined as the infants with duodenal atresia tend to lose fluids and electrolytes produced by the stomach due to a large amount of gastric aspiration.
  • Hematocrit has to be checked prior to general anesthesia.
  • Blood glucose may be low in premature babies; they tend to develop hypoglycemia as there is a limited supply of glycogen.
  • Blood type and cross match.
  • Karyotype analysis is the appropriate method of examination in the case of trisomy 21.
  • Rectal biopsy conducted in order to exclude Hirschsprung disease especially in the patients with Down syndrome.
  • Immunohistochemistry of proximal and distal duodenal samples of affected infants reflects changes and transformation of neuronal cells, intestinal cell Cajai, hypertrophy of circular muscles, which witness about the persistent disorder of the duodenal motoric after primary surgery.

Imaging studies

duodenal atresia ultrasonography

Image: “Duodenal atresia neonate ultrasound double bubble.” by Kinderradiologie Olgahospital Klinikum Stuttgart – Own work. License: CC BY-SA 3.0

Radiography is an informative method of examination in duodenal atresia, which finds dilated stomach and the first part of the duodenum (“double bubble”), and absence of air behind the second bubble.

Ultrasonography is efficient in the intrauterine diagnostic of duodenal atresia, apart from the specific appearance of the abdomen; there is polyhydramnios (excess of amniotic fluid, 40% of cases), which is a prominent symptom of the defect. This method is preferred in esophageal and duodenal atresia.

Barium enema examination is an additional study that makes distant duodenal atresia as well as malpositioned cecum evident. However, in the diagnostics of malrotation and volvulus barium enema is not that informative.

Management of Duodenal Atresia

There is no conservative treatment available for this condition.

Surgical intervention

Type of duodenal obstruction Preoperative management Surgical treatment Postoperative period Prognosis
Duodenal atresia IV fluids hours and nasogastric suction 24-48 hours Diamond-shaped duodenoduodenostomy No feeding per os for 2-4 days after operation; nasogastric suction. Benign, if there are no serious associated congenital defects
Jejunoileal atresia IV fluids, STAT surgery for the patients with obvious symptoms; nasogastric suction Ladd’s procedure: sometimes a second laparotomy is required No feeding per os; nasogastric suction Benign, if there is no large bowel resection
Jejunoileal atresia IV fluids and nasogastric suction for 24-48 hours Resection and anastomosis No feeding per os for 2-4 days after operation; nasogastric suction. Benign, if there is no large loss of the bowel

In spite of the benign prognosis after the surgery, 22% of babies may develop distant complications:

  • Blind-loop syndrome
  • Megaduodenum with altered duodenal motility
  • Gastritis with duodenal-gastric reflux
  • Peptic ulcer
  • Esophagitis and gastroesophageal reflux
  • Pancreatitis
  • Cholecystitis
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