Pediatric Duodenal Atresia and Stenosis Surgery Clinical Presentation

Updated: May 09, 2018
  • Author: Nicola Lewis, MBBS, FRCS, FRCS(Paed Surg); Chief Editor: Eugene S Kim, MD, FACS, FAAP  more...
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Presentation

History and Physical Examination

In 38-55% of patients, intrinsic duodenal obstruction is associated with another significant congenital anomaly. [1, 11, 12, 13] Approximately 30% of cases are associated with Down syndrome, and 23-34% of cases are associated with isolated cardiac defects. Esophageal atresia may be present in 7-12% of patients. [14] Other gastrointestinal anomalies include the following:

  • Malrotation
  • Anterior portal vein
  • Second distal web
  • Anorectal anomalies [15]
  • Intestinal atresias
  • Cloacal anomalies
  • Renal tract anomalies

Duodenal atresia is associated with prematurity and low birth weight. [16, 12] Rarely, duodenal atresia is seen as a part of Feingold syndrome. [17]

Duodenal atresia

Duodenal atresia is detected antenatally in 32-57% of patients. [11, 18] Sonographic features of high intestinal obstruction (ie, duodenal obstruction with a dilated stomach [double-bubble sign]) become apparent in the third trimester. Polyhydramnios develops in 32-59% of cases; in the presence of polyhydramnios, normal findings on ultrasonography of the fetus do not exclude duodenal atresia. [19, 11, 5] A similar appearance can be observed in fetuses with a choledochal cyst, external duodenal compression, and a normal stomach with a sharp incisura. Approximately 80% of cases are diagnosed antenatally, with confirmation following delivery. [18]

Antenatal diagnosis of duodenal atresia should lead to a search for other associated anomalies and amniocentesis for karyotype analysis.

After delivery, a thorough physical examination should be performed, including careful examination of the anus.

Healthy newborn infants have gastric aspirates that measure less than 5 mL. Congenital intestinal obstruction is associated with gastric aspirates that measure more than 30 mL. [20] An infant with a gastric aspirate larger than 30 mL in the delivery room or newborn nursery should be evaluated for duodenal atresia and other causes of upper intestinal obstruction.

Symptoms of upper intestinal obstruction commence within the first 24 hours after birth. However, patients may present hours or days after delivery. Sustained vomiting (bilious or nonbilious) is the most common symptom, occurring in approximately 85% of cases. [2, 1, 3] Nonbilious vomiting occurs when atresia is present above the papilla of Vater. Vomiting is associated with variable dehydration, changes in serum electrolytes, and weight loss.

Normal meconium may be observed in the early stages. [19, 3] The high level of the obstruction makes global abdominal distention an infrequent finding, but fullness in the epigastrium, caused by the dilated duodenum and stomach, may be noted.

Differentials include malrotation and volvulus, intestinal atresia or stenosis in other locations, and extrinsic duodenal obstruction, duodenal duplication, or congenital bands.

Duodenal stenosis

The incomplete nature of the obstruction in duodenal stenosis results in a variable and often delayed presentation. It usually results in recurrent episodes of vomiting, aspiration, or failure to thrive. Some patients present in adulthood with gastroesophageal reflux, peptic ulceration, or obstruction of the duodenum proximal to the stenosis by a bezoar.