Pediatric Duodenal Atresia and Stenosis Surgery 

Fonkalsrud et al reviewed 503 cases of congenital duodenal obstruction treated between 1957 and 1967.^[1] Of patients who were surgically treated, 64% survived. Deaths were attributed to associated malformations, respiratory complications, prematurity, and anastomotic complications.

More recent survival rates for infants born with duodenal atresia or stenosis are approximately 90-95%.^{[2, 3]} Increased survival rates can be attributed to advances in respiratory care, hyperalimentation, improved pediatric anesthesia, improvements in the recognition and management of associated anomalies, and more refined surgical techniques (eg, the diamond-shaped anastomosis^[4] ).

In 1733, Calder described the first 2 recorded cases of duodenal atresia. The first successfully treated case was reported by Vidal in 1905; a gastrojejunostomy was performed. In 1914, Ernest performed the first successful duodenojejunostomy in an infant with duodenal atresia. Current surgical management more commonly includes duodenoduodenostomy and duodenoplasty.

See Pathophysiology.

Frequency

The incidence of duodenal atresia is 1 case per 5,000-10,000 live births.

Most cases of duodenal atresia are sporadic. Investigations of familial cases of duodenal atresia suggest an autosomal recessive inheritance in these individuals.^{[1, 5]}

In 1900, Tandler described the traditionally accepted theory on the normal development of the duodenum.^[6] The duodenum develops from the caudal part of the foregut and the cranial part of the midgut. At 4 weeks' gestation, it consists of an epithelial tube surrounded by mesenchyme. At 5-6 weeks' gestation, the epithelium proliferates while the surrounding mesenchymal walls are still narrow; the epithelial cells fill the lumen, completely obliterating it. Subsequent epithelial apoptosis at 8-10 weeks' gestation leads to vacuolation and recanalization of the duodenum. Failure of vacuolation may lead to intrinsic duodenal obstruction.

In 38-55% of patients, intrinsic duodenal obstruction is associated with another significant congenital anomaly.^{[1, 7, 8, 9]} 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.^[10] Other GI anomalies include malrotation, anterior portal vein, second distal web, anorectal anomalies, intestinal atresias, cloacal anomalies, and renal tract anomalies. Duodenal atresia is associated with prematurity and low birth weight.^{[11, 8]} Rarely, duodenal atresia is seen as a part of Feingold syndrome.^[12]

Duodenal atresia

Duodenal atresia is prenatally detected in 32-57% of patients.^{[7, 13]} 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.^{[14, 7, 15]} 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 prenatally diagnosed with confirmation following delivery.^[13]

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

Following 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 greater than 30 mL.^[16] An infant with a gastric aspirate that measures greater 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.^{[14, 3]} The high level of the obstruction makes global abdominal distension 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.

The definitive management of patients with intrinsic duodenal obstruction is surgical correction.

Duodenal atresia or stenosis usually occurs in the first or second part of the duodenum, most often near the papilla of Vater. The common bile duct may open into an intraluminal mucosal web.

The 3 anatomic types of duodenal atresia as described by Gray and Skandalakis (see following image) are as follows:

• Type 1: The most common type is formed by a membrane composed of mucosa and submucosa. This membrane traverses the internal diameter of the duodenum. The duodenum and stomach proximal to the obstruction are dilated and hypertrophied. The duodenum distal to the obstruction is narrowed. A variation of this occurs when the membrane is elongated in the shape of a windsock, and the site of origin of the membrane is proximal to the level of obstruction.
• Type 2: The atretic ends of the duodenum are connected by a fibrous cord.
• Type 3: Complete separation of the atretic segments occurs. Most of the biliary duct anomalies associated with duodenal atresia are observed in type 3 defects.^[15]

Various biliary tract and pancreatic anomalies have been demonstrated in patients with duodenal atresia or stenosis. These include stenosis and duplication of the distal common bile duct, choledochal cysts, and annular pancreas. Of note, air in the distal duodenum and gallbladder on plain radiography is suggestive of a bifid common bile duct. Double duodenal atresia or stenosis is less frequently reported.^[17]

In the patient with associated tracheoesophageal fistula, ligation of the fistula should precede correction of the duodenal atresia. This can be performed on 2 occasions or simultaneously. Repair of the atresia prior to ligation of the tracheoesophageal fistula could lead to duodenal rupture.