Pediatric Duodenal Atresia and Stenosis Surgery Treatment & Management

The definitive management of patients with intrinsic duodenal obstruction is surgical correction. In a patient with associated tracheoesophageal fistula, ligation of the fistula should precede correction of the duodenal atresia. This can be performed on two occasions or simultaneously. Repair of the atresia before ligation of the tracheoesophageal fistula could lead to duodenal rupture.

Duodenal webs can be diagnosed and excised by an expert surgical endoscopist. This is feasible in patients who present after the neonatal period with duodenal stenosis. The morbidity and complications associated with a megaduodenum may require further surgical intervention.

Transanastomotic feeding tubes or gastrostomies were used in the past but have been demonstrated to offer no clear advantage; instead, they result in a delay in establishing oral feedings and an increase in the duration of hospitalization. ^{[30, 3, 31]}

Soutter and Askew reported successful results with a transumbilical approach to duodenal atresia. ^[32]

Operative management of duodenal atresia is determined by the anatomic findings and associated anomalies noted upon laparotomy. Bypass procedures for duodenal atresia or stenosis include duodenoduodenostomy and duodenojejunostomy. Although many surgeons prefer the former for duodenal atresia, there is evidence to suggest that the two procedures have similar outcomes. ^[33] Type 1 duodenal atresia can also be managed by performing a simple duodenotomy with web excision.

Preparation for surgery

Orogastric decompression of the stomach and fluid resuscitation should be promptly initiated. Orogastric losses are monitored and replaced. Broad-spectrum antibiotics and 1 mg vitamin K are administered. Parenteral nutrition is instituted on the first day of life via a peripherally inserted central catheter. When stable, the infant is taken to the operating room.

Operative details

Once precautions have been taken against hypothermia, the abdomen is entered via a transverse right-upper-quadrant incision. The stomach and first part of the duodenum are usually dilated and thickened and must be decompressed via the orogastric tube.

The gallbladder and spleen are examined. The viscera are examined for other anomalies, including malrotation, an anterior portal vein, and an anular pancreas. The liver is padded and gently retracted superiorly. The duodenum is mobilized via the Kocher maneuver.

The site of obstruction is detected by noting the discrepancy in the size of the bowel above and below the obstruction and by passing the orogastric tube down to the level of the obstruction. In patients with a windsock web, an indentation at a site proximal to the site of obstruction may be observed. This marks the site of origin of the web and the location where the duodenotomy should be made.

The duodenotomy is made, and the papilla of Vater is identified by gently pressing on the gallbladder and observing the proximal and distal segments. In patients with type 3 defects, dual biliary ducts may be present.

Once it has been confirmed that no distal obstruction is present, a proximal transverse–to–distal longitudinal (diamond-shaped) anastomosis may be created with interrupted stitches. A direct duodenoduodenostomy is believed to result in a earlier recovery of anastomotic function than a duodenojejunostomy. ^[13]

During the diamond-shaped anastomosis, the midpoint of the proximal incision is approximated to the end of the distal incision. ^[4] This creates a larger stoma and allows the proximal duodenum to overlie the distal duodenum. (See the image below.) As an alternative, a standard side-to-side duodenoduodenostomy may be performed.

Alternatively, type 1 defects may be treated by excising the web. An anterior duodenotomy is performed, and the web is opened along the lateral side. The web is carefully excised, leaving the medial portion containing the papilla in situ.

When the proximal duodenum is floppy and dilated, an antimesenteric duodenoplasty may be performed either by excising excess tissue or by plicating the anterior wall with interrupted sutures or a stapling device over a dilator. This is believed to decrease the complications of a megaduodenum. ^[34]

If an anular pancreas is present, it should not be divided. The anastomosis is formed anterior to the pancreatic mass.

If a preduodenal portal vein is identified, it should not be divided.  The anastomosis is formed anterior to the vein.

In patients with malrotation, a Ladd procedure is performed; Ladd peritoneal bands are divided, the small-bowel mesentery is widened, an appendectomy is performed, and the cecum and colon are placed on the left.

Laparoscopic approaches

Several reports and case series have looked at short-term outcome for laparoscopic duodenoduodenostomy. ^{[35, 36]} The basic principles described above for the open technique are carried out; however, this procedure requires advanced laparoscopic skills. ^[37] This approach is facilitated by the use of 3-mm short instruments, better visualization at laparoscopy, the use of nitinol U-clips (Medtronic Surgical, Minneapolis, MN), and the decompressed distal bowel associated with the atresia.

Theoretically, assessment of the distal bowel for associated atresias is more difficult; however, these short-term reports documented no missed distal atresias with the laparoscopic approach. Long-term outcomes and the ease with which other pediatric surgeons adopt this approach will determine how widely available laparoscopic duodenoduodenostomy becomes in the future. Laparoscopic duodenojejunostomy has also been performed ^[38] ; it is surgically simpler than laparoscopic duodenoduodenostomy and appears to yield comparable results. ^[37]

In a small single-center series (N = 29), Parmentier et al compared laparoscopic repair (n = 10) with laparotomy repair (n = 19) for treatment of congenital duodenal atresia or stenosis. ^[38] They found laparoscopy to be safe and reproducible in this setting and to yield outcomes comparable to those of laparotomy. Laparoscopy was not associated reductions in time to full oral intake or length of stay.

Cho et al found laparoscopic duodenoduodenostomy to be comparable to to the equivalent open procedure for repair of congenital duodenal obstruction (duodenal atresia, stenosis, or web or anular pancreas) in neonates, though operating time was longer for the former. ^[39]  

A single-center study by Son et al found that laparoscopic surgical treatment of neonatal congenital duodenal obstruction was associated with lower postoperative morbidity, shorter recovery time, reduced postoperative hospital stay, and better postoperative cosmesis than laparotomy. ^[40]

A study of 44 patients undergoing duodenal atresia repair compared the outcomes of laparoscopic repair using a miniature stapler (LA-MS; n = 10) with those of laparoscopic handsewn repair (LA-HS; n = 21) and open repair (OR; n = 13). ^[41]  Mean operating time was significantly shorter in the LA-MS group than in the LA-HS group (145 ± 37 min vs 201 ± 47 min). Overall complication rate was similar between groups. Time to initiation of feeds and time to full feeds were significantly shorter in the LA-MS group than in the OR group (5 vs 11.9 days and 14.5 vs 24.4 days, respectively). 

The orogastric tube is left on free drainage. The patient is not given oral feedings until bowel sounds are heard, stool is passed, and the gastric drainage is limited (< 1 mL/kg/hr of clear or pale-green fluid). This may take 7-10 days but can be prolonged in the premature infant with other significant anomalies that necessitate venous access for parenteral nutrition. Patients with marked delay in establishing enteral feeding and delayed initiation of parenteral nutrition may show poor weight gain over long periods and increased sepsis. ^[42]

Oral feedings are gradually introduced, commencing with clear fluids and aspirating the stomach prior to each feed. These infants can be placed in a right-side-down position after feeds to enhance gastroduodenal emptying. Some surgeons choose to interrogate the anastomosis between postoperative days 5 and 7 by performing a limited upper contrast study before initiating feeds.

Early postoperative complications are frequently related to prematurity, very low birth weight, ^[43] coexisting congenital anomalies, and parenteral nutrition. Intestinal obstruction secondary to adhesions may also occur in the early postoperative period.

Long-term complications occur at any time from a few months to years after the primary procedure. Hence, long-term follow-up is compulsory for infants treated for intrinsic duodenal obstruction (see Long-Term Monitoring).

Late complications include the following ^{[34, 10, 44]} :

• Duodenal dysmotility
• Megaduodenum with blind loop syndrome
• Duodenogastric reflux and gastritis
• Peptic ulcer disease
• Gastroesophageal reflux
• Cholelithiasis
• Cholecystitis

Poor peristalsis in the proximal duodenum leads to functional obstruction in some patients. This may predispose the patient to blind loop syndrome and duodenogastric reflux. Alkaline biliary reflux leads to gastritis and peptic ulceration.

Reports of functional obstructions occurring more than 20 years after primary repair suggest that survivors of duodenal atresia should receive the following long-term follow-up care:

• Clinical examination - Special attention is paid to the abdominal examination and the nutritional status of the patient
• Radiologic examination - A barium meal may demonstrate a mildly dilated duodenum, megaduodenum, delayed emptying, diminished peristalsis, slight luminal narrowing, or bezoars
• Endoscopy - Findings on esophagogastroduodenoscopy (EGD) may include esophageal irritation, reflux gastritis, megaduodenum, or delayed emptying