Pediatric Duodenal Atresia 

Updated: Jul 07, 2020
Author: Frederick Merrill Karrer, MD, FACS; Chief Editor: Carmen Cuffari, MD 

Overview

Background

Relatively speaking, congenital duodenal atresia is one of the more common intestinal anomalies treated by pediatric surgeons, occurring 1 in 5,000-10,000 live births.[1]  In 25-40% of cases, the anomaly is encountered in an infant with trisomy 21 (Down syndrome).[2]  The definitive intervention to correct the anomaly in the newborn is surgical and typically consists of duodenoduodenostomy.

History of the Procedure

Calder published the first report of duodenal obstruction in 1733 when he described two children with "preternatural confirmation of the guts."[3] Both infants died, as did infants with this defect subsequently reported. Scattered reports of duodenal obstruction appeared in the European literature over ensuing years. In 1916, the first survivor was reported, yet survival in the early 20th century remained rare.

Significant improvement in morbidity and mortality has occurred only over the last 50 years.[4]  Because of progress in pediatric anesthesia, neonatology, and surgical techniques, current survival is about 90% in infants who present with duodenal obstruction. At present, the standard operative procedure consists of duodenoduodenostomy via a right supraumbilical incision, although more recent advancements have enabled surgeons to repair the defect by minimally invasive means.[5, 6, 7]

Relevant Anatomy

Duodenal atresia is subcategorized into three types[1, 8] :

  • Type 1 (most common): A mucosal web or diaphragm obstructs the duodenal lumen, causing proximal dilatation and elongation of the web. Due to the stretching and elongation of the diaphragm, the mucosal web occasionally has the appearance of a windsock. The true site of obstruction will be proximal to the distal aspect of the dilated bowel. The web may also be fenestrated, leading to only a partial obstruction.
  • Type 2: The proximal and distal segments of the duodenum are connected by an atretic cord.
  • Type 3: The proximal and distal segments of the duodenum are completely separated. This malformation may be associated with other anomalies of the biliary system. [9, 10]

See the following image.

Pediatric duodenal atresia. Three anatomic types o Pediatric duodenal atresia. Three anatomic types of duodenal atresia are recognized. In type 1 atresia, a membrane traverses the internal diameter of the duodenum. This membrane may be elongated, giving rise to the windsock type 1 duodenal atresia. In type 2 atresia, the atretic ends of the duodenum are connected by a fibrous cord. In type 3 atresia, the atretic segments are completely separated.

 

Problem

The differential diagnosis of neonatal upper gastrointestinal obstruction or vomiting includes the following:

Duodenal obstructions can be complete or partial. Intrinsic duodenal obstructions may be attributed to atresia or mucosal webs. Even with annular pancreas, the obstruction is due to an associated intrinsic narrowing of the duodenum within the annular portion. Duodenal extrinsic obstruction can occur in association with malrotation or a preduodenal portal vein.

Duodenal atresia can take many forms, but proximal and distal intestinal segments always end blindly.[11]  The intestine on either side of the defect may be in apposition (type 1), or separated by a fibrous cord (type 2), or a gap (type 3).[12]  Hallmarks of duodenal atresia are a dilated proximal segment and a decompressed distal segment. Although obstruction may occur anywhere within the duodenum, it is most common in the vicinity of the ampulla of Vater. 

Stenosis may manifest as a stricture or a fenestrated intraluminal diaphragm (ie, web). The fenestration within the diaphragm is usually singular and centrally located within the lumen of the duodenum, although variations have been reported. A windsock abnormality is a thin diaphragm that has ballooned distally as a result of peristalsis. Together, both duodenal atresia and stenosis are a common etiology of intestinal obstruction in the newborn.[5]

Pathophysiology

Duodenal maldevelopment occurs secondary to either inadequate endodermal proliferation (gut elongation outpaces proliferation) or failure of the epithelial solid cord to recanalize (failure of vacuolization). Multiple investigators have demonstrated that the epithelium of the duodenum proliferates during days 30-60 of gestation, completely plugging the duodenal lumen. A subsequent process termed vacuolization occurs whereby the solid duodenum is recanalized. Vacuolization is believed to occur by way of apoptosis, or programmed cell death, which occurs during normal development within the lumen of the duodenum. Occasionally, duodenal atresia is associated with annular pancreas—pancreatic tissue that surrounds the entire circumference of the duodenum. This is likely due to failure of duodenal development rather than robust and/or abnormal growth of the pancreatic buds and can also be associated with other biliary anomalies.[9, 13]

At the cellular level, the gastrointestinal (GI) tract develops from the embryonic gut, which is composed of an epithelium derived from endoderm, surrounded by cells of mesodermal origin. Cell signaling between these two embryonic layers appears to play a critical role in coordinating patterning and organogenesis of the duodenum. Sonic hedgehog genes encode members of the hedgehog family of cell signals. Both are expressed in gut endoderm, whereas target genes are expressed in discrete layers in the mesoderm. Mice with genetically altered sonic hedgehog signaling display duodenal stenosis, which suggests that genetic defects in the sonic hedgehog family of genes may influence the development of duodenal abnormalities.

Epidemiology

Reported incidence rates for duodenal atresia range from 1 in 5,000 to 10,000 live births; rates published in the United States and internationally do not appear to differ.[14]  Duodenal atresia is not usually regarded as a familial condition, despite isolated reports of this condition in multiple siblings.

Etiology

Although the underlying cause of duodenal atresia remains unknown, its pathophysiology has been well described. Frequent association of duodenal atresia or stenosis with other neonatal malformations suggests both anomalies are due to a development error in the early period of gestation. However, duodenal atresia differs from other atresias of the small and large bowel, which are isolated anomalies caused by mesenteric vascular accidents during later stages of development. No predisposing maternal risk factors are known. Although 25-40% of patients with duodenal atresia have Down syndrome (trisomy 21), this condition is not an independent risk factor for developing duodenal atresia.[2, 5, 15]

Presentation

Duodenal atresia is a disease of newborn infants. Cases of duodenal stenosis or perforated duodenal web rarely remain undiagnosed until childhood or adulthood; these cases represent the exception rather than the rule.[16]  Duodenal atresia appears to be equally distributed between infants of both sexes, with no reported predilection for one race.

The use of modern ultrasonography has allowed many infants with duodenal obstruction to be identified prenatally.[17]  In a large cohort study of 18 different congenital malformation registries from 11 European countries, 52% of infants with duodenal obstruction were identified in utero.[18]

Duodenal obstruction is characterized by a double-bubble sign on prenatal ultrasonography. The first bubble corresponds to the stomach and the second to the post-pyloric dilated duodenum. Prenatal diagnosis allows the mother the opportunity to receive prenatal counseling and to consider delivery at or near a tertiary care facility that is able to care for infants with gastrointestinal (GI) anomalies.[18]

Presenting symptoms and signs of duodenal atresia are the result of proximal intestinal obstruction. Duodenal atresia is typically characterized by the onset of vomiting within hours of birth. Whereas the vomitus is most often bilious, it may be nonbilious because 15% of defects occur proximal to the ampulla of Vater.[19]  Occasionally, infants with duodenal stenosis escape detection of an abnormality until childhood or, rarely, adulthood before a partial obstruction is noted. Nevertheless, one should assume any child with bilious vomiting has a proximal GI obstruction until proven otherwise, and further workup should be initiated expeditiously.

Once delivered, an infant with duodenal atresia typically has a scaphoid abdomen. One may occasionally note epigastric fullness from dilation of the stomach and proximal duodenum. Gastric aspiration volume in a newborn of more than 20 mL is suggestive of intestinal obstruction; normally, aspirates should be minimal.[20]  There is usually no alteration of passing meconium within the first 24 hours of life. Dehydration, weight loss, and electrolyte imbalance soon follow unless fluid and electrolyte losses are adequately replaced. If intravenous hydration is not begun, a hypokalemic/hypochloremic metabolic alkalosis develops, as with other etiologies of proximal GI obstruction.

Timing of Surgical Repair

Although duodenal atresia is a surgically treated disease, operating on an infant with duodenal obstruction in the middle of the night is usually unnecessary. The exception is the infant with duodenal obstruction due to malrotation/volvulus, which can evaluated with an upper gastrointestinal contrast study.

Over 50% of infants with duodenal atresias have concomitant anomalies. The most common limitations that apply to timing the repair include stabilization of the fluid and electrolyte balance and exclusion of overwhelming congenital defects that would preclude the use of a general anesthetic (ie, complex congenital heart disease). Surgical correction can begin any time after these issues are addressed and optimized.

Prior to repair, infants can be maintained on orogastric suction and intravenous nutrition with aggressive repletion of fluid and electrolyte losses while any life-threatening issues are addressed.

Prognosis

The overall mortality for infants with duodenal atresia was 33% in a large series published in 1967. Today, the early mortality associated with this condition has declined to approximately 3% in most series.

Most deaths occurring in association with duodenal atresia are attributed to the presence of other associated anomalies (usually complex cardiac defects). Improvement in survival is most likely a result of advances in neonatal intensive care, nutritional support, and pediatric anesthesia. Long-term survival is excellent, at reported rates of greater than 90%.

 

Workup

Laboratory Studies

The following studies are indicated in duodenal atresia. Histologic examination is rarely performed or necessary, because repair does not involve removal of the obstruction.

Serum electrolytes

If duodenal atresia is diagnosed early, electrolyte and fluid balance should be normal. If the diagnosis is delayed at all, laboratory assessment of electrolyte and fluid status is imperative for an infant with duodenal atresia.

Prolonged vomiting can result in a hypokalemic/hypochloremic metabolic alkalosis with paradoxical aciduria.

Chromosome analysis

When trisomy 21 is suspected, a full genetic analysis should be performed. However, it is not necessary to obtain such an analysis prior to operative repair of the duodenal anomaly.

Imaging Studies

Prenatal ultrasonography

Perform prenatal ultrasonography during any pregnancy with associated polyhydramnios. Examination of a fetus with duodenal atresia may reveal a dilated fluid-filled stomach and duodenum in addition to other (eg, cardiac) abnormalities. Note that prenatal ultrasonography does not reliably detect duodenal stenosis.

The absence of the above findings does not rule out duodenal obstruction. Fetuses with duodenal atresia may have normal ultrasonographic findings in the presence of fetal vomiting. Monitor mothers with amniotic fluid abnormalities with repeat scans.

Diagnosis prior to birth enables prenatal consultation with a pediatric surgeon as well as provides parents an opportunity to discuss plans for postnatal care and management.

Erect and recumbent plain radiography of the abdomen

When duodenal atresia is suspected, erect and recumbent plain radiography of the abdomen should be the first imaging study obtained after birth.

A characteristic finding of duodenal obstruction is the double-bubble image of an air-filled stomach proximal to an air-filled first portion of the duodenum. The absence of gas in the remaining small and large bowel suggests atresia, whereas scattered amounts of gas distal to the obstruction suggest stenosis or malrotation/volvulus.

Cardiac and/or renal ultrasonography

More than 50% patients with duodenal atresia have other congenital anomalies.[1, 12]

Ultrasonography of the heart and kidneys may be warranted to identify potentially life-threatening abnormalities prior to definitive repair of the duodenal obstruction.

Upper gastrointestinal (GI) contrast evaluation

An upper GI contrast study may be useful to rule out the presence of malrotation with midgut volvulus or to confirm the presence of duodenal obstruction (see below), particularly if distal air is present.

Pediatric duodenal atresia. Upper gastrointestinal Pediatric duodenal atresia. Upper gastrointestinal series demonstrating duodenal atresia.

 

 

Treatment

Medical Therapy

Surgery is the definitive treatment for neonatal duodenal obstruction. However, adequate intravenous hydration, total parenteral nutrition, and gastric decompression are essential until the neonate has been stabilized for surgical repair.

Surgical Therapy

Duodenal atresia and stenosis are treated surgically. In patients with duodenal obstruction, a duodenoduodenostomy is the most commonly performed procedure for operative repair. Currently, a duodenojejunostomy is not commonly performed due to its higher risk of long-term complications, such as delayed return of bowel function and blind loop syndrome.[1, 21]

Duodenal repair may be performed via a right supraumbilical incision or laparoscopically, depending on surgeon preference.[22, 23, 24, 25, 26]  Generally, advancements in neonatal intensive care, parenteral nutrition, and refinement of surgical technique have contributed to improved outcomes in neonatal surgical patients.[27]

Preoperative Details

Little preoperative preparation is necessary if the diagnosis is secured within the first 24 hours. Placement of an orogastric (OG) tube and maintenance of intravenous (IV) hydration is mandatory in all infants with duodenal obstruction. If prolonged OG suction is necessary, IV fluid replacement of the gastric aspirate with one half normal saline with added potassium should be administered.

Prior to proceeding with operative repair, the surgeon should ensure that both fluid and electrolyte derangements are adequately corrected. In addition, the surgeon should also perform a thorough examination of the infant, with special attention to cardiac and pulmonary function.

Intraoperative Details

As with all neonatal surgery, preserving body temperature is essential. Coordination with an anesthesiologist with specialized training in neonatal surgery is advantageous, because advances in pediatric anesthesia, such as the use of inhaled agents with limited cardiac depression and the use of multimodal pain management, have improved perioperative mortality and morbidity in infants.[28]

Open repair

Enter the abdomen through a transverse skin incision begun 2 cm above the umbilicus from the midline and extending approximately 5 cm into the right upper quadrant (see the image below). Divide the abdominal musculature transversely using cautery. For adequate exposure, carefully retract the liver superiorly and pack the Morison pouch (hepatorenal fossa or recess) with laparotomy pads.

Pediatric duodenal atresia. Incision for duodenal Pediatric duodenal atresia. Incision for duodenal exposure.

Next, mobilize the duodenum using the Kocher maneuver. For type I atresia, advancing an orogastric (OG) or feeding tube is helpful to determine the location of the obstruction without opening the stomach. The stomach and proximal duodenum are often thickened and dilated. Adequate mobilization is critical when a significant gap is present between the proximal and distal ends.

The authors prefer a duodenoduodenostomy for repair, when possible. This may be performed in either a diamond-shaped fashion (authors' preference) or a side-to-side anastomosis. For the side-to-side technique (see the image below), make parallel incisions in both the proximal and distal segments.

Pediatric duodenal atresia. Side-to-side duodenodu Pediatric duodenal atresia. Side-to-side duodenoduodenostomy.

Gentle pressure on the gallbladder assists in locating the ampulla of Vater prior to anastomosis. This allows the surgeon to avoid placement of sutures that might result in biliary obstruction. Additionally, examining the distal segment for other atresia or webs is recommended, because 1-3% of patients with duodenal atresia have an additional distal small intestinal atresia.[29]  This assessment is facilitated by passing a small red rubber catheter or feeding tube through the distal segment.

A single layer, diamond-shaped duodenoduodenostomy repair (see image below) with 4-0 or 5-0 absorbable suture is preferable. This is created by making a transverse incision in the proximal duodenum and a longitudinal incision in the distal segment. Stay sutures on the proximal segment are often helpful before proceeding with the anastomosis. Some surgeons prefer a two-layer closure; the internal layer is completed with running sutures, and Lembert sutures are used for the outer layer.

Pediatric duodenal atresia. Diamond-shaped duodeno Pediatric duodenal atresia. Diamond-shaped duodenoduodenostomy.

In patients with a duodenal web, the surgeon can identify the site of the web's origin by passing the OG tube through the pylorus into the duodenum and noting the indentation of the duodenal wall caused by tenting of the web prior to opening the bowel. A duodenotomy can be performed along the site of this indentation. Again, before undertaking repair, examine the duodenum for the location of the ampulla and for distal obstruction. Excision of the web should proceed from the lateral duodenal wall, leaving the medial third of the web alone to avoid damaging the sphincter of Oddi or ampulla. Oversew the resection line with 4-0 Vicryl suture, and close the duodenotomy either longitudinally or transversely in one layer as described above.

When performing a repair in the presence of an annular pancreas or preduodenal portal vein, do not divide these tissues. Instead, a diamond-type or side-to-side duodenoduodenostomy anterior to the annular pancreas or preduodenal portal vein is recommended. Patients who present with an associated malrotation should undergo a Ladd procedure at the time of duodenal repair.

If possible, the authors prefer placement of a small, transanastomotic feeding tube (5F silastic nasojejunal feeding tube) across the anastomosis to facilitate postoperative enteral feeding. The authors also always leave an OG tube in place for gastric decompression. Although gastrostomy tubes were often used in the past, complications associated with their placement and long-term problems with gastroesophageal reflux (following gastrostomy) prompted the authors to avoid these adjuncts, except in cases where gastrostomy is likely to be needed in the future (eg, an infant with trisomy 21 and complex congenital heart disease).

Close the abdominal wound in layers. Close the peritoneum and posterior fascia separately from the anterior fascia, using 4-0 absorbable suture. Close the skin with a running subcuticular suture.

Laparoscopy

For the laparoscopic approach, neonatal laparoscopic instruments (3 mm) and trocars are used. The patient is placed supine at the end of the operating table. The operating surgeon stands at the patient's feet. The abdomen is insufflated through a 5-mm umbilical port via a modified Hasson technique. Two other ports, one 3 mm and one 5 mm, are placed in the right and left mid-abdomen, respectively. The left port is placed for the introduction of suture. The liver may be retracted either via an additional port or a transabdominal suture around the falciform ligament.

After the duodenum is mobilized, the site of the obstruction typically becomes easily visible. A standard diamond anastomosis is then performed most commonly using interrupted sutures.[22, 30, 31]  As with the open repair, stay sutures are placed at each corner to facilitate the anastomosis. The distal bowel is then visually examined to identify another distal atretic segment or an unusual caliber change suggestive of a web.[32] Once completed, the ports are removed and the sites are closed with absorbable suture.

Postoperative Details

Nutrition should be provided by intravenous alimentation or via a trans-anastomotic feeding tube. Maintain low intermittent suction on an orogastric (OG) tube until stool is passed, and the drainage from the OG is less than 1 mL/kg/h and is clear. Feeding can then be advanced slowly by mouth.

Follow-up

Infants are seen 2 weeks following discharge from the neonatal intensive care unit to assess wound healing and to ensure adequacy of nutrition and gastrointestinal function. Thereafter, infants are monitored on a yearly basis to assess for long-term complications of duodenal repair and to ensure that current practices are not contributing to long-term morbidity.

Complications

Despite improvements in early mortality rates, as many as 22% of children with duodenal atresia may incur late complications following repair. 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. Blind-loop syndrome due to previous duodenojejunostomy repair can be corrected by conversion to a duodenoduodenostomy.

Many patients have a very dilated proximal duodenum at the time of initial repair. The proximal dilatation improves with relief of the obstruction after repair in most infants. Only a small number of infants develop megaduodenum later in life, therefore the authors do not recommend routine duodenoplasty at the initial operation.[33, 34, 35]

However, in patients with an extensively floppy and distended duodenum (megaduodenum) with persistent symptoms of obstruction, an antimesenteric tapering duodenoplasty can be used to address duodenal dysmotility.[1]  An autostapling device is the most common method to resect excess duodenal tissue. Alternatively, resection with a two-layer closure or plication with interrupted sutures over a dilator can be used.

Future and Controversies

Reports of experience with laparoscopic repair of congenital duodenal obstructions in the early 2000s noted a steep learning curve and longer operative times compared with open repair.[29, 35]  However, more recent observational comparisons between open and laparoscopic approaches suggest an advantage with laparoscopic repair in terms of lower morbidity, reduced time to full feeding, and decreased use of parenteral nutrition.[7, 36, 37, 38, 39, 40, 41]  Future larger randomized prospective studies may be needed to fully clarify any potential advantage between the techniques.

Traditionally, laparoscopic diamond-shaped duoduodenostomy utilizes intracorporeal suturing for the anastomosis. Rare case reports using surgical U-clips (duodenoduodenostomy) or laparoscopic miniature staplers (duodenojejunostomy) for the creation of the anastomosis have reported these methods as feasible techniques.[42, 43]  Advocates of these methods theorize limiting tissue trauma from intracoporeal knot-tying may reduce the rate of anastomotic leak.

Although laparoscopic repair offers excellent visualization of the anastomosis, placement of a transanastomotic feeding tube is more difficult. The use of transanastomotic feeding tubes has been shown to decrease time to full feeds and the use of parenteral nutrition.[44, 45] Transanastomotic feeding tubes can also be useful for inspection of the distal bowel for additional type I atresia.

Alternatively, endoscopic excision or dilatation of a partial obstruction due to duodenal webs is possible with advanced endoscopic expertise. Endoscopic dilatation of a fenestrated duodenal web may require multiple dilations over time.[46, 47]  Although there may be a benefit to this minimally invasive technique, wide adoption of this practice has yet to take place due to safety concerns related to fear of damaging the ampulla when excising the membrane, perforation of the duodenal wall, hemorrhage, or missing a distal obstruction.[45, 46, 47]