Duodenal Atresia

Author: Frederick Merrill Karrer, MD, The David R and Kiku Akers Chair in Pediatric
Surgery, The Children's Hospital; Head, Division of Pediatric Surgery, Professor of Surgery and
Pediatrics, University of Colorado School of Medicine
Coauthor(s): D Dean Potter, MD, Fellow in Pediatric Surgery, The Children's Hospital; Casey
M Calkins, MD, Assistant Professor of Surgery, Division of Pediatric Surgery, Department of
Pediatric Surgery, Medical College of Wisconsin; Consulting Staff, Children's Hospital of
Wisconsin

Introduction
Relatively speaking, congenital duodenal atresia is one of the more common intestinal anomalies
treated by pediatric surgeons, occurring 1 in 2500-5000 live births. In 25-40% of cases, the
anomaly is encountered in an infant with trisomy 21 (Down syndrome).1 The definitive
intervention to correct the anomaly is surgical and consists of duodenoduodenostomy in the
newborn period.

History of the Procedure

Calder published the first report of duodenal obstruction in 1733 when he described 2 children
with "preternatural confirmation of the guts." Both infants died, as did subsequently reported
infants with this defect. 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. Morbidity and mortality significantly improved only over the last 50
years.2 Because of progress in pediatric anesthesia, neonatology, and surgical techniques, survival
is about 90% in infants who present with this anomaly. The standard operative procedure today
consists of duodenoduodenostomy via a right upper quadrant incision, although recent
advancements have enabled some surgeons to repair the defect by minimally invasive means.3,4

Problem

Differential diagnosis of neonatal upper GI obstruction includes the following:

Esophageal atresia
Malrotation with midgut volvulus
Pyloric stenosis
Duodenal atresia and stenosis
Annular pancreas
Preduodenal portal vein
 Any intestinal atresia
Duodenal duplication
Foreign body obstruction
Hirschsprung disease
Gastroesophageal reflux

Duodenal obstruction may be complete or incomplete.

Complete duodenal obstruction.

Complete duodenal obstruction.
Incomplete duodenal obstruction (duodenal stenosis).

Incomplete duodenal obstruction (duodenal stenosis).

Duodenal atresia is an example of complete intrinsic obstruction. Duodenal stenosis is an

example of an incomplete intrinsic abnormality; however, duodenal extrinsic stenosis can occur
in association with malrotation or a preduodenal portal vein. Strictly speaking, annular pancreas
does not cause an extrinsic duodenal obstruction because the duodenum within the collar of an
annular pancreas is intrinsically narrowed.
Duodenal atresia can take many forms, but proximal and distal intestinal segments always end
blindly. The intestine on either side of the defect may be in apposition (type 1), separated by a
fibrous cord (type 2), or gap (type 3). Regardless of atresia severity, the proximal intestinal
segment is typically dilated and the distal segment empty; these are hallmarks of duodenal
atresia. 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 perforated intraluminal diaphragm. The perforation

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 comprise
a frequent cause of intestinal obstruction in the newborn.3

Frequency

Reported incidence rates range from 1:2,500 to 1:40,000 live births; published rates in the United
States and internationally do not appear to differ. 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. 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 up to one third of patients with
duodenal atresia have Down syndrome (trisomy 21), it is not an independent risk factor for
developing duodenal atresia.3

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 30-60 days' gestation, completely plugging the duodenal lumen. A subsequent process
termed vacuolation occurs whereby the solid duodenum is recanalized. Vacuolation 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
pancreaspancreatic 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.
At the cellular level, the 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.

Presentation

Duodenal atresia is a disease of newborn infants. Cases of duodenal stenosis or perforated

duodenal web (diaphragm) rarely remain undiagnosed until childhood or adulthood; these cases
represent the exception rather than the rule. 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. 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.5
Duodenal obstruction is characterized by a double-bubble sign on prenatal ultrasonography. The
first bubble corresponds to the stomach and the second to the postpyloric and prestenotic dilated
duodenal loop. 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 GI anomalies.5,6

Presenting symptoms and signs are the result of high intestinal obstruction. Duodenal atresia is
typically characterized by onset of vomiting within hours of birth. While vomitus is most often
bilious, it may be nonbilious because 15% of defects occur proximal to the ampulla of
Vater. Occasionally, infants with duodenal stenosis escape detection of an abnormality and
proceed into childhood or, rarely, into 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 begun 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.
Passing meconium within the first 24 hours of life is not usually altered. Dehydration, weight
loss, and electrolyte imbalance soon follow unless fluid and electrolyte losses are adequately
replaced. If intravenous (IV) hydration is not begun, a hypokalemic/hypochloremic metabolic
alkalosis with paradoxical aciduria develops, as with other high GI obstruction. An orogastric
(OG) tube in an infant with suspected duodenal obstruction typically yields a significant amount
of bile-stained fluid.

Indications
Although duodenal atresia is a surgically treated disease, operating on an infant with duodenal
obstruction in the middle of the night is unnecessary. Only 2 limitations apply to timing the
repair: stabilization of the fluid and electrolyte balance and exclusion of overwhelming
congenital defects that would preclude use of a general anesthetic (ie, complex congenital heart
disease). Correction can begin any time after these issues are addressed and optimized.

Relevant Anatomy
Relevant anatomy of duodenal atresia is addressed in Problem.

Contraindications
Contraindications to immediate repair include electrolyte or fluid balance disturbances; severe
cardiac defects, which should be repaired prior to addressing the duodenal abnormality; and
severe respiratory insufficiency that would preclude a safe operation. Infants can be maintained
on orogastric OG suction and intravenous nutrition with aggressive repletion of fluid and
electrolyte losses while these life-threatening issues are addressed.

Duodenal Atresia: Treatment

Author: Frederick Merrill Karrer, MD, The David R and Kiku Akers Chair in Pediatric
Surgery, The Children's Hospital; Head, Division of Pediatric Surgery, Professor of Surgery and
Pediatrics, University of Colorado School of Medicine
Coauthor(s): D Dean Potter, MD, Fellow in Pediatric Surgery, The Children's Hospital; Casey
M Calkins, MD, Assistant Professor of Surgery, Division of Pediatric Surgery, Department of
Pediatric Surgery, Medical College of Wisconsin; Consulting Staff, Children's Hospital of
Wisconsin
Treatment
Medical Therapy

No medical therapies are available for the definitive treatment of duodenal atresia or stenosis; all
treatment is surgical. Adequate intravenous (IV) 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. A duodenojejunostomy is
now uncommonly performed due to its higher risk of long-term complications. Duodenal repair
may be performed via a right upper quadrant incision, an umbilical incision, or laparoscopically,
depending on surgeon preference.
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. The surgeon should also perform a
thorough examination of the infant, with special attention to cardiac and pulmonary function
before undertaking duodenal repair.

Intraoperative Details

As with all neonatal surgery, pay attention to preserving body temperature. Coordination with
an anesthesiologist with specialized training in neonatal surgery is advantageous when possible
because advances in pediatric anesthesia have contributed to improved overall survival of these
infants. When ready to proceed, the abdomen is entered 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 Media file 3).

Incision for duodenal exposure.

[ CLOSE WINDOW ]
Incision for duodenal exposure.

Divide the abdominal musculature transversely using cautery. For adequate exposure, carefully
retract the liver superiorly and pack Morisons pouch with laparotomy pads.

Thoroughly explore the abdomen for evidence of other abnormalities. Then mobilize the
duodenum using the Kocher maneuver and use an OG tube to determine the location of the
obstruction without opening the stomach. The stomach and proximal duodenum are often
thickened and dilated. When a significant gap is present between the proximal and distal ends,
the distal duodenum must be adequately mobilized.

The authors prefer a duodenoduodenostomy for repair, when possible. This may be performed in
either a side-to-side or a diamond-shaped fashion (authors' preference). For the side-to-side
technique (see Media file 4), make parallel incisions in both the proximal and distal segments.
Side-to-side duodenoduodenostomy.

[ CLOSE WINDOW ]
Side-to-side duodenoduodenostomy.

With gentle pressure on the gallbladder, document the site of the ampulla of Vater. Examine the
distal segment for other atresias or webs by passing a small red rubber catheter through the distal
duodenotomy because 1-3% of patients with duodenal atresia have an additional distal small
intestinal atresia.When ready to proceed, the authors' preference is a single layer anastomosis
with 4.0 or 5.0 PDS or Vicryl suture. Some surgeons prefer a 2-layer closure; the internal layer is
completed with a running 4.0 Vicryl, and Lembert sutures of 5.0 silk are used for the outer layer.
The authors prefer a diamond-shaped repair (see Media file 5), whereby the duodenotomies are
created differently.

Diamond-shaped duodenoduodenostomy.

[ CLOSE WINDOW ]
Diamond-shaped duodenoduodenostomy.

Make a transverse incision in the caudal end of the proximal duodenum and a longitudinal
incision of the same length in the distal segment. Stay sutures on the proximal segment are often
helpful prior to proceeding with the anastomosis. The latter can be accomplished in a single layer
(authors' preference) or double layer as described above. When completed, the
duodenoduodenostomy assumes the shape of a diamond.

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. A duodenotomy can be performed along the site of this
indentation. Again, prior to repair, examine the distal duodenum for a second defect. Thereafter,
the surgeon must identify the ampulla and note its relationship to the web because the medial
portion of most of these defects is located close to the ampulla. Accordingly, 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 Dexon and close
the duodenotomy either longitudinally or transversely in one layer as described above.

In patients with an annular pancreas, pancreatic tissue should not be divided for fear of
pancreatic fistula. Instead, a diamond-type or side-to-side duodenoduodenostomy is
recommended. Patients who present with associated malrotation should undergo a Ladd
procedure at the time of duodenal repair. Although gastrostomy tubes were often used in the past,
complications associated with their placement and long-term problems with gastroesophageal
reflux (following gastrostomy) have prompted the authors to avoid these adjuncts, except in
cases where gastrostomy is likely to be needed in the future (ie, an infant with trisomy 21 and
complex congenital heart disease).7

If possible, the authors prefer placement 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. One should consider
placing a peripheral intravenous central catheter (PICC) or central intravenous catheter at the
time of operation because of the expected prolonged ileus and the need for parenteral nutrition.

Many patients have a very dilated proximal duodenum at the time of initial repair. 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. An autostapling device is the most common method to resect excess duodenal tissue.
Alternatively, resection with a 2-layer closure or plication with interrupted sutures over a dilator
can be used. In most cases, the proximal dilatation of the duodenum resolves with time after a
successful duodenoduodenostomy. A small number of infants develop megaduodenum later in
life. However, the authors do not recommend duodenoplasty at the initial operation.8,9

Close the abdominal wound in layers. Close the peritoneum and posterior fascia separately from
the anterior fascia, using 4.0 PDS or Vicryl suture. Close the skin with a running subcuticular
suture of 5.0 Vicryl.

An umbilical approach for the treatment of pyloric stenosis was described in 1986; recently, this
incision has been applied to other intra-abdominal anomalies such as duodenal atresia and
stenosis. A semicircular umbilical incision is made in a skin fold and the peritoneum is entered in
the midline. The pliability of the neonatal abdominal wall allows retraction to expose the right
upper quadrant. Repair of the duodenal anomaly proceeds in a similar fashion as described
above.10

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. Two other ports, one 3
mm and one 5 mm are placed in the right lower quadrant and left mid quadrant, respectively. The
left mid-quadrant port is placed for the introduction of suture. At times, a fourth port is placed in
the right upper quadrant to retract the liver. After the duodenum is Kocherized, the site of the
obstruction typically becomes easily visible. A standard diamond anastomosis is then performed
using interrupted sutures or u-clips (see Media file 6).11

Upper GI contrast study following laparoscopic duodenal atresia repair. No leak is present,
and an open anastomosis is shown. The white arrow highlights the anastomosis, which was
performed using u-clips.

[ CLOSE WINDOW ]

Upper GI contrast study following laparoscopic duodenal atresia repair. No leak is present,
and an open anastomosis is shown. The white arrow highlights the anastomosis, which was
performed using u-clips.
As with the open repair, stay sutures are placed at each corner to facilitate the anastomosis. The
distal bowel is then examined to identify another distal atretic segment or suggestion of a web.
Once completed, the ports are removed and the sites are closed with absorbable suture.

One retrospective case series compared the right upper quadrant incision to a laparoscopic repair
for duodenal atresia or stenosis.12 Fourteen patients were in the open group, and 15 patients were
in the laparoscopic cohort. No anastomotic leaks were reported in either group. Patients that
underwent a laparoscopic repair were advanced to full feeding quicker (9 d vs 17 d) and were
discharged from the hospital sooner (13 d vs 20 d) compared with patients who underwent open
repair.

Postoperative Details

Nutrition should be provided by intravenous alimentation or via a transanastomotic feeding tube.

Maintain low intermittent suction on an OG tube until stool is passed and drainage from the OG
is less than 1 mL/kg/h and is clear. Feeding can then be advanced slowly by mouth.

Follow-up

See infants 2 weeks following discharge from the neonatal intensive care unit to assess wound
healing and ensure adequacy of nutrition and gastrointestinal function. Thereafter, see infants on
a yearly basis to assess for the 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 may incur late
complications. 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 can be corrected by
conversion to a duodenoduodenostomy. Megaduodenum with abnormal duodenal motility can be
addressed by performing a tapering duodenoplasty. Today, these issues may be addressed at the
time of initial operation by performing the duodenoduodenostomy along with duodenoplasty
when necessary.13,14,15

Duodenal Atresia: Follow-up

Author: Frederick Merrill Karrer, MD, The David R and Kiku Akers Chair in Pediatric
Surgery, The Children's Hospital; Head, Division of Pediatric Surgery, Professor of Surgery and
Pediatrics, University of Colorado School of Medicine
Coauthor(s): D Dean Potter, MD, Fellow in Pediatric Surgery, The Children's Hospital; Casey
M Calkins, MD, Assistant Professor of Surgery, Division of Pediatric Surgery, Department of
Pediatric Surgery, Medical College of Wisconsin; Consulting Staff, Children's Hospital of
Wisconsin
Outcome and Prognosis
The overall mortality rate for infants with duodenal atresia was 33% in a large series published in
1967. Today, the early mortality rate 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 multiple associated anomalies (usually complex cardiac defects). Improvement in
survival rates is most likely a result of advances in neonatal care such as high-frequency
ventilation, surfactant supplementation, nutritional support, pediatric anesthesia, and
sophisticated cardiac surgery. Long-term survival is excellent at rates reported between 86% and
90%.

Future and Controversies

Endoscopic excision of a duodenal web is possible but is not widely practiced and is of
questionable efficacy because of the precision required to avoid damaging the ampulla when
excising the membrane. Laparoscopic duodenoduodenostomy is a reasonable undertaking when
the surgeon is comfortable with advanced minimally invasive techniques in infants. The ideal
candidate for a laparoscopic intervention is an infant of reasonable size (>2.5 kg) without
significant congenital cardiac disease. The latter may preclude maintenance of a
pneumoperitoneum, which is necessary to complete the procedure safely.

Timing for the initiation of oral feeds has been traditionally based on return of bowel function
(ie, passage of stool, decreased orogastric [OG] tube aspirate volume and change from bilious to
clear gastric fluid). However, routine postoperative fluoroscopic evaluation for duodenal leak
after laparoscopic repair has contributed to earlier initiation of oral feeds and quicker discharge
from the hospital. Routine use of fluoroscopic evaluation, regardless of the technique of
duodenal repair, may reduce the need for parenteral nutrition and decrease the length of hospital
stays.12

The value of gastrostomy at the time of duodenal repair remains controversial. In 1969, some
80% of pediatric surgeons surveyed routinely used gastrostomy. Some purport large-caliber
gastrostomy offers better gastric drainage when compared to an OG tube, but the authors have
had little difficulty in achieving adequate gastric decompression with OG tubes. The authors
believe that a gastrostomy tube adds no advantage in postoperative management, and it may
contribute to late development of gastroesophageal reflux.

The authors use a transanastomotic feeding tube when it can be easily accomplished at the time
of surgery. If the tube does not easily pass into the upper jejunum, attempts to place it are
abandoned. A transanastomotic tube protects the anastomosis in the early postoperative period
when reinstituting feeding and has low risk of causing postoperative complications. Previous
worries of anastomotic complications following transanastomotic tube placement are no longer
justified. The slender silicone character of newer tubes remains supple in the lumen of the bowel
unlike earlier polythene and plastic tubes, which hardened in situ. Nevertheless, pediatric
surgeons remain divided in their use of transanastomotic tubes.