Background

Duodenal atresia represents complete obliteration of the duodenal lumen. A duodenal

diaphragm (or duodenal web) is thought to represent a mild form of atresia. Duodenal
stenosis (incomplete obstruction of the duodenal lumen) is discussed with duodenal
atresia because the 2 disorders together represent a spectrum of similar intrauterine
events.
Annular pancreas occurs when pancreatic tissue surrounds the second portion of the
duodenum. If the encirclement is complete, it ma be associated with complete or
incomplete duodenal obstruction. !ince duodenal atresia or duodenal stenosis occurs in
all cases of annular pancreas, the anomalous pancreas should be considered a secondar
change rather than a primar cause of duodenal obstruction.
"athophsiolog
#he etiolog of duodenal atresia and stenosis is unknown. $ailure of recanali%ation of the
duodenal lumen remains the favored theor, compared with intrauterine vascular
ischemia.
During the third week of embronic development, the second portion of the duodenum, at
the &unction of the foregut and midgut, forms biliar and pancreatic buds, which
are derived from endoderm. During the ne't ( weeks, these buds differentiate into the
hepatobiliar sstem, with the development and subse)uent fusion of the 2 pancreatic
anlagen. *oncurrentl, the epithelium of the duodenum undergoes active proliferation,
which, at times, completel obliterates the duodenal lumen. +acuoli%ation, followed b
recanali%ation, reestablishes the hollow viscus.
#he second part of the duodenum is the last to recanali%e. #he earl forming biliar
sstem consists of 2 channels arising from the embronic duodenum. #his structure
creates a narrow segment of bowel, appro'imatel ,.-2. mm in length, that is interposed
between the 2 biliar channels. #his narrow region is the area most prone to problems,
with recanali%ation and with atresia formation. #he ampulla of +ater usuall is
immediatel ad&acent to or traverses the medial wall of the diaphragm. #he presence of a
bifid biliar sstem, or the insertion of - duct above the atresia and - duct below it, is
rare, occurring when both biliar duct anlagen remain patent. #he presence of bile above
and below the atresia indicates a bifid biliar sstem.
$re)uenc
United States
#he incidence of duodenal atresia is - per /,,, births. Intrinsic congenital duodenal
obstruction constitutes two thirds of all congenital duodenal obstructions (duodenal
atresia, (,0/,12 duodenal web, 3.0(.12 annular pancreas, -,03,12 duodenal stenosis, 40
2,1).
International
#he incidence in $inland of congenital obstruction (intrinsic, e'trinsic, combined) is - per
3(,, live births.
5ortalit65orbidit
If duodenal atresia or significant duodenal stenosis is left untreated, the condition rapidl
becomes fatal as a result of electrolte loss and fluid imbalance.
7ne half of the neonates with duodenal atresia or stenosis are born prematurel.
8dramnios occurs in appro'imatel (,1 of neonates with duodenal obstruction.
Duodenal atresia or duodenal stenosis is most commonl associated with trisom
2-. About 2203,1 of patients with duodenal obstruction have trisom 2-. 7ther
problems associated with trisom 2- include cardiac defects (most commonl
ventricular septal defects and endocardial defects), as well as 8irschsprung
disease.
9ace
:o racial predilection e'ists.
!e'
#he incidence of duodenal atresia and duodenal stenosis is appro'imatel e)ual in males
and females.
Age
Infants with duodenal atresia present with vomiting in their first few hours of life, but
patients with duodenal stenosis present at various ages. #he clinical findings depend on
the degree of stenosis. 7ccasionall, with duodenal web or duodenal stenosis,
presentation occurs in adulthood.
Anatom
In most cases, duodenal atresia occurs below the ampulla of +ater. In a ver few cases,
the atresia occurs pro'imal to the ampulla.
*linical Details
Bile0stained vomit in neonates aged 2( hours or ounger is the tpical presentation of
atresia or severe stenosis. 5inimal duodenal obstruction in mild stenosis or duodenal
membrane ma have few smptoms. In a few cases, the atresia is pro'imal to the ampulla
of +ater and the vomit is free of bile.
Both duodenal anomalies can be associated with other ;I and biliar tract abnormalities
(malrotation, esophageal atresia, ectopic anus, annular pancreas, gallbladder or biliar
atresia, vertebral anomalies). In addition, duodenal atresia can be associated with a
duodenal diaphragm, as well as with congenital abnormalities in other sstems. <'amples
include +A#<9 (vertebral defects, anal atresia, tracheoesophageal fistula with
esophageal atresia, radial and renal anomalies) association and +A*#<9= (vertebral,
anal, cardiac, tracheal, esophageal, renal, limb) sndrome.
Anomalies of the kidnes can occur in +A#<9 association2 the most common of these
renal abnormalities include aplasia, dsplasia, hdronephrosis, ectopia, persistent
urachus, vesicoureteral reflu', and ureteropelvic obstruction.
A few familial cases have been reported.
"referred <'amination
"lain radiographs that demonstrate a double0bubble appearance with no distal gas are
characteristic of duodenal atresia. Distal bowel gas indicates stenosis, incomplete
membrane, or a hepatopancreatic ductal anomal. 7ccasionall, a radiograph must be
obtained with the patient in the erect or the decubitus position to delineate the duodenal
component. If a combination of esophageal atresia and duodenal atresia is present,
ultrasonograph is preferred.
=imitations of #echni)ues
:o oral contrast materials are necessar in the evaluation of complete duodenal
obstruction. 7ccasionall, a small amount of positive contrast material can be instilled
through a feeding tube into the distal stomach and duodenum to differentiate the
diaphragm from a long stenosis.
7ccasionall, barium enema e'amination is suggested as an ad&unct stud in the
evaluation of duodenal atresia. Barium enema findings can demonstrate a malpositioned
cecum, but this is not alwas diagnostic of malrotation and volvulus. In addition, if a
microcolon is demonstrated, the presence of additional, more distal atresias can be
suggested. !uccus entericus ma be prevented from reaching the colon because of the
additional area of bowel obstruction. 5ultiple atresias are present in appro'imatel -.1
of patients. 8owever, most surgeons can determine the presence of malrotation and
additional atresias at the time of surger.
#o the author>s knowledge, in onl - instance was the addition of the barium enema
e'amination beneficial to the preoperative e'amination of a patient with trisom 2-. #his
case involved the coe'istence of 8irschsprung disease. (#he presence of trisom
2- makes the likelihood of 8irschsprung disease in a patient -.0fold greater.) If these
conditions are known prior to surger, the surgeon can create a tempori%ing colostom at
the time of duodeno&e&unostom and spare the child from undergoing additional
anesthesia.
Dd/7ther "roblems to Be *onsidered
Annular pancreas
Duodenal diaphragm
Duodenal stenosis
"reduodenal portal vein
=add bands
Duplication of duodenum
#umor of duodenum
8ematoma of duodenum
9etroperitoneal tumor
RADIOGRAPH
$indings
#he double0bubble sign represents dilatation of the stomach and duodenum. #his
configuration most commonl occurs with duodenal atresia and an annular pancreas. An
annular pancreas is almost alwas associated with duodenal atresia.
In a few cases of duodenal atresia, air can be observed distal to the area of obstruction. #he
anomalous hepatopancreatic ducts permit movement of air through a ?0shaped ductal
sstem, with - limb pro'imal to the obstruction and - limb distal to the atresia.
@hen duodenal atresia is combined with esophageal atresia, no air is observed in the
stomach. Because the stomach is obstructed at both ends, the infant presents with a large,
opa)ue upper midabdominal mass. If esophageal atresia e'ists along with a distal fistula, air
is found in the stomach and duodenum.
Duodenal obstruction in the neonate ma be partial or complete, and it ma be secondar to
intrinsic or e'trinsic abnormalities. #he duodenal bulb ma be larger in duodenal atresia than
in obstructions of the duodenum. Increased intramural pressure in duodenal obstruction can
result in gastric pneumatosis.
Degree of *onfidence
7nce the radiographic finding of a double0bubble sign without distal gas is determined, the
diagnosis of duodenal atresia is evident, and usuall, no additional, contrast0enhanced
studies are needed.
$alse "ositives6:egatives
Duodenal atresia that is associated with a ?0shaped biliar duct connection can have air
distal to the point of duodenal atresia. #his finding ma suggest stenosis rather than atresia.
5edia file -A Types of duodenal recanalization anomalies. A. Diaphragm, B. Solid cord
and atresia, C. Segmental asence. Dilatation of the pro!imal normal segment is seen in
each type.
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5edia file 2A Anteroposterior radiograph of the adomen depicts the doule"ule
sign of duodenal atresia. #ote the flattened acetaular angles and roadened ilia of
trisomy $%.
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5edia file 3A &ateral radiograph demonstrates the doule"ule sign of duodenal
atresia.
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5edia file (A Anteroposterior pro'ection of the chest and adomen demonstrates the
doule"ule sign of duodenal atresia in the adomen, as (ell as a pro!imal dilated
pouch )right arro(* resulting from esophageal atresia+ this displaces the trachea )left
arro(* to(ard the right side in the superior mediastinum. The presence of air in the
stomach and duodenum is related to the e!istence of a distal fistula.
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5edia file .A Barium air contrast,enhanced study of the duodenal memrane )arro(s*
in the lateral pro'ection sho(s a small amount of arium e!iting into the more distal
o(el. Coincidentally, $ coronal cleft -erterae are noted.
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5edia file /A Anteroposterior radiograph demonstrates an enlarged duodenum,
representing duodenal stenosis. Air is oser-ed distally.
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5edia file 4A Diagram of a hepatopancreatic ductal anomaly, (hich permits air to
mo-e distal to the ostruction in duodenal atresia.
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