Laporan Kasus

Maj Kedokt Indon, Volum: 58, Nomor: 11, Nopember 2008
Duodenal Atresia in
A Newborn Baby
Thermiany Anggri Sundari,* W. Retayasa,* M. Kardana,* N. Sukerena,** N. Sudira***
*Department of Child Health, Medical School of Udayana University/Sanglah Hospital, Denpasar,
**Department of Pediatric Surgery, Medical School of Udayana University/Sanglah Hospital, Denpasar,
***Department of Radiology, Medical School of Udayana University/Sanglah Hospital, Denpasar.
Abstract: Duodenal atresia (DA) is thought to arise from failure to recanalize the lumen after the
solid phase of intestinal development during the 4
th
and 5
th
week of gestation. A case of duodenal
atresia (DA) in a newborn baby boy was reported. He was born in Sanglah Hospital on 23
th
of
August, 2005, with bile-stained vomitus suspected duodenal atresia. On physical examination,
there was jaundice on sclera, the bowel sound was normal, there was no abdominal distention,
the skin was jaundice 2
nd
grade in Kramer. The plain abdominal radiograph showed double
bubble sign and no gas revealed in postgastric cavity. The operative finding of duodenal atresia
was then followed by duodenoduodenostomy (diamond-shapel anastomosis).
Keywords: duodenal atresia, double bubble appearance, diamond-shapel anastomosis
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Maj Kedokt Indon, Volum: 58, Nomor: 11, Nopember 2008
Atresia Duodenum pada Bayi Baru Lahir
Thermiany Anggri Sundari,* W. Retayasa,* M. Kardana,*
N. Sukerena,** N. Sudira***
*Bagian/SMF Ilmu Kesehatan Anak Fakultas Kedokteran Universitas Udayana/
RSUP Sanglah, Denpasar,
**Bagian Bedah Anak Fakultas Kedokteran Universitas Udayana/
RSUP Sanglah, Denpasar,
***Bagian Radiologi Fakultas Kedokteran Universitas Udayana/RSUP Sanglah, Denpasar
Abstrak: Atresia duodenum (AD) diduga timbul dari kegagalan rekanalisasi lumen setelah fase
padat pada perkembangan usus selama masa kehamilan minggu ke-4 dan ke-5. Sebuah laporan
kasus atresia duodenum (AD) pada seorang bayi laki-laki yang baru lahir. Bayi tersebut lahir di
Rumah Sakit Sanglah pada 23 Agustus 2005 dengan keluhan muntah berwarna hijau yang
diduga merupakan suatu atresia duodenum. Pada pemeriksaan fisik didapatkan kuning pada
sklera, bising usus dalam batas normal, tidak didapatkan adanya distensi abdomen, dan tampak
kuning pada kulit (Kramer 2). Pada pemeriksaan foto polos abdomen didapatkan tanda gelembung
ganda yang disebabkan karena lambung dan duodenum bagian proksimal mengembang dan
terisi udara. Pada tindakan pembedahan laparotomi ditemukan adanya atresia duodenum dan
kemudian dilakukan duodenoduodenostomi (anastomosis bentuk wajik).
Kata kunci: atresia duodenum, tanda gelembung ganda, anastomosis bentuk wajik
Introduction
Duodenal obstruction in the neonate may be partial or
complete and secondary to intrinsic or extrinsic abnormali-
ties of the gut.
1
A stenosis is an incomplete obstruction with
a small opening secondary to a diaphragm or web whereas
an atresia is a complete obstruction. Extrinsic duodenal ob-
struction is usually due to congenital peritoneal bands as-
sociated with intestinal malrotation, annular pancreas, or
duodenal duplication. Intrinsic obstruction is a result of
stenosis, mucosal diaphragm (so called wind sock defor-
mity), or duodenal atresia.
2
There are several types of intes-
tinal atresias: duodenal atresia (50%), jejunal atresia (36%),
and ileal atresia (14%).
3
Duodenal atresia (DA) is thought to arise from failure
to recanalize the lumen after the solid phase of intestinal
development during the 4
th
and 5
th
week of gestation.
1
DA is
the most common cause of duodenal obstruction occurring
in approximately 1 in 10 000 live births and accounts for 25-
40% of all intestinal atresias and affect males and females
equally.
1,2
DA is four times more common than duodenal
stenosis. DA is commonly associated with other congenital
anomalies (30%). Other congenital anomalies that are asso-
ciated with DA include malrotation (20%), esophageal atre-
sia (10-20%), congenital heart disease (10-15%), and anorec-
tal and renal anomalies (5%). Half of the patients are born
prematurely. Down syndrome occurs in 20-30% patients with
DA.
2
The etiology of DA is unknown. The duodenum begins
to develop during the fourth week from the caudal portion of
the foregut, the cranial part of the midgut, and the splanch-
nic mesenchym associated with the primitive gut. The com-
mon bile duct enters the duodenum at the junction of the
distal foregut with the midgut. The proliferation of the epi-
thelial cells during the 4
th
and 5
th
weeks narrows the lumen of
the duodenum and is the solid cord phase of development.
Failure of recanalization of the duodenal lumen remains the
favored theory compared with intrauterine vascular ischemia.
1
DA maybe proximal or distal to the ampulla of Vater but occur
most frequently distal to the ampulla of Vater.
2
The hallmark clinical presentation of DA is bilious vom-
iting without abdominal distention. Bile-stained vomitus in
neonates is usually noted on the first day of life but in a few
case if the atresia is proximal to the ampulla of vater, the
vomitus is free of bile.
1,4
A history of polyhydramnios is
present in half the pregnancies and is caused by a failure of
absorption of amniotic fluid in the distal intestine. Jaundice
is present in one third of the infants.
1
Plain abdominal radiograph that demonstrate the double
bubble appearance with no distal gas are characteristic of
DA.
5,6
This configuration most commonly occurs with duode-
Duodenal Atresia in A Newborn Baby
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Maj Kedokt Indon, Volum: 58, Nomor: 11, Nopember 2008
Duodenal Atresia in A Newborn Baby
nal atresia and annular pancreas. Malrotation with volvulus
may also result in duodenal obstruction and a consequent
double bubble. Duodenal atresia and malrotation may coex-
ist. Once the radiographic finding of double bubble sign
without distal gas is determined, the diagnosis of duodenal
atresia is evident and usually no additional contrast enhanced
studies are needed. The exact cause of the obstruction may
not be known until laparotomy.
4
Differential diagnosis of duodenal obstruction includes
duodenal atresia, duodenal stenosis, annular pancreas, mal-
rotation with or without midgut volvulus.
4
The initial treatment of DA is intravenous fluid replace-
ment. If DA is left untreated, the condition is rapidly fatal
owing to electrolyte loss and fluid imbalance. Definitive cor-
rection of DA is usually postponed to evaluate and treated
these life-threatening anomalies. The usual surgical repair
for DA is duodenoduodenostomy.
1,2
The prognosis is primarily dependent on the presence
of associated anomalies. The mortality rate (35-40%) is sig-
nificantly affected by prematurity, down syndrome, and as-
sociated congenital anomalies.
2
The purpose of this paper is to report a case of duode-
nal atresia. The rarity of this case in the main reason of this
case presentation.
Report of the Case
WY, was a newborn baby boy, he was born at Sanglah
Hospital on 23
th
of August 2005. He had vomit feel a few
hours after breastfeeding on the first day. The vomiting was
profuse and projectile, 4-6 times daily with bile-stained vomi-
tus but without blood, the volume about 5-10 mL of each
vomiting.
He was the first child in the family, no history of gas-
trointestinal congenital anomalies in both of his parents
family. He was born spontaneously; body weight was 3200
g, body length was 49 cm, and at term. There was no history
of specific illnesses suffered by his mother during preg-
nancy and history of maternal polyhidramnios was unknown.
The amount of amniotic fluid was sufficient and clear.
Meconeum passed normally.
On the first day of life, after getting bile-stained vomi-
tus, the physical examination revealed that the baby was
alert, the heart rate was 130 times per minute, regular, the
respiration rate was 40 times per minute, regular. The body
temperature was 37
o
C. The head was normal in shaped and
the hair was strong, black in colour, and the fontanel was
flat. There was no jaundice on sclera, nor conjunctiva injec-
tion, anemia, and sunken. The pupil light reflect was normal.
The ear, nose, and throat examination were in normal limit.
The sucking reflex was normal. There was no lymph nodes
enlargement found on the neck. The chest was symmetrical
both on rest and movement, breath sound was
bronchovesicular, no evidence of rales or wheezing, the heart
sound first and second were normal, regular and no murmur
in auscultation was heard. There were no lymph nodes en-
largement found on both of axillaes. There was no epigastric
distention, the skin elasticity was normal, the bowel sound
was normal, liver and spleen were not palpable. There were
no lymph nodes enlargement found on both of the inguinals
region. The extremities were normal and the power, tonus,
and reflex of the right superior and inferior extremities were
normal. On the 2
nd
day of life, jaundice was appearance on
the sclera and also on the skin with second grade in Kramer.
The laboratories finding on 24
th
of August, 2005, revealed:
the leukocyte count was 9.4 K/mL, the hemoglobin level was
18.5 g/dL, the haematocrite was 54 %, platelet count was 249
K/mL, the electrolytes serum revealed sodium chloride was
139 mmol/L, potassium was 3.8 mmol/L, chlorida was 96 mmol/
L. The total bilirubin level was 11 mg/dL, direct bilirubin level
was 0.35 mg/dL, indirect bilirubin level was 10.6 mg/dL, globu-
lin was 3 mg/dL, protein total was 7.1 g/dL, albumin level was
4 g/dL, bleeding time was 2 minutes, clothing time was 8
minutes, blood sugar was 125 mg/dL.
The plain abdominal radiograph was done on 24
th
of
August 2005, showed: double bubble sign, - no sign of gas
revealed in post gastric cavity. There was no evidence of
free air in the abdominal cavity but was a sign of duodenal
agenesis.
Fig 1. A Plain Abdominal X-Ray
Based on clinical manifestation, laboratories finding, and
the plain abdominal radiograph, a working diagnosis was
established. Differential diagnosis includes duodenal atre-
sia, severe duodenal stenosis, annular pancreas, malrotation
with or without midgut volvulus. The patient was given in-
travenous fluid drip (IVFD) Dextros 10% 250 ml/day or 10
microdrop per minute, nasogastric decompression is to pre-
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Maj Kedokt Indon, Volum: 58, Nomor: 11, Nopember 2008
Duodenal Atresia in A Newborn Baby
vent aspiration, and he was referred to Pediatric Surgery
Department and by Pediatric Surgeon planned laparotomy.
On 25
th
of August, 2005, laparotomy was performed.
The surgery showed that duodenal atresia was found distal
to the ampulla of Vater. Then duodenoduodenostomy (dia-
mond shape anastomosis) was done to repair the anomaly.
Fig 2. The Operative
The patient was observed in the High Care Intensive
Unit (HCU) post operation. There was no complication found
in this patient. The patient was given IVFD Dextros 10% 360
mL/day or 12 microdrop per minute, calcium gluconas 10 ml
in 500 mL dextros 10%, and KCL 10 mL in 500 ml dextros 10%,
aminofusin paed, Kedacillin® injection 250 mg three times
daily.
On 26
th
of August, 2005, the patient was alert, there was
no bleeding of wound operation, the bowel sound was nor-
mal, and he had flatus and his defecation was normal. On 27
th
of August, 2005, realimentation was done by giving the pa-
tient breastfeeding gradually, starting from 5 cc every 2 hours.
The patient was discharge from hospital in a good condition
after 9

days post operation, there was no complication from
the operation wound, and he was breastfed well.
Discussion
Approximately 50% of patients with duodenal atresia
have some form anomaly: Down syndrome, malrotation,
esophageal atresia, annular pancreas, renal anomalies, con-
genital heart disease, imperforate anus, and up to 40% have
trisomy 21.
1,2
In this case there were no other congenital
anomalies.
Infants with DA present with bilious vomiting in their
first few hours of life but patients with duodenal stenosis
present at various ages depend on the degree of stenosis. In
a very few cases, the atresia is proximal to the ampulla of
Vater and the vomitus is free of bile. Bilious vomiting without
abdominal distention on the first day of life is the hallmark of
duodenal atresia. Other manifestation include polyhydram-
nios which is present in 50% of cases, jaundice, and intoler-
ance to feeding.
1,6
In this case, bilious vomiting without ab-
dominal distention is evident within hours of birth. There
was jaundice in second grade of Kramer which was showed
on the second day of life.
Meconeum stools may be passed initially if the obstruc-
tion is in the upper part of the small intestine or if the ob-
struction developed late in intrauterine life.
1
In this case,
meconeum passage was normal because duodenal atresia
was found distal to the ampulla of Vater.
Polyhydramnion, an excessive accumulation of amni-
otic fluid within the amniotic cavity, is not associated with an
identifiable cause in the majority of the cases. However, in
about one-third of the cases an underlying cause is present.
5
History of maternal polyhydramnios in this case is unknown
and antenatal ultrasonography was not done.
Radiographically, duodenal atresia is suggested by the
presence of the double bubble sign which results from accu-
mulation of gas in the stomach and proximal duodenum. Be-
cause of the atresia, bowel gas does not enter the remainder
of the bowel until after the first day of life which is associ-
ated with complete or near complete duodenal obstruction.
This lesion can usually be identified with certainty on plain
abdominal radiograph. Upright and contrast radiography
using air or radioopaque contrast may confirm the diagnosis
but in this case was not done.
7
Plain abdominal radiograph
that demonstrate the double-bubble appearance with no dis-
tal gas are characteristic of duodenal atresia. Distal bowel
gas indicates stenosis, incomplete membran, or a
hepatopancreatic ductal anomaly. Barium enema findings can
demonstrate a malpositioned caecum but this is not always
diagnostic of malrotation and volvulus. However, most sur-
geons can determine the presence of malrotation, annular
pancreas at the time of surgery.
5
In this case, the plain ab-
dominal radiograph showed double bubble sign, there was
no gas revealed in postgastric cavity suspected agenesis
duodenal. Contrast radiography was not done in this case.
True atresias usually occurs between the second and
third portions of the duodenum. The procedure of choise for
DA is duodenoduodenostomy (interrupted single or two layer
anastomosis) that is constructed using the most dependent
portion of the proximal atretic segment to minimize stasis.
Kimura proposed the construction of a diamond-shaped anas-
tomosis in order to create a larger intraluminal orifice.
8
In this
case, at laparotomy the duodenum was seen to end in a blind
pouch at about the junction of the second and third portion.
There was a separation between the blind pouch and the
blind-ending distal duodenum and no intraluminal gas was
visible. The colon was normal in position and contained
meconeum. The liver and gallbladder were normal. There was
no web neither nor annular pancreas. Duodeno-
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Duodenal Atresia in A Newborn Baby
Maj Kedokt Indon, Volum: 58, Nomor: 11, Nopember 2008
duodenostomy (diamond shape anastomosis) was performed
in this case and there were no anomalies so the differential
diagnosis were excluded. The differential diagnosis includes
duodenal atresia, severe duodenal stenosis, annular pan-
creas, malrotation with or without midgut volvulus. In this
case, those could be excluded because this patient had the
hallmark clinical presentation of duodenal atresia was bil-
ious vomiting without abdominal distention on the first day
of life and based on the result after laparotomy.
Duodenal atresia can be fatal unless promptly diag-
nosed and treated surgically. Death occurs in the newborn
secondary to emesis, aspiration, and electrolyte imbalance.
Longterm complications following repaired duodenal atre-
sia include stasis in the proximal megaduodenum. Although
mortality rate in this patient population has been reduced 5-
8%, late complication maybe observed including foregut
motility disorder (gastroesophageal reflux, duodenal stasis,
duodenal gastric reflux), gastritis, and biliary tractus disor-
ders (including cholecystitis and cholelitiasis). Stagnation
and functional obstruction in the proximal duodenum is the
main factor influencing the morbidity rate.
8
Operative mortality for neonates with duodenal atresia
was 4% and the longterm survival rate for children with
duodenal atresia was 86%.
9
Suggesting that longterm follow
up for this case is extremely important.
The patient postoperative course was uneventful.
There was no bleeding of wound operation; the bowel sound
was normal; he had flatus; and his defecation was also nor-
mal. He was discharge in good condition on the 9
th
day. In
this case, the prognosis is good.
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