J Ped Surg Case Reports 3 (2015) 489e491

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Journal of Pediatric Surgery CASE REPORTS

journal homepage: www.jpscasereports.com

Combined duodenal and jejunal atresia forming a bile filled cyst,

presenting as an abdominal mass: A case report and review of the
literature
Ahmad Zaghal a, Timothy M. Bahr b, Graeme J. Pitcher a, *
a
Deparment of Surgery, Division of Pediatric Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA 52242, USA
b
Phoenix Children’s Hospital, Phoenix, AZ 85006, USA

a r t i c l e i n f o a b s t r a c t

Article history: The simultaneous occurrence of duodenal and jejunoileal atresia in the newborn is very rare. This is not
Received 17 August 2015 surprising considering the unrelated embryogenesis behind the development of the two congenital
Received in revised form anomalies. We present a case of duodenal atresia associated with an apple peel jejunoileal atresia in a
21 September 2015
newborn presenting with a prenatal diagnosis of an abdominal cystic mass and a palpable abdominal
Accepted 26 September 2015
mass at birth. Exploration revealed a type 3 duodenal atresia followed by a type 3b proximal jejunal
atresia (apple peel configuration), with a dilated short segment of bowel between the two. This segment
of bowel was the cystic structure detected prenatally and preoperatively on exam. Double foregut
Key words:
Duodenal atresia
atresias should be considered in the differential diagnosis of right upper quadrant cystic masses in the
Jejunoileal atresia newborn period.
Congenital abdominal cyst Ó 2015 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND
license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

The embryogenesis of congenital duodenal atresia/web and abdominal palpable mass at birth, with an abdominal cystic mass
congenital jejunoileal atresia is thought to be markedly different. detected on prenatal ultrasound that was intra-operatively found to
The embryogenesis of duodenal atresia and stenosis is believed to be bile-filled. To our knowledge, no case of duodenal atresia and
be due to the failure of recanalization of the fetal duodenum at jejunoileal atresia presenting initially as a palpable abdominal mass
around 8e10 weeks of gestation. This is typically a peri-ampullary has been reported.
malformation [1] and is commonly associated with other anomalies
and chromosomal abnormalities, especially trisomy 21. The
1. Case report
embryogenesis of jejunoileal stenosis and atresia is thought to be
due to intrauterine ischemic insult to the midgut affecting intestinal
A one-day-old preterm (33 weeks gestation by 12 week ultra-
segments after their development [2]. Intrauterine vascular
sound) male infant weighing 2292 g was referred to our neonatal
disruption can lead to ischemic necrosis of the bowel and then to
intensive care unit because of a large palpable abdominal cystic
resorption of the affected segments. Duodenal atresia occurs in
mass (8
5 cm) seen on prenatal ultrasound. Pregnancy was
approximately 1 in 6000 to 1 in 10,000 live births [3]. Jejunoileal
complicated by maternal gestational diabetes.
atresia is similarly common affecting approximately 1 in 5000 live
On examination, the infant had normal and stable vital signs and
births. The incidence of both anomalies occurring simultaneously is
required no respiratory support to maintain acceptable hemoglobin
unknown and has only been reported in scattered cases throughout
saturation. At the time of examination there were light green as-
the literature.
pirates from the nasogastric tube, and he has not passed meconium
We present a case of duodenal atresia associated with a prox-
yet. The abdomen was soft, non-distended, and non-tender with
imal jejunal atresia with apple peel configuration presenting as an
active bowel sounds. There was a palpable soft mid-abdominal
mass, mobile in the cranio-caudal and medio-lateral directions.
Basic metabolic panel and liver function tests were within normal
* Corresponding author. Deparment of Surgery, Division of Pediatric Surgery,
University of Iowa Hospitals and Clinics, 200 Hawkins Drive, Iowa City, IA 52242,
limits.
USA. Tel.: þ1 319 356 1766. Ultrasound of the abdomen showed a large complex cystic mass
E-mail address: graeme-pitcher@uiowa.edu (G. J. Pitcher). extending from above the urinary bladder to just underneath the

2213-5766/Ó 2015 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
http://dx.doi.org/10.1016/j.epsc.2015.09.013
490 A. Zaghal et al. / J Ped Surg Case Reports 3 (2015) 489e491
gallbladder, measuring approximately 8.3
4.5 cm containing
mostly fluid with debris (sludge-like) in the dependent portions.
Plain abdominal X-ray showed a distended stomach and a dis-
tended proximal duodenum, with no distal air.
Upon surgical exploration patient was found to have (Fig. 1):
 Type 3 duodenal atresia
 Dilated short segment of bowel (distal duodenum/proximal
jejunum) between a proximal type 3 duodenal atresia and a
distal type 3b jejunal atresia (apple peel configuration), forming
an enteric cystic structure (about 8 cm in diameter) containing
dark green bile
 Bile duct (CBD) opened into this enteric cystic structure
 Type 3b proximal jejunal atresia (Apple peel configuration)
 Annular pancreas
We underwent exploratory laparotomy, diamond-shaped duo-
denoduodenostomy, excisional duodenoplasty, duodeno-jejunos-
tomy, and appendectomy (Fig. 2).
The postoperative course was uneventful. The patient was dis-
charged home at around 1 month of age. At the time of discharge he
was on breast milk, feeding ad libitum with a minimum of 2 sup-
plemental bottle feedings per day of 24 kcal fortified breast milk,
which were well tolerated.
2. Discussion and review of literature
The differential diagnosis of an abdominal mass in a newborn
includes renal masses, other solid organ tumors (neuroblastoma,
hepatoblastoma), choledochal cyst, urachal cyst, omental cyst,
mesenteric cyst, and duplication cyst among other possible causes.
The differential diagnosis of a bile-filled abdominal cystic mass in a
newborn, as in the case of this patient, is more specific for chol-
edochal cyst and duplication cyst. The co-occurrence of choledochal
cyst and duodenal atresia has been described in a limited number of
single-case reports in the literature [4].
Fig. 1. Diagram of intraoperative findings of duodenal atresia with bifid common bile
duct entering the intervening bowel segment forming a giant duodenal cyst.
Diamond-shaped
Duodenoduodenostomy
Tapered Duodenoplasty
Duodeno-jejunostomy
Excised Cyst
Fig. 2. Diagram of operative repair performed showing tapering of the cyst to use its
wall to maintain duodenal and biliary continuity.
MEDLINE, the bibliographic database of the National Library of
Medicine was searched at length for primary literature discussing
concomitant duodenal and jejunoileal atresias. To our knowledge,
no other case has been documented in which multiple intestinal
atresias presented as this patient did e as a palpable abdominal
mass.
Some case series provide insight into the sparseness of multiple
atresias. The cases most similar to ours were described in a case
series by St. Peter et al. In their review they summarized 408 cases
of duodenal atresia. Two of which had a second atresia (0.5%) and
both were type IIIb jejunal atresia [5]. Dalla Vecchia et al. discussed
277 cases of intestinal atresias over 25 years. Of the 138 found to be
duodenal obstruction, five (3.6%) also had jejunoileal atresia. The
nature of the duodenal obstruction and the jejunoileal atresia in
these five cases was not specified [6]. In another large series of 169
patients with duodenal atresia, three patients were found to have a
second downstream mucosal web [7].
A few single-case reports revealed anatomy similar to our pa-
tient, but none documented the finding of a palpable abdominal
mass. Gross et al. documented two siblings with similar findings.
The first had a dilated proximal duodenum followed by a collapsed
duodenal loop (duodenal atresia). The first 3 cm of the jejunum
immediately after the ligament of Treitz were dilated followed by a
narrow-caliber small bowel (jejunal atresia). The second sibling was
found to have duodenal atresia with an annular pancreas and first-
loop jejunal atresia with malrotation of the bowel [8]. We were able
to find 4 other cases of duodenal atresia with apple peel configu-
ration of the remaining small bowel [9e12].
The association of duodenal atresia with jejunoileal atresia,
despite being rare, has led some authors to question the current
belief that embryogenesis of duodenal atresia is caused by failure of
 A. Zaghal et al. / J Ped Surg Case Reports 3 (2015) 489e491
recanalization, and that vascular accidents may be the underlying
cause of duodenal atresia in this group of patients [12].
We believe that our patient had a bifid (Y-configuration) distal
common bile duct with one limb entering the enteric cyst (evi-
denced by the presence of bile in the enteric cyst), and another limb
entering the duodenum proximal to the duodenal atresia (evi-
denced by the preoperative bilious nasogastric aspirates). Such a
variation in biliary ductal anatomy has been reported in several
infants with duodenal atresia in the literature. This variation is
presumed to be rare despite the fact that the precise incidence is
not known [13].
3. Conclusions
The simultaneous occurrence of duodenal atresia and jejunoileal
atresia is rare, with very sparse number of similar cases reported in
the literature. This rare occurrence can present in the form of a
palpable abdominal mass in a newborn which might create a
diagnostic challenge.
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