

Volume 53, Issue 10

October 2017
Pages 1032–1034
Letter to the Editor
Annular Pancreas Combined with Intestinal
Malrotation and Duodenal Duplication: A Rare Case
of Multiple Alimentary Tract Anomalies in a
Preadolescent
Authors
Dr Yin Zhou,
Ms Wenqiong Xin,
Dr Guozhu Yang,
Dr Shuguang Jin,
Dr Lin Zhong,
Professor Yuan Li
 First published: 4 October 2017
 DOI: 10.1111/jpc.13687

Dear Editor,

Annular pancreas (AP) is a rare congenital anomaly that is estimated to occur in 1 of 12 000–
15 000 newborns.[1] It is thought to represent an aberration in the development of the ventral
pancreatic bud and is characterised by an extension of pancreatic tissue around the second
part of the duodenum. Malrotation of the intestinal tract is a congenital anomaly that refers
to either lack of or incomplete rotation of the foetal intestines around the axis of the superior
mesenteric artery during foetal development.
An 11-year-old boy was admitted because of a complaint of repeated nausea and vomiting
for more than 10 years, which became frequent in the month prior to admission. The vomiting
was bilious and non-projectile, and occurred five or six times per day, always accompanied
with post-prandial, mild-epigastric abdominal pain and intermittent cramping, which lasted
several minutes to an hour. The pain could be relieved after vomiting. The patient appeared
weak, and his abdomen was soft, without tenderness, palpable masses or organomegaly.
Abdominal contrast-enhanced computed tomography (CT) depicted an enlarged pancreatic
head, which partly surrounded the second segment of the duodenum (Fig. 1a). All the colons
were on the right side, and the small bowels were on the left side of the abdominal cavity
(Fig. 1b). An upper gastrointestinal tract barium study revealed that the duodenum and
jejunum were distributed abnormally in the cavity. No duodenal extension or stenosis was
found. Based on these findings, the boy was diagnosed as having AP and intestinal anomaly,
and exploratory operation was performed. The ectopic pancreatic tissue was soft and partly
encircling the duodenum at the second part (Fig. 1c). The proximal and distal duodena from
the AP had the same size as the cavity, and no narrowing was detected at the encircled
duodenum. A globular cyst, which measured 20 mm in diameter, was protruding from the
serosa at the joint of the third and fourth parts of the duodenum (Fig. 1d). A small amount of
adhesion was found between the duodenum and jejunum. Both the descending and sigmoid
colons were in the right abdominal cavity, and the terminal ileum entered the caecum from
the right side (Fig. 1e). Then, the duplication cyst was completely resected, and Ladd's
procedure was performed. Post-operative histopathological examination of the excised cyst
wall revealed the duodenal mucosa, submucosa and muscle coats, which supported the
diagnosis of duodenal duplication, and no evidence of canceration. The patient recovered
smoothly, and his symptoms were relieved. Three months after the operation, the patient was
readmitted because of repeated nausea and vomiting. Gastroscopy revealed gastroesophageal
reflux disease and chronic superficial gastritis. The duodenal lumen was normal, and no
neoformation was detected. An upper gastrointestinal tract barium study revealed that the
passing of barium through the pylorus was delayed (Fig. 1f). Repeated nausea and vomiting
could not be alleviated using palliative treatment, and a second exploratory operation was
performed. The gastric lumen was enlarged, and the small intestine was empty; the head of
the pancreas appeared much harder than that in the first operation. A duodenoduodenostomy
was performed. The patient recovered well and was discharged 10 days after the operation.
The patient has been well during 1-year follow-up.
Figure 1.
Pre-operative computed tomography image showing enlargement of the pancreatic head (black arrow),
which partly encircles the second segment of the duodenum (black dotted arrow) (a). All the colons (white
arrow) are seen in the right lower quadrant, and the small bowels (white dotted arrow) are distributed in
the left abdominal cavity (b). During surgery, an enlarged and soft pancreatic head (black arrow) was
found to partly surround the second part of the duodenum (black dotted arrow) (c), a globular cyst (black
arrow) was found between the third and fourth parts of the duodenum (d), the terminal ileum (black arrow)
entered the caecum from the right side, and the descending colon (black dotted arrow) was in the right
lower quadrant of the abdominal cavity (e). The contrast barium radiography shows obstruction of the
second duodenum associated with proximal dilatation of the duodenal bulb and stomach, classically
known as the ‘double bubble’ sign (f).
Coincidence of AP, intestinal malrotation and duodenal duplication in preadolescents is
rare.[1, 2] Pre-operative radiological evaluation, careful evaluation of the gastrointestinal
tract and a delicate operative technique are necessary to avoid misdiagnosis, missed diagnosis
or repeated operations.[3, 4]
Acknowledgement
This study was supported by grants from the National Natural Science Fund of China
(NSFC key project 81470886).

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