Cystic dystrophy of the duodenal wall: imaging features and differential diagnosis

e-Poster: P-131

Congress: ESGAR 2009

Type: Educational Exhibit

Topic: Diagnostic / GI Tract / Small Bowel

Authors: J. Brito1, L. Curvo-Semedo2, M. Lima1, J.F. Costa2, B.J.A.M. Gonçalves2, B. Graça2, M.F.S. Seco2
, F. Caseiro-Alves2; 1Angra Do Heroismo/PT, 2Coimbra/PT

MeSH:

Duodenum [A03.556.124.684.124]

Duodenal Diseases [C06.405.469.275]

Diagnosis, Differential [E01.171]

Keyword: Cystic dystrophy of the duodenal wall

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1. Learning objectives

To review the cross-sectional imaging features of cystic dystrophy of the duodenal wall (CDDW), with
emphasis on its differential diagnosis.

2. Background

Cystic dystrophy of the duodenal wall (CDDW) is believed to result from heterotopic pancreatic tissue
situated in the suprapapillary area of the 2nd portion of the duodenum.

CDDW is one of the interrelated benign conditions that affect this area including pancreatic
hamartoma, para-duodenal wall cyst, myoadenomatosis, and groove pancreatitis, which have been
collectively termed “paraduodenal pancreatitis”. They share similar pathologic findings and it is not
yet well understood whether they represent distinct disease entities or a spectrum of the same disease
process.

Clinical aspects

CDDW mainly affects men in their fifth decade. About 75% of patients present chronic alcoholism.

Symptoms can be epigastric pain, vomiting and nausea from intolerance of food intake (by duodenal
compression) or jaundice (by biliary compression). Weight loss can be related to pain, leading to
auto-limitation of food intake and vomiting due to duodenal stenosis. Most of the symptoms are also
compatible with chronic alcoholic pancreatitis which is associated in about 90% of patients.

Pathological aspects

Pancreatic heterotopia: Pancreatic heterotopia is most commonly seen in the duodenum, pylorus and
gastric antrum ([Fig. 1] Fig. 1), and by definition lacks anatomical and vascular continuity with the
pancreas proper.

Autopsy studies suggest that ectopic pancreatic tissue is quite common (1% to over 13%) but its
clinical manifestation is very rare.

Despite the comparable incidence of pancreatic heterotopia in duodenum and stomach, there are
only few reports of cystic dystrophy involving gastric ectopic pancreas.

Duodenal cysts: Ectopic exocrine lobules may have excretion channels too small to function.
Accumulation of pancreatic secretions can then lead to repeated episodes of obstructive acute
pancreatitis and formation of retention cysts imbedded in the thickened duodenal wall. Histologically,
cysts are dilated pancreatic-type ducts lined with its typical cuboid epithelium. In some of these ducts
partial or total erosion of the epithelium may occur, thus creating a pseudocyst-like appearance.

Thickened duodenal wall: In CDDW the duodenal wall in the vicinity of the minor papilla shows
proliferation of myoid cells. Brunner’s gland hyperplasia is typically prominent and contributes further
to thickening of the duodenal wall.

Associated chronic pancreatitis: CDDW can be associated with a healthy pancreas or more generally
(90%) with a pancreas suffering from chronic pancreatitis. Examining series of chronic pancreatitis
patients shows that the frequency of CDDW is to the order of 25%. For some authors, CDDW is an
extension of pre-existing main gland chronic pancreatitis. For others, the chronic pancreatitis evolves
independently in the ectopic gland, leading to upstream compression of the pancreatic duct.
Treatment

Besides non-specific treatment with abstention from alcohol, management practices include:
octreotide, endoscopic fenestration of the cysts, and surgery.

3. Imaging findings OR Procedure details

CDDW cross-sectional imaging features:

CDDW diagnosis is often missed and established late due to the non-specific clinical manifestations or
attribution of symptoms to chronic pancreatitis. This emphasizes the importance of its recognition on
cross-sectional imaging studies.

Main imaging findings on CDDW are:

1. Duodenal cysts;
2. Thickening of the duodenal wall;
3. Associated features.

1. Duodenal cysts.

The cysts within the duodenal wall may be tiny ([Fig. 2] Fig. 2) or quite large ([Fig. 3] Fig. 3). Its usual
location is the mesenteric side of the 2nd portion, less commonly in the antimesenteric side. Lesions
may be spherical ([Fig. 4] Fig. 4) or have elongated or plurilobular morphology ([Fig. 5] Fig. 5).
Sometimes a multilocular cystic “mass” may be identified ([Fig. 5] Fig. 5).

Endoscopic US (EUS) is currently considered the exploration of choice to search for CDDW, mainly
because of its capacity to precisely locate anechoic lesions in the duodenal wall. We consider
conventional US as well a utile technique, because these patients are generally thin, thus allowing
visualization of the duodenal wall with a high-frequency probe through a transparietal approach ([Fig.
2] Fig. 2).

Cysts have fluid density on CT and high intensity signal on the T2-w sequences on MRI ([Fig. 6] Fig. 6).

2. Thickening of duodenal wall.

Thickening usually affects the inner border of the second portion ([Fig. 7] Fig. 7).

US, particularly endoscopic US, may enable visualization of the duodenal wall thickening ([Fig. 8] Fig. 8
).

However, CT and MRI allow more information, particularly after intravenous administration of iodine
or gadolinium, respectively. The duodenal wall may present a slow and late enhancement in the
venous phase and even more in the late phase ([Fig. 6] Fig. 6), presumably due to delayed blood
circulation caused by fibrous tissue proliferation and secondary arteries constriction.

This fibrous nature of the duodenal wall thickening also explains the low signal intensity observed in
all sequences on plain MRI.

3. Associated features.

Accompanying features suggestive of chronic pancreatitis are frequently observed ([Fig. 7] Fig. 7).
Some patients also present dilatation of the biliary tree.
Inflammatory changes may also result in narrowing of the duodenal lumen with gastric distension (
[Fig. 6] Fig. 6), a feature that may preclude EUS and ERCP due to the duodenal stenosis. This
emphasizes the importance of non-invasive methods, particularly MRCP, in the imaging workup of
these patients.

CDDW differential diagnosis:

Main CDDW differential diagnosis include:

1. Pancreatic tumors
2. Regional cystic lesions
3. Duodenal tumors
4. Other causes of wall thickening

1. Pancreatic tumors

The characteristic location and oblong morphology of CDDW is important for the radiological
differentiation from pancreatic tumors.

In addition, since the cysts originate from the duodenum, they may shift the gastroduodenal artery
forward and to the left ([Fig. 7] Fig. 7), exactly the opposite of what happens if the pathology
originates from the pancreatic head; in this case, the vessel is shifted to the right. The common bile
duct should also appear separate from the duodenum on MRCP.

Ductal adenocarcinoma

Although cystic spaces are not typical for pancreatic adenocarcinoma ([Fig. 9] Fig. 9), a cephalic tumor
with upstream duct dilatation and central tumor necrosis may mimic a cystic lesion. In addition,
distinction between fibrous tissue in CDDW and scirrhous adenocarcinoma of the pancreatic head
invading the groove is difficult on CT and MRI. Both lesions show hypointensity on T1-weighted
images and may also exhibit delayed enhancement at the late phase on dynamic CT and MRI.

In CDDW the main pancreatic duct may appear normal or it may exhibit only mild upstream dilatation.
However, this dilatation is not as marked as expected with adenocarcinoma. More frequently, CDDW
has associated ductal anomalies related to chronic pancreatitis ([Fig. 7] Fig. 7). Unfortunately,
radiological signs of chronic pancreatitis and pancreatic carcinoma show significant similarities. In this
setting the “penetrating duct sign” may be useful, particularly when there is a pseudotumoral
pancreatitis.

In addition, CDDW does not necessarily involve biliary obstruction, a feature expectable with a
cephalic ductal adenocarcinoma.

Widening of space between distal pancreatic and common bile ducts and duodenal lumen on MRCP is
another sign not commonly seen in cases of pancreatic cancer, but observed in most patients with
CDDW.

An important differentiating clue is the normal appearance of the peripancreatic vessels that may be
displaced in CDDW, but they do not show signs of obstruction or encasement ([Fig. 7] Fig. 7). In
contrast, pancreatic carcinoma is expected to invade along large peripancreatic vessels that are
obstructed or encased.

Cystic pancreatic tumors

The most challenging differential diagnosis of CDDW are cystic neoplasms of the pancreas, which
encompasses a wide variety of neoplasms, including serous cystadenoma, mucinous cystic neoplasm,
IPMT, solid and papillary epithelial neoplasm, and cystic islet cell tumor. Differential diagnosis may be
challenging if the pancreatic tumor is located near the pancreato-duodenal grove.

Serous cystadenoma is more frequent in women over the age of 60 years, has a typical honeycombed
appearance due to multiple cysts varying in size from 0,2 to 2,0 cm, and it may show a centralstellate
scar with calcification. Differential diagnosis is more difficult regarding the macrocystic variant of
serous cystadenoma ([Fig. 10] Fig. 10), which frequently is located in the pancreatic head.

Mucinous cystic neoplasms ([Fig. 11] Fig. 11) have large cystic spaces with multiple enhancing
septations and solid intramural nodules, a feature not observed in CDDW. Calcifications can be seen in
10-25% and are peripherally located.

IPMT tipically occur in elderly men. Its typical location in uncinate process, grapelike appearance, and
communication with the pancreatic duct (as detected with thin-section MDCT or MRCP) generally
allow diagnosis.

Solid and papillary epithelial neoplasm is tipically found in young women. The tumor may have a
variety of internal appearances, from purely cystic to completely solid, but is usually surrounded by a
thick, well-defined rim. The appearance of the internal architecture tipically depends on the degree of
hemorrhage and necrosis of the tumor.

Islet cell tumor may have a cystic appearance. Its solid component preserves the typical hypervascular
behaviour, allowing differentiation from CDDW.

2. Regional cystic lesions

Pseudocyst

A pancreatic pseudocyst ([Fig. 12] Fig. 12) generally presents as a unilocular round or oval fluid
collection following pancreatitis, trauma or surgery. As the majority of patients with CDDW have a
history of pancreatitis, pseudocyst is an important alternative diagnosis.

As mentioned earlier, some duodenal cysts in the setting of CDDW may present total erosion of the
cuboid epithelium, thus creating a pseudocyst-like appearance. Therefore, the location of the lesions
within the duodenal wall, which gives them an elongated or bilobate morphology, is the most
important distinguishing feature.

Choledocal cyst

Usually diagnosed in the postnatal period, choledocal cysts ([Fig. 13] Fig. 13) are more commonly
unilocular and its relation to the common bile duct is generally evident in MRCP.

Duodenal duplication cyst

Usually diagnosed in the postnatal period due to intestinal obstruction. Rarely may it be an incidental
finding at abdominal CT in the adult life, presenting as a well-circumscribed cystic mass with fluid
attenuation and a 1-2 mm thick wall of functional mucosa and a muscular layer of smooth muscle. It is
typically unilocular, without communication with the duodenal lumen.

Duodenal diverticulum

60% of duodenal diverticula are located in the 2nd portion, commonly within 2,0 cm of the ampulla of
Vater. It may resemble a masslike structure interposed between the duodenum and the pancreas.
Differential diagnosis must be considered when it contains fluid ([Fig. 14] Fig. 14). The presence of air,
air-fluid level, contrast material, or debris is unequivocal ([Fig. 15] Fig. 15).

Gastroduodenal artery pseudoaneurysm

A pseudoaneurysm of gastroduodenal artery ([Fig. 16] Fig. 16) is a potential complication of

pancreatitis and can present as a cystic lesion in the pancreatoduodenal grove. Diagnosis is allowed
by Doppler US or by contrast-enhanced CT or MRI, showing internal flow and continuity with the
artery.

3. Duodenal tumors

Duodenal tumors are solid lesions, generally without cystic component. However, a variety of
duodenal tumors may cause duodenal wall thickening, thus being included on differential diagnosis
of CDDW.

Duodenal GIST’s are usually small round or ovoid tumours with early and homogeneous enhancing
pattern ([Fig. 17] Fig. 17). Cyst formation reflects a rapid increase in size, more frequently representing
malignant transformation. Despite cystic changes, the solid component of the lesion remains
hypervascular, contrarily to CDDW ([Fig. 18] Fig. 18).

Duodenal lipomas are easily identified due to its homogeneous fat content (-50 to -100 HU) ([Fig. 19]
Fig. 19), very different from the fluid density of cyst content in CDDW.

Duodenal polyps are intraluminal filling defects with no associated bowel wall thickening or invasion.
They may be single or multiple, sessile or pedunculated, usually solid, allowing differential diagnosis
from CDDW. However, juvenile polyps have central fluid-filled cavities ([Fig. 20] Fig. 20) that must be
differentiated from CDDW.

Duodenal adenocarcinoma may manifest either as a polypoid mass or as an irregular, concentric or

asymmetric thickening over a short segment of the bowel wall causing deformity of the lumen ([Fig.
21] Fig. 21). Different degrees of bowel obstruction may be seen proximal to the tumour site. If the
tumour is located in the 2nd portion, obstruction of the biliary tract and pancreatic duct may occur.
Additional CT findings, such as invasion of retroperitoneal fat planes or surrounding organs, lymph
node enlargement, vascular encasement and distant metastases help in establishing diagnosis and
predicting tumour resectability.

Ampullary carcinoma may appear to protrude into the duodenal lumen in imaging ([Fig. 22] Fig. 22).
This is because ampullary carcinomas have a tendency toward intraluminal growth, with extraluminal
extension being relatively infrequent. However, bowel obstruction does not usually develop, even
with large tumours, perhaps because the lesions are soft. The tumour mass itself is often unapparent
at imaging due to its frequently small size. When a mass is not seen, marked and abrupt dilatation of
the distal bile duct may suggest the diagnosis. Bile duct dilatation may be accompanied by dilatation
of the pancreatic duct, a feature known as double duct sign.

Duodenal lymphoma may present with several imaging features. More frequently it appears as: a) a
large, segmental, circumferential wall thickening that seldom causes obstruction ([Fig. 23] Fig. 23); or
b) a large eccentric mass with necrosis, cavitation and extension into adjacent tissues. Aneurysmal
dilatation of the affected duodenal segment is a diagnostic clue. Additional lymphadenopathy and
splenomegaly also suggest the diagnosis. The presence of nodes on both sides of the plane of the
mesenteric vessels (the “sandwich” or “burger” sign) is a common finding in non-Hodgkin’s
lymphoma.
Duodenal neuroendocrine tumours may manifest as solitary/multiple duodenal polyps or as a mural
mass ([Fig. 24] Fig. 24). They enhance during the arterial and portal venous phases and experience
contrast washout during the equilibrium phase. This is an important feature to consider when imaging
patients suspected of having a duodenal neuroendocrine tumour or ampullary/ periampullary
abnormality because these lesions may be difficult to detect at CT when they are small. Contrarily to
those arising in the jejunum and ileum, duodenal tumours do not present with bowel kinking,
mesenteric desmoplasia, elastic vascular sclerosis, or intestinal ischemia. These differences reflect
absence of local serotonin production in the duodenal lesions.

4. Other causes of wall thickening

Groove pancreatitis

Groove pancreatitis ([Fig. 25] Fig. 25) is a form of segmental pancreatitis that affects the groove
between the head of pancreas, the duodenum, and the common bile duct. The microscopic features
of groove pancreatitis are similar to those observed in cystic dystrophy of the duodenal wall. Groove
pancreatitis is therefore believed to be also a complication of heterotopic pancreatic tissue. However,
in these cases cystic lesions can be absent.

Annular pancreas

Annular pancreas is a congenital abnormality that generally presents with duodenal obstruction in the
newborn period or early childhood. Cross sectional imaging is rarely needed for such diagnosis.

Duodenitis

Duodenitis from inflammation without ulcer formation is manifested by focal or diffuse thickening of
the duodenal wall. Imaging displays wall thickening and intense enhancement of the duodenal wall
due to active inflammation ([Fig. 26] Fig. 26). Cystic lesions are not a usual feature.

Duodenal ulcers

The finding of bowel wall thickening and adjacent inflammatory changes due to ulcer penetration is
non-specific. Wall thickening, however, rarely involves the mesenteric border of the 2nd duodenal
portion, because ulcer more often affects the duodenal bulb.

The diagnostic of perforation of a duodenal ulcer is more specific due to wall thickening, periduodenal
fluid, retroperitoneal gas ([Fig. 27] Fig. 27), free intraperitoneal gas ([Fig. 28] Fig. 28) or leakage of orally
administered contrast medium.

Duodenal Crohn’s disease

The presence of duodenal stenosis reflects stricturing disease. Parietal thickening with the target sign
is observed after iodine administration and is due to enhanced mucosa and serosa with a hypodense
and oedematous submucosa between them ([Fig. 29] Fig. 29). Imaging may also show fistulas,
abscesses and/or involvement of other bowel segments, features that support the correct diagnosis.

Secondary involvement from extrinsic inflammatory diseases

The most common inflammatory process affecting the duodenum is secondary involvement from
pancreatitis ([Fig. 30] Fig. 30) and, less frequently, from acute cholecystitis. Pancreatic inflammation
may be associated with a phlegmon along the groove and release of exocrine enzymes that cause
mild to severe duodenal oedema which may obstruct the gastric outlet. Clinical and laboratory
settings give the diagnosis.

Duodenal trauma

Duodenal trauma may present with thickening of the duodenal wall due to oedema ([Fig. 31] Fig. 31)
or haematoma. Generally, history provides the correct diagnosis.

4. Conclusion

CDDW is most probably an under-diagnosed disease that can be mistaken for other pathologies,
including malignancy.

In the majority of cases, however, state-of-the-art cross-sectional imaging can provide an accurate
non-invasive diagnosis, which may provide guidance to a proper management of patients.

5. References

1. Arrivé L, Saint-Maurice JP. CT features of cystic dystrophy of the duodenal wall. Radiology. 1998
Sep;208(3):830-1.
2. Chevallier P, Oddo F, Hastier P, Chevallier A, Diaine B, Coussement A. [Ultrasonographic and MRI
aspects of cystic duodenal dystrophy with aberrant pancreatic tissue]. J Radiol. 1999;80(1):50-2.
3. Galloro G, Napolitano V, Magno L, Diamantis G, Nardone G, Bruno M, Mollica C, Persico G.
Diagnosis and therapeutic management of cystic dystrophy of the duodenal wall in heterotopic
pancreas. A case report and revision of the literature. JOP. 2008 Nov 3;9(6):725-32.
4. Graziani R, Tapparelli M, Malago R, Girardi V, Frulloni L, Cavallini G, Pozzi Mucelli R. The various
imaging aspects of chronic pancreatitis. JOP. 2005;6(1 Suppl):73-88.
5. Jouannaud V, Coutarel P, Tossou H, Butel J, Vitte RL, Skinazi F, Blazquez M, Hagege H, Bories C,
Rocher P, Belloula D, Latrive JP, Meurisse JJ, Eugene C, Dellion MP, Cadranel JF, Pariente A;
Association Nationale des Hepato-Gastroenterologues des Hopitaux generaux. Cystic dystrophy
of the duodenal wall associated with chronic alcoholic pancreatitis. Clinical features, diagnostic
procedures and therapeutic management in a retrospective multicenter series of 23 patients.
Gastroenterol Clin Biol. 2006;30(4):580-6.
6. Jovanovic I, Knezevic S, Micev M, Krstic M. EUS mini probes in diagnosis of cystic dystrophy of
duodenal wall in heterotopic pancreas: a case report. World J Gastroenterol.
2004;10(17):2609-12.
7. Lopez-Pelaez MS, Hoyos FB, Isidro MG, Unzurrunzaga EA, Lopez Ede V, Collazo YQ. Cystic
dystrophy of heterotopic pancreas in stomach: radiologic and pathologic correlation. Abdom
Imaging. 2008 Jul-Aug;33(4):391-4.
8. Pessaux P, Lada P, Etienne S, Tuech JJ, Lermite E, Brehant O, Triau S, Arnaud JP.
Duodenopancreatectomy for cystic dystrophy in heterotopic pancreas of the duodenal wall.
Gastroenterol Clin Biol. 2006;30(1):24-8.
9. Procacci C, Graziani R, Zamboni G, Cavallini G, Pederzoli P, Guarise A, Bogina G, Biasiutti C,
Carbognin G, Bergamo-Andreis IA, Pistolesi GF. Cystic dystrophy of the duodenal wall: radiologic
findings. Radiology. 1997;205(3):741-7.
10. Thomas H, Marriott P, Portmann B, Heaton N, Rela M. Cystic dystrophy in heterotopic pancreas: a
rare indication for pancreaticoduodenectomy. Hepatobiliary Pancreat Dis Int. 2009
Apr;8(2):215-7.
11. Triantopoulou C, Dervenis C, Giannakou N, Papailiou J, Prassopoulos P. Groove pancreatitis: a
diagnostic challenge. Eur Radiol. 2009 Feb 24. [Epub ahead of print] PubMed PMID: 19238393.

6. Author Information
Jorge Brito, MD;

Luís Curvo-Semedo, MD;

Miguel Lima, MD;

João Filipe Costa, MD;

Belarmino Gonçalves, MD;

Bruno Graça, MD;

Miguel Seco, MD;

Filipe Caseiro-Alves, PhD.

7. Mediafiles

Fig. 1

A. Gastric heterotopic pancreas (arrowheads): a casual endoscopic finding in an asymptomatic

patient. Note pylorus (thin arrow).
Fig. 2

44-year-old alcoholic man with chronic pancreatitis. A-C. Transparietal US performed with
multi-frequency convex (A-B) and high-frequency linear (C) probes clearly depicts intramural cystic
lesions in the duodenal wall. Some lesions extend to the pyloric area and gastric antrum. Note
pancreatic calcifications due to chronic pancreatitis.
Fig. 3

61-year-old alcoholic man with chronic pancreatitis. A-C. Abdominal CT shows duodenal intramural
cyst-like lesion with fluid density (arrow). The cystic lesion narrow the duodenal lumen (asterisk).
Note pancreatic calcification due to chronic pancreatitis and a small amount of fluid surrounding
the duodenum.
Fig. 4

37-year-old alcoholic man. A-C. Abdominal CT shows parietal thickening and several cystic lesions
surrounding the duodenum (thin arrows), best evaluated after contrast administration (B-C). The
cysts have a subserosal distribution with a preferential localization in the mesenteric side, although
some of them reach the antimesenteric side of the 2nd portion of the duodenum. There is also a
small amount of fluid surrounding the duodenum. D. Transabdominal ultrasound shows a
mass-like lesion (arrows) with some cystic images in its interior. E. Endoscopic ultrasound showed
the cystic nature of the lesions but failed to demonstrate its precise subserosal location.
Fig. 5

75-year-old alcoholic man. A-C. Abdominal CT shows duodenal intramural cyst-like lesions with
fluid density (thin arrows), best depicted after iodinated contrast material administration (B). The
cysts narrow and shift the duodenal lumen (asterisk) to the right. The plurilobular morphology of
the lesion is best appreciated in the coronal MIP reconstruction (C). D-F. The cystic lesions (thin
arrows) present low signal intensity on T1-w (D) and high signal intensity on T2-w (E-F) images.
Fig. 6

45-year-old alcoholic man with chronic pancreatitis. A. Contrast-enhanced abdominal CT shows

gastric dilatation due to duodenal stenosis. B-E. Fat-supressed T2-weighted image (B) clearly shows
a cystic lesion (asterisks) between the pancreatic head and the duodenal loop. Gadolinium
enhanced T1-weighted images shows hypointense duodenal wall (arrowheads) in the arterial
phase (C), with progressive enhancement in the portal venous phase (D) and maximum
enhancement in the late phase (E), reflecting the presence of fibrous changes.
Fig. 7

34-year-old alcoholic man with chronic pancreatitis. A-C. Abdominal CT shows cyst-like lesion with
fluid density (asterisks) within a thickened duodenal wall. Note pancreatic calcifications and main
pancreatic duct dilatation due to chronic pancreatitis. D. Coronal MPR from the same study clearly
shows that the gastroduodenal artery is located to the left of the cystic lesions, supporting a
duodenal origin.
Fig. 8

34-year-old alcoholic man with chronic pancreatitis (same patient of Fig. 7). A-C. Abdominal CT
performed 6 months later shows wall thickening of the medial border of the second duodenal
portion (arrowheads). The thickened wall has no longer cystic lesions, although a tiny hypodense
image is still identified. D. These imaging features are also observed in endoscopic US: an
asymmetrically thickened duodenal wall (arrowheads) with a tiny hypoechoic lesion inside,
presumably corresponding to an empty cystic cavity.
Fig. 9

80-year-old woman with biliary obstruction. A. Ultrasound sowed a hypoechoic mass in the
pancreatic head (arrow). B-D. CT shows the soft tissue density of the mass (arrows), which remains
hypodense during all phases of the dynamic study. CT clearly reveals tumour invasion of the
medial wall of the 2nd portion of the duodenum, best recognised in the arterial phase. E-F.
Percutaneous transhepatic cholangiogram shows complete obstruction of biliary duct by the
tumour. A percutaneously applied metallic prosthesis crossed the tumour re-establishing
bilioenteric flow.
Fig. 10

Macrocystic serous cystadenoma. A. Ultrasound sows a complex cystic lesion in the pancreatic area.
B-C. CT shows a multiloculated cystic mass with thin septa and no evidence of internal vegetations.
Although it is centred in the pancreatic head, it is extremely difficult to achieve a non-invasive
diagnostic of this lesion.
Fig. 11

Mucinous cystic neoplasm. A-C. CT shows a multiloculated cystic mass with enhancing septa and
internal nodules. The lesion is centred in the pancreatic head and shows scattered calcifications.
Fig. 12

Pancreatic pseudocyst. A. Contrast-enhanced CT shows a unilocular cyst with a smooth thin wall,
associated to inflammatory changes of the peri-pancreatic fat.
Fig. 13

Type I-A choledochal cyst. A. Projective MR cholangiogram shows saccular dilatation involving
most of the common bile duct.
Fig. 14

63-year-old woman with duodenal diverticulum. A-B. Abdominal CT shows a sacular outpouching
from the medial wall of the 2nd portion of the duodenum containing an air-fluid level
(arrowheads). The thin and regular borders of the diverticulum enhance after iodine administration
(B). Note the relationship with the choledocal (thick arrow) and pancreatic (thin arrow) ducts. C-D.
Coronal thick-slab heavily T2-w image (C) shows a fluid-filled lesion (arrowhead), which clearly
shows a typical air-fluid level on the axial T2-w image (arrowhead in D).
Fig. 15

A. 67-year-old man. The duodenal diverticulum (arrowhead) is filled with gas. B. 54-year-old man.
The duodenal diverticulum (arrowhead) is filled with debris and gas, resembling the “small bowel
faeces sign”.
Fig. 16

Man with recent history of acute pancreatitis. A. US reveals a cystic lesion (arrowhead) in the
pancreatoduodenal groove. B. Doppler interrogation reveals flow inside the lesion. C-D. Plain CT (C)
shows a mass-like image adjacent to the cephalic portion of the pancreas, which presents intense
arterial enhancement (arrowhead) after iodine administration (D). E. MRI angiography clearly
depicts the pseudoaneurysm (arrowhead) with origin in the gastroduodenal artery.
Fig. 17

65-year-old woman presenting with melenae and iron deficiency anaemia. A. Barium study shows
well-circumscribed mass (arrow) in the transition from the 2nd to the 3rd portion of the duodenal
loop. B. Endoscopy confirms the presence of a submucosal tumour (curved arrow) covered with
normal mucosa. C-E. Abdominal CT study before (C) and after (D) iodine administration shows a
well-defined soft tissue mass (arrowheads) with intense and homogeneous enhancement. The
absence of exoenteric growth is best appreciated in coronal MPR image (E). F. Histological analysis
(100x) reveals c-kit (CD117) expression by tumour cells.
Fig. 18

54-year-old woman recently explored for biliary disease. During surgery a retroperitoneal tumour
was identified with evidence of intra-abdominal spread. A-D. Contrast-enhanced CT shows a large
well-circumscribed duodenal tumour with a primarily exoenteric growth pattern and lobulated
contour (arrowheads). The tumour presents a strong predominantly peripheral enhancement in
the arterial phase of the dynamic study (A) with central unenhanced areas even in the venous
phase (B), a feature related with haemorrhage and necrosis. Coronal MIP (C) clearly shows increase
in the number and calibre of the vessels (curved arrow) supplying the lesion (arrowheads) and a
small hypervascular nodule in the wall of jejunum representing metastasis (thin arrow). Oblique
MPR (D) shows several small hypervascular parietal metastases in the 2nd portion of the
duodenum (thin arrows), in addition to the large tumour with exoenteric growth pattern
(arrowheads). E. PET-CT shows abnormal FDG metabolism in the tumour but failed to detect
metastases. F. Endoscopy reveals a voluminous submucosal tumour with a bleeding ulcerated area
(curved arrow).
Fig. 19

A-C. Incidental CT finding in a 66-year-old woman. A. Plain abdominal CT shows a polypoid mass in
the descending duodenum with homogeneous fat attenuation (arrowhead). B. After intravenous
iodine administration the lesion does not enhance (arrowhead). C. Endoscopic ultrasound shows
the parietal mass (arrowheads) with a heterogeneously echogenic texture suggestive of fat
content. Due to its characteristic attenuation on CT, the mass was diagnosed as lipoma, remaining
stable for the last 3 years. D-E. Incidental CT finding in a 67-year-old woman with uterine
malignancy. D. Contrast enhanced CT shows a mass with homogeneous fat attenuation in the
horizontal duodenum (arrow). E. Coronal T2-w image (HASTE) shows the intraluminal mass (arrow)
with high signal intensity similar to mesenteric fat.
Fig. 20

46-year-old man with juvenile polyposis of the stomach and duodenum. A. Abdominal X-ray shows
a stomach with augmented volume (arrowheads) due to gastric outflow obstruction. B-C. Plain (B)
and contrast-enhanced CT (C) shows several polyps. The biggest is located in the duodenal bulb
and presents central fluid density (asterisk), a feature also observed in endoscopic ultrasound (D) as
anechoic images corresponding to fluid-filled cavities (asterisk). E. Endoscopy frame shows the
multiplicity of polypoid lesions. F. Gross specimen clearly shows the major polyp (asterisk)
prolapsed through the pylorus (thick arrows) to the duodenal bulb. G. Histology showing the
empty cavities of the polyps (asterisks).
Fig. 21

67-year-old man with melenae and abdominal pain. A-C. Abdominal CT performed before (A) and
after (B) intravenously administered iodinated contrast shows an irregular, asymmetric thickening
of the 3rd portion of duodenum, causing deformity of the lumen (arrowheads). Encasement of the
superior mesenteric artery (thin arrow) turns the lesion surgically unresectable. C. Adjacent slice
shows also encasement and thrombosis of the superior mesenteric vein (thick arrow). D.
Endoscopy clearly shows a bleeding neoplasm and provides material to histological analysis.
Fig. 22

55-year-old woman with ampullary carcinoma. A-C. Plain (A) and contrast-enhanced CT (B-C)
reveals a soft-tissue mass (arrowheads) originating in the medial wall of the descending duodenum
protruding into the water-filled duodenum. D. Contrast-enhanced CT shows dilatation of the main
pancreatic duct and of the common bile duct, a feature known as the “double-duct sign”. E.
Endoscopic US shows a mass (arrowheads) involving the papilla, with internal vegetations (thin
arrow) in the lumen of the common bile duct.
Fig. 23

64-year-old woman with duodenal lymphoma. A-B. Contrast-enhanced abdominal CT shows

concentric thickening of the duodenal wall (arrowheads) with irregular narrowing of the lumen.
Lymphadenopathy (thin arrows) also suggests the diagnosis of lymphoma. C. Barium study shows
widening of the C-loop with mucosal irregularity but absent obstructive signs. D. Endoscopy shows
luminal narrowing and extense mucosal ulceration. E. Histology (H&E 100x) revealed a
non-Hodgkin’s T-cell type lymphoma.
Fig. 24

85-year-old woman with von Recklinghausen's disease. A-B. Abdominal CT shows a mass (arrows)
in the medial wall of the 2nd portion of the duodenum. The tumour displays intense contrast
enhancement in the arterial phase (A), with retention in the venous phase (B) of the dynamic study.
The high spatial resolution provided by CT clearly shows that the lesion is independent from the
pancreas. C-G. The lesion (arrows) displays low signal intensity on T1-w (C) and high signal intensity
on T2-w (D) images. The dynamic study following gadolinium shows intense enhancement of the
lesion in the arterial phase (E), with retention in the portal venous phase (F) and some loss during
the equilibrium phase (G). H-I. The lesion (arrows) is well-defined and hypoechoic either in
trans-abdominal (H) as in endoscopic ultrasound (I). J. Somatostatin-receptor (octreotide)
scintigraphy confirms the neuroendocrine nature of the tumour.
Fig. 25

A-C. 40-year-old alcoholic man with groove pancreatitis. Despite the clinical and laboratory
findings highly suggestive of pancreatitis, CT reveals the pancreas with normal volume,
morphology and texture. There is retroperitoneal fluid in the right anterior pararenal space and in
the pancreatoduodenal groove (thin arrows). The posterior wall of the descending duodenum is
slightly thickened and the pancreatoduodenal groove reveals discrete late enhancement
suggestive of fibrotic changes. Endoscopy revealed a normal duodenal mucosa. D. Another case of
groove pancreatitis shows the gastroduodenal artery (thin arrow) shifted to the left, reflecting the
duodenal origin of the pathologic process.
Fig. 26

50-year-old alcoholic man with chronic pancreatitis. A-E. CT shows marked thickening and intense
enhancement of the duodenal wall observed in both the arterial (A) and portal venous phase (B-E),
displaying a pseudo-tumoral appearance (arrows). Note also ascites and severe gastritis, which
manifests by thickening and layering of the gastric wall with enhancing mucosa and low
attenuating submucosa due to oedema and inflammation. The retroperitoneal fluid collection in
(A) corresponds to a pancreatic pseudocyst.
Fig. 27

76-year-old male with perforated duodenal peptic ulcer. A-B. Endoscopy shows a large, deep,
necrotic ulceration on the posterior wall of duodenal bulb, prone to perforation. C. The upper GI
study performed with water-soluble contrast agent shows an apparently retroperitoneal leakage of
contrast medium (thick arrow). D. Plain abdominal CT clearly shows the site of the duodenal
perforation (thin arrows) and accumulation of gas and contrast in the right anterior pararenal space
(asterisk).
Fig. 28

80-year-old female with perforated duodenal peptic ulcer. A-C. Contrast-enhanced CT shows
parietal thickening of the duodenal bulb (curved arrow) and small amount of intraperitoneal gas
(thin arrows). D. Chest X-ray shows a small amount of gas beneath the right hemidiaphragm (thin
arrow). At surgery a perforated duodenal ulcer was identified. E. Histology (H&E 50x) shows
inflammatory changes with granulation tissue (thick arrows) and necrosis corresponding to the
margins of the perforation.
Fig. 29

46-year-old woman with Crohn’s disease. A-B. Contrast-enhanced abdominal CT shows gastric
outlet obstruction (A) and a stricture in the 2nd portion of the duodenum (B). The strictured
segment shows the target sign (curved arrow), which corresponds to the enhanced mucosa and
serosa with a hypodense and oedematous submucosa between them. C. Barium study shows the
short stricture (curved arrow) and dilatation of the proximal duodenum. D. Pathologic study of the
surgical specimen revealed typical transparietal features of Crohn’s disease, with a very thickened
submucosal layer.
Fig. 30

A-B. 45-year-old man with acute pancreatitis. The inflammatory process centred in the pancreas
involves also the duodenal loop, which presents a slightly thickened wall and a hyperenhanced
mucosa. Note the gastroduodenal artery (thin arrow) shifted to the right, reflecting the pancreatic
origin of the pathologic process.
Fig. 31

35-year-old female victim of car accident. A. Chest X-ray shows gastric tube with a thoracic loop
(thin arrow). B. Contrast-enhanced CT confirms traumatic diaphragmatic hernia; note the
“dependent viscera sign” (arrowheads). C. The 2nd portion of duodenum is surrounded by fluid
and its wall presents the target sign (thick arrow). This target sign is composed of inner and outer
rings of high attenuation, corresponding to the preferentially enhancing mucosa and muscularis
propria, respectively. Between them lies a middle ring of gray attenuation representing oedema in
the submucosa. A myriad of pathologic conditions can produce this sign, representing acute bowel
injury. D. The upper GI study shows uniform, regular, fold thickening.