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DOI: 10.1148/rg.263055164
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Right arrow Gastrointestinal Radiology

Multimodality Imaging of Pancreatic and Biliary Congenital Anomalies1

Koenraad J. Mortelé, MD, Tatiana C. Rocha, MD, Jonathan L. Streeter, MD, and Andrew J. Taylor, MD

1 From the Department of Radiology, Division of Abdominal Imaging and Intervention (K.J.M., J.L.S.), and the Department of Radiology, 3D and Image Processing Center (T.C.R.), Brigham and Women’s Hospital, Harvard Medical School, 75 Francis St, Boston, MA 02115; and the Department of Radiology, Clinical Science Center, University of Wisconsin, Madison, Wis (A.J.T.). Presented as an education exhibit at the 2004 RSNA Annual Meeting. Received August 18, 2005; revision requested October 5 and received January 11, 2006; accepted January 11. All authors have no financial relationships to disclose.

Figure 1A
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Figure 1a.  Drawings illustrate the normal embryologic development of the pancreas and biliary tree. The ventral pancreatic bud (arrow in a and b) and biliary system arise from the hepatic diverticulum, and the dorsal pancreatic bud (arrowhead in a and b) arises from the dorsal mesogastrium. (c) After clockwise rotation of the ventral bud around the caudal part of the foregut, there is fusion of the dorsal pancreas (located anterior) and ventral pancreas (located posterior). (d) Finally, the ventral and dorsal pancreatic ducts fuse, and the pancreas is predominantly drained through the ventral duct, which joins the common bile duct (CBD) at the level of the major papilla. The dorsal duct empties at the level of the minor papilla.

 

Figure 1B
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Figure 1b.  Drawings illustrate the normal embryologic development of the pancreas and biliary tree. The ventral pancreatic bud (arrow in a and b) and biliary system arise from the hepatic diverticulum, and the dorsal pancreatic bud (arrowhead in a and b) arises from the dorsal mesogastrium. (c) After clockwise rotation of the ventral bud around the caudal part of the foregut, there is fusion of the dorsal pancreas (located anterior) and ventral pancreas (located posterior). (d) Finally, the ventral and dorsal pancreatic ducts fue, and the pancreas is predominantly drained through the ventral duct, which joins the common bile duct (CBD) at the level of the major papilla. The dorsal duct empties at the level of the minor papilla.

 

Figure 1C
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Figure 1c.  Drawings illustrate the normal embryologic development of the pancreas and biliary tree. The ventral pancreatic bud (arrow in a and b) and biliary system arise from the hepatic diverticulum, and the dorsal pancreatic bud (arrowhead in a and b) arises from the dorsal mesogastrium. (c) After clockwise rotation of the ventral bud around the caudal part of the foregut, there is fusion of the dorsal pancreas (located anterior) and ventral pancreas (located posterior). (d) Finally, the ventral and dorsal pancreatic ducts fuse, and the pancreas is predominantly drained through the ventral duct, which joins the common bile duct (CBD) at the level of the major papilla. The dorsal duct empties at the level of the minor papilla.

 

Figure 1D
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Figure 1d.  Drawings illustrate the normal embryologic development of the pancreas and biliary tree. The ventral pancreatic bud (arrow in a and b) and biliary system arise from the hepatic diverticulum, and the dorsal pancreatic bud (arrowhead in a and b) arises from the dorsal mesogastrium. (c) After clockwise rotation of the ventral bud around the caudal part of the foregut, there is fusion of the dorsal pancreas (located anterior) and ventral pancreas (located posterior). (d) Finally, the ventral and dorsal pancreatic ducts fuse, and the pancreas is predominantly drained through the ventral duct, which joins the common bile duct (CBD) at the level of the major papilla. The dorsal duct empties at the level of the minor papilla.

 

Figure 2A
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Figure 2a.  Normal pancreatic anatomy. (a) Axial reformatted computed tomographic (CT) image shows the typical size, location, and lobulated appearance of the normal pancreas. (b) CT scan shows the normal triangular appearance of the uncinate process (arrow), with a straight posteromedial border and a gently concave anteromedial border (arrowhead). (c) Drawing illustrates the various parts (head, neck, body, and tail) of the pancreas.

 

Figure 2B
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Figure 2b.  Normal pancreatic anatomy. (a) Axial reformatted computed tomographic (CT) image shows the typical size, location, and lobulated appearance of the normal pancreas. (b) CT scan shows the normal triangular appearance of the uncinate process (arrow), with a straight posteromedial border and a gently concave anteromedial border (arrowhead). (c) Drawing illustrates the various parts (head, neck, body, and tail) of the pancreas.

 

Figure 2C
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Figure 2c.  Normal pancreatic anatomy. (a) Axial reformatted computed tomographic (CT) image shows the typical size, location, and lobulated appearance of the normal pancreas. (b) CT scan shows the normal triangular appearance of the uncinate process (arrow), with a straight posteromedial border and a gently concave anteromedial border (arrowhead). (c) Drawing illustrates the various parts (head, neck, body, and tail) of the pancreas.

 

Figure 3A
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Figure 3a.  Normal pancreatic ductal anatomy. (a) Drawing illustrates the MPD, the duct of Wirsung, and the duct of Santorini. The latter empties at the minor papilla. (b) Coronal magnetic resonance (MR) cholangiopancreatogram shows the normal bifid configuration of the pancreas with main drainage of the gland through the duct of Wirsung (arrow). The duct of Santorini (arrowhead) drains into the minor papilla. (c) Coronal MR cholangiopancreatogram shows a narrowed intersphincteric segment of the CBD (arrow) and pancreatic duct due to contraction of the sphincter of Oddi. (d) Drawing illustrates the sphincter of Oddi complex (arrow) encompassing the distal CBD and pancreatic duct.

 

Figure 3B
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Figure 3b.  Normal pancreatic ductal anatomy. (a) Drawing illustrates the MPD, the duct of Wirsung, and the duct of Santorini. The latter empties at the minor papilla. (b) Coronal magnetic resonance (MR) cholangiopancreatogram shows the normal bifid configuration of the pancreas with main drainage of the gland through the duct of Wirsung (arrow). The duct of Santorini (arrowhead) drains into the minor papilla. (c) Coronal MR cholangiopancreatogram shows a narrowed intersphincteric segment of the CBD (arrow) and pancreatic duct due to contraction of the sphincter of Oddi. (d) Drawing illustrates the sphincter of Oddi complex (arrow) encompassing the distal CBD and pancreatic duct.

 

Figure 3C
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Figure 3c.  Normal pancreatic ductal anatomy. (a) Drawing illustrates the MPD, the duct of Wirsung, and the duct of Santorini. The latter empties at the minor papilla. (b) Coronal magnetic resonance (MR) cholangiopancreatogram shows the normal bifid configuration of the pancreas with main drainage of the gland through the duct of Wirsung (arrow). The duct of Santorini (arrowhead) drains into the minor papilla. (c) Coronal MR cholangiopancreatogram shows a narrowed intersphincteric segment of the CBD (arrow) and pancreatic duct due to contraction of the sphincter of Oddi. (d) Drawing illustrates the sphincter of Oddi complex (arrow) encompassing the distal CBD and pancreatic duct.

 

Figure 3D
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Figure 3d.  Normal pancreatic ductal anatomy. (a) Drawing illustrates the MPD, the duct of Wirsung, and the duct of Santorini. The latter empties at the minor papilla. (b) Coronal magnetic resonance (MR) cholangiopancreatogram shows the normal bifid configuration of the pancreas with main drainage of the gland through the duct of Wirsung (arrow). The duct of Santorini (arrowhead) drains into the minor papilla. (c) Coronal MR cholangiopancreatogram shows a narrowed intersphincteric segment of the CBD (arrow) and pancreatic duct due to contraction of the sphincter of Oddi. (d) Drawing illustrates the sphincter of Oddi complex (arrow) encompassing the distal CBD and pancreatic duct.

 

Figure 4A
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Figure 4a.  CT scans show the three possible anatomic relationships between the CBD (arrow) and the pancreas: partial coverage of the CBD by pancreatic tissue (a), total coverage (b), and no coverage (c).

 

Figure 4B
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Figure 4b.  CT scans show the three possible anatomic relationships between the CBD (arrow) and the pancreas: partial coverage of the CBD by pancreatic tissue (a), total coverage (b), and no coverage (c).

 

Figure 4C
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Figure 4c.  CT scans show the three possible anatomic relationships between the CBD (arrow) and the pancreas: partial coverage of the CBD by pancreatic tissue (a), total coverage (b), and no coverage (c).

 

Figure 5
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Figure 5.  Normal biliary anatomy. Coronal MR cholangiopancreatogram shows the left hepatic duct (LHD) and RHD (long arrow) forming the common hepatic duct (CHD). Note that the RHD is formed by two branches: the RPD (short arrow), which drains posterior segments VI and VII; and the RAD (arrowhead), which drains anterior segments V and VIII.

 

Figure 6A
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Figure 6a.  Variant pancreatic ductal anatomy. Coronal MR cholangiopancreatograms show a dominant dorsal duct with a "santorinicele" (arrow in a) and ansa pancreatica (arrow in b).

 

Figure 6B
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Figure 6b.  Variant pancreatic ductal anatomy. Coronal MR cholangiopancreatograms show a dominant dorsal duct with a "santorinicele" (arrow in a) and ansa pancreatica (arrow in b).

 

Figure 7
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Figure 7.  Variant pancreatic ductal anatomy. MR cholangiopancreatogram shows a change in the caliber of the pancreatic duct (arrow) at the level of fusion between the MPD and the duct of Wirsung. Note the lack of upstream dilatation, which indicates that this change in caliber represents an anatomic variant.

 

Figure 8
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Figure 8.  Variant pancreatic ductal anatomy. Coronal endoscopic retrograde cholangiopancreatographic (ERCP) image shows duplicate pancreatic ducts (arrows) in the tail of the pancreas.

 

Figure 9A
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Figure 9a.  Variant biliary ductal anatomy. Coronal MR cholangiopancreatograms show the RPD (arrow in a) draining into the LHD and a medial and low insertion of the cystic duct (arrow in b).

 

Figure 9B
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Figure 9b.  Variant biliary ductal anatomy. Coronal MR cholangiopancreatograms show the RPD (arrow in a) draining into the LHD and a medial and low insertion of the cystic duct (arrow in b).

 

Figure 10A
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Figure 10a.  Abnormal common channel. (a) Drawing illustrates a long common channel (>15 mm). Note that the sphincter of Oddi does not reach the confluence (arrow) of the ducts. (b) ERCP image obtained in a child shows a long common channel (arrowhead) and the presence of a CBD web (arrow).

 

Figure 10B
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Figure 10b.  Abnormal common channel. (a) Drawing illustrates a long common channel (>15 mm). Note that the sphincter of Oddi does not reach the confluence (arrow) of the ducts. (b) ERCP image obtained in a child shows a long common channel (arrowhead) and the presence of a CBD web (arrow).

 

Figure 11A
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Figure 11a.  Pancreas divisum. (a) Coronal MR cholangiopancreatogram shows drainage of the pancreas through the duct of Santorini (arrow). Note that the MPD is not fused with the duct of Wirsung (arrowhead). (b) CT scan obtained in a patient with acute recurrent pancreatitis and pancreas divisum shows a dilated duct of Santorini (arrowhead).

 

Figure 11B
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Figure 11b.  Pancreas divisum. (a) Coronal MR cholangiopancreatogram shows drainage of the pancreas through the duct of Santorini (arrow). Note that the MPD is not fused with the duct of Wirsung (arrowhead). (b) CT scan obtained in a patient with acute recurrent pancreatitis and pancreas divisum shows a dilated duct of Santorini (arrowhead).

 

Figure 12A
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Figure 12a.  Annular pancreas. (a) Axial T2-weighted MR image shows the pancreas (arrow) encircling the descending portion of the duodenum. (b) Coronal MR cholangiopancreatogram shows the duct of Wirsung (arrowhead) encircling the duodenum.

 

Figure 12B
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Figure 12b.  Annular pancreas. (a) Axial T2-weighted MR image shows the pancreas (arrow) encircling the descending portion of the duodenum. (b) Coronal MR cholangiopancreatogram shows the duct of Wirsung (arrowhead) encircling the duodenum.

 

Figure 13
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Figure 13.  Ectopic pancreas. CT scan obtained in a patient with abdominal pain shows ectopic pancreatic tissue (arrow) within the small bowel mesentery. Surgery helped confirm the presence of an inflamed ectopic pancreas.

 

Figure 14A
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Figure 14a.  Pancreatic hypoplasia. (a) CT scan shows the pancreatic head (arrow) and absence of the pancreatic neck, body, and tail. (b) CT scan obtained in a patient with heterotaxia syndrome shows a truncated mid-line pancreas (arrow).

 

Figure 14B
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Figure 14b.  Pancreatic hypoplasia. (a) CT scan shows the pancreatic head (arrow) and absence of the pancreatic neck, body, and tail. (b) CT scan obtained in a patient with heterotaxia syndrome shows a truncated mid-line pancreas (arrow).

 

Figure 15A
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Figure 15a.  Von Hippel–Lindau disease. (a) Contrast material–enhanced CT scan shows enhancing septa separating congenital pancreatic cysts. (b) Axial T2-weighted MR image shows cystic replacement of the pancreas and a serous microcystic pancreatic adenoma (arrow).

 

Figure 15B
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Figure 15b.  Von Hippel–Lindau disease. (a) Contrast material–enhanced CT scan shows enhancing septa separating congenital pancreatic cysts. (b) Axial T2-weighted MR image shows cystic replacement of the pancreas and a serous microcystic pancreatic adenoma (arrow).

 

Figure 16A
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Figure 16a.  Choledochal cysts. (a, b) Drawing (a) and coronal MR cholangiopancreatogram (b) show a type I choledochal cyst (arrow in b). (c, d) Drawing (c) and ERCP image (d) show a diverticulum (arrow in d) of the EBD, a finding that represents a type II choledochal cyst. (e, f ) Drawing (e) and ERCP image (f ) show a choledochocele, or type III choledochal cyst (arrow in f ).

 

Figure 16B
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Figure 16b.  Choledochal cysts. (a, b) Drawing (a) and coronal MR cholangiopancreatogram (b) show a type I choledochal cyst (arrow in b). (c, d) Drawing (c) and ERCP image (d) show a diverticulum (arrow in d) of the EBD, a finding that represents a type II choledochal cyst. (e, f ) Drawing (e) and ERCP image (f ) show a choledochocele, or type III choledochal cyst (arrow in f ).

 

Figure 16C
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Figure 16c.  Choledochal cysts. (a, b) Drawing (a) and coronal MR cholangiopancreatogram (b) show a type I choledochal cyst (arrow in b). (c, d) Drawing (c) and ERCP image (d) show a diverticulum (arrow in d) of the EBD, a finding that represents a type II choledochal cyst. (e, f ) Drawing (e) and ERCP image (f ) show a choledochocele, or type III choledochal cyst (arrow in f ).

 

Figure 16D
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Figure 16d.  Choledochal cysts. (a, b) Drawing (a) and coronal MR cholangiopancreatogram (b) show a type I choledochal cyst (arrow in b). (c, d) Drawing (c) and ERCP image (d) show a diverticulum (arrow in d) of the EBD, a finding that represents a type II choledochal cyst. (e, f ) Drawing (e) and ERCP image (f ) show a choledochocele, or type III choledochal cyst (arrow in f ).

 

Figure 16E
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Figure 16e.  Choledochal cysts. (a, b) Drawing (a) and coronal MR cholangiopancreatogram (b) show a type I choledochal cyst (arrow in b). (c, d) Drawing (c) and ERCP image (d) show a diverticulum (arrow in d) of the EBD, a finding that represents a type II choledochal cyst. (e, f ) Drawing (e) and ERCP image (f ) show a choledochocele, or type III choledochal cyst (arrow in f ).

 

Figure 16F
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Figure 16f.  Choledochal cysts. (a, b) Drawing (a) and coronal MR cholangiopancreatogram (b) show a type I choledochal cyst (arrow in b). (c, d) Drawing (c) and ERCP image (d) show a diverticulum (arrow in d) of the EBD, a finding that represents a type II choledochal cyst. (e, f ) Drawing (e) and ERCP image (f ) show a choledochocele, or type III choledochal cyst (arrow in f ).

 

Figure 17A
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Figure 17a.  (a, b) Drawing (a) and coronal MR cholangiopancreatogram (b) show type IV choledochal cysts. (c, d) Drawing (c) and ERCP image (d) show a type V choledochal cyst (Caroli disease). (e) Caroli disease. Contrast-enhanced T1-weighted MR image shows saccular dilatation of the IBD and the "central dot sign" (arrow).

 

Figure 17B
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Figure 17b.  (a, b) Drawing (a) and coronal MR cholangiopancreatogram (b) show type IV choledochal cysts. (c, d) Drawing (c) and ERCP image (d) show a type V choledochal cyst (Caroli disease). (e) Caroli disease. Contrast-enhanced T1-weighted MR image shows saccular dilatation of the IBD and the "central dot sign" (arrow).

 

Figure 17C
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Figure 17c.  (a, b) Drawing (a) and coronal MR cholangiopancreatogram (b) show type IV choledochal cysts. (c, d) Drawing (c) and ERCP image (d) show a type V choledochal cyst (Caroli disease). (e) Caroli disease. Contrast-enhanced T1-weighted MR image shows saccular dilatation of the IBD and the "central dot sign" (arrow).

 

Figure 17D
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Figure 17d.  (a, b) Drawing (a) and coronal MR cholangiopancreatogram (b) show type IV choledochal cysts. (c, d) Drawing (c) and ERCP image (d) show a type V choledochal cyst (Caroli disease). (e) Caroli disease. Contrast-enhanced T1-weighted MR image shows saccular dilatation of the IBD and the "central dot sign" (arrow).

 

Figure 17E
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Figure 17e.  (a, b) Drawing (a) and coronal MR cholangiopancreatogram (b) show type IV choledochal cysts. (c, d) Drawing (c) and ERCP image (d) show a type V choledochal cyst (Caroli disease). (e) Caroli disease. Contrast-enhanced T1-weighted MR image shows saccular dilatation of the IBD and the "central dot sign" (arrow).

 

Figure 18A
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Figure 18a.  Uneven fatty replacement of the pancreas. (a) Drawings illustrate the four different patterns of fatty replacement of the pancreas (gray areas). The percentage of cases of uneven pancreatic lipomatosis represented by each type is also indicated. (b) CT scan shows type 1a fatty replacement of the pancreatic head (arrow).

 

Figure 18B
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Figure 18b.  Uneven fatty replacement of the pancreas. (a) Drawings illustrate the four different patterns of fatty replacement of the pancreas (gray areas). The percentage of cases of uneven pancreatic lipomatosis represented by each type is also indicated. (b) CT scan shows type 1a fatty replacement of the pancreatic head (arrow).

 

Figure 19A
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Figure 19a.  Pancreatic head configurations. (a) Drawings illustrate the normal appearance of the pancreatic head and the three variant (pseudomass) appearances. (b) CT scan shows a posterior configuration (arrow).

 

Figure 19B
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Figure 19b.  Pancreatic head configurations. (a) Drawings illustrate the normal appearance of the pancreatic head and the three variant (pseudomass) appearances. (b) CT scan shows a posterior configuration (arrow).

 





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