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Complete Agenesis of the Dorsal Pancreas

Images

Fig 1: US Abdomen

Fig 2a: Gadolinium Enhanced MRI

Fig 2b:Gadolinium Enhanced MRI

Fig 3a: T2-weighted MRI

Fig3b: MRCP


Clinical History:

A 36-year-old man referred to the emergency department for epigastric pain after large meals.


Findings:

Figure 1: Abdominal US of the pancreas in an axial plane reveals hypoechoic pancreatic head in its normal position, but the body and tail not adequately visualized. Figure 2a: Axial contrast enhanced T1-weighted MR image shows enlarged and enhancing pancreatic head (arrow). Figure 2b: Axial contrast enhanced T1-weighted MR image at the level of splenic vein shows non visualization of pancreatic body and tail. Figure 3a: Axial SSFSE MR image demonstrates bright pancreatic duct in the head of the pancreas (arrow) Figure 3b: Coronal MRCP revealed isolated pancreatic duct in the head of the pancreas (arrow). No evidence of dorsal pancreatic duct.


Diagnosis:

Complete Agenesis of the Dorsal Pancreas


Discussion:

Anomalies of pancreas are occasionally reported. The mechanism and etiology are unknown. The relatively complicated embryogenesis of this organ- with its two ventral buds and dorsal bud rotation of the right ventral bud around the duodenum and eventually fusion with the dorsal analage, all takes place with a very low frequency of anatomic variation. Complete agenesis of pancreas are extremely rare, in which there is a regression of dorsal pancreatic duct of endodermal cells which normally arises from posterior duodenal wall to form upper portion of pancreas. This dorsal bud usually provides upper part of the head, the isthmus, body, tail of the pancreas and drains through santorini duct and minor papilla. The ventral bud forms the major part of the head and uncinate process. In patients with complete agenesis of the dorsal pancreas, neck, body, tail of the pancreas, accessory pancreatic duct (duct of Santorini), and the minor duodenal papilla are absent. While in partal agenesis, there is a remnant of the duct of santorini, and the minor duodenal papilla.US is the first modality for abdominal pain. But pancreas is sometimes not adequately visualized due to excessive bowel gas disturbances. While CT is valuable in depicting the pathology but information regarding the pancreatic duct anatomy on these images are difficult. MRI including MRCP is rapid and non invasive method of evaluation not only the pancreatic ductal system but also the parenchymal abnormality. It is important to recognize this anomaly because it can stimulate pseudotumoral enlargement of pancreatic head which has been noted in other congenital pancreatic anomalies such as annular and divisum pancreas. ERCP was once thought to be the gold standard in evaluating biliary tree due its superior resolution. However this technique is invasive not devoid of morbidity risk, operator dependent and also it requires catheterization of the minor duodenal papilla. But current improved MRCP using single shot rapid acquisition with relaxation is superior for conspicuity of the pancreatic duct. In summary with current noninvasive imaging modalities, we anticipate more cases will come into view. Hence better understanding of such rare congenital anomaly will be achieved.


References / Suggested Reading:

1.Gold RP. Agenesis and pseudo-agenesis of the dorsal pancreas. Abdom Imaging 1993; 18:141–144.

2 Schnedl Wi, Reisinger EC, Schreiber F, et al. Complete and partial agenesis of the dorsal pancreas in one family. Gastrointest Endosc 1995; 42:485-487

3. Adda G, Hannoun L, Loygue J. Development of the human pancreas: variations and pathology. A tentative classification. Anat clin 1984; 5:275-83.

4 Herman TE, Siegel MJ. Polysplenia syndrome with congenital short pancreas. AJR 1991; 156:799-800

5.Wilding R, Schnedl WJ, Reisinger EC, et al.Agenesis of the dorsal pancreas in a woman with diabetes mellitus and in both of her sons. Gastroenterology 1993; 104:1182-86

6. Deignan RW, Nizzero A, Malone DE. Case report: agenesis of the dorsal pancreas-a cause of diagnostic error on abdominal sonography. Clin Radiol 1996; 51:145


Author

Sachit K Verma, MD and Donald G. Mitchell, MD

MD

Thomas Jefferson University Hospital, Philadelphia, PA 19107

drrajeshsharma's picture
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Joined: 08/10/2007
Great case

Great case  and  beautiful images.

Dr.Rajesh Sharma MD, DMRD, Department of Radiodiagnosis, Government Medical College, Jammu (J&K) India

jdogra's picture
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Joined: 05/23/2007
Excellent case

Excellent case. Thank you for your submission.

Dr. Vikram Singh Dogra

Professor of Radiology, Urology & BME
Associate Chair for Education and Research.
Department of Imaging Sciences
University of Rochester School of Medicine