J Korean Endocr Soc.  2010 Mar;25(1):78-83. 10.3803/jkes.2010.25.1.78.

A Case of Complete Agenesis of the Dorsal Pancreas in a Patient with Newly Diagnosed Diabetes Mellitus

• Kim DP
• Park KS
• Kim DS
• Ko BS
• Lee JH
• Lee JH
• Shin JH
• Kim BJ
• Kim HJ

• KMID: 2177868
• DOI: http://doi.org/10.3803/jkes.2010.25.1.78

Abstract

Agenesis of the dorsal pancreas is a rare congenital anomaly caused by underdevelopment or agenesis of the dorsal pancreatic bud that forms the upper head, neck, body and tail of the pancreas. We report a case of agenesis of the dorsal pancreas, which was found under examination of diabetes mellitus (DM). A 16-year-old girl was transferred to our hospital because of a positive urine glucose reading during a school-conducted examination. Abdominal computed tomography and magnetic resonance cholangiopancreatography revealed the deficit of the pancreatic body and tail. Diabetes-associated autoimmune antibodies were negative in a blood test. Decreased beta-cell function was demonstrated by oral glucose tolerance and glucagon stimulation tests. Although the notion that agenesis of the dorsal pancreas leads to decreased endocrine or exocrine function is controversial, the results of this study suggest that we should consider these causes of diabetes mellitus. When treating a young patient with diabetes mellitus, we should consider causes of diabetes mellitus such as congenital anomaly or maturity onset diabetes, in addition to type 1 and type 2 diabetes mellitus.

Keyword

Agenesis of dorsal pancreas; Diabetes mellitus; Insulin

MeSH Terms

Adolescent
Antibodies
Cholangiopancreatography, Magnetic Resonance
Congenital Abnormalities
Diabetes Mellitus
Diabetes Mellitus, Type 2
Glucagon
Glucose
Glucose Tolerance Test
Head
Hematologic Tests
Humans
Insulin
Neck
Pancreas
Antibodies
Congenital Abnormalities
Glucagon
Glucose
Insulin
Pancreas

Figure

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  Fig. 1 Pedigree of the patient reveals that there is no family history of diabetes mellitus.

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  Fig. 2 Abdominal CT scan shows (A) normal pancreatic head without abnormal calcification or ductal dilatation (arrow), and (B) nonvisualization of pancreatic body and tail at the level of splenic vein (arrow).

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  Fig. 3 Magnetic resonance cholangiopancreatography (MRCP). A. T2-weighted image shows normal pancreatic duct in pancreas head. B. In maximum intensity projection image, pancreatic duct of body and tail and Santorini duct cannot be seen.

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