Agenesis of the dorsal pancreas and associated diseases.
BACKGROUND
Agenesis of the dorsal pancreas is a very rare congenital pancreatic malformation and is associated with some other diseases.


METHODS
A PubMed search revealed 53 cases of agenesis of the dorsal pancreas.


RESULTS
In 28 patients with this congenital malformation hyperglycemia was demonstrated, 27 had abdominal pain, 16 had pancreatitis, 14 had an enlarged or prominent pancreatic head visible on computed tomography, and in a few cases, polysplenia, which may occur with various congenital anomalies of visceral organs, was described.


CONCLUSIONS
Difficulties involved in obtaining a firm diagnosis have led to a variety of terms being used to describe this congenital disease. Diagnosis of agenesis of the dorsal pancreas is inconclusive without demonstration of the absence of the dorsal pancreatic duct. Here we describe the embryological development of the pancreas, the so-far known cases of agenesis of the dorsal pancreas with associated medical problems, and the diagnostic measures to find the right conclusions.
Schnedl
Wolfgang J
WJ
Department of Internal Medicine, Medical University, Auenbruggerplatz 15, A-8036, Graz, Austria. w.schnedl@dr-schnedl.at
Piswanger-Soelkner
Claudia
C
Wallner
Sandra J
SJ
Reittner
Pia
P
Krause
Robert
R
Lipp
Rainer W
RW
Hohmeier
Hans E
HE
D003920:Diabetes Mellitus / Q000151:congenital*; D006801:Humans; D010179:Pancreas / Q000002:abnormalities*; D010182:Pancreatic Diseases / Q000150:complications / Q000151:congenital*