Complete agenesis of the dorsal pancreas

Abstract

Pancreatic anomalies are occasionally reported, but complete agenesis of the dorsal pancreas is extremely rare. We report a 47-year-old woman with complete agenesis of the dorsal pancreas. This patient initially presented with jaundice. Computed tomography did not reveal the pancreatic corpus or tail. Endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography did not visualize the dorsal pancreatic duct. Choledochojejunostomy was performed because she had obstructive jaundice. At laparotomy, there was an enlarged pancreatic head, but no distal pancreas was seen. Histological examination of the pancreatic biopsy specimen showed scattered islets of Langerhans in diffuse fibrosis, with destruction of the glandular parenchyma. This case was diagnosed as complete agenesis of the dorsal pancreas.

Reduction of Hepatic Glycogen Synthesis and Breakdown in Patients with Agenesis of the Dorsal Pancreas

“…Most reports describe single, but not familiar, patients who were diagnosed because of jaundice (36), pancreatitis (11), weight loss, and diabetes mellitus (10). Partial agenesis of the dorsal pancreas like that in the older son has been reported (37), but was not familiar.…”

Agenesis of Dorsal Pancreas

“…The dorsal bud forms the upper part of the head, body and tail of the pancreas which drains through the Santorini duct. The ventral bud gives rise to the major part of the head and uncinate process which drains through Wirsung duct [1] . Agenesis of the ventral pancreas and complete agenesis of the pancreas are lethal conditions [2] .…”

“…Failure of the ventral and dorsal pancreatic ducts to fuse is called pancreas divisum. In this entity, the ventral duct drains into major papilla, while the dorsal duct drains into separate minor papilla [1] . Atrophy of the body and the tail of the pancreas secondary to chronic pancreatitis and sparing of the pancreatic head is called pseudo-agenesis [3] .…”

“…The patient has a suspicion of lymphoma with gastroscopy. The biochemical evaluation of the patient revealed mild elevation of fasting plasma glucose (126 mg/dL; reference range: 70-115 mg/dL [1] ). Pancreatic amylase and lipase levels in serum were within normal limits.…”

“…A few case reports have been published in the literature about this anomaly. Agenesis of the dorsal pancreas is mostly asymptomatic but abdominal pain, pancreatitis and diabetes mellitus may be associated [1] . We present a case of asymptomatic dorsal pancreatic agenesis.…”