Agenesis of the dorsal pancreas presenting with diabetic ketoacidosis – a case report and literature review

Abstract

Background: Agenesis of the dorsal pancreas (ADP) is clinically rare, and it is usually accompanied by abdominal pain. Various disorders of glucose metabolism associating with ADP have been reported, but there are only two studies reporting a correlation between ADP and DKA in English literature. Case presentation: We present a case of a patient with ADP accompanied by abdominal pain and diabetic ketoacidosis as the initial clinical presentation. A 30-year-old man presented with a 3-month history of recurrent onset of persistent mild epigastric pain, which worsen when eating. Laboratory tests revealed metabolic acidosis, hyperglycemia, and ketonuria. Phase contrast CT and MRCP showed the absence of the body and tail of the pancreas, as well as the dorsal pancreatic duct. The C-peptide release test indicated β-cell dysfunction. A combination therapy of insulin, pancreatic enzyme supplements, and mosapride citrate were administrated and the pain gradually resolved. Conclusions: As glucose metabolism disorders can vary across different individuals, we advise clinicians to consider the diagnosis of ADP for a patient who presents with a glucose metabolism disorder accompanied by abdominal pain, pancreatitis or steatorrhea.

Agenesis of the Dorsal Pancreas

“…Steatorrhoea is a manifestation of exocrine deficiency and is commonly managed with supplementation of pancreatic enzymes. ADP has been associated with include polysplenia, ectopic spleen, bowel malrotation, horseshoe kidney, congenital heart disease and heterotaxy [7], none of which were noted on our patient's CT abdomen and MRCP. There has been an increased frequency of incidental diagnosis of ADP [6,7].…”

“…Associations between embryogenesis of the pancreas and genes such as HNF1B [3] and GATA6 [4], and signalling pathways such as retinaldehyde dehydrogenase 2 (Raldh2) [5] have been reported in the literature. Yet this condition remains rare with less than one hundred and twenty cases reported in the literature [6][7][8][9].…”

“…Approximately half of patients with ADP will develop diabetes mellitus, and up to half of them will require insulin therapy [7][8][9]. The age of onset of diabetes mellitus associated with ADP ranges between 28 to 39 years [7][8][9].…”

“…Approximately half of patients with ADP will develop diabetes mellitus, and up to half of them will require insulin therapy [7][8][9]. The age of onset of diabetes mellitus associated with ADP ranges between 28 to 39 years [7][8][9]. Although beta cell dysfunction and insulin deficiency have been implicated in the pathophysiology of diabetes mellitus among patients with ADP, there have only been three studies that reported a correlation between this congenital malformation and diabetic ketoacidosis [7,9].…”

“…The age of onset of diabetes mellitus associated with ADP ranges between 28 to 39 years [7][8][9]. Although beta cell dysfunction and insulin deficiency have been implicated in the pathophysiology of diabetes mellitus among patients with ADP, there have only been three studies that reported a correlation between this congenital malformation and diabetic ketoacidosis [7,9]. Studies conducted on patients who have undergone surgical resection of the pancreas for other indications demonstrate that the pancreatic tail in humans is more densely populated with islets of Langerhans than other parts of the pancreas.…”