Agenesis of the dorsal pancreas: systematic review of a clinical challenge

Abstract

Background: Agenesis of the dorsal pancreas is a rare malformation. Since 1911 and until 2008, 53 cases have been reported. Several authors have recently described the association of this anomaly with neoplasia of the ventral pancreas, thus we performed a systematic review of the literature from 2008 to 2015.Methods: A systematic review of the Medline and ISI Web of Science Databases from 2008 until 2015 was carried out, and 30 articles which met the inclusion criteria were identified that included a total of 53 patients: 7 children and 46 adults.Conclusions: Although dorsal pancreatic agenesis is a rare malformation, given its association with non-alcoholic pancreatitis and neoplasia of the residual pancreas, physicians should maintain an expectant attitude.

Use of hyperglycemic clamp to assess pancreatectomy and islet cell autotransplant in patient with heterotaxy syndrome and dorsal pancreas agenesis leading to chronic pancreatitis

“…This finding is at variance with previous cases of HS and DPA that were associated with diabetes 13,14 . Also, in isolated DPA, more than half of patients have diabetes at the time of diagnosis 5,6 . For these reasons, deceased donors with DPA are in general not considered suitable for whole organ pancreas transplantation and are also discouraged from islet isolation; one report, however, did mention a successful islet cell allotransplantation following isolation from a deceased donor with partial DPA 15 .…”

“…DPA most frequently presents with abdominal pain or diabetes mellitus if not diagnosed as an incidental finding. DPA is associated with acute, recurrent, or chronic pancreatitis in up to 30% of cases 5,6 …”

Agenesis of dorsal pancreas in a young adult: a rare cause of diabetes mellitus

“…Dorsal pancreatic agenesis is exceedingly rare with less than 100 cases reported in the world literature 1. Patients with dorsal pancreatic agenesis may be asymptomatic or may present with epigastric discomfort, hyperglycaemia, and acute or chronic pancreatitis 1. Our patient was diagnosed with type 1 diabetes mellitus 8 years ago at the age of 17 years and since then he was on insulin.…”

“…Complete pancreatic and ventral pancreatic agenesis are incompatible with life. Dorsal pancreatic agenesis is exceedingly rare with less than 100 cases reported in the world literature 1. Patients with dorsal pancreatic agenesis may be asymptomatic or may present with epigastric discomfort, hyperglycaemia, and acute or chronic pancreatitis 1.…”

Agenesis of the dorsal pancreas associated with mucinous cysts and chronic calcific non-alcoholic pancreatitis

“…Dear Editor, Dorsal pancreatic agenesis (DPA) is a congenital malformation which has been described in 107 cases (1,2). Here we present the case of DPA associated with chronic calcific non-alcoholic pancreatitis and mucinous cysts in the residual pancreas.…”