Incidentally detected dorsal pancreatic agenesis: hidden clinical implications

Dorsal pancreatic agenesis (DPA) is a rare congenital anomaly characterised by the partial or complete absence of the pancreatic body and tail, with < 100 cases documented globally. Patients with incidentally detected DPA face significant long-term health risks, including diabetes mellitus (10-83%), exocrine pancreatic insufficiency (~ 30%), and pancreatitis (~ 40%). Furthermore, 15-20% of these patients may develop pancreatic ductal adenocarcinoma. This study aims to highlight the importance of screening and establishing guidelines for the long-term monitoring of individuals incidentally diagnosed with DPA.

Over five years (2018-2024), three cases of incidentally detected DPA were identified at our institution. A male in his late 60s presented with hematuria, revealing complete DPA during imaging for bladder carcinoma. Another male in his late 60s had incidental DPA diagnosed alongside a left renal mass, while a female in her late 20s was found to have DPA during evaluation for nephrolithiasis. All patients received lifestyle counselling and were advised a structured follow-up regimen involving HbA1c monitoring, stool fat analysis, abdominal ultrasound, and echocardiography every 3 to 6 months for the first 1-2 years.

Regular follow-up for patients with incidentally detected DPA is crucial due to the associated risk of DM and pancreatic insufficiency. Our findings support a proactive monitoring strategy, which ensures timely detection and management of potential complications, ultimately enhancing patient outcomes. Future research should aim to establish standardised guidelines for the long-term management of DPA, particularly in asymptomatic patients.