Literature review on clinical features and associated complications of dorsal pancreas agenesis

Abstract

Objective To analysis the clinical features of dorsal pancreas agenesis (DPA) and the associated diabetes, pancreatitis and other congenital organ malformations. Methods Chinese databases of Sinomed, CQVIP and CNKI using the term of short pancreas, pancreas agenesis, bulbar pancreas and dorsal pancreas, and English databases of PubMed using the term of dorsal pancreas agenesis, short pancreas and pancreas hypoplasia were searched. The clinical manifestation, pancreatic head characteristics and associations with diabetes, pancreatitis and other congenital organ malformations were analyzed. Results Six related publications from Chinese databases were searched and 21 patients were included with 2 cases excluded. Sixty-one publications from English database were searched and 71 patients were included. Thus, a total of 91 patients with DPA were analyzed. Abdominal pain was the most common manifestation, which was reported by 61.5% of the patients. 15.3% patients were identified during regular physical examination. Other manifestations including jaundice, fatigue, abdominal discomfort and diabetes were rare. After removing cases with insufficient information, 39 patients (61.9%) carried abnormal pancreatic head. Prevalence of diabetes or impaired glucose tolerance was 56.7% and the percentage of insulin-dependent diabetes in patients with abnormal glycaemia was 47.3% (n=18). 20 patients (26.7%) were associated with pancreatitis, including 15 patients (75.0%) with acute pancreatitis, 1 patient (5.0%) with recurrent pancreatitis, and 4 patients (20.0%) with chronic pancreatitis. Thirty-three patients (36.2%) suffered other congenital organ malformations, including 21 patients (63.6%) with splenic malformation, 8 patients (24.2%) with heart malformation, and 17 patients (51.5%) with multi-organs malformations like gastrointestinal malformation, azygos vein and inferior cava vena fusion, duodenal and biliary atresia and renal absence. Conclusions The main diagnostic criteria of DPA was the absence of dorsal pancreatic duct. Diabetes was the most common complication followed by pancreatitis. Key words: Pancreas; Congenital abnormalities; Diabetes mellitus; Pancreatitis