Identification and management of pancreas divisum

Abstract

Pancreas divisum, the most common congenital malformation of the pancreas, occurs due to a failure of fusion of the ductal systems of the dorsal and ventral pancreatic buds in the seventh week of intra-uterine life. This leads to a dominant dorsal pancreatic duct draining though the minor papilla and a small ventral pancreatic duct draining through the major papilla. The prevalence in western populations is about 10% and more than 95% of these patients are without pancreatic symptoms, with the anomaly found incidentally on abdominal imaging for an unrelated indication. The etiological role and clinical significance of pancreas divisum in relation to pancreatic disease has not yet been clearly defined, but may predispose to pancreatic disease in co-existence with other factors. Secretin-enhanced Magnetic Resonance Cholangiopancreatography is the non-invasive imaging modality of choice to identify pancreas divisum. Patients may be offered minor papilla therapy when they present with recurrent acute pancreatitis, severe acute pancreatitis and can be considered for therapy in the setting of chronic pancreatitis and chronic abdominal pain of pancreatic origin. Minor papilla endotherapy (sphincterotomy and/or stenting) via Endoscopic Retrograde Cholangiopancreatography and minor papilla surgical therapy have comparable outcomes with endotherapy typically considered first-line due to a favorable adverse event profile. The response to therapy is variable with maximal benefit seen in patients with recurrent acute pancreatitis and least with chronic pancreatic-type abdominal pain. Data supporting either therapy are of low quality as they are predominantly retrospective with a sub-optimal follow up period. Surgical options including a pancreatojejunostomy (Puestow or Frey procedure) or a total pancreatectomy with auto-islet cell transplantation may be considered in a subset of patients.

Clinical outcome of endoscopic therapy in patients with symptomatic pancreas divisum: a Dutch cohort study

“…Success rates were reported to be 76 %, 52 %, and 48 % for patients with RAP, CP and CAP, respectively. Important to note is that if strict objective outcome measures were used, instead of subjective measurements like self-reported pain or opioid usage, a smaller percentage of the patients reached clinical success in this review 17 , which is in line with the outcome of our current study.…”

Synchronous Periampullary Tumors in a Patient With Pancreas Divisum and Neurofibromatosis Type 1

“…On the other hand, acute and chronic pancreatitis and associated pancreatic injury are risk factors for the development of pancreatic cancer (Becker et al, 2014;Kirkegård et al, 2018). In this context, it has been reported that pancreas divisum acts as modulator of pancreatitis risk in carriers of additional genetic variants such as the one described here in the CFTR gene (Bertin et al, 2012;Gutta et al, 2019).…”

Idiopathic recurrent acute pancreatitis in the context of pancreas divisum: A case report

“…Pancreas divisum represents the most common congenital anomaly in pancreatic ductal anatomy in which two distinct dorsal (duct of Santorini) and ventral ducts (Wirsung duct) fail to fuse. This condition may occur in approximately 10% of the population [5] . In such cases, the dorsal duct plays the main role in drainage of pancreatic secretions through minor papilla and a small ventral duct opens to the major papilla [6] .…”

“…Endoscopic therapy includes endoscopic interventions include minor papillotomy, trans minor papilla dorsal duct stenting, and balloon dilation of the minor papilla. A surgical minor papilla procedure such as surgical sphincteroplasty is reserved for patients who fail endotherapy [5] .…”