CASE 13694 Published on 17.06.2016

Disconnected pancreatic duct syndrome: imaging findings and endoscopic treatment


Abdominal imaging

Case Type

Clinical Cases


Tonolini Massimo, M.D.1; Vella Adriana, M.D.1; Bareggi Emilia, M.D.2; Gambitta Pietro, M.D.2

"Luigi Sacco" University Hospital,
(1) Radiology Department
(2) Gastroenterology Department
Via G.B. Grassi 74
20157 Milan, Italy;

56 years, male

Area of Interest Pancreas ; Imaging Technique Fluoroscopy, MR, CT
Clinical History

Middle-aged male patient with history of prolonged hospitalization and medical treatment at another institution because of severe necrotic-haemorrhagic acute pancreatitis (AP) from biliary sludge, and allergy to iodinated contrast medium.
Currently suffering from persistent abdominal pain and biochemical abnormalities ten months after the initial AP episode.

Imaging Findings

Previous CT reports described severe AP with pleural effusions and 8 cm dominant peripancreatic post-necrotic fluid collection (PPNFC), which was initially treated with endoscopic ultrasound-guided cystogastrostomy with metallic stent (Fig. 1), achieving incomplete cyst resolution.
Transferred to our hospital, the patient underwent pancreatic necrosectomy and positioning of pigtail stent through the cystogastrostomy (Fig. 2a-b); after initial clinical improvement both stents were endoscopically removed (Fig. 2c).
A month later, multidetector CT (Fig. 3) and MRI (Fig. 3a-g) showed the reappearance of sizeable, elongated PPNFC located above the pancreatic body-tail, which showed preserved anatomic continuity and enhancement without appreciable ductal dilatation. Additional MR-cholangiopancreatography (MRCP, Fig. 3h-i) showed mild Wirsung duct dilatation at the pancreatic head, plus segmental discontinuity of the main pancreatic duct (MPD) at the body.
Endoscopic retrograde cholangiopancreatography (Fig. 4a) confirmed MRCP findings and allowed definitive treatment by positioning of long stent through the disrupted MPD, ultimately resulting in relieved complaints and laboratory changes.


The disconnected pancreatic duct syndrome (DPDS) is an anatomic condition characterised by absent continuity between viable secreting pancreatic tissue and the gastrointestinal tract. This increasingly recognized condition occurs as a complication of acute necrotizing pancreatitis (ANP), occasionally following pancreatic traumatic injury with segmental necrosis [1-3].
Mostly seen in adults, DPDS generally presents late (2-9 months) after alcohol-related, gallstone or idiopathic ANP. The commonest presentation is persistent illness and non-resolving collection despite ineffectual percutaneous, endoscopic or surgical procedures. Meanwhile, patients remain at risk of delayed bleeding or infection. Alternatively, DPDS may occur in the setting of chronic pancreatitis (CP). Long-term complications include persistent symptoms, development of CP, pancreatic atrophy, diabetes (16-53% of patients), rarely left-sided portal hypertension. The mortality is non-negligible (up to 2%) [1-3].
Traditionally, the DPDS hallmark was endoscopic retrograde cholangiography (ERCP) demonstration of main pancreatic duct (MPD) discontinuity at the neck (58%), body-tail (23%) or mid-body (19%). Contrast extravasation at the site of ductal disruption at ERCP was reported in up to half of patients, particularly in the distal MPD [2-4].
Contrast-enhanced multidetector CT represents the mainstay technique to assess severity, complications and prognosis of acute pancreatitis [5, 6]. DPDS is present in up to 16% of patients with walled-off pancreatic necrosis (WOPN). Making the diagnosis of DPDS is important since—unlike other post-inflammatory peripancreatic fluid collections—conservative management is generally unsuccessful. Unfortunately, despite most patients receiving serial CT studies, DPDS is missed at primary CT interpretation in the majority of cases, thus contributing to diagnostic delay: therefore, the radiologists’ awareness is advisable. DPDS is suggested by viable, enhanced pancreatic parenchyma after ANP plus a non-resolving collection of greatly variable size (4-74 cm2). As this case exemplifies, collections may or may not be located along the expected course of the MPD. Although false positives may result from partial duct disruption, additional investigation with MR-cholangiopancreatography (MRCP) may reveal ductal discontinuity which requires ERCP for diagnosis confirmation and treatment [2-4].
Managing DPDS requires either redirection of secretions into the digestive tract, or resection of the viable disconnected pancreatic segment. Repeated surgery is reported in 6% of patients. Surgery may perform internal drainage into a Roux-en-Y jejunal limb, or distal pancreatic resection. Endoscopic treatment includes ultrasound-guided transgastric drainage of WOPN with pigtail stents and ERCP-assisted stenting of the MPD, is increasingly recognized as the best therapeutic option and is successful in 75% of patients [1, 2, 7-11].

Differential Diagnosis List
Disconnected pancreatic duct syndrome following acute necrotizing pancreatitis
Pancreatic pseudocyst
Walled-off pancreatic necrosis
Peripancreatic abscess
Final Diagnosis
Disconnected pancreatic duct syndrome following acute necrotizing pancreatitis
Case information
DOI: 10.1594/EURORAD/CASE.13694
ISSN: 1563-4086