An Atypical Complication of Chronic Pancreatitis

Abstract

Question: A 55-year-old man with a history of chronic alcoholic pancreatitis presented with a 4-day history of progressively worsening abdominal pain, nausea, and vomiting. Physical examination revealed tenderness in the epigastrium, without guarding or rebound. Laboratory tests were significant for the following: white blood cell count, 16,600/μL; amylase level, 452 U/L; lipase level, 437 U/L; and C-reactive protein level, 32.5 mg/dL. Contrast-enhanced computed tomography (CT) of the abdomen showed a dilated main pancreatic duct, peripancreatic fluid collections, and fluid collection at the tail of the pancreas adjacent to the splenic hilum (Figure A). Because the patient was hemodynamically stable, he was initially managed conservatively, but the cystic lesion at the tail of the pancreas enlarged, and a follow-up CT performed 1 week later showed the cystic lesion extending into the splenic parenchyma (Figure B) and the left chest (Figure C). The patient’s clinical condition was deteriorating. What is the diagnosis and what should be the next steps in management? See the Gastroenterology website (www.gastrojournal.org) for more information on submitting to Gastro Curbside Consult. Thoracentesis of the left pleural effusion had a high amylase content (66,162 U/L). These findings confirmed the diagnosis of a pancreatic pseudocyst with acute exacerbation of chronic pancreatitis involving the spleen and the chest. Magnetic resonance cholangiopancreatography showed communication between the pancreatic duct and the pseudocyst. Endoscopic retrograde pancreatography revealed strictures of the main pancreatic duct in the pancreatic head and body, and a flow of contrast medium from the main pancreatic duct into the pseudocyst was visualized (Figure D, arrow). An endoscopic pancreatic sphincterotomy was performed and a 7-French (Fr), 9-cm straight plastic pancreatic stent was placed into the main pancreatic duct. However, there was no improvement in the patient’s condition, and the size of the pseudocyst remained unchanged. Endoscopic ultrasound (EUS)–guided transmural drainage was not performed because the stomach and the pseudocyst were not in close proximity. Therefore, the patient underwent ultrasound-guided percutaneous trans-splenic pseudocyst drainage (7-Fr) and percutaneous pleural drainage (20-Fr), with immediate decompression of the pseudocyst (Figure E) and symptomatic relief. Drainage tubes were removed when there was no further drainage, and he was asymptomatic at discharge. A follow-up CT at 2 months revealed that the pseudocyst had resolved completely. The pancreatic stent was removed 1 year after discharge, resulting in the recurrence of acute pancreatitis. The pancreatic duct strictures had not improved, and an 8.5-Fr pancreatic duct stent was replaced. The pancreatic stent was thereafter replaced regularly every 4–6 months. He was still doing well during a 10-year follow-up period with no recurrence of pseudocyst. Pancreatic pseudocysts are a common complication of acute or chronic pancreatitis. Most pseudocysts are found in peripancreatic region, but they can also be found in atypical locations such as the liver, spleen, mediastinum, pelvis, kidney, and retroperitoneum. Although uncommon, due to the close proximity of the pancreatic tail and the splenic hilum, splenic complications such as isolated splenic vein thrombosis, intrasplenic pseudocysts, splenic rupture, infarction, and necroses as well as splenic hematoma and severe bleeding from eroded splenic vessels may occur in the course of acute or chronic pancreatitis.1Lankisch P.G. The spleen in inflammatory pancreatic disease.Gastroenterology. 1990; 98: 509-516Abstract Full Text PDF PubMed Scopus (73) Google Scholar Pseudocysts that are symptomatic or present with complications such as infection, bleeding, rupture, or fistulization to adjacent hollow structures require intervention.2Dumonceau J.M. Delhaye M. Tringali A. et al.Endoscopic treatment of chronic pancreatitis: European Society of Gastrointestinal Endoscopy (ESGE) guideline - updated August 2018.Endoscopy. 2019; 51: 179-193Crossref PubMed Scopus (151) Google Scholar Management options are considered based on the timing, their size and location, relationship with adjacent anatomic structure, and symptoms. The treatment strategy includes conservative treatment, percutaneous, endoscopic, and surgical drainage, or excision. Medical therapy consists of enteral or parenteral nutrition, enzyme supplementation, and somatostatin analogues. It has been reported that there is no significant difference in pseudocyst resolution between surgical and endoscopic approaches; thus, the minimally invasive approach is recommended. Endoscopic drainage has become the preferred therapeutic modality. At present, EUS-guided transmural drainage is the first-line treatment for pseudocysts and walled-off pancreatic necrosis. Endoscopic transpapillary drainage is also an effective treatment when the pseudocyst and the main pancreatic duct are connected.2Dumonceau J.M. Delhaye M. Tringali A. et al.Endoscopic treatment of chronic pancreatitis: European Society of Gastrointestinal Endoscopy (ESGE) guideline - updated August 2018.Endoscopy. 2019; 51: 179-193Crossref PubMed Scopus (151) Google Scholar Prolonged stenting (6–12 months) is effective in treating for remodeling pancreatic duct strictures.3Strand D.S. Law R.J. Yang D. et al.AGA Clinical practice update on the endoscopic approach to recurrent acute and chronic pancreatitis: Expert review.Gastroenterology. 2022; 163: 1107-1114Abstract Full Text Full Text PDF PubMed Scopus (3) Google Scholar In addition to plastic stents, placement of a fully covered self-expanding metal stent has also become a management option for refractory benign pancreatic duct strictures.2Dumonceau J.M. Delhaye M. Tringali A. et al.Endoscopic treatment of chronic pancreatitis: European Society of Gastrointestinal Endoscopy (ESGE) guideline - updated August 2018.Endoscopy. 2019; 51: 179-193Crossref PubMed Scopus (151) Google Scholar,3Strand D.S. Law R.J. Yang D. et al.AGA Clinical practice update on the endoscopic approach to recurrent acute and chronic pancreatitis: Expert review.Gastroenterology. 2022; 163: 1107-1114Abstract Full Text Full Text PDF PubMed Scopus (3) Google Scholar When transpapillary drainage can be technically difficult due to severe strictures, pancreatic duct stones, or surgically altered anatomy, EUS-guided pancreatic duct drainage is becoming an alternative technique.2Dumonceau J.M. Delhaye M. Tringali A. et al.Endoscopic treatment of chronic pancreatitis: European Society of Gastrointestinal Endoscopy (ESGE) guideline - updated August 2018.Endoscopy. 2019; 51: 179-193Crossref PubMed Scopus (151) Google Scholar However, if the pseudocyst does not connect with the pancreatic duct and is located away from the stomach or duodenum, percutaneous or surgical drainage could be considered. Surgery may be required if the other modalities of treatment have failed, cystic neoplasm cannot be ruled out, or complications have occurred such as perforation or hemorrhage not controlled by embolization. Pseudocyst management often necessitates multiple interventions. The approach should be individualized for each patient. A multidisciplinary strategy involving the expertise of therapeutic endoscopists, interventional radiologists, and pancreaticobiliary surgeons is highly preferred to provide the best possible outcome.