Case Presentation: Patient is a 43-year-old female with history of recurrent pancreatitis who presented with diffuse abdominal pain and shortness of breath. She initially presented 6 weeks prior with epigastric pain radiating to the back. The pain was intermittent for a week but had become more severe and constant. This was associated with fever, nausea and vomiting. There is no family history of autoimmune, gastric, or hepatobiliary disease. She endorsed remote heavy alcohol use but currently admits to a few glasses of wine occasionally. Physical exam was remarkable for diffuse abdominal tenderness. Labs included total bilirubin 2.5, ALP 200, AST 156, ALT 59, lactate 2.1 and lipase 273. CT scan of the abdomen revealed ascites and pancreatic inflammation with a peri-pancreatic fluid collection but no calcifications suggestive Acute interstitial pancreatitis with pseudocyst. She was treated with endoscopic ultrasound-guided pancreatic pseudocyst cystogastrostomy. Subsequent MRCP demonstrated improving ascites with no clear leak. She was discharged on day 12 in good condition, but was readmitted for abdominal pain twice over the next two weeks. During these admissions, the pseudocyst had improved and ERCP was repeated to remove the cystgastrostomy stent. At the present [fourth] admission, she complained of with severe abdominal pain and shortness of breath with vital signs notable for HR 129, RR 25, and SPO2 94% on 4L of O2 via nasal canula. Exam was remarkable for decreased breath sounds of the right lung field and abdominal distension with diffuse tenderness. CT of the chest and abdomen/pelvis demonstrated residual pancreatitis, stable small pseudocysts, ascites, intra-peritoneal fluid, and a new large right pleural effusion. Thoracostomy was performed in the ED and 2L of serous fluid was drained. The chest tube was removed the next day but required reinserted due to rapid re-accumulation. The pleural fluid was positive for amylase and lipase. Repeat ERCP demonstrated pancreas divisum with a strictured ventral duct and a leaking dorsal duct suggestive of possible pancreatico-pleural fistula.
Discussion: Pancreatic divisum (PD) is one of the most common congenital anomalies and may be present in 20% of cases of recurrent pancreatitis. Conversely, pancreatico-pleural fistula (PPF) is a rare complication and cause of pleural effusion in pancreatitis. It is characterized by a pathologic connection between the pancreas and pleural space that allows for leakage of pancreatic fluid to the thoracic cavity, resulting in pleural effusions of one or both lung cavities; unilateral left effusions are most common. Patients usually have a history of chronic pancreatitis and present with respiratory symptoms including pleuritic chest pain and shortness of breath. Abdominal complaints are less common and prompt concern for concurrent ascites. Suspicion for PPF is warranted when pleural fluid is positive for pancreatic enzymes. Diagnosis can be confirmed with MRCP-guided identification of a disruption in the pancreatic duct or the wall of a pseudocyst.
Conclusions: Here we present a case of acute recurrent pancreatitis complicated by recurrent pleural effusions and ascites secondary to PPF in a previously undiagnosed complete PD. This aims to increase awareness of this uncommon clinical entity, its complex course, and the need for a multidisciplinary approach. Due to the disease’s intricate progression, a high index of suspicion should exist for patients found with pleural effusion in setting of recurrent pancreatitis.
