Acute recurrent pancreatitis complicated by pancreatic-portal venous fistulisation, secondary chronic portal vein thrombosis, multiple hepatic abscesses and newly diagnosed cirrhosis
- 1 Medicine, University of Miami Health System, Miami, Florida, USA
- 2 Internal Medicine, University of Miami Health System, Miami, Florida, USA
- Correspondence to Dr Tess Calcagno; tesscalcagno@med.miami.edu
Abstract
Pancreatic-portal vein fistula, portal vein thrombosis and liver abscesses are rare complications of acute pancreatitis which occur in the setting of localised inflammation of the pancreatic tissues and surrounding structures. We discuss a 34-year-old woman with a medical history of intermittently controlled HIV and alcohol use disorder who presents with severe epigastric pain diagnosed with acute pancreatitis. Concerning CT findings showing hypoattenuating liver lesions likely to be abscesses and multiple pancreatic pseudocysts led us to order an MRI which showed thrombosis of the portal vein, porto-pancreatic pseudocyst fistulation and cirrhotic changes. Patient was treated conservatively in the hospital and ultimately given a course of antibiotics for hepatic abscesses. Workup for new diagnosis of cirrhosis revealed positive antimitochondrial antibodies, raising suspicion for autoimmune hepatitis possibly triggered by immune reconstitution in the setting of HIV infection. Patient was discharged on oral antibiotic therapy and home antiretroviral therapy.
Background
Commonly known local complications of pancreatitis include pancreatic pseudocyst, walled-off necrosis, peripancreatic fluid collections and acute necrotic collections.1 However, we present a case in which three rare local complications of pancreatitis present concurrently. The incidence of acute pancreatitis in the USA is 4.9–35 per 100 000. It is associated with a significant degree of morbidity and mortality and serves as one of the most common gastrointestinal causes of hospitalisation.2 It is important to increase awareness of rare complications seen in pancreatitis to familiarise other clinicians of potential anatomical derangements which can occur in concert with pancreatic inflammation.
Case presentation
A woman in her thirties with a medical history of intermittently controlled HIV (CD4 638 on hospital admission, but 117 3 months earlier), previous hospitalisation for alcoholic pancreatitis and chronic alcohol use presents to the emergency room complaining of severe epigastric pain, nausea and vomiting. Vital signs on admission included a heart rate of 101 BPM, blood pressure of 136/86 mm Hg, temperature of 36.9°C and a respiratory rate of 16 breaths/min on room air. Presenting labs included a basic metabolic panel (BMP) and complete blood count (CBC) which were within normal limits. Importantly, she did not have leucocytosis, leucopenia or hypocalcaemia. Lipase was mildly elevated at 468 units/L. Other notable labs on presentation included elevated aspartate aminotransferase (450 IU/L), and low alanine aminotransferase and aspartate aminotransferase, 19 IU/L and 15 IU/L, respectively. Right upper quadrant ultrasound is done in the emergency room to assess the gallbladder and biliary tree revealed no evidence of cholecystitis or cholelithiasis. The CT scan on presentation showed multiple pancreatic pseudocysts, the largest one measuring up to 5.9 cm with main pancreatic duct dilatation. There were hypoattenuating lesions in the right lobe of the liver as well as intrahepatic and extrahepatic ductal dilatation. The patient was treated conservatively for an episode of acute pancreatitis. The pancreatic pseudocyst was not thought to be contributing to her symptoms and endoscopic drainage was not indicated.
On hospital day 2, the patient had been improving clinically, reporting less epigastric pain. She continued to be mildly tachycardic, normotensive and afebrile throughout the hospital stay. MRI was done for further evaluation of hypoattenuating liver lesions in the setting of intrahepatic and extrahepatic duct dilation. Further evaluation with MRI showed thrombosis of the portal venous system with cavernous transformation and high T2 signal intensity inseparable from the 6 cm pseudocyst in the pancreatic neck, likely representing pancreatic pseudocyst-portal vein fistula. The MRI also revealed cirrhotic morphology of the liver with peripheral enhancing fluid collections in segment 7 and 8, likely representing abscesses. The patient was ultimately diagnosed with alcoholic pancreatitis complicated by pancreatic pseudocysts and hepatic abscesses.
The patient continued to be treated conservatively with intravenous fluids and opioid pain medications. Symptoms improved gradually with conservative therapy. Porto-pancreatic fistula was not drained by interventional percutaneous drainage or endoscopic ultrasound and fine needle aspiration. Imaging was reviewed with the interventional radiology team and the patient was determined to not be a candidate for percutaneous drainage of pancreatic cyst due to acute inflammatory state. Imaging was also reviewed by hepatology. The patient was not a candidate for endoscopic drainage with fine needle aspiration due to haemorrhagic features seen in the cyst. Furthermore, given haemodynamic stability, stable haemoglobin and cavernous transformation of thrombosed portal vein, there was low concern for significant bleeding through pseudocyst-portal vein fistula that would require intervention.
Anticoagulation was not used to treat portal vein thrombosis due to chronicity. Hepatic abscesses were not drained or sampled due to challenging location and small size, and instead were treated with ceftriaxone and metronidazole intravenous for 3 days with the transition to oral ciprofloxacin and metronidazole on discharge. Further workup for cirrhosis complications and aetiology was done during the same hospital stay. Ascitic fluid tap revealed 244 neutrophils and no growth on culture. Positive antimitochondrial antibodies (titres>1:40) suggested a potential autoimmune condition contributing to cirrhotic pathology. Of note, the patient had intermittently controlled HIV with CD4 count on admission of 638, and a CD4 count of 117 documented 3 months prior. Immune reconstitution syndrome was on our differential for the cause of a flare of previously undiagnosed autoimmune hepatitis.
Total hospital stay was 6 days. Vital signs at discharge included a heart rate of 81 BPM, blood pressure of 116/86 mm Hg, temperature of 36.8°C and a respiratory rate of 16 breaths/min on room air. Laboratory findings included a BMP and CBC still within normal limits. Aspartate aminotransferase trended down to (150 IU/L), alanine aminotransferase and aspartate aminotransferase were like presenting labs at 12 IU/L and 19 IU/L, respectively. Patient was discharged with a plan for a follow-up MRI in 4 weeks to monitor resolution of disease. She also planned to follow-up with a hepatologist to provide further care for managing diagnostic workup and complications of cirrhosis. Additionally, she planned to seek outpatient rehabilitation services to decrease alcohol intake with the help of a familial support system.
The patient was followed closely in the ambulatory setting and personally contacted at days 30, and 60 postdischarge. She has not used alcohol in over 60 days since admission to the hospital and is proud of her diligence in addressing this matter. She has not been readmitted to the hospital in 60 days. Patient denies postdischarge abdominal pain, vomiting, diarrhoea, changes in mental status or pruritus. MRI is done 60 days after discharge revealed resolution of hepatic abscesses and a decrease in size of the pancreatic pseudocyst with fistula to the portal vein after conservative therapy. However, it also revealed a new collection near the pancreatic head consistent with chronic pancreatitis and worsening ascites consistent with cirrhotic pathology. The patient has an appointment scheduled with hepatology to discuss imaging findings and undergo further specialised evaluation for new-onset cirrhosis.
Investigations
CT abdomen+pelvis with intravenous contrast only
Multiple pancreatic pseudocysts, the largest one measuring up to 5.9 cm with main pancreatic duct dilatation. Hypoattenuating lesion in the right lobe of the liver as well as intrahepatic and extrahepatic ductal dilatation. Underlying malignancy should be excluded. Further evaluation with MRI and magnetic resonance cholangiopancreatography (MRCP) is recommended. Worsening ascites throughout the abdomen and pelvis and mild diffuse mesenteric stranding (figure 1).
Day 1 CT abdomen+pelvis with intravenous contrast. Multiple pancreatic pseudocysts, the largest one measuring up to 5.9 cm (labelled) with main pancreatic duct dilatation. Hypoattenuating lesion in the right lobe of the liver as well as intrahepatic and extrahepatic ductal dilatation.
MR abdomen with+without contrast
(1) Thrombosis of the portal venous system with cavernous transformation and high T2 signal intensity, inseparable from the 6 cm pseudocyst in the pancreatic neck, likely representing pancreatic pseudocyst-portal vein fistula. (2) Multiple pancreatic and peripancreatic pseudocysts with main pancreatic ductal dilation. (3) Cirrhotic morphology of the liver with peripheral enhancing fluid collections in segment 7 and 8, likely representing abscesses. Anasarca and diffuse mesenteric stranding, which could be reactive in the setting of chronic pancreatitis (figure 2).
Day 2 MR abdomen with+without contrast. Thrombosis of the portal venous system with cavernous transformation and high T2 signal intensity, inseparable from the 6 cm pseudocyst (labelled) in the pancreatic neck, likely representing pancreatic pseudocyst-portal vein fistula.
Eight-week interval outpatient MR abdomen with+without contrast
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Interval decrease in size of segment 7 fluid collection, now subcentimetre and resolution of segment 8 abscess.
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Worsening ascites and new 8.5 cm collection near the pancreatic head.
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Interval decrease in size of the pancreatic pseudocyst with fistula to the portal vein. All other peripancreatic pseudocysts show minimal mixed interval changes as detailed above (figure 3).
60-day follow-up MR abdomen with+without contrast. Interval decrease in size of the pancreatic pseudocyst with fistula to the portal vein. 5.5 cm pancreatic cyst in the head with fistula to portal vein, previously 6.3 cm.
Differential diagnosis
Diagnosis: acute recurrent pancreatitis complicated by pancreaticoportal venous fistulisation, chronic portal vein thrombosis, multiple hepatic abscesses and newly diagnosed cirrhosis.
Prior to imaging, our differential included acute pancreatitis, peptic ulcer disease, acute cholecystitis, choledocholithiasis, ascending cholangitis and partial mucosal rupture of the oesophagus. However, in the setting of chronic heavy alcohol use, and previous episodes of acute alcoholic pancreatitis, alcoholic pancreatitis was the highest on our differential. This diagnosis was confirmed with CT scan showing inflammatory pancreatic changes. This was determined to be a complicated case of pancreatitis superimposed on previously undiagnosed cirrhosis.
Unique complications included pancreatic portal venous fistulisation, chronic portal vein thrombosis and multiple hepatic abscesses. These complications likely developed in the setting of recurrent episodes of acute pancreatitis and alcoholic liver injury. Our differential for cirrhotic changes included alcoholic hepatitis and autoimmune hepatitis possibly worsened in the setting of immune reconstitution inflammatory syndrome (IRIS).
Treatment
Pharmacological therapy with metronidazole 500 mg intravenous three times a day+ciprofloxacin 500 mg intravenous two times a day for treatment of empiric treatment of undrained hepatic abscesses. Pharmacological therapy with oxycodone 10 mg every 8 hours as needed for pain. Although we were concerned for potential IRIS, we continued the patient on bictegravir 50 mg, emtricitabine 200 mg and tenofovir alafenamide 25 mg daily. IRIS is treated primarily through therapy directed at the underlying pathology. The patient had no further hospital admissions at 2 months from discharge and reports diminished abdominal pain. She maintains antiretroviral therapy.
Discussion
The pathogenesis of acute pancreatitis is thought to involve acinar cell injury and trypsinogen activation to trypsin leading to a local inflammatory reaction which involves fat and tissue destruction.3 The Revised Atlanta Classification in 2012 classifies acute pancreatitis as two of the following: onset of persistent abdominal pain, serum lipase three times the upper limit of normal and characteristic findings on imaging. Our patient met the criteria through imaging and characteristic abdominal pain. Common local complications of acute pancreatitis are pancreatic pseudocysts, acute necrotic collections and walled off necrosis. Pseudocysts are organised collections with non-epithelial walls which are thought to arise from acute peripancreatic fluid collections. Most resolve on their own, and only 50% will cause clinical complications.4 Pancreatic portal fistulas are rare complications of pancreatic pseudocysts, can sometimes form instead with pancreatic ducts, and have been associated with secondary portal vein thrombus formation as well as hepatic abscess formation.
The mechanism behind pancreatic-portal fistula induction of portal thrombosis and hepatic abscess formation as seen in our patient is unclear. It is also challenging to definitively conclude that pancreatic portal fistula leads to the formation of both the portal vein thrombosis and hepatic abscesses or if there are three separate pathologies presenting in concert. However, previous case reports have shown similar pathologies to follow a parallel aetiologic progression.
Fung et al present a case of a 58-year-old woman with a pancreatic pseudocyst and portal venous fistula with portal vein thrombus who received cyst-gastrostomy and died 1 week later due to massive gastrointestinal haemorrhage.5 Mina et al present a case of a 75-year-old woman with a medical history of recurrent pancreatitis who was found to have a pancreatic portal fistula with acute portal vein thrombus. Like our patient, the woman received supportive medical management. However, given the acuity of the portal vein thrombus in this patient, she was treated with anticoagulation for 4 months which led to resolution of symptoms and formation of collateral flow on follow-up imaging.6 Hector et al present a 55-year-old man with chronic alcoholic pancreatitis who was found to have a pancreatic duct to portal vein fistula with concurrent portal vein thrombosis and hepatic abscesses. He was treated with plastic stent placement through endoscopic retrograde cholangiopancreatography (ERCP) to close the communication. This contrasts with our patient who had a fistula between a pancreatic pseudocyst, not a pancreatic duct. Interestingly, the serial cross-sectional imaging studies done by the radiologist who authored this report confirmed the pancreatic duct-to-portal vein fistula occurred prior to the development of thrombus and abscesses.7
Hepatic abscesses have also been reported as a complication of acute pancreatitis in the absence of pancreatic portal vein fistulisation. Shweta et al reports a 32-year-old woman who presented with alcoholic pancreatitis complicated by multifocal hepatic abscesses and pancreatic necrosis. An exploratory laparotomy to debride the necrosis and drain the abscesses was completed and the patient was discharged on pancreatic enzyme therapy.8
Portal vein thrombosis has also been reported as a complication of acute pancreatitis in absence of pancreatic portal vein fistulisation. Hugo et al report a patient with known hepatocellular carcinoma who presented with acute pancreatitis and was found to have portal vein thrombosis secondary to inflammation around the main portal trunk. Follow-up imaging revealed resolution of thrombosis without anticoagulation or intervention after 46 days.9
Our case was complicated by newly diagnosed cirrhotic changes found incidentally on imaging. The cause of our patients newly diagnosed cirrhotic likely evolved from a combination of alcoholic hepatitis and autoimmune hepatitis possibly worsened by IRIS in the setting of recent CD4 T-cell count increase. IRIS is characterised as worsening of a previously indolent condition, usually infection, after initiation of antiretroviral therapy. Infection is the most common manifestation of IRIS, however, it has also been seen in patients with underlying autoimmune disease such as autoimmune hepatitis.10 Moy et al present a 33-year-old woman with HIV (CD4 count of 17) who recently started combination antiretroviral therapy that presented with fever, and chills and was found to have elevated liver function tests (LFTs), a positive antimitochondrial antibody and autoimmune hepatitis in the setting IRIS.11 In our patient, complete workup for definitive diagnosis of hepatic pathology including tissue biopsy diagnosis was held until outpatient evaluation. She was not given empiric steroid therapy to treat suspected autoimmune hepatitis due to concern for possible infectious aetiology of hepatic abscesses.
Patient perspective
It was and still is the worst experience I’ve ever had in my life! It’s only the beginning and I wake up praying that nothing or no one will ever make me have to pick up another drink that may end my life! It’s not easy knowing I must change so many things to make sure I stay on the right path to a better lifestyle! I can’t do the things I love to do or eat. But I know I can do it and I will one day at a time.
Learning points
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Appreciate the spectrum of complications seen because of acute and recurrent pancreatitis.
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The paradigm for treatment of acute pancreatitis with fluids and pain management sometimes extends into cases of complicated pancreatitis.
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Recognise pancreatic pseudocyst-portal vein fistulization±portal vein thrombus as a potential complication of acute pancreatitis.
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Understand the role of inflammation in the development of localised complications of acute and recurrent pancreatitis.
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Appreciate the importance of screening for underlying autoimmune conditions in the setting of pancreatitis with concurrent cirrhosis.
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Recognise autoimmune disease in HIV-positive patients with rapidly increasing CD4 T-cell counts.
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Awareness of uniquely described clinical manifestations may expedite medical management and decrease hospital length of stay.
Ethics statements
Patient consent for publication
Footnotes
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Contributors TC, SM and LO analysed an interpreted patient data, imaging and treatment plan. TC was the major contributor in writing and editing the manuscript. All authors read and approved the final manuscript.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.
References
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