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Septic Pylephlebitis and Cavernous Transformation Presenting as Pyrexia of Unknown Origin: A Diagnostic Challenge
Aditi Sarker1, Prodipta Chowdhury2, Md Razibul Alam1
1Department of Gastroenterology Bangabandhu Sheikh Mujib Medical University Dhaka Bangladesh.
Abstract:
Acute cholangitis classically presents with fever, jaundice, and right upper quadrant pain, but atypical or subacute disease may present as pyrexia of unknown origin (PUO) and remain unrecognized until vascular complications develop. We report a 45-year-old man referred for weight loss, malaise, and suspected non-cirrhotic portal hypertension after upper gastrointestinal endoscopy showed esophageal varices despite a normal FibroScan. Detailed history revealed intermittent fever for 2 months. Laboratory evaluation demonstrated marked systemic inflammation, with leukocytosis, neutrophilia, ferritin 1095 ng/mL, and C-reactive protein > 200 mg/L, together with a cholestatic-hepatitic liver enzyme pattern and preserved liver synthetic function. Blood and urine cultures were negative. Initial ultrasonography showed bile duct dilatation without a definite obstructing lesion, delaying diagnosis. Subsequent magnetic resonance cholangiopancreatography and contrast-enhanced computed tomography demonstrated biliary abnormality with portal vein thrombosis and periportal collateralization, consistent with septic pylephlebitis, cavernous transformation, and portal biliopathy secondary to occult cholangitis. The patient was treated with intravenous broad-spectrum antibiotics and anticoagulation, with clinical improvement and partial portal vein recanalisation on follow-up ultrasonography at 3 months. This case highlights that occult cholangitis should be considered in patients with PUO and cholestatic liver biochemistry, even in the absence of classic biliary symptoms, and that early cross-sectional biliary imaging may allow timely recognition of pylephlebitis before chronic portal hypertensive and biliary sequelae become established.
Insights
Occult cholangitis can present as pyrexia of unknown origin (PUO) with liver enzyme changes. Early cross-sectional imaging is crucial for diagnosing this condition and preventing complications like portal vein thrombosis.
Area of Science:
- Gastroenterology
- Hepatology
- Infectious Disease
Background:
- Acute cholangitis typically presents with fever, jaundice, and RUQ pain.
- Atypical or subacute cholangitis may manifest as pyrexia of unknown origin (PUO), delaying diagnosis and leading to vascular complications.
- Non-cirrhotic portal hypertension with esophageal varices can be an indirect sign of underlying biliary pathology.
Purpose of the Study:
- To highlight the importance of considering occult cholangitis in patients presenting with PUO and cholestatic liver biochemistry.
- To emphasize the role of early cross-sectional biliary imaging in diagnosing occult cholangitis and associated pylephlebitis.
- To illustrate the potential for severe vascular complications if occult cholangitis remains unrecognized.
Main Methods:
- Case report of a 45-year-old man with PUO, weight loss, and suspected non-cirrhotic portal hypertension.
- Detailed patient history, laboratory evaluation (including inflammatory markers and liver function tests), and serial imaging (ultrasonography, MRCP, contrast-enhanced CT).
- Treatment involved intravenous antibiotics and anticoagulation.
Main Results:
- The patient presented with PUO, elevated inflammatory markers, and a cholestatic-hepatitic liver enzyme pattern.
- Initial ultrasonography was inconclusive, but subsequent MRCP and CT revealed biliary abnormalities, portal vein thrombosis, and signs of septic pylephlebitis and portal biliopathy secondary to occult cholangitis.
- Treatment led to clinical improvement and partial portal vein recanalization.
Conclusions:
- Occult cholangitis should be suspected in patients with PUO and cholestatic liver enzymes, even without classic biliary symptoms.
- Early cross-sectional biliary imaging is vital for timely diagnosis of occult cholangitis and associated pylephlebitis.
- Prompt recognition and treatment can prevent the development of chronic portal hypertensive and biliary sequelae.
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