Porto pulmonary hypertension due to progression of secondary biliary cirrhosis following Kasai procedure

Primary sclerosing cholangitis (PSC) is a chronic progressive cholestatic disease characterized by inflammation and fibrosis that may involve the entire biliary tree. The fibrosis causes diffuse narrowing of the intrahepatic and extrahepatic bile ducts, and the resulting biliary stasis leads to secondary biliary cirrhosis and associated complications. This review addresses PSC through its epidemiology, etiopathogenesis, diagnosis, differential diagnosis, management, complications, and prognosis. Figures show pouchoscopy, liver biopsy, and endoscopic retrograde cholangiopancreatography; direct peroral cholangioscopy visualization of cholangiocarcinoma; and algorithms depicting diagnosis of cholangiocarcinoma and screening for inflammatory bowel disease (IBD) in patients with PSC. Tables list characteristics of IBD in PSC, prevalence of antibodies in PSC, differential diagnosis of PSC, diagnostic approach in PSC, and risk of cancers in PSC. This review contains 6 highly rendered figures, 5 tables, and 91 references.

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Mucormycosis in a liver allograft after liver transplantation for secondary biliary cirrhosis due to bile duct injury

Journal of Clinical Case reports and reviews ◽

10.36879/jccrr.20.000152 ◽

2020 ◽

pp. 1-3

Keyword(s):

Bile Duct ◽

Bile Duct Injury ◽

Percutaneous Biopsy ◽

Exploratory Laparotomy ◽

Biliary Cirrhosis ◽

Organic Failure ◽

Opportunistic Mycoses ◽

Rhizopus Sp ◽

Abdominal Packing ◽

Secondary Biliary Cirrhosis

Introduction: Mucormycosis refers to a group of opportunistic mycoses that occur generally in immunocompromised patients and are caused by Mucorales, ubiquitous filamentous fungi with broad, thin-walled, sparsely septate, ribbon-like hyphae. Case report: A 57-year-old man with a history of secondary biliary cirrhosis due to inadvertent bile duct injury during cholecystectomy. He was referred to our center and underwent LT on June 2018. Due to severe coagulopathy he underwent exploratory laparotomy and abdominal packing for 48 hours. He recovered with good liver function and LFT´s with a tendency towards normalization. On post op day 8 the patient presented an episode of fever and a CT scan was performed showing a large zone of hypoperfusion with bubbles of gas in the liver dome. A percutaneous biopsy was taken for cultures. Preliminary results reported a filamentous fungus and liposomal amphotericin b was initiated with the suspicion of mucormycosis. The patient remained afebrile and asymptomatic. After 5 days of treatment a new image was performed, and progression of the lesion was noticed, due to these findings the patient was taken to the OR for surgical debridement. Involvement of the liver dome and diaphragm was noticed and a non-anatomic hepatectomy was performed. After surgery the patient required increasing amounts of vasopressors. Despite all the support he progressed to multiple organic failure and finally expired. The product of hepatectomy confirmed the diagnosis of mucormycosis (Rhizopus sp). Discussion: Despite all the efforts the patients’ clinical condition deteriorated after surgery showing the high mortality rate in liver transplant recipients that has been reported of 50 to 100%.

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