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home - Liver - Liver Masses - Carolis Disease Written by Dr Sebastian Zeki
Knowledge


Knows the epidemiology pathology clinical presentation and natural
history of benign tumours of the liver

Can define a programme of investigation and characterisation of
benign liver lesions including haemangioma focal modular
hyperplasia and adenoma

Skills
Demonstrates ability to make an appropriate differential diagnosis
Formulates appropriate plan of management
Behaviours
Recognises importance of the role of multidisciplinary team in
diagnosis and management

Also...

Knowledge


Understands the epidemiology risk factors pathology prevalence
and range of presentations of HCC

Knows the appropriate investigation and staging of the disease
Aware of treatment options including trans-arterial
chemoembolisation (TACE) radiofrequency ablation (RFA) local
ethanol injection

Appreciates the indications and contraindications of each and how
the most appropriate is selected Aware of surgical treatment options

Aware of role of surveillance and referral for specialist multidisciplinary management including liaison with oncology

Skills
Appreciates the indications and contraindications of each modality of
treatment and how the most appropriate is selected

Understands the process of selection of patients for liver resection or
transplantation

Behaviours
Appreciates Involvement of multi-disciplinary team in management
decisions close liaison with surgical radiology oncology and
pathology colleagues

Carolis Disease

Diagnosis Cysts can be seen on USS/ ERCP/ MRI. A liver biopsy is rarely required. Treatment Treatment is as per chronic cholestasis. An unexplained clinical deterioration/ new biliary stricture suggests cholangiocarcinoma although surveillance is not recommended. Removal of stones in both CBD and IHD as necessary. Partial hepatectomy may be curative if the cysts are in a single lobe of the liver. Complications of portal hypertension/ recurrent biliary infec - tions may need liver transplantation. Caroli's disease (Type 5 coledochal cyst) Pathological Features: Segmental, saccular dilatations of the large intrahepatic bile ducts in left lobe. Dilated ducts lined by biliary epithelium that may be hyperplastic and ulcerated. Congenital fibrosis. Hypoplasia of the portal vein branches. Acute and chronic inflammatory cell infiltrate around the dilated bile ducts. Chromosome 6 ARPKD fibrocystin Regulation of cell prolif - eration, and of cellular adhesion and repulsion No homology with the proteins responsible for autosomal dominant polycystic kidney disease. The pathogenesis is related to an arrest or derangement in remod - eling of the ductal plate of the larger intrahepatic bile ducts during development. Prognosis This depends on severity and coexisting renal dysfunction. The risk of cholangiocarcinoma is increased (up to 7 %). Amyloidosis has also been described due to the inflamm - tion from chronic or recurrent cholangitis. Clinical Presentation Features: Renal disease/ portal hypertension. Can cause biliary stagnation and cholangitis. Enlarged liver. Portal hypertension Inc alkaline phosphatase, direct bilirubin, and neutrophilia. Written by Dr Sebastian Zeki

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