Caroli's disease classification: Difference between revisions

Jump to navigation Jump to search
 
Line 16: Line 16:
:::* [[Renal]] [[cystic]] lesions also often occur
:::* [[Renal]] [[cystic]] lesions also often occur
::* [[Hepatic]] function is preserved in most, but many patients have recurrent [[cholangitis]], [[liver]] [[abscesses]] and [[portal hypertension]].
::* [[Hepatic]] function is preserved in most, but many patients have recurrent [[cholangitis]], [[liver]] [[abscesses]] and [[portal hypertension]].
* The [[cysts]] are nonobstructive saccular or fusiform dilatations of the [[intrahepatic]] ducts.
* The [[cysts]] are nonobstructive [[saccular]] or [[fusiform]] dilatations of the [[intrahepatic]] ducts.
* The disease may be unilobed or bilobed.  Unilobed disease is most commonly localized to the left lobe of the [[liver]].
* The disease may be unilobed or bilobed.  Unilobed disease is most commonly localized to the left lobe of the [[liver]].
* Some reviews have reported that pure [[Caroli’s disease]] is not that common, and more patients actually have Type IVA disease, with both intra- and extra-hepatic [[cysts]].
* Some reviews have reported that pure [[Caroli’s disease]] is not that common, and more patients actually have Type IVA disease, with both intra- and extra-hepatic [[cysts]].
* Intrahepatic [[biliary]] cystic disease should be differentiated from [[polycystic liver disease]], an autosomal dominant disorder characterized by cystic collections of [[serous]] fluid not in communication with the [[biliary tree]].
* Intrahepatic [[biliary]] cystic disease should be differentiated from [[polycystic liver disease]], an autosomal dominant disorder characterized by cystic collections of [[serous]] fluid not in communication with the [[biliary tree]].
* The incidence of [[malignant]] transformation in intrahepatic cysts is ~7% in Caroli’s disease.
* The incidence of [[malignant]] transformation in intrahepatic [[cysts]] is ~7% in Caroli’s disease.


==References==
==References==

Latest revision as of 20:04, 11 March 2022

Caroli's disease Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Caroli's disease from other Diseases

Epidemiology and Demographics

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Treatment

Medical Therapy

Surgery

Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Caroli's disease classification On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Caroli's disease classification

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Caroli's disease classification

CDC on Caroli's disease classification

Caroli's disease classification in the news

Blogs on Caroli's disease classification

Directions to Hospitals Treating Caroli's disease

Risk calculators and risk factors for Caroli's disease classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Rinky Agnes Botleroo, M.B.B.S.

Classification

  • Simple type

:* Periportal fibrosis type

  • Also associated with:
  • The cysts are nonobstructive saccular or fusiform dilatations of the intrahepatic ducts.
  • The disease may be unilobed or bilobed. Unilobed disease is most commonly localized to the left lobe of the liver.
  • Some reviews have reported that pure Caroli’s disease is not that common, and more patients actually have Type IVA disease, with both intra- and extra-hepatic cysts.
  • Intrahepatic biliary cystic disease should be differentiated from polycystic liver disease, an autosomal dominant disorder characterized by cystic collections of serous fluid not in communication with the biliary tree.
  • The incidence of malignant transformation in intrahepatic cysts is ~7% in Caroli’s disease.

References