Korean J Gastroenterol.  2011 Jan;57(1):51-53. 10.4166/kjg.2011.57.1.51.

Caroli Syndrome with Autosomal Recessive Polycystic Kidney Disease

• Ko JS

• KMID: 991470
• DOI: http://doi.org/10.4166/kjg.2011.57.1.51

Abstract

No abstract available.

MeSH Terms

Caroli Disease/complications/*diagnosis
Child
Cholangiopancreatography, Magnetic Resonance
Creatinine/blood
Female
Humans
Liver/pathology
Liver Transplantation
Polycystic Kidney, Autosomal Recessive/complications/*diagnosis/ultrasonography
Tomography, X-Ray Computed

Figure

• 

  Fig. 1. (A) A transverse ultrasonography of the liver showed coarse echogenicity of the liver parenchyme and mild intrahepatic duct dilatation (arrows). (B) Longitudinal ultrasonography showed enlarged kidney with increased echogenicity resulting from the multiple acoustic interfaces of the cysts.

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  Fig. 2. Dilataion of the intrahepatic and extrahepatic bile ducts was demonstrated on MRCP (A). Note the multiple cysts in the kidneys on coronal T2 HASTE image (arrows, B).

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  Fig. 3. Histology of liver showed diffuse periportal fibrosis, some cystically dilated bile ducts containing bile plugs, and bile ductular proliferation (H&E stain, ×200).

Reference

References

1. Taylor AC, Palmer KR. Caroli's disease. Eur J Gastroenterol Hepatol. 1998; 10:105–108.

2. Kaplan BS, Kaplan P, de Chadarevian JP, Jequier S, O'Regan S, Russo P. Variable expression of autosomal recessive polycystic kidney disease and congenital hepatic fibrosis within a family. Am J Med Genet. 1988; 29:639–647.

3. Dayton MT, Longmire WP Jr, Tompkins RK. Caroli's Disease: a premalignant condition? Am J Surg. 1983; 145:41–48.

4. Chapman RW. Risk factors for biliary tract carcinogenesis. Ann Oncol. 1999; 10(Suppl 4):308–311.

5. Ulrich F, Pratschke J, Pascher A, et al. Longterm outcome of liver resection and transplantation for Caroli disease and syndrome. Ann Surg. 2008; 247:357–364.

6. Habib S, Shakil O, Couto OF, et al. Caroli's disease and orthotopic liver transplantation. Liver Transpl. 2006; 12:416–421.

7. De Kerckhove L, De Meyer M, Verbaandert C, et al. The place of liver transplantation in Caroli's disease and syndrome. Transpl Int. 2006; 19:381–388.

8. Millwala F, Segev DL, Thuluvath PJ. Caroli's disease and outcomes after liver transplantation. Liver Transpl. 2008; 14:11–17.

9. Meier C, Deutscher J, Müller S, et al. Successful liver transplantation in a child with Caroli's disease. Pediatr Transplant. 2008; 12:483–486.