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. 1984 Dec;200(6):718–723. doi: 10.1097/00000658-198412000-00008

Successful treatment of Caroli's disease by hepatic resection. Report of six patients.

N Nagasue
PMCID: PMC1250588  PMID: 6508401

Abstract

Caroli's disease is a congenital disease of cystic or saccular dilatation of the intrahepatic bile ducts. There are two disease entities: a simple type and a periportal fibrosis type. Frequent complications with the simple type are recurrent cholangitis, liver abscess, intraductal lithiasis, abdominal pain, and fever that often lead to fatal sepsis. Development of portal hypertension and esophageal varices is usually a final feature of the periportal fibrosis type. Malignancies are also possible complications with Caroli's disease. During the recent 13 years, the author had experiences with eight patients with Caroli's disease of the simple type; six of these eight underwent hepatic resection: right lobectomy in two, left lobectomy in three, and left lateral segmentectomy in one. Other two patients died of sepsis and cholangiocellular carcinoma, respectively. All six patients with hepatic resections were relieved from the disabling symptoms after surgery and have had no recurrent hepatobiliary problems for 3 months to 13 years. Hepatic resection may be indicated for more patients than previously assumed in the treatment of Caroli's disease of the simple type.

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Selected References

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  1. Adson M. A., Nagorney D. M. Hepatic resection for intrahepatic ductal stones. Arch Surg. 1982 May;117(5):611–616. doi: 10.1001/archsurg.1982.01380290067012. [DOI] [PubMed] [Google Scholar]
  2. CAROLI J., COUINAUD C., SOUPAULT R., PORCHER P., ETEVE J. Une affection nouvelle, sans doute congénitale, des voies biliaires: la dilatation kystique unilobaire des canaux hépatiques. Sem Hop. 1958 Feb 18;34(8/2):496–502/SP. [PubMed] [Google Scholar]
  3. Caroli J. Diseases of the intrahepatic biliary tree. Clin Gastroenterol. 1973 Jan;2(1):147–161. [PubMed] [Google Scholar]
  4. Chaudhuri P. K., Chaudhuri B., Schuler J. J., Nyhus L. M. Carcinoma associated with congenital cystic dilation of bile ducts. Arch Surg. 1982 Oct;117(10):1349–1351. doi: 10.1001/archsurg.1982.01380340067016. [DOI] [PubMed] [Google Scholar]
  5. Dayton M. T., Longmire W. P., Jr, Tompkins R. K. Caroli's Disease: a premalignant condition? Am J Surg. 1983 Jan;145(1):41–48. doi: 10.1016/0002-9610(83)90164-2. [DOI] [PubMed] [Google Scholar]
  6. GLENN F., MOODY F. G. Intrahepatic calculi. Ann Surg. 1961 May;153:711–724. doi: 10.1097/00000658-196105000-00010. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Kaiser J. A., Mall J. C., Salmen B. J., Parker J. J. Diagnosis of Caroli disease by computed tomography: report of two cases. Radiology. 1979 Sep;132(3):661–664. doi: 10.1148/132.3.661. [DOI] [PubMed] [Google Scholar]
  8. Longmire W. P., Jr, Mandiola S. A., Gordon H. E. Congenital cystic disease of the liver and biliary system. Ann Surg. 1971 Oct;174(4):711–726. doi: 10.1097/00000658-197110000-00014. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Mittelstaedt C. A., Volberg F. M., Fischer G. J., McCartney W. H. Caroli's disease: sonographic findings. AJR Am J Roentgenol. 1980 Mar;134(3):585–587. doi: 10.2214/ajr.134.3.585. [DOI] [PubMed] [Google Scholar]
  10. Musante F., Derchi L. E., Bonati P. CT cholangiography in suspected Caroli's disease. J Comput Assist Tomogr. 1982 Jun;6(3):482–485. doi: 10.1097/00004728-198206000-00007. [DOI] [PubMed] [Google Scholar]
  11. Nagase M., Hikasa Y., Soloway R. D., Tanimura H., Setoyama M., Kato H. Gallstones in Western Japan. Factors affecting the prevalence of intrahepatic gallstones. Gastroenterology. 1980 Apr;78(4):684–690. [PubMed] [Google Scholar]
  12. Pridgen J. E., Jr, Aust J. B., McInnis W. D. Primary intrahepatic gallstones. Arch Surg. 1977 Sep;112(9):1037–1043. doi: 10.1001/archsurg.1977.01370090019004. [DOI] [PubMed] [Google Scholar]
  13. Sato T., Suzuki N., Takahashi W., Uematsu I. Surgical management of intrahepatic gallstones. Ann Surg. 1980 Jul;192(1):28–32. doi: 10.1097/00000658-198007000-00005. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Schrumpf E., Bergan A., Blomhoff J. P., Hoel P. S., Kolmannskog F. Partial hepatectomy in Caroli's disease. Scand J Gastroenterol. 1981;16(5):581–585. doi: 10.3109/00365528109182015. [DOI] [PubMed] [Google Scholar]
  15. Simi M., Loriga P., Basoli A., Leardi S., Speranza V. Intrahepatic lithiasis. Study of thirty-six cases and review of the literature. Am J Surg. 1979 Mar;137(3):317–322. doi: 10.1016/0002-9610(79)90058-8. [DOI] [PubMed] [Google Scholar]
  16. Song E., Kew M. C., Grieve T., Isaacson C., Myburgh J. A. Primary squamous cell carcinoma of the liver occurring in association with hepatolithiasis. Cancer. 1984 Feb 1;53(3):542–546. doi: 10.1002/1097-0142(19840201)53:3<542::aid-cncr2820530328>3.0.co;2-3. [DOI] [PubMed] [Google Scholar]
  17. Sorensen K. W., Glazer G. M., Francis I. R. Diagnosis of cystic ectasia of intrahepatic bile ducts by computed tomography. J Comput Assist Tomogr. 1982 Jun;6(3):486–489. doi: 10.1097/00004728-198206000-00008. [DOI] [PubMed] [Google Scholar]
  18. Vallent K., Vadon G., Szebeni A., Papp J., Jánossa M. Mit Hepato-Lobektomie operierter Fall einer congenitalen, cystischen, intrahepatischen Gallengangserweiterung (Caroli-Syndrom). Chirurg. 1982 Mar;53(3):131–136. [PubMed] [Google Scholar]

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