Caroli disease is a rare affection. It’s commonly
associated to polycystic kidneys. We report a case of a nine-year-old girl. She
was hospitalized at the pediatric unit of the Cocody Teaching Hospital for an
intermittent fever. During the clinical examination, we found a likely
cirrhotic hepatomegaly associated to an edematous-ascitic syndrome. Biological
exams showed an infectious syndrome with ahyperleukocytosis at 13.000 per mm3, a
hyperneutrophilia at 9.600 per mm3. Serological viral markers of B,
C and D hepatitis were negative. We saw an intra-hepatic cystic picture at the
liver CT-scan with a vascular lesion at his center called a “dot sign”. The
diagnosis of Caroli disease could be evocated.
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Steinbruck, K., Enne, M., Martinho, J.M., Fernandes, R. and Pacheco-Moreira, L.F. (2011) Living Donor Liver Transplantation for Caroli’s Disease: A Report of Two Cases. ISRN Surgery, 2011, Article ID: 106487. https://doi.org/10.5402/2011/106487
Iancu, C., Bodea, R., Muresan, T., Iancu, D., Boruah, P. and Al Hajjar, N. (2010) Diffuse Form of Caroli’s Disease: Therapeutical Approach in a Female Patient with Recurrent Cholangitis. The Journal of Gastrointestinal and Liver Diseases, 19, 457-460.
Last, R.D., Hill, J.M., Roach, M., et al. (2006) Congenital Dilatation of the Large and Segmental Intrahepatic Bile Ducts (Caroli’s Disease) in Two Golden Retriever Littermates. Journal of the South African Veterinary Association, 77, 210-214. https://doi.org/10.4102/jsava.v77i4.379
Srinath, A. and Shneider, B.L. (2012) Congenital Hepatic Fibrosis and Autosomal Recessive Polycystic Kidney Disease. Journal of Pediatric Gastroenterology and Nutrition, 54, 580-587. https://doi.org/10.1097/MPG.0b013e31824711b7
Gunay-Aygun, M., Font-Montgomery, E., Lukose, L., et al. (2012) Characteristics of Congenital Hepatic Fibrosis in a Large Cohort of Patients With Autosomal Recessive Polycystic Kidney Disease. Gastroenterology, 144, 112-121. https://doi.org/10.1053/j.gastro.2012.09.056