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Revista colombiana de Gastroenterología
Print version ISSN 0120-9957On-line version ISSN 2500-7440
Abstract
VACCA-CARVAJAL, Bryan Felipe; VASQUEZ-ILES, José Mario; RANGEL-PEREIRA, María Gabriela and VARGAS-RODRIGUEZ, Ledmar Jovanny. Characterization of patients with Caroli’s disease: Systematic review. Rev. colomb. Gastroenterol. [online]. 2021, vol.36, n.2, pp.180-190. Epub Nov 24, 2021. ISSN 0120-9957. https://doi.org/10.22516/25007440.612.
Caroli’s disease (CD) is a rare congenital disease, which presents with multifocal segmental intrahepatic bile duct dilatation. It was first described by Jacques Caroli in 1958 as a saccular or fusiform dilatation of the intrahepatic bile ducts.
Objective:
To characterize the population that has been diagnosed with Caroli’s disease.
Materials and methods:
Systematic review.
Results:
66 articles were analyzed. The age group most affected was children under 10 years old, where a higher prevalence was evidenced in males. This condition was associated with other comorbidities such as polycystic kidney in 20%. The most frequent manifestation was hepatomegaly (44.7%), followed by fever (42.4%), and abdominal pain in the right upper quadrant (41.2%). The most used diagnostic method was magnetic resonance imaging in 73.8% of the sample. The findings showed predominance of intra-hepatic dilatation in 76.5%. The most widely used treatment was antibiotic therapy to treat recurrences due to cholangitis.
Conclusion:
Caroli’s disease has an extremely low incidence and occurs more frequently in the American continent, affecting mainly patients in the first decade of life, with a predilection for the male sex. It is characterized by a dilatation of the intrahepatic ducts that can affect other organs such as the kidneys, causing renal cysts.
Keywords : Caroli’s disease; Cystic liver disease; Intrahepatic dilatation; Portal hypertension; Hepatomegaly; Cystic dilation; Newborn diseases.
