Síndrome de Caroli: un caso de comienzo en la infancia y buena evolución

Rivero, M.J.; Román, E.; Cilleruelo, M.L.; Sánchez, F.; Barrio, J.

doi:10.1016/S1695-4033(00)77416-9

Anales de Pediatría (English Edition)

ISSN: 2341-2879

The Spanish Pediatric Association (Asociación Española de Pediatría, AEP) has as one of its main objectives the dissemination of rigorous and up-to-date scientific information on the various areas of pediatrics. Annals of Pediatrics is an open access journal that serves as the Association's Scientific Expression Organ and constitutes the vehicle through which members communicate. Annals of Pediatrics publishes, in English and Spanish, original research papers on pediatrics, as well as review articles prepared by experts in each specialty, and guidelines or positioning documents prepared by the different Societies/Specialized Sections integrated into the Spanish Pediatric Association. The journal, a reference for Spanish-speaking pediatrics, is indexed in the most important international databases: Index Medicus/Medline IBECS, IME, SCOPUS, Science Citation Index Expanded, Journal Citations Report, Embase/Excerpta Medica, Directory of Open Access Journals (DOAJ).

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Index Medicus/Medline IBECS, IME, SCOPUS, Science Citation Index Expanded, Journal Citations Report, Embase/Excerpta Medica, Directory of Open Access Journals (DOAJ)

La enfermedad de Caroli es una rara entidad que se incluye entre las malformaciones fibroquísticas de la vía biliar. El patrón ecográfico consiste en dilataciones evidentes de los ductos biliares. Aunque se piensa que es una enfermedad congénita, frecuentemente se manifiesta enadultos jóvenes, y existen pocos casos descritos en niños.Presentamos un caso de un niño de 10 años con síndrome de Caroli (enfermedad de Caroli, fibrosis hepáticacongénita y poliquistosis renal) con buena evolución.

Palabras clave:

Síndrome de Caroli

Malformaciones fibroquísticas

Ductos biliares

Caroli's disease is a rare entity that is included in the fibropolycystic abnormalities of the bile ducts. Ultrasonographic patterns consist of evident dilatation of the bileducts. Although it is thought to be a congenital disease, itusually presents in young adults and few cases have been reported in children.We present the case of a 10-year-old boy with Caroli's syndrome (Caroli's disease, congenital hepatic fibrosisand polycystic renal disease). Evolution was favorable.

Key words:

Caroli's syndrome

Fibropolycystic abnormalities

Bile ductus

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