Enfermedad y síndrome de Caroli. Presentación de dos casos y revisión de la literatura
Keywords:
ictericia colestásica, Caroli, enfermedad fibropoliquistica (es)Downloads
Se presentan dos casos clínicos, con cuadro de ictericia obstructiva prolongada, por malformación de la las vías biliares intrahepáticas. El primero corresponde a una mujer adulta con quistes y litiasis de los conductos biliares mayores intrahepáticos y cuyas imágenes (ecografía y TAC) establecieron el diagnóstico de Enfermedad de Caroli y permitieron una resección parcial de la lesión con mejoría clínica. El segundo pertenece a una niña de cuatro años, quien clínicamente además del cuadro ictérico tenía hipertensión portal; en ella el diagnóstico se hizo mediante la necropsia, que mostró várices esofágicas sangrantes y además de las lesiones quísticas con litiasis intrahepática, un severo cuadro de fibrosis hepática congénita asociada, que explica el proceso hipertensivo portal. Estas dos lesiones hepáticas conforman el diagnóstico de Síndrome de Caroli. Se analizan los aspectos clínicos en cada uno de ellos, los hallazgos histopatológicos y se revisa además la patogenia de las entidades.
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