Enfermedad de Caroli: A propósito de un caso clínico

La enfermedad de Caroli es una malformacion congenita quistica de la via biliar intrahepatica, de transmision autosomica recesiva. Se distinguen dos formas: fibrosis hepatica simple, y una forma en que predomina una fibrosis periportal conocida como "fibrosis hepatica congenita", individualizada en la literatura como sindrome de Caroli. La mayoria de los pacientes presenta compromiso de ambos lobulos del higado, aun cuando hay casos de enfermedad localizada en un lobulo o segmento, de preferencia el izquierdo. Puede manifestarse en cualquier epoca de la vida aunque es rara a partir de los 50 anos y la forma de presentacion mas frecuente se caracteriza por episodios de fiebre en relacion con crisis colangiticas secundarias a estasis biliar. En la forma compleja predominan manifestaciones de hipertension portal. Las complicaciones tardias mas habituales son la cirrosis biliar secundaria, los abscesos intrahepaticos y los tumores, siendo el mas frecuente el colangiocarcinoma. Se presenta caso paciente femenina de 45 anos de edad, sin antecedentes de importancia quien ingresa al servicio de cirugia general por presentar dolor de aparicion insidiosa de tres dias de evolucion, en hipocondrio derecho, moderada intensidad, caracter opresivo, irradiado a flanco y region lumbar derecha, sin atenuantes, exacerbado con el movimiento, sin concomitantes. Al examen fisico: TA 150/80 mmHg abdomen globoso propio de paniculo adiposo, doloroso a la palpacion profunda en hipocondrio y flanco derecho, timpanismo generalizado, borde hepatico palpable 2 cm. por debajo de reborde costal. Paraclinicos dentro de valores normales. Ultrasonido el cual reporta multiples imagenes anecoicas en ambos lobulos hepaticos; diagnostico sugestivo de Enfermedad de Caroli. Colangioresonancia reporto enfermedad multiquistica hepatica en relacion a Enfermedad de Caroli. Actualmente, se encuentra asintomatica en espera de resolucion por trasplante Hepatico. SUMMARY Caroli´s disease is a cystic congenital malformation of the intrahepatic biliary tract, of autosomal recessive transmission. There are two kinds: simple hepatic fibrosis, and a form in which predominates a well-known periportal fibrosis known as “congenital hepatic fibrosis”, This entity has been named Caroli’s syndrome. In most patients, it is present in both lobes of the liver, even though there are cases where the disease is located in only one lobe or segment, of preference the left one. It can appear at any time in life, however, it is rare after 50 years of age, The most common form is characterized by episodes of fever related to cholangitis, secondary to biliary stasis. In more complex forms, portal hypertension is the main finding. The more frequent complications are: secondary biliary cirrhosis, intrahepatic abscesses and tumors, colangiocarcinoma being the most frequent. This case is about a female patient of 45 years old, without relevant antecedents. She complained of insidious pain during the last three days, before admission, located in the right hypochondrium, of moderate intensity, and oppressive character, radiated to the right flank and right lumbar region without attenuation. it increased with movement without other accompanying symptoms. Physical examination: TA 150/80 mmHg, painfulabdomen to the deep palpation in hypochondrium and right flank, hepatic border 2 cm. below costal rim. Laboratory test were normal. Ultrasound showed anechoic images in both hepatics lobes, suggestive of Caroli´s disease. Images from magnetic resonance reported hepatic cystic disease related to Caroli’s disease. Presently she is asymptomatic, and waiting for a hepatic transplant.