ABSTRACT
Resumen Introducción: La enfermedad de Caroli es un trastorno congénito poco usual, el cual cursa con dilatación segmentaria multifocal de los conductos biliares intrahepáticos. Fue descrita por Jacques Caroli en 1958 como una dilatación sacular, segmentada o fusiforme de los ductos biliares intrahepáticos. Objetivo: Caracterizar a la población que ha padecido la enfermedad de Caroli. Metodología: Revisión sistemática de la literatura. Resultados: Se analizaron 66 artículos, el grupo de edad más afectado es los menores de 10 años, en el que se evidenció una mayor prevalencia en varones, y se encontró asociado con otras comorbilidades como el riñón poliquístico (20 %). La manifestación más frecuente fue la hepatomegalia (44,7 %), seguido de la fiebre (42,4 %) y el dolor abdominal en el hipocondrio derecho (41,2 %). Como método diagnóstico más utilizado se encontraba la resonancia magnética (73,8 %); dentro de los hallazgos predominó la dilatación intrahepática (76,5 %) y el manejo más empleado fue la antibioticoterapia para tratar las recurrencias por colangitis. Conclusión: La enfermedad de Caroli tiene una baja prevalencia, se presenta con mayor frecuencia en el continente americano, afecta principalmente a la primera década de la vida, tiene predilección por el sexo masculino y se caracteriza por una dilatación de los conductos intrahepáticos que pueden afectar a otros órganos como los riñones, lo que produce quistes renales.
Abstract 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.
Subject(s)
Humans , Male , Female , Bile Ducts, Intrahepatic , Abdominal Pain , Caroli Disease , Dilatation , Fever , Hepatomegaly , Disease , Cysts , DiagnosisABSTRACT
BACKGROUND: Cystic liver disease is a rare disease entity, but its surgical management can vary from partial excision to hepatic lobectomy. This study was conducted to evaluate proper surgical management of cystic liver disease. METHODS: The authors reviewed the medical records of 26 patients who underwent operative management for cystic liver disease, consisting of 15 cases of simple cyst, 7 cases of biliary cystadenoma, 1 case of biliary cystadenocarcinoma, 1 case of polycystic disease and 2 cases of Caroli's disease. RESULTS: The 26 patients had a mean age of 57, with a male to female ratio of 1:4. Patients' main symptoms were right upper quadrant pain or discomfort, and epigastric pain. Their main physical findings were palpable mass and direct tenderness, but 23% of patients were aymptomatic and 35% had no physical findings. Preoperative diagnosis was made with abdominal ultrasound and computed tomogram. Intervening septum was identified in 27% of simple cysts and 100% of biliary cystadenoma. However neither diagnostic tool has 100% diagnostic accuracy. All cysts except polycystic disease were located at either both hepatic lobes but more right lobe in simple cysts. The mean size of all cysts were 10.2cm ranging from 2-to-22cm without any difference among simple cysts, cystadenomas, and cystadenocarcinoma. Operative methods for simple cysts were partial cystectomy with marsulpialization, total cystectomy and hepatic segmentectomy in 87% of cases, of which 2 cases were performed by laparoscopic approach. However partial cystectomy was confined to unilocular simple cyst. There was no recurrence of simple cyst postoperatively. Biliary cystadenoma were managed by total cyst excision, hepatic segmentectomy or lobectomy in 86% of cases, but one cystadenoma was managed by partial cystectomy with marsulpialization had a persisting cyst. One patient with polycystic liver disease died because of overwhelming sepsis with postoperative acute renal failure. CONCLUSION: For unilocular simple cyst, partial cystectomy with marsulpialization can be an acceptable surgical procedure, even with a laparoscopic approach. But for biliary cystadenoma, the cyst should be totally removed at least to prevent recurrence and transformation to cystadenocarcinoma. Therefore, correct preoperative diagnosis is a prerequisite for appropriate selection of operative methods.