Caroli’s disease: Description of a case with a benign clinical course
A 12-year-old boy was admitted to the Pediatrics Department of our Hospital, because of high fever (40.5°C) with shivers and moderate pain in the right upper quadrant of the abdomen, for the previous two days. The pain increased during the rise in fever. Before his admission a course of antibiotics (amoxicilline-clavulanic acid) had preceded without any response. On physical examination the liver and spleen were just palpable, liver (+1 cm) and spleen (+1cm) under costal margins. Interestingly a slight tenderness in the right hypochondrium was found only as the fever rose. No jaundice, ascites, splenomegaly or venous dilatation of abdominal wall were observed. The physical examination of the other systems was normal. Blood pressure was repeatedly between 100-110 / 60-70 mmHg.\nA month before this episode, he had been hospitalized again because of an episode with similar clinical picture (fever for 8 days: 38-40.5°C, with shivers, muscle aches and diffuse abdominal pain) and he was treated for suspected bacteremia for 10 days. The laboratory findings on these two admissions appear on and it is noteworthy that during the second admission the laboratory findings of bacterial infection were more obvious (although with negative cultures) in addition to a slight elevation of transaminases. His medical history was extremely interesting in that he had had two consecutive hospitalizations at the age of 2.5 years, because of suspected bacteremia. At that time he presented again with elevated WBC, CRP, ESR, negative cultures, normal immune profile and normal liver function tests, so an ultrasound (U/S) of the abdomen was not considered necessary. In the intermediate period (the last 10 years) he was asymptomatic with normal growth (weight and height: between 50th to 75th percentile, according to our national charts) and without any other chronic illness.\nDuring the last hospitalization (4th episode), because of the localization of moderate pain in the right upper quadrant of abdomen during the rise in fever, an U/S of the abdomen was performed and revealed two small cystic formations in the superior part of right upper lobe of the liver.\nAccording to the clinical picture and to the above U/S findings, more extensive radiological investigation was performed. CT scan of abdomen revealed several small cystic formations in the VII segment of the liver. MRI of the liver showed several well-defined round lesions (maximum diameter 2 cm), hyperintense on T2 and hypointense on T1 -weighted images. There was no contrast enhancement after intravenous contrast (Gadolinium DTPA) injection, therefore these findings were thought to be cysts [, ].\nIn addition, there were no specific findings from other organs (kidneys, pancreas, spleen etc) or signs of portal hypertention.\nA subsequent MRCP revealed communication of the cystic formations with the biliary tree [], compatible with Caroli’s disease.\nThe child was treated for cholangitis. He was afeverish on the 4th day of treatment. The abdominal pain subsided later (on 9th day).\nHe was discharged in generally good condition with the advice of frequent follow-up studies, including liver function tests (AST, ALT, γGT, CEA, CA19-9, aFP etc.) and U/S of the liver.\nSince, he has been followed-up systematically for ten years and remains asymptomatic, with normal blood pressure, normal growth, normal laboratory findings and unchanged imaging (U/S and MRI) parameters, in follow-up studies.