Caroli’s Disease vs Caroli Syndrome: Clinical and Radiological Spectrum, with disease Outcome at a Teaching Hospital in Lahore

Abstract

Background: Caroli disease and Caroli syndrome are part of the clinical spectrum of ductal plate (DP) malformations, also referred to, as the fibrocystic liver diseases. This article aimed to study both these conditions, in terms of clinical features and disease outcome at a teaching hospital. Objective: To describe the clinical spectrum, laboratory and imaging findings, management course and outcome of fibrocystic liver diseases in children, at the University of Child Health Sciences, Lahore, Pakistan. Study Design: Cross-sectional Study. Settings: Department of Pediatric Gastroenterology & Hepatology, University of Child Health Sciences, Lahore Pakistan. Duration: January 2014 to July 2017. Methods: The study included patients with Caroli disease and Caroli Syndrome, confirmed on the basis of characteristic findings on magnetic resonance cholangiopancreatography. SPSS v.20 was used for statistical analysis. Results: The mean age of 15 patients included in the study was 6.54 ± 4.18 years. Among them, 7/15 (46.7%) had Caroli’s disease (CD), whereas 8/15 (53.3%) had Caroli’s syndrome (CS). Male to female ratio was 4:1. CS pre-dominantly affected male children (8:0), while CD affected both genders almost equally (4:3). CD commonly presented with recurrent cholangitis (6/7, 85%), while CS as portal hypertension (7/8, 87.5%). Renal involvement was found in 5/15(33.33%) patients; mostly in CS (4/5,80%) and one (6.66%) patient progressed to end stage renal disease. One patient of CD underwent lobectomy and one died of end stage liver disease. Two patients of CS were treated with shunt surgery with partial splenectomy. Six patients are doing well after endoscopic variceal band ligation and propranolol prophylaxis. Conclusion: Caroli disease and Caroli syndrome are two sides of the same coin; it is important to differentiate the two disorders, especially from management point of view. CS is more common in our set up, leading to portal hypertension, which can be treated with sclerotherapy, endoscopic band ligation or shunt surgery. CD leads to cholangitis, which is managed with antibiotics.