DOI: 10.36347/sjmcr.2023.v11i04.068

Pages: 691-694

Introduction: Caroli disease is a fibrocystic liver disease characterized by congenital nonobstructive cystic dilatation of the intrahepatic bile ducts. Its association with congenital hepatic fibrosis defines Caroli syndrome. The more common Caroli syndrome is associated with cystic renal dilatations in 60-80% of patients with polycystic kidney disease and Cacchi-Ricci disease as the most frequently noted renal anomalies. Objective: We want to define this uncommon entity and to describe the radiological characteristics which can, in the absence of their knowledge, be a source of confusion for the radiologist and pose problems of diagnostic delay. Case Report: The patient was a male infant, 1 year and 10 months old, with a history of 2 episodes of urinary tract infections at the age of 3 months and 6 months, and a 13-year-old sister with polycystic hepatorenal disease. The patient was admitted to the pediatric emergency department for acute fever associated with fluid diarrhea evolving for 3 days and irritability and crying at the time of urination. A cytobacteriological examination of the urine showed a turbid appearance of the urine with isolation of Escherichia Coli on culture. Abdominal ultrasound revealed bilateral nephromegaly, with diffuse microcystic hyperechogenicity of medullary pyramids generating multiple comet tail images with a "sponge kidney" appearance as well as a homogeneous splenomegaly and a normal sized liver of heterogeneous echostructure with cystic dilatation of the intrahepatic bile ducts which are centered by hyperechoic punctuations realizing the "Dot Sign". The individualization of this sign at an early age associated with splenomegaly allowed the diagnosis of a Caroli syndrome. While the diffuse medullary hyperechogenicity in the kidney allowed to evoke its association with polycystic kidney disease, especially the recessive one. Discussion and Conclusion: Caroli syndrome is a rare entity of autosomal recessive transmission. ..........