Incidentally detected polycystic kidney and Caroli's disease

Shiran Shetty^a, Salil Pandey^b, Venkatakrishnan Leelakrishnan^a

PSG Institute of Medical Science and Research, Tamil Nadu, India

Departments of ^aGastroenterology (Shiran Shetty, Venkatakrishnan Leelakrishnan); ^bRadiology (Salil Pandey), PSG Institute of Medical Science and Research, Peelamedu, Coimbatore, Tamil Nadu, India

Correspondence to: Shiran Shetty, Department of Gastroenterology, PSG Hospitals, PSG Institute of Medical Sciences & Research, Avinashi Road, Peelamedu, Coimbatore, Tamil Nadu, India 641 004, Tel.: +91 422 257 0170 Fax: +91 422 259 4400,
email: drshiran@gmail.com

Conflict of Interest: None

Received 20 May 2013; accepted 18 June 2013

Congenital cystic dilatation of the intrahepatic biliary ducts [Caroli's disease (CD)] has been seldom seen in clinical practice. It is often associated with autosomal recessive polycystic kidney disease and congenital hepatic fibrosis. Here we report an image of a female asymptomatic patient with CD and polycystic kidney disease.

A 51-year-old female patient with a one month history of pruritus visited our hospital for a routine health check up. She was undergoing treatment with her dermatologist for pruritus only with a prescription for local application. Her past medical history was unremarkable. Clinical examination was normal with only scratch marks. Laboratory investigations revealed normal hemogram, mildly elevated serum creatinine 1.3 (0.6-1.0) and normal electrolytes. Liver function test showed normal bilirubin with alanine aminotransferase 64 (5-30), AST 55 (5-30) and alkaline phosphatase 260 (30-120) gamma-glutamyltransferase 314 (5-30) with normal albumin level. All viral serology was normal. MRI/MRCP showed enlarged kidneys with parenchymal replaced by cysts along with hepatic cyst communicating with biliary tree as shown in Fig. 1.

Cystic lesions of the liver and kidney are increasingly being detected with the availability of newer imaging techniques [1]. Fibropolycystic disease can exist singly or  in combination. They are usually inherited and consist of polycystic disease, congenital hepatic fibrosis, CD and choledochal cysts. CD is associated with renal involvement in up to 60%, and includes medullary sponge kidney disease or polycystic kidney disease [2]. CD can be asymptomatic or can present with cholangitis, intra hepatic stone formation or portal hypertension. CD is characterized by multifocal segmental dilatation of intrahepatic bile ducts affecting all or parts of the liver. Two forms of CD are described, the pure form and that associated with periportal fibrosis. The disease may diffusely affect the liver or be localized to one lobe or segment. Less than 20% of all reported cases of CD are monolobar type. Here we present an interesting image of CD localized mainly in the left lobe.

References

1. Rodríguez Rodríguez E, Santolaria Fernández F, González Reimers E, et al. Caroli’s disease, congenital hepatic fibrosis and renal polycystosis: a little frequent association. Rev Esp Enferm Dig 1996;88:873-876.

2. Shedda S, Robertson A. Caroli’s syndrome and adult polycystic kidney disease. ANZ J Surg 2007;77:292-294.