Polycystic Liver Disease: A Case Report

Randy Adiwinata,Natalin Allorerung,Michael Tendean,Andrea Livina,Fandy Gosal,Pearla Lasut,Jeanne Winarta,Luciana Rotty,Bradley Jimmy Waleleng,Jonathan Arifputra,Andrew Waleleng

doi:10.24871/2222021159-163

Abstract

Polycystic liver disease is characterized by multiple cystic lesions on the liver. Liver cysts are typically incidental findings, with occasional complications including cyst hemorrhage, infection and rupture. Polycystic liver disease may be part of autosomal dominant polycystic liver disease (ADPLD). Autosomal dominant polycystic liver disease is considered rare autosomal dominant disease, with prevalence of 1/100,000-1,000,000. Without family history of polycystic liver disease, ADPLD is defined as the presence of more than 20 liver cysts with no renal cysts, however up to third of ADPLD may have small number of renal cysts without kidney function impairment. This case of a 73-year-old woman with symptomatic polycystic liver disease, and we performed cyst fenestration-deroofing via laparoscopic.

Highlights

Polycystic liver disease (PLD) is a rare disorder which may be found incidentally during abdominal imaging examination
Distinguishing autosomal dominant polycystic kidney disease (ADPKD) with autosomal dominant polycystic liver disease (ADPLD) is important as the monitoring protocol, management, and prognosis may vary greatly.[2]
USG examination demonstrated multiple hepatic cystic lesion on both liver lobes sized 7.5 mm to 74.6 mm and simple left kidney cyst sized 25.7 mm x 20.6 mm. She was diagnosed with acute acalculous cholecystitis, due to no cholelithiasis was found on USG and positive for cholecystitis signs-symptoms, and polycystic liver disease suggestive for ADPLD

Summary

INTRODUCTION

Polycystic liver disease (PLD) is a rare disorder which may be found incidentally during abdominal imaging examination. PLD may be part of autosomal dominant polycystic liver disease (ADPLD) or extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD); with the latter is more common.[1,2]. Distinguishing ADPKD with ADPLD is important as the monitoring protocol, management, and prognosis may vary greatly.[2] ADPKD is a multisystemic and progressive disease characterized by cyst formation in kidney and other organs which warranted routine renal function monitoring because the risk of developing end stage renal disease.[1]. Evaluation regarding the burden of PLD is warranted; because PLD is often mistakenly as benign disease and no treatment required. Liver transplantation is the only definitive cure for PLD.[1,2,3] Until now, there is no specific guideline published for PLD. We present a recently symptomatic 73-year-old-female with PLD and acute cholecystitis

CASE ILLUSTRATION

Abdominal USG is the first line of imaging modality

CONCLUSION