Autosomal recessive polycystic kidney disease (
ARPKD) is characterized by enlarged kidneys with dilated collecting ducts and
congenital hepatic fibrosis. There is a variable rate of progression of kidney and
liver disease.
Portal hypertension and
Caroli's disease occur from liver involvement that contributes to morbidity and mortality. Approximately 40 % of patients have a severe disease phenotype leading to rapid onset of
end-stage kidney disease (ESKD) and signs of
portal hypertension and the rest may have predominant involvement of either the kidney or liver. It is important for the physician to establish the extent of organ involvement before deciding on the ultimate plan of management, especially when
transplantation is required. Isolated
renal transplantation can be considered when liver involvement is minimal. If
hepatobiliary disease is prominent, and kidney function is preserved, management options are based on individual characteristics. In the presence of significant
liver disease and ESKD, consideration should be given to combined liver
kidney transplantation, which can be beneficial in eliminating the consequences of both kidney and
liver disease. However, this is a complex
surgical procedure that needs to be performed at experienced transplant centers. Improvement in surgical techniques has considerably improved short-term graft survival with the added advantage of the liver offering immunologic protection to the kidney allograft.