As with ADPKD, no directed treatment is available to prevent or slow cyst formation, and thus care should be directed toward managing complications of renal or hepatic dysfunction. Patients who present early in life with renal dysfunction require aggressive support to maintain adequate nutritional status and avoid sustained fluid or electrolyte abnormalities. Patients who have portal hypertension complications may require intervention, such as portosystemic shunting or variceal sclerotherapy. The timing of ESRD onset is variable, but when it occurs dialysis and renal transplantation become the only remaining therapeutic options.