Treatment of Symptomatic Polycystic Liver Disease with Hepatomegaly

This protocol addresses the management of symptomatic polycystic liver disease (PLD) in patients presenting with significant hepatomegaly, in the specific context where neither cyst hemorrhage nor cyst infection is present.

In symptomatic patients, the primary clinical goal is to reduce the burden of hepatomegaly. The absence of active cyst hemorrhage and cyst infection defines the eligible population for this treatment pathway.

Treatment approach (partial)

The approach for this scenario involves a surgical intervention targeting the diseased native liver. The complete protocol — including eligibility criteria, surgical specifics, and considerations when kidney transplantation is indicated concurrently — is available via the full regimen.

Full algorithm and details available below ↓

Treatment goal

Relief of symptoms of hepatomegaly.

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References

DOI: 10.1016/j.jviscsurg.2018.07.004

In symptomatic patients, the treatment goal is to reduce the volume of hepatomegaly, except for the two special cases of hemorrhage and infection.

LT is an effective treatment for hepatomegaly with excellent short- and long-term relief of symptoms.

The technique of LT for PLD consists of explantation of the diseased native liver, a source of hemorrhage requiring transfusion that has been constant in our experience.

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