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Polycystic liver disease

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of polycystic liver disease are prepared by our editorial team based on guidelines from the European Association for the Study of the Liver (EASL 2022), the American College of Gastroenterology (ACG 2014), and the Spanish Working Group on Inherited Kidney Diseases (SWG-IKD 2014).
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Screening and diagnosis

Indications for screening
As per EASL 2022 guidelines:
Obtain abdominal ultrasound to screen for PLD in all patients diagnosed with autosomal dominant polycystic kidney disease.
B
Do not obtain genetic testing for the screening of PLD.
D
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Classification and risk stratification

Severity assessment
As per EASL 2022 guidelines:
Consider using disease-specific symptom severity questionnaires upon the emergence of symptoms to assess disease severity in patients with PLD.
C
Use disease-specific symptom severity questionnaires to assess treatment efficacy in patients with PLD.
A
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Diagnostic investigations

Diagnostic imaging: as per EASL 2022 guidelines, obtain ultrasound as the first-line imaging for the diagnosis of PLD.
B
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  • Evaluation of mucinous cystic neoplasms

Medical management

General principles: as per EASL 2022 guidelines, decide on the choice of treatment based on symptoms and complications related to the presence of cysts in combination with liver phenotypes:
Situation
Guidance
Symptoms
Abdominal fullness
Lack of appetite or early satiety
Acid reflux
Nausea and vomiting
Pain in rib cage, sides, abdomen or back
Shortness of breath
Limited mobility, fatigue
Anxiety about the future
Concern or dissatisfaction with abdomen size
Problems with intercourse
Involuntary weight loss
Complications
Jaundice
Hepatic venous outflow obstruction
Portal hypertension
Recurrent cyst infection
Recurrent cyst hemorrhage
Phenotypes
Numerous small-to-medium-sized cysts throughout the liver
Large or strategically located cyst percutaneously accessible
Single or multiple large superficial cysts
Small-to-medium-sized cysts clustered in a few segments in the presence of some less affected liver segments
Massive PLD
Severely affecting the QoL or accompanied by recurrent complications
B
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Nonpharmacologic interventions

Nutrition and physical activity: as per EASL 2022 guidelines, provide intensive nutrition and exercise rehabilitation under the supervision of dietitians and physical therapists in patients with PLD and sarcopenia.
B
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Therapeutic procedures

Drainage: as per ACG 2014 guidelines, consider performing aspiration or surgical resection/deroofing of dominant cysts based on the patient's clinical presentation and underlying hepatic reserve.
C
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Surgical interventions

Cyst fenestration and resection: as per ACG 2014 guidelines, consider performing deroofing, surgical resection, or aspiration of dominant cysts based on the patient's clinical presentation and underlying hepatic reserve.
C
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Specific circumstances

Patients with cyst infection, antibiotic therapy: as per EASL 2022 guidelines, administer fluoroquinolones and third-generation cephalosporins for 4-6 weeks
B
as empirical first-line antibiotics in patients with hepatic cyst infection.
B
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  • Patients with cyst infection (drainage)

  • Patients with cyst infection (secondary prophylaxis)

  • Patients with cyst hemorrhage

  • Patients with Caroli disease/syndrome

  • Patients with mucinous cystic neoplasms

Patient education

Preconception counseling
As per EASL 2022 guidelines:
Provide preconception counseling concerning the risk of passing on PLD to the newborn in patients with PLD.
B
Do not counsel patients with PLD against pregnancy.
D
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Follow-up and surveillance

Indications for referral: as per EASL 2022 guidelines, refer patients with symptomatic PLD to centers of expertise.
B
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