EASL Clinical Practice Guidelines on the management of cystic liver diseases

Drenth, Joost P.H.; Barten, Thijs; Hartog, Hermien; Nevens, Frederik; Taubert, Richard; Balcells, Roser Torra; Vilgrain, Valérie; Böttler, Tobias

doi:10.1016/j.jhep.2022.06.002

Cited by 44 publications

(41 citation statements)

References 231 publications

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“…Approximately 95–98% of PLD cases are clinically asymptomatic, but a small proportion develop disabling symptoms and a significant reduction in quality of life. 7 , 110–112 The number, size, location, and distribution of the cysts determine the spectrum of symptoms, with most being associated with mass effect or compression imposed by hepatomegaly. 113 Abdominal distension usually occurs in patients with significant hepatomegaly.…”

Section: Clinical Presentationmentioning

confidence: 99%

“… 110 , 116 , 133 Liver volume increases during the reproductive period and stabilizes in postmenopause, secondary to reduced endogenous estrogen synthesis. 7 , 134 There is no consensus on the effects of pregnancy on liver volume in women with DHP. Pregnancy is not contraindicated in these women, but they should receive preconception counseling about the risk of transmitting the disease to the newborn.…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Pregnancy is not contraindicated in these women, but they should receive preconception counseling about the risk of transmitting the disease to the newborn. 7 …”

Section: Clinical Presentationmentioning

confidence: 99%

“…Currently, two validated instruments have been used: the polycystic liver disease questionnaire (PLD-Q) and polycystic liver disease complaint-especific assessment (POLCA). 7 …”

Section: Clinical Presentationmentioning

confidence: 99%

“… 155–157 It is important to point out that the levels of CA 19–9, whether in the blood or in the cystic fluid, do not allow the differentiation between hepatic cysts (solitary or associated with PLD) and mucinous cystic neoplasms. 7 …”

Section: Diagnosismentioning

confidence: 99%

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Polycystic Liver Disease: Pathophysiology, Diagnosis and Treatment

Norcia¹,

Watanabe²,

Filho³

et al. 2022

HMER

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Polycystic liver disease (PLD) is a clinical condition characterized by the presence of more than 10 cysts in the liver. It is a rare disease Of genetic etiology that presents as an isolated disease or assoc\iated with polycystic kidney disease. Ductal plate malformation, ciliary dysfunction, and changes in cell signaling are the main factors involved in its pathogenesis. Most patients with PLD are asymptomatic, but in 2-5% of cases the disease has disabling symptoms and a significant reduction in quality of life. The diagnosis is based on family history of hepatic and/or renal polycystic disease, clinical manifestations, patient age, and polycystic liver phenotype shown on imaging examinations. PLD treatment has evolved considerably in the last decades. Somatostatin analogues hold promise in controlling disease progression, but liver transplantation remains a unique curative treatment modality.

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Section: Clinical Presentationmentioning

confidence: 99%

Section: Clinical Presentationmentioning

confidence: 99%

“…Pregnancy is not contraindicated in these women, but they should receive preconception counseling about the risk of transmitting the disease to the newborn. 7 …”

Section: Clinical Presentationmentioning

confidence: 99%

“…Currently, two validated instruments have been used: the polycystic liver disease questionnaire (PLD-Q) and polycystic liver disease complaint-especific assessment (POLCA). 7 …”

Section: Clinical Presentationmentioning

confidence: 99%

Section: Diagnosismentioning

confidence: 99%

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Polycystic Liver Disease: Pathophysiology, Diagnosis and Treatment

Norcia¹,

Watanabe²,

Filho³

et al. 2022

HMER

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Patient reported outcome measure thresholds: The next step in the management of polycystic liver disease

Duijzer

Gevers

2023

UEG Journal

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Diagnosis and treatment of patients with suspected mucinous cystic neoplasms of the liver: a retrospective cohort study

Furumaya,

Schulz,

Verheij

et al. 2024

Langenbecks Arch Surg

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Purpose Mucinous cystic neoplasms of the liver (MCN-L) are hepatic cysts with a low malignant potential. The recent European Association for the Study of the Liver (EASL) guidelines provide guidance on the imaging features and surgical management of MCN-L, yet are hampered by a lack of studies adhering to the revised World Health Organization (WHO) criteria. This study attempted to validate the new 2022 EASL-guidelines in a retrospective cohort study of patients who underwent surgery for suspected MCN-L. Methods Patients undergoing surgery for suspected MCN-L in a single center between 2010 and 2020 were included. Imaging features were assessed according to the EASL guidelines and were compared to final pathological diagnoses, according to the WHO criteria. Results In total, 35 patients were included. In three patients, there were no worrisome imaging features, yet final pathological diagnosis showed MCN-L. Contrarily, six patients with worrisome imaging features did not have MCN-L. Five patients were diagnosed with MCN-L on final pathology. The sensitivity of the EASL-guidelines for the diagnosis of MCN-L was 40% (95%CI: 5.3–85%) and the specificity was 80% (95% CI: 61–92%). Conclusion Although the new EASL-guidelines provide some guidance, they could not reliably distinguish MCN-L from other cysts in our series. Thus, preoperative diagnosis of MCN-L remains challenging and we should be careful in selecting surgical strategies based on these criteria.

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EASL Clinical Practice Guidelines on the management of cystic liver diseases

Cited by 44 publications

References 231 publications

Polycystic Liver Disease: Pathophysiology, Diagnosis and Treatment

Polycystic Liver Disease: Pathophysiology, Diagnosis and Treatment

Patient reported outcome measure thresholds: The next step in the management of polycystic liver disease

Diagnosis and treatment of patients with suspected mucinous cystic neoplasms of the liver: a retrospective cohort study

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