Questionnaire PLD-complaint-specific assessment identifies need for therapy in polycystic liver disease: A multi-centric prospective study.

BACKGROUND AND AIMS: Polycystic liver disease (PLD) can lead to extensive hepatomegaly. Symptom relief is the primary goal of the treatment. The role of the recently developed disease-specific questionnaires for identification of the thresholds and the assessment of therapy needs further investigation. METHODS: A five-year prospective multi-centric observational study in 21 hospitals in Belgium gathered a study population of 198 symptomatic PLD-patients of whom the disease-specific symptom questionnaire PLD-complaint-specific assessment (POLCA) scores were calculated. The thresholds of the POLCA score for the need for volume reduction therapy were analyzed. RESULTS: The study group consisted of mostly (82.8%) women with baseline mean age of 54.4 years ±11.2, median liver volume expressed as height-adjusted total liver volume(htLV) of 1994 mL (interquartile range [IQR] 1275; 3150) and median growth of the liver of +74 mL/year (IQR +3; +230). Volume reduction therapy was needed in 71 patients (35.9%). A POLCA severity score (SPI) ≥ 14 predicted the need for therapy both in the derivation (n = 63) and the validation cohort (n = 126). The thresholds to start somatostatin analogues (n = 55) or to consider liver transplantation (n = 18) were SPI scores of ≥14 and ≥ 18 and the corresponding mean htLVs were 2902 mL (IQR 1908; 3964) and 3607 mL (IQR 2901; 4337), respectively. Somatostatin analogues treatment resulted in a decrease in the SPI score -6.0 versus + 4.5 in patients without somatostatin analogues (p < 0.01). Changes in the SPI score were significantly different between the liver transplantation group and no liver transplantation group, +4.3 ± 7.1 versus -1.6 ± 4.9, respectively, (p < 0.01). CONCLUSION: A polycystic liver disease-specific questionnaire can be used as a guide on when to start a volume reduction therapy and to assess the effect of treatment.

Thrombosis of a portal vein aneurysm: a case report with literature review.

OBJECTIVES: Portal vein aneurysm is an unusual vascular dilatation of the portal vein. The etiology, diagnosis and management are ill-defined. METHODS: A case of a portal vein aneurysm complicated with complete thrombosis is presented with a literature review providing an overview of the etiology, clinical presentation and management. RESULTS: Portal venous aneurysms represent approximately 3% of all venous aneurysms with a reported prevalence of 0.06%. The reported incidence is on the rise with increasing use of modern imaging techniques in clinical practice. Usually, portal vein aneurysms are incidental findings and patients are asymptomatic. They can be congenital or acquired and portal hypertension represents the most frequent cause of the acquired version. Various complications such as biliary tract compression, portal vein thrombosis, and rupture can occur. Treatment options are conservative management or surgery. Surgical treatment is currently reserved for symptomatic patients with severe abdominal pain, symptoms of pressure effect or with expanding aneurysms, and/or complications such as thrombosis or rupture. CONCLUSION: Conservative management seems the best option in the majority of patients. A multidisciplinary approach discussing the best option on a case-by-case base in light of their individual underlying risk and symptoms is advised.