Background: Polycystic liver in the adult (PLA) is a rare disease characterized by chronic liver enlargement.
Objective: To analyse gastroenterologists´ involvement in, experience with, and attitude toward diagnosing, monitoring, andtreating patients with PLA in Spain.
Methods: Each of seven study coordinators contacted 15 specialists in their geographic area about participating in the study via an online structured survey.
Results: Of the 105 clinics contacted, 88 completed the questionnaire, with a mean of 3 patients being followed per practice, although 6 clinics were following more than 20 patients with PLA. Patients were being followed mainly by the Department of Hepatology (81 %) and/or the Department of Gastroenterology (33 %). The majority of patients were diagnosed (98 %) and monitored (97 %) using liver ultrasound. When diagnosed, 76 % of patients were under 50 years of age, females predominating.The primary treatment objective for the patients was symptomatic management. Pharmacotherapy was prescribed by 28 % of physicians: Somatostatin analogues, primarily, followed by mTOR inhibitors. One-third of the clinics indicated that they had patients who had undergone liver transplant and/or surgery.
Conclusions: Ultrasound is the diagnosing and monitoring method of choice. Among the clinics using pharmacotherapy for symptomatic management, somatostatin analogues were the drugs of choice. These clinics´ infrequent use of invasive procedures suggests that they perceive the various invasive techniques as not very effective.