Low Phospholipid-Associated Cholelithiasis: Contribution of Imaging in Two Cases

Abstract

Low phospholipid-associated cholelithiasis (LPAC) is a genetic disease responsible for the development of intrahepatic lithiasis. It is retained in the presence of two of the following three criteria: age of onset of biliary symptoms less than 40 years; echogenic intrahepatic images or microlithiasis; and the recurrence of biliary clinical signs after cholecystectomy. The majority of clinical situations are simple and not serious, but some complicated forms may require more invasive endoscopic or surgical treatments. By presenting two case studies, we illustrated and summarized the different aspects of this entity.

Low phospholipids associated cholelithiasis syndrome in a young women: A rare case report

“…LPAC (Low Phospholipid-Associated Cholelithiasis) syndrome, or genetic cholesterolic lithiasis, is a very particular form of biliary lithiasis that was first described in 2001 by the team of the Saint-Antoine hospital [1 , 9] . It is a genetic disease responsible of the formation of intrahepatic stones, characterized by the association of ATP-binding cassette subfamily B member4 (ABCB4) and a low level of bile phospholipids with symptomatic and recurrent cholelithiasis [8] . The prevalence of LPAC syndrome is unknown but considered quite low [6] .…”

“…In a young asyptomatic subject, a normal screening ultrasound may be repeated few years later because suggestive radiological signs may occur later. In asymptomatic parents with intrahepatic lithiasis, it is reasonable to propose treatment with UDCA [8 , 9] .…”

“…Diagnosis and treatment of low phospholipid-associated cholelithiasis syndrome (LPAC) is easy, but the majority of cases are underestimated because they are not diagnosed [8] . We report a clinical case of a 43-year-old female patient with LPAC syndrome diagnosed after cholecystectomy.…”