Laparoscopic intercostal segment 7 resection for intrahepatic lithiasis due to low phospholipid-associated...

Low phospholipid-associated cholelithiasis (LPAC) syndrome is a rare biliary disorder caused by mutations in the ABCB4 gene, which encodes the MDR3 phosphatidylcholine transporter. It primarily affects young adults and may persist or recur following cholecystectomy. LPAC is characterized by intrahepatic lithiasis and recurrent biliary symptoms.

We report the case of a 27-year-old woman with a history of recurrent acute pancreatitis and prior laparoscopic cholecystectomy, who presented with ongoing episodes of biliary colic. Laboratory tests revealed leukocytosis (12,000/μL), while liver function tests remained within normal limits. Abdominal ultrasound identified multiple echogenic foci with posterior acoustic shadowing in segment VII of the liver, along with bile duct ectasia. Further evaluation with computed tomography and magnetic resonance imaging excluded alternative diagnoses. A laparoscopic, ultrasound-guided resection of segment VII was performed. Histopathological analysis confirmed hepatolithiasis with associated acute and chronic cholangitis.

LPAC syndrome presents both diagnostic and therapeutic challenges. Although its prevalence is low-estimated at approximately 1 % among patients with recurrent biliary symptoms after cholecystectomy-early recognition is essential for appropriate management. In selected cases with localized intrahepatic lithiasis and persistent symptoms, surgical resection may offer an effective therapeutic option.

This case highlights the importance of considering LPAC in young patients with unresolved biliary symptoms post-cholecystectomy and demonstrates the feasibility of minimally invasive liver resection in specialized hepatobiliary centers.