Laparoscopic intercostal segment 7 resection for intrahepatic lithiasis due to low phospholipid-associated cholelithiasis syndrome. A case report

TL;DR

A rare liver condition called LPAC syndrome was successfully treated with a minimally invasive liver surgery in a young woman who had ongoing biliary symptoms after gallbladder removal.

Contribution

This case report demonstrates the effectiveness of laparoscopic intercostal segment VII resection for treating localized intrahepatic lithiasis in LPAC syndrome.

Findings

Laparoscopic resection of liver segment VII was safely performed and confirmed to treat intrahepatic lithiasis.

Histopathology confirmed hepatolithiasis with acute and chronic cholangitis in the patient.

Minimally invasive liver surgery is a feasible option for selected LPAC syndrome cases with localized disease.

Abstract

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.

•LPAC syndrome is rare (1%), affects young women, with intrahepatic microlithiasis and biliary symptoms a rare condition with a prevalence of 1%, primarily affects young women and is characterized by intrahepatic microlithiasis and recurrent biliary symptoms following cholecystectomy•We report a 27-year-old woman with complicated intrahepatic lithiasis, treated with lap hepatectomy.•Lateral intercostal lap liver resection is safe and effective in experienced hands.

LPAC syndrome is rare (1%), affects young women, with intrahepatic microlithiasis and biliary symptoms a rare condition with a prevalence of 1%, primarily affects young women and is characterized by intrahepatic microlithiasis and recurrent biliary symptoms following cholecystectomy

We report a 27-year-old woman with complicated intrahepatic lithiasis, treated with lap hepatectomy.

Lateral intercostal lap liver resection is safe and effective in experienced hands.

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