Vargas Aignasse Ramiro, Gasque Rodrigo Antonio, Viscido Germán Rodrigo, Chianalino Maria Eugenia, Ávila Micaela Noemí, Alvarez Fernando Andrés
Hepato-pancreato-biliary Surgery Section, General Surgery Department, Clínica Universitaria Reina Fabiola, Universidad Católica de Córdoba, Argentina.
Int J Surg Case Rep. 2025 Jul;132:111490. doi: 10.1016/j.ijscr.2025.111490. Epub 2025 Jun 7.
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.
低磷脂相关胆石症（LPAC）综合征是一种罕见的胆道疾病，由ABCB4基因突变引起，该基因编码多药耐药蛋白3（MDR3）磷脂酰胆碱转运体。它主要影响年轻人，胆囊切除术后可能持续存在或复发。LPAC的特征是肝内结石和反复出现的胆道症状。
我们报告一例27岁女性，有复发性急性胰腺炎病史且曾行腹腔镜胆囊切除术，现出现持续性胆绞痛发作。实验室检查显示白细胞增多（12,000/μL），而肝功能检查仍在正常范围内。腹部超声在肝脏VII段发现多个伴有后方声影的强回声灶，以及胆管扩张。计算机断层扫描和磁共振成像的进一步评估排除了其他诊断。进行了腹腔镜超声引导下的VII段切除术。组织病理学分析证实为肝内结石伴急性和慢性胆管炎。
LPAC综合征在诊断和治疗方面都具有挑战性。尽管其患病率较低——据估计在胆囊切除术后有反复胆道症状的患者中约为1%——但早期识别对于恰当的管理至关重要。在某些有局限性肝内结石和持续症状的病例中，手术切除可能是一种有效的治疗选择。
本病例强调了在胆囊切除术后有未解决胆道症状的年轻患者中考虑LPAC的重要性，并证明了在专业肝胆中心进行微创肝切除的可行性。
