Low Phospholipid-Associated Cholelithiasis (LPAC) syndrome is a rare biliary disorder related to ABCB4 gene mutations, leading to impaired biliary phospholipid secretion and recurrent intrahepatic lithiasis in young adults. While ursodeoxycholic acid (UDCA) is the standard treatment, some patients experience persistent or complicated disease requiring endoscopic management. The therapeutic contribution of endoscopic retrograde cholangiopancreatography (ERCP) in LPAC remains poorly defined. This study aimed to assess the clinical outcomes and therapeutic role of ERCP in LPAC patients.

A retrospective study was conducted at Ibn Sina University Hospital, Rabat, including all patients diagnosed with LPAC who underwent ERCP between 2022 and 2025. Data regarding demographics, clinical presentation, laboratory and imaging findings, ERCP indications, interventions, complications, and outcomes were analyzed. The primary endpoint was therapeutic efficacy, defined as symptom relief and biochemical improvement.

Among 16 patients followed for LPAC syndrome, ERCP was performed in 9 patients. The male-to-female ratio was 0.8, with a mean age of 40.4 years (range 26–54). Prior cholecystectomy had been performed in 55.5% (5/9) of patients, and 44.4% (4/9) had a family history of biliary lithiasis (including jaundice, pregnancy-related cholestasis, or cholecystectomy). All patients experienced recurrence of symptoms after cholecystectomy.

Clinically, 44.4% (4/9) presented with pancreatic-type pain, 33.3% (3/9) with hepatic colic, and 22.2% (2/9) with isolated cholestatic jaundice. Diagnosis was confirmed by ultrasound, which demonstrated a “comet tail” appearance in all patients. Laboratory tests showed cytolysis and cholestasis in 66.6% (6/9) of cases.

The indications for ERCP were cholangitis in 55.5% (5/9), recurrent pancreatitis in 11.1% (1/9), choledocholithiasis 33.3% (3/9) . ERCP was performed prior to starting medical therapy in 66.6% (6/9) of patients. Endoscopic interventions included sphincterotomy in 88.8% (8/9) ,Dilatation was performed in 22.2% (2/9) patients 

Clinical improvement was observed in 77.7% (7/9) of patients, with normalization of laboratory tests was achieved in 44.4% (4/9) of patients. Symptom recurrence occurred in 44.4% (4/9) of cases; however, this mainly reflected repeated episodes in a single outpatient who required multiple additional ERCP procedures. Overall, the final outcome was favorable in 44.4% (4/9) of patients, while intermittent hepatic colic persisted in 22.2% (2/9). Three patients (33.3%) were lost to follow-up.

ERCP provides significant therapeutic benefit in LPAC patients, particularly for post-cholecystectomy recurrences and complications such as cholangitis or pancreatitis. It ensures effective biliary drainage with an acceptable safety profile. Despite occasional recurrences, ERCP should be considered an integral component of multidisciplinary LPAC management. Larger prospective studies are needed to define standardized endoscopic strategies for this rare condition.