Background and Objectives: Hepatolithiasis (HL), or intrahepatic bile duct stone disease, shows regional variation and is a rare condition in Western countries. While cases from East Asia are often linked to chronic biliary infections and brown pigment stones, Western HL more frequently involves cholesterol or black pigment stones, typically in the context of prior cholecystectomy, biliary interventions, or congenital anomalies. The disease is generally associated with significant morbidity, including recurrent cholangitis, biliary strictures, and risk of cholangiocarcinoma. This study aimed to characterize HL disease in an Italian case series. Materials and Methods: We retrospectively reviewed 1450 patients with biliary stone disease treated between 2010 and 2024. HL was diagnosed in 14 patients (0.96%). Clinical records, imaging (ultrasound, CT, magnetic resonance cholangiopancreatography—MRCP, cholangiography), bile cultures, and stone composition (categorized as cholesterol, brown pigment, black pigment, or mixed using FTIR/XRD) were analyzed. Results: Among the 14 patients (mean age: 60.1 years; 64.3% female), 71.4% presented with recurrent cholangitis, while 28.6% were asymptomatic. Stones were left-sided in 57.1%, right-sided in 21.4%, and bilateral in 21.4%. Stone composition was cholesterol/mixed in 50%, brown pigment in 35.7%, and black pigment in 14.3%. Risk factors for bile stasis were present in 71.4% of cases. Bile cultures (available in nine cases) were positive in 77.8%. MRCP was highly effective for diagnosis. Hepatectomy achieved complete resolution in 35.7% of patients with unilobar disease; endoscopic/percutaneous therapy had a 44.4% recurrence rate. Interestingly, no cholangiocarcinoma was observed over a median follow-up of 4.8 years. Conclusions: Western HL is a rare, heterogeneous disease with distinct features. Cholesterol-predominant, infection-negative cases suggest a metabolic or surgical etiology. Hepatectomy offers durable outcomes in unilobar disease. Advanced imaging (MRCP, cholangioscopy) and personalized strategies are key to effective management.

Pigment gallstones represent a heterogeneous group of concretions, classically divided into black and brown types, whose morphology and microstructure offer critical clues about their underlying pathogenesis. Gallstone formation (lithogenesis) is a complex process triggered when the physicochemical equilibrium of bile is disrupted. Background/Objectives: The spicules observed on the surface of certain black pigment gallstones have traditionally been attributed to the branching capacity of cross-linked bilirubin polymers. However, a growing body of experimental and spectroscopic evidence suggests that inorganic calcium salts, particularly calcium carbonate and calcium phosphate, play a central role in the formation of the distinctive spiculated or “coral-like” architecture. Materials and Methods: In our study, we examined a case series of 1350 consecutive patients with gallstone disease, identifying 81 patients who presented with solitary black pigment stones. We systematically explored the association between high calcium content, specifically calcium carbonate, and the occurrence of spiculated morphology. Our analyses demonstrated a robust correlation between an elevated concentration of calcium carbonate and the presence of well-defined spicules. Results: These results support the hypothesis that mineral elements, rather than organic bilirubin polymers, act as crucial determinants of the peculiar crystalline structure observed in a significant subset of pigment stones. Spiculated stones, due to their small size and sharp projections, have a higher likelihood of migrating, increasing the risk of potentially life-threatening complications, such as acute cholangitis and gallstone pancreatitis. Conclusions: Our findings, consistent with recent advanced crystallographic analyses, underscore the importance of considering mineral composition in the diagnosis and management of cholelithiasis. Understanding the factors that drive calcium carbonate precipitation is essential for developing new preventive and therapeutic strategies, aiming to modulate bile chemistry and reduce the risk of calcium-driven lithogenesis.