The Nowhere Stone: Unusually Located Intrahepatic Calculi in a Patient with Low Phospholipid-associated Cholelithiasis Syndrome

ABSTRACT

Intrahepatic calculi or hepatolithiasis is an uncommon phenomenon which is found in relatively higher rates in eastern Asian countries. Patients usually present with features of cholangitis or intrahepatic abscess and are at risk for developing cholangiocarcinoma. Correct diagnosis and management often pose challenges. A 42-year-old female presented with a history of episodic upper abdominal pain since 8 years. Initial imaging studies showed evidence of biliary calculi, but were confounding in their localization. Magnetic resonance cholangiopancreatography showed two large calculi in the right biliary system proximal to the confluence, along with choledocholithiasis. Endoscopic retrograde cholangiopancreatography and common bile duct clearance were done; however, the patient underwent hepatic sectionectomy as a curative procedure. ABCB4 mutation was detected on DNA analysis and hence aided in making the diagnosis of low phospholipid-associated cholelithiasis. Additional imaging studies are warranted in doubtful cases. Difficult accessibility of the calculi may often limit endoscopic therapeutic options and pave the way for surgical interventions. As there is an increased risk of cholangiocarcinoma in these patients, regular follow-up is essential.

Keywords

ABCB4 - gallstones - hepatolithiasis - intrahepatic calculi - magnetic resonance cholangiopancreatography

• REFERENCES

• 1 di Carlo I, Sauvanet A, Belghiti J. Intrahepatic lithiasis: A western experience. Surg Today 2000; 30: 319-22
• 2 Ran X, Yin B, Ma B. Four major factors contributing to intrahepatic stones. Gastroenterol Res Pract 2017; 2017: 7213043
• 3 Sakpal SV, Babel N, Chamberlain RS. Surgical management of hepatolithiasis. HPB (Oxford) 2009; 11: 194-202
• 4 Vetrone G, Ercolani G, Grazi GL, Ramacciato G, Ravaioli M, Cescon M. et al. Surgical therapy for hepatolithiasis: A western experience. J Am Coll Surg 2006; 202: 306-12
• 5 Uchiyama K, Onishi H, Tani M, Kinoshita H, Ueno M, Yamaue H. Indication and procedure for treatment of hepatolithiasis. Arch Surg 2002; 137: 149-53
• 6 Fan YF, Fang CH, Chen JX, Xiang N, Yang J, Li KX. Application of three-dimensional visualization technology in precise diagnosis and treatment for hepatolithiasis. Nan Fang Yi Ke Da Xue Xue Bao 2011; 31: 949-54
• 7 Jarufe N, Figueroa E, Muñoz C, Moisan F, Varas J, Valbuena JR. et al. Anatomic hepatectomy as a definitive treatment for hepatolithiasis: A cohort study. HPB (Oxford) 2012; 14: 604-10
• 8 Chen DW, Tung-Ping Poon R, Liu CL, Fan ST, Wong J. Immediate and long-term outcomes of hepatectomy for hepatolithiasis. Surgery 2004; 135: 386-93
• 9 Prakash K, Ramesh H, Jacob G, Venugopal A, Lekha V, Varma D. et al. Multidisciplinary approach in the long-term management of intrahepatic stones: Indian experience. Indian J Gastroenterol 2004; 23: 209-13
• 10 Ramesh H, Prakash K, Kuruvilla K, Philip M, Jacob G, Venugopal B. et al. Biliary access loops for intrahepatic stones: Results of jejunoduodenal anastomosis. ANZ J Surg 2003; 73: 306-12