Massive Extra- and Intrahepatic Lithiasis in a Young Patient: a Case of LPAC-Syndrome?
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Massive Extra- and Intrahepatic Lithiasis in a Young Patient: a Case of LPAC-Syndrome? Antonio Cubisino 1,2
&
Antoine Gautier 1 & Piera Leon 1
Received: 30 September 2020 / Accepted: 31 October 2020 # 2020 The Society for Surgery of the Alimentary Tract
A 36-year-old woman with no past medical or surgical history presented to our institute with 1-day history of persistent abdominal pain with nausea and vomiting. Laboratory examination revealed leukocytosis, neutrophilia, and an elevated C-reactive protein as well as cholestasis (ALT 82 U/L, AST 125 U/L, GGT 843 U/L, total bilirubin 26 mg/dl). Abdominal echography was inconclusive; therefore, a computer tomography (CT) scan was performed, and it revealed a dilated common bile duct (CBD) and probable impacted CBD lithiasis. The cholangio-MRI confirms the CBD dilatation with a massive lithiasis into the extra- and intrahepatic biliary ducts. (Fig. 1a).
Guarantor of the article is Antonio Cubisino, MD * Antonio Cubisino [email protected] 1
Division of HBP Surgery and Transplantation, Department of Surgery, Hôpital Saint Eloi, CHU-Montpellier, 80 Av. Augustin Fliche, 34295 Montpellier, France
2
HBP and Transplantation Surgery Unit, Department of Digestive Surgery and Transplantation, Saint-Eloi Hospital, University of Montpellier, 80 avenue Augustin Fliche, 34295 Montpellier, France
A laparoendoscopic rendezvous with a laparoscopic cholecystectomy and an intraoperative cholangiography is performed (Fig. 1b). Endoscopic large sphincterotomy with stone extraction completely resolved the biliary obstruction. A 9-Fr biliary stent was finally placed. Post-operative course was regular with no complication occurred. Patient was discharged home with ursodeoxycholic acid 600 mg daily treatment. Low phospholipid-associated cholelithiasis (LPAC) could be evocated in this case. LPAC is a genetic disease responsible for the development of intrahepatic lithiasis and has to be suggested in any case of intrahepatic lithiasis occurring in a patient aged less than 30 years old.1 According to the LPANGH cohort (LPAC cohort of the Association Nationale des Hépato-Gastro-entérologues des Hôpitaux Généraux de France), LPAC concerns approximately 1% of symptomatic cholelithiasis’ patients. The diagnosis of LPAC syndrome is defined by the existence of two out of the three following criteria: the onset of biliary symptoms in patients younger than 40 years old, recurrent biliary pain subsequent to cholecystectomy, and typical radiological signs of intrahepatic lithiasis. In a retrospective multicenter study, Dong et al. recently reported the clear association between LPAC syndrome and lithiasis of the common bile duct in young non-cholecystectomized patients. They suggest a more
J Gastrointest Surg
Fig. 1 Cholangio-MRI and laparoendoscopic rendezvous of extra- and intrahepatic biliary ducts
articulated diagnostic score for the diagnosis of LPAC, incorporating two additional criteria and attributing different degrees of relevance to the diagnostic items. Supplementa
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