A rare presentation of hepatolithiasis in an adolescent patient: A case report
| Autor: | Kwun Wah Wen, Jorge Mena, Sunita P. Ho, Jonathan Freise, Carlos U. Corvera, Marshall L. Stoller |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
Urologic Diseases
Hepatic duct Abdominal pain medicine.medical_specialty Adolescent medicine.medical_treatment Clinical Sciences Context (language use) Article Primary sclerosing cholangitis 03 medical and health sciences 0302 clinical medicine Rare Diseases Case report medicine 2.1 Biological and endogenous factors Intrahepatic stone Aetiology MRCP magnetic resonance cholangiopancreatography SEM Scanning Electron Microscopy Hepatolithiasis Micro-XCT Micro X-Ray Computerized Tomography Magnetic resonance cholangiopancreatography medicine.diagnostic_test business.industry General surgery Prevention Liver Disease medicine.disease EDX Electron Dispersive X-Ray PSC primary sclerosing cholangitis 030220 oncology & carcinogenesis Etiology 030211 gastroenterology & hepatology Surgery medicine.symptom Differential diagnosis Hepatectomy business Digestive Diseases |
| Zdroj: | International Journal of Surgery Case Reports |
| Popis: | Author(s): Freise, Jonathan; Mena, Jorge; Wen, Kwun Wah; Stoller, Marshall; Ho, Sunita; Corvera, Carlos | Abstract: IntroductionHepatolithiasis (intrahepatic stones) is rare in adolescent patients and requires complex management strategies to prevent recurrent infections and progression to hepatic fibrosis. Surgical management is often required. In cases of unclear etiology, further work-up is indicated to provide insight into future management. In this report we describe an extensive stone analysis.Presentation of caseA 20-year-old Caucasian female presented with known hepatolithiasis and multiple prior recurrent bouts of abdominal pain requiring hospitalization. Magnetic resonance cholangiopancreatography (MRCP) demonstrated an abnormal left-sided hepatic biliary ductal system dilatation. She was treated surgically with a formal left hepatectomy and preservation of the caudate lobe. The right ductal system had no stones or evidence of inflammation, and her bile and stones cultures were negative for organism growth. An extensive analysis demonstrated stone composition primarily of cholesterol.DiscussionAdolescent presentations of hepatolithiasis are rare and considerations in the differential diagnosis include primary sclerosing cholangitis, bile acid transporter defects, and other known genetic diseases. This case is unique because only the left half of the intrahepatic ductal system had evidence of stone disease and the bile was sterile. A detailed stone analysis demonstrating cholesterol supersaturation provides additional context though the etiology remains unclear in this case and will require lifelong follow-up.ConclusionEarly-onset hepatolithiasis is rare and requires expert management, and in some cases definitive surgical management with life-long follow-up. Extensive stone analysis and genetic testing can be performed to help identify disease etiology in unique cases. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|