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Bourabaa S, Laalou T, Mansouri A, Hamid M, Settaf A. Surgical management of Caroli disease in a low-mid income country: a single-center study and review of literature. BMC Surg 2025; 25:214. [PMID: 40375264 PMCID: PMC12082854 DOI: 10.1186/s12893-025-02948-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/10/2024] [Accepted: 05/05/2025] [Indexed: 05/18/2025] Open
Abstract
INTRODUCTION Caroli disease is an uncommon congenital condition characterized by non-obstructive intrahepatic bile duct dilation. When coupled with liver fibrosis or cirrhosis, it is termed Caroli syndrome. This disorder can lead to the development of gallstones, inflammation of the bile ducts, and an elevated susceptibility to cholangiocarcinoma. Typically, Caroli disease presents with involvement in less than 20% of the liver, predominantly affecting a single lobe (either left or right). Monolobar disease can often be effectively addressed through liver resection, while bilobar disease may necessitate the consideration of liver transplantation. METHODS A retrospective study was undertaken involving patients diagnosed with Caroli disease who underwent liver resection. The research included cases from Surgery B Department at Ibn Sina University Hospital in Rabat, covering the period from January 2010 to January 2023. RESULTS Nine patients who underwent liver resection for Caroli disease were identified, with an average age of 54 years (range: 17-76), and 44.4% (n = 4) being females. The study comprised 6 cases with disease limited to the left lobe and 3 to the right. The average time interval between initial symptoms and the definitive diagnosis was 4 years (range: 0-24 years). Surgical procedures included left lobectomy in 4 cases, left hepatectomy in 3 cases, right hepatectomy in 1 case, and sub-segmentectomy in 2 cases. Biliodigestive anastomosis was performed in 4 cases. Complications occurred in 2 patients (22.2%), and synchronous cholangiocarcinoma was observed in a single case (11.1%). CONCLUSION Consideration of Caroli disease as part of the differential diagnosis is crucial in cases of recurrent cholangitis. Liver resection stands out as the treatment of choice for patients with localized Caroli disease. The critical importance of early intervention is highlighted by the potentially fatal consequences of delayed diagnosis or treatment.
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Affiliation(s)
- Soukayna Bourabaa
- General Surgery Department, Ibn Sina University Hospital, Rabat, Morocco.
- Mohammed V University, Rabat, Morocco.
| | - Talha Laalou
- General Surgery Department, Ibn Sina University Hospital, Rabat, Morocco
- Mohammed V University, Rabat, Morocco
| | - Abderrahman Mansouri
- General Surgery Department, Ibn Sina University Hospital, Rabat, Morocco
- Mohammed V University, Rabat, Morocco
| | - Mohamed Hamid
- General Surgery Department, Ibn Sina University Hospital, Rabat, Morocco
- Mohammed V University, Rabat, Morocco
| | - Abdellatif Settaf
- General Surgery Department, Ibn Sina University Hospital, Rabat, Morocco
- Mohammed V University, Rabat, Morocco
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Fahrner R, Dennler SGC, Inderbitzin D. Risk of malignancy in Caroli disease and syndrome: A systematic review. World J Gastroenterol 2020; 26:4718-4728. [PMID: 32884228 PMCID: PMC7445861 DOI: 10.3748/wjg.v26.i31.4718] [Citation(s) in RCA: 17] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/20/2020] [Revised: 05/15/2020] [Accepted: 08/01/2020] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Congenital intrahepatic bile duct dilatation without fibrosis is called Caroli disease (CD), and is called Caroli syndrome (CS) when it has fibrotic and cirrhotic liver morphology. The development of intrahepatic carcinoma is described in both conditions, but the reported incidence varies extensively. Potential risk factors for the malignant transformation were not described. Furthermore, conservative or surgical treatment is performed depending on the extent of cystic malformation, hepatic dysfunction and structural hepatic changes, but little is known about which treatment should be offered to patients with CD or CS and cancer.
AIM To further investigate the malignant transformation in these conditions.
METHODS A systematic review of the current literature until January 2019 was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. A search using Medline (PubMed) was performed using a combination of Medical Subject Headings terms “caroli disease”, “caroli syndrome”, “tumor”, “malignant”, and “cholangiocarcinoma”. Only human studies published in English were used for this systematic review. The following parameters were extracted from each article: year of publication, type of study, number of patients, incidence of malignant tumor, duration of symptoms, age, sex, diagnostics, identification of tumor, surgical therapy, survival and tumor recurrence.
RESULTS Twelve retrospective studies reporting the courses of 561 patients (53% females) were included in this systematic review. With a mean age of 41.6 years old (range 23 to 56 years old), patients were younger than other populations undergoing liver surgery. Depending on the size of the study population the incidence of cholangiocarcinoma varied from 2.7% to 37.5% with an overall incidence of 6.6%. There were only few detailed reports about preoperative diagnostic work-up, but a multimodal work-up including ultrasound of the liver, computed tomography, magnetic resonance imaging and endoscopic retrograde cholangiopancreatography was used in most studies. Disease duration was variable with up to several years. Most patients had episodes of cholangitis, sepsis, fever or abdominal pain. Tumor detection was an incidental finding of the surgical specimen in most cases because it is currently often impossible to detect tumor manifestation during preoperative diagnostics. Liver resection or liver transplantation was performed depending on the extent of the biliary pathology and additional alterations of the liver structure or function. No postoperative adjuvant chemotherapy was reported, but chemotherapy was administered in selected cases of tumor recurrence. Overall survival rates after one year were low at 36% and a high recurrence rate of up to 75% during the observation period.
CONCLUSION Only few retrospective studies reported a low tumor incidence. Despite the high rate of mortality and tumor recurrence, definite surgical treatment should be offered as soon as possible.
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Affiliation(s)
- René Fahrner
- Department of Surgery, Bürgerspital Solothurn, Solothurn 4500, Switzerland
| | - Sandra GC Dennler
- Department of Surgery, University Hospital Dresden, Dresden 01307, Germany
| | - Daniel Inderbitzin
- Department of Surgery, Bürgerspital Solothurn, Solothurn 4500, Switzerland
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Liu B, Cao PK, Wang YZ, Wang WJ, Tian SL, Hertzanu Y, Li YL. Modified percutaneous transhepatic papillary balloon dilation for patients with refractory hepatolithiasis. World J Gastroenterol 2020; 26:3929-3937. [PMID: 32774067 PMCID: PMC7385557 DOI: 10.3748/wjg.v26.i27.3929] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/06/2020] [Revised: 05/24/2020] [Accepted: 07/04/2020] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Some patients with hepatolithiasis cannot tolerate surgery due to severe cardiac or pulmonary comorbidities, or cannot be endoscopically treated because of altered gastrointestinal anatomies.
AIM To propose a modified percutaneous transhepatic papillary balloon dilation procedure, and evaluate the clinical efficacy and safety of this modality.
METHODS Data from 21 consecutive patients who underwent modified percutaneous transhepatic papillary balloon dilation with hepatolithiasis were retrospectively analyzed. Using auxiliary devices, intrahepatic bile duct stones were pushed into the common bile duct and expelled into the duodenum with an inflated balloon catheter. The outcomes recorded included success rate, procedure time, hospital stay, causes of failure, and procedure-related complications. Patients with possible long-term complications were followed up for 2 years.
RESULTS Intrahepatic bile duct stones were successfully removed in 20 (95.23%) patients. Mean procedure time was 65.8 ± 5.3 min. Mean hospital stay was 10.7 ± 1.5 d. No pancreatitis, gastrointestinal, or biliary duct perforation was observed. All patients were followed up for 2 years, and there was no evidence of reflux cholangitis or calculi recurrence.
CONCLUSION Modified percutaneous transhepatic papillary balloon dilation was feasible and safe with a small number of patients with hepatolithiasis, and may be a treatment option in patients with severe comorbidities or in patients in whom endoscopic procedure was not successful.
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Affiliation(s)
- Bin Liu
- Department of Interventional Medicine, The Second Hospital of Shandong University, Jinan 250033, Shandong Province, China
- Interventional Oncology Institute of Shandong University, Jinan 250033, Shandong Province, China
| | - Pi-Kun Cao
- Department of Cancer Center, The First Affiliated Hospital of Shandong First Medical University, Jinan 250014, Shandong Province, China
| | - Yong-Zheng Wang
- Department of Interventional Medicine, The Second Hospital of Shandong University, Jinan 250033, Shandong Province, China
- Interventional Oncology Institute of Shandong University, Jinan 250033, Shandong Province, China
| | - Wu-Jie Wang
- Department of Interventional Medicine, The Second Hospital of Shandong University, Jinan 250033, Shandong Province, China
- Interventional Oncology Institute of Shandong University, Jinan 250033, Shandong Province, China
| | - Shi-Lin Tian
- School of Medicine, Shandong University, Jinan 250014, Shandong Province, China
| | - Yancu Hertzanu
- Department of Interventional Medicine, The Second Hospital of Shandong University, Jinan 250033, Shandong Province, China
- Interventional Oncology Institute of Shandong University, Jinan 250033, Shandong Province, China
| | - Yu-Liang Li
- Department of Interventional Medicine, The Second Hospital of Shandong University, Jinan 250033, Shandong Province, China
- Interventional Oncology Institute of Shandong University, Jinan 250033, Shandong Province, China
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Fahrner R, Dennler SGC, Dondorf F, Ardelt M, Rauchfuss F, Settmacher U. Liver resection and transplantation in Caroli disease and syndrome. J Visc Surg 2019; 156:91-95. [PMID: 29929811 DOI: 10.1016/j.jviscsurg.2018.06.001] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
INTRODUCTION Caroli disease (CD) is a congenital dilatation of the intrahepatic bile ducts. In combination with liver fibrosis or cirrhosis, it is called Caroli syndrome (CS). Infectious complications and intrahepatic cholangiocarcinoma are secondary problems. The aim of this study was to analyse the clinical pattern and outcome in patients with CD/CS who underwent liver surgery. METHODS Between January 2004 and December 2016, 21 patients with CD/CS were treated with liver resection or transplantation (LTX) and post-operative data of patients with CD/CS were retrospectively analysed in a database. RESULTS Two patients underwent LTX, and 19 patients underwent liver resection due to CD/CS. During follow-up, one patient developed lung cancer nine years after LTX. Patients resected due to CD/CS were predominantly females (74%) with an overall low incidence of co-morbidities. The median post-operative Clavien-Dindo score was 1 (range: 0-3). There was no death during a median follow-up period of over five years. In four patients, cholangiocarcinoma was confirmed. Tumor recurrence was seen in three patients, and was treated with chemotherapy or repeated liver resection. CONCLUSIONS LTX and liver resections due to CD/CS are rare and associated with an acceptable post-operative morbidity and low mortality. Surgical treatment should be performed as early as possible to avoid recurrent episodes of cholangitis or carcinogenesis.
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Affiliation(s)
- R Fahrner
- Department of General, Visceral and Vascular Surgery, Jena University Hospital, Am Klinikum 1, 07747 Jena, Germany.
| | - S G C Dennler
- Department of General, Visceral and Vascular Surgery, Jena University Hospital, Am Klinikum 1, 07747 Jena, Germany
| | - F Dondorf
- Department of General, Visceral and Vascular Surgery, Jena University Hospital, Am Klinikum 1, 07747 Jena, Germany
| | - M Ardelt
- Department of General, Visceral and Vascular Surgery, Jena University Hospital, Am Klinikum 1, 07747 Jena, Germany
| | - F Rauchfuss
- Department of General, Visceral and Vascular Surgery, Jena University Hospital, Am Klinikum 1, 07747 Jena, Germany
| | - U Settmacher
- Department of General, Visceral and Vascular Surgery, Jena University Hospital, Am Klinikum 1, 07747 Jena, Germany
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Tabibian JH, Hilscher MB, Martin JA. Cholangiohepatitis With Extensive Intrahepatic Choledocholithiasis. Clin Gastroenterol Hepatol 2017; 15:A31-A32. [PMID: 28754269 DOI: 10.1016/j.cgh.2017.06.048] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/28/2017] [Accepted: 06/29/2017] [Indexed: 02/07/2023]
Affiliation(s)
- James H Tabibian
- Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota; Division of Gastroenterology, Olive View-UCLA Medical Center, Sylmar, California
| | - Moira B Hilscher
- Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota
| | - John A Martin
- Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota
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Abstract
Caroli's disease is a rare congenital disease with multiple segmental dilatations of the bile ducts. It may present in two forms: one (the simple form) with segmental dilatations only, and the other with dilatations combined with fibroangioadenomatosis, frequently complicated by portal hypertension. The disease is thought to be recessively inherited, but few familial cases have been reported. We have observed two sisters with the disease, one with the simple form, the other with fibroangioadenomatosis. Their case histories and diagnostic findings, with special reference to ultrasonography and computed tomography, are presented, and the potential for diagnosis by different imaging modalities is discussed.
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Surgical Management of Caroli's Disease: Single Center Experience and Review of the Literature. J Gastrointest Surg 2015; 19:2019-27. [PMID: 26302876 DOI: 10.1007/s11605-015-2918-9] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/26/2015] [Accepted: 08/10/2015] [Indexed: 02/07/2023]
Abstract
BACKGROUND Caroli's disease is a rare congenital condition characterized by non-obstructive dilatation of intrahepatic ducts. In Caroli's syndrome, there is additionally an associated congenital hepatic fibrosis. METHODS With institutional review board approval, we identified all patients with Caroli's disease and syndrome. RESULTS Nine patients were identified, seven males and two females, with a median age of 40 years. Final pathological diagnoses included Caroli's disease (n = 6) and Caroli's syndrome (n = 3). Patients presented with deranged liver function, cholangitis, cholangiocarcinoma, abdominal pain, cirrhosis, or were diagnosed incidentally. Four patients underwent resection and two underwent liver transplantation. Of the resection group, two patients subsequently underwent transplantation for recurrent cholangitis due to anastomotic stricture in one patient and for end-stage liver disease in the other. All patients with Caroli's syndrome underwent liver transplantation. Three patients died during follow-up at 26.2, 7.8, and 3 months post-diagnosis with recurrence of cholangiocarcinoma, liver failure, and metastatic cholangiocarcinoma, respectively. Six patients are alive with a median follow-up of 60 months since presentation (range = 10-134 months). CONCLUSIONS Caroli's disease and syndrome have a varied presentation. Most individuals with Caroli's disease may be adequately treated by resection, but transplantation is required for Caroli's syndrome patients due to the associated hepatic fibrosis.
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Jani A, Delaney D, Boardman P, Sharma RA. Sub-hepatic cholesterol stones indicate a sinister underlying diagnosis. QJM 2012; 105:699-700. [PMID: 21642362 DOI: 10.1093/qjmed/hcr079] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
Affiliation(s)
- A Jani
- Somerville College, University of Oxford, Woodstock Road, Oxford OX2 6HD, UK
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Harring TR, Nguyen NTT, Liu H, Goss JA, O'Mahony CA. Caroli disease patients have excellent survival after liver transplant. J Surg Res 2012; 177:365-72. [PMID: 22640887 DOI: 10.1016/j.jss.2012.04.022] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/06/2012] [Revised: 03/22/2012] [Accepted: 04/11/2012] [Indexed: 02/08/2023]
Abstract
BACKGROUND Caroli disease (CD) is characterized by dilation of the intrahepatic biliary tree, which may result in malignancy. Treatments include management of symptoms and hepatic resection to decrease disease burden. In patients with CD not amenable to these treatments, orthotopic liver transplantation (OLT) has been used. This study examines if OLT is a reasonable treatment for patients with CD. MATERIALS AND METHODS The United Network of Organ Sharing/Organ Procurement and Transplantation Network database between September 30, 1987 and March 31, 2011 was queried. Cases without patient or allograft survival time or without a diagnosis were excluded from analysis. Patients with CD were compared to patients with primary biliary cirrhosis (PBC), secondary biliary cirrhosis (BC), primary sclerosing cholangitis (PSC), and all indications for OLT. Survival analysis was performed by log-rank test and Kaplan-Meier. RESULTS One hundred forty patients with CD were compared to 4797 patients with PBC, 489 patients with secondary BC, 6033 patients with PSC, and 92,210 patients post-OLT. Patient and allograft survivals of CD patients at 1, 3, 5, and 10 y are, respectively, 88.5%, 83.4%, 80.9%, and 77.8%; and 81.2%, 74.8%, 70.6%, and 67.9%. CD patients have significantly improved patient and allograft survivals after OLT compared to patients with secondary BC (P = 0.003, P = 0.015) and all other patients undergoing OLT (P = 0.003, P = 0.026). There is a trend towards long-term improved patient and allograft survival in transplanted patients with CD compared to patients with PBC and PSC. CONCLUSIONS These results suggest that OLT should be considered an effective treatment modality for patients with CD resulting in excellent long-term outcomes.
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Affiliation(s)
- Theresa R Harring
- Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas, USA
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10
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Jin S. Etiology and pathogenesis of hepatolithiasis. Shijie Huaren Xiaohua Zazhi 2012; 20:3324. [DOI: 10.11569/wcjd.v20.i34.3324] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
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Steinbrück K, Enne M, Fernandes R, Martinho JM, Pacheco-Moreira LF. Living Donor Liver Transplantation for Caroli's Disease: A Report of Two Cases. ISRN SURGERY 2011; 2011:106487. [PMID: 22084740 PMCID: PMC3195350 DOI: 10.5402/2011/106487] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 02/20/2011] [Accepted: 03/24/2011] [Indexed: 12/21/2022]
Abstract
Caroli's disease (CD) is a rare autosomal recessive disorder characterized by intrahepatic cystic dilatation of the bile ducts. Patients with bilobar or progressive disease may require orthotopic liver transplantation (OLT). In the MELD era, living donor liver transplantation (LDLT) raised as the ultimate treatment option for these patients, once their MELD score is usually low. Herein, we describe 2 cases of patients (a 2-year-old girl and a 19-year-old teenager) that successfully underwent LDLT as a treatment for diffuse CD. The good postoperative courses of the two cases indicate that LDLT is a feasible option in the treatment of this disorder, even in complicated or early age patients.
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Affiliation(s)
- Klaus Steinbrück
- Liver Transplantation Unit, Bonsucesso Federal Hospital, Health Ministry, Rio de Janeiro, 21041-030, RJ, Brazil
| | - Marcelo Enne
- Liver Transplantation Unit, Bonsucesso Federal Hospital, Health Ministry, Rio de Janeiro, 21041-030, RJ, Brazil
| | - Reinaldo Fernandes
- Liver Transplantation Unit, Bonsucesso Federal Hospital, Health Ministry, Rio de Janeiro, 21041-030, RJ, Brazil
| | - Jose M. Martinho
- Liver Transplantation Unit, Bonsucesso Federal Hospital, Health Ministry, Rio de Janeiro, 21041-030, RJ, Brazil
| | - Lúcio F. Pacheco-Moreira
- Liver Transplantation Unit, Bonsucesso Federal Hospital, Health Ministry, Rio de Janeiro, 21041-030, RJ, Brazil
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Hori T, Oike F, Ogura Y, Ogawa K, Hata K, Yonekawa Y, Egawa H, Takada Y, Kaido T, Uemoto S. Liver Transplantation for Congenital Biliary Dilatation. THE JAPANESE JOURNAL OF GASTROENTEROLOGICAL SURGERY 2011; 44:1219-1230. [DOI: 10.5833/jjgs.44.1219] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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Abstract
OBJECTIVE To assess the preoperative disease characteristics as well as the rate of postoperative complications, patient survival, and course of symptoms after liver resection or orthotopic liver transplantation (OLT) for Caroli disease (CD) or syndrome (CS). SUMMARY BACKGROUND DATA The clinical course of monolobar or diffuse CD or CS is often characterized by multiple conservative treatment attempts and interventions with recurrent episodes of cholangitis and a serious reduction in quality of life. The role and effectiveness of surgical treatment is still not well defined. PATIENTS AND METHODS Between June 1989 and December 2002, we treated 44 consecutive patients with CD or CS who had failure of conservative treatment before and were referred for surgical intervention. Demographic and clinical data, operative procedures and related morbidity, course of symptoms, and long-term follow-up were reviewed. Four patients with palliative resection for cholangiocarcinoma and incidental diagnosis of CD were excluded from the analysis. RESULTS Twenty-two women and 18 men had a median period of 26.5 months from onset of symptoms to surgical therapy. Their median age at therapy was 49 years and 80% of the patients had monolobar disease with a left-right ratio of 2.6 to 1. Thirty-three (82.5%) patients underwent liver resection, while 4 (10%) patients received OLT for diffuse disease. Biliodigestive anastomosis alone was performed in 3 (7.5%) patients with contraindications to OLT. Patients (37.5%) had minor postoperative complications, which were treated conservatively, while 2 (5%) transplanted patients had a reoperation due to intraperitoneal bleeding. After a median follow-up of 86.5 months, we observed a favorable patient and graft survival. Three deaths during follow-up were not related to treatment or disease complications. Follow-up of disease-related symptoms, biliary complications, and antibiotic treatment revealed a significant improvement. CONCLUSION Our data show that liver resection for monolobar CD or CS and OLT for diffuse manifestations can achieve excellent long-term patient survival with marked symptom relief. Because of life-threatening long-term complications such as biliary sepsis and development of cholangiocarcinoma, timely indication for surgical treatment is crucial.
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Habib S, Shakil O, Couto OF, Demetris AJ, Fung JJ, Marcos A, Chopra K. Caroli's disease and orthotopic liver transplantation. Liver Transpl 2006; 12:416-21. [PMID: 16498655 DOI: 10.1002/lt.20719] [Citation(s) in RCA: 46] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Caroli's disease is a rare congenital hepatic disease, characterized by segmental dilatation of the biliary tree. Patients who have recurrent bouts of biliary infection, particularly those with complications related to portal hypertension, may require orthotopic liver transplantation (OLT). Few case reports have described the outcome of OLT in patients with Caroli's disease and to date there is no large series reported in the literature. We retrospectively analyzed the outcome of OLT in patients with Caroli's disease who underwent OLT between 1982 and 2002 at Starzl Transplantation Institute, University of Pittsburgh. Patients were identified and data was collected by computerized search of the electronic database system. All patients had confirmation of diagnosis by histopathology of explanted liver. A total of 33 patients with Caroli's disease were listed for liver transplantation, 3 of whom were excluded, as they were not transplanted. A total of 90% had signs of hepatic decompensation at the time of OLT. Median posttransplantation follow-up was 7.7 yr. Short-term graft and patient survival at 1 month was 83% and 86%, whereas overall long-term graft survival rates at 1, 5, and 10 yr were 73%, 62%, and 53%, respectively, and patient survival rates were 76%, 65%, and 56%, respectively. Long-term outcome in patients who survived the first year after transplantation was significantly better. Their survival rate at 5 and 10 yr was 90% and 78%. On univariable analysis, recipient age, donor male gender, coexistent congenital hepatic fibrosis, and re-OLT were associated with poor patient survival. Eight patients were retransplanted, 3 of whom had primary nonfunction. A total of 13 patients died; the most common cause of death being sepsis and cardiovascular complications. Patients who died of sepsis had cholangitis pre-OLT. In conclusion, OLT is a form of curative and life-saving therapy in patients with Caroli's disease, especially in those with decompensated liver disease. Overall survival is better with liver transplantation and is comparable with the survival of recipients who undergo OLT for other etiologies of chronic liver disease. Survival was poor in patients with congenital hepatic fibrosis (Caroli's syndrome) and in those who had cholangitis at the time OLT.
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Affiliation(s)
- Shahid Habib
- Division of Gastroenterology, Hepatology & Nutrition, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
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Herman P, Bacchella T, Pugliese V, Montagnini AL, Machado MAC, da Cunha JEM, Machado MCC. Non-Oriental Primary Intrahepatic Lithiasis: Experience with 48 Cases. World J Surg 2005; 29:858-62; discussion 863-4. [PMID: 15951933 DOI: 10.1007/s00268-005-7759-3] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
Abstract
An experience with the diagnosis and treatment of patients with non-Oriental primary intrahepatic lithiasis (PIHL) is described. A group of 48 native Brazilian patients with symptomatic PIHL were studied, and the patients' characteristics, diagnoses, treatment protocols based on the presentation of the disease, prognostic factors, and late results were analyzed. Liver resection was performed in patients with an irreversible lesion, such as parenchymal atrophy or biliary stenosis; and biliary drainage procedures were employed in patients with bilateral disease. Late results were considered good when no postoperative symptoms were observed and poor if there was pain recurrence or cholangitis. Overall good results were observed in 73.4% of the patients. Good late results were observed in 94.1% and 62.1% of the patients with unilateral and bilateral stones, respectively. None of the analyzed parameters (gender, age, previous biliary surgery, bilirubin level, serum leukocyte counts, prothrombin activity, previous history of cholangitis, stone location) predicted poor late results, but the risk for patients with bilateral stones to develop late complications was 7.2 times higher than for those with unilateral disease. Non-Oriental PIHL is a rare disease, and the patients' characteristics are similar to those of patients with Oriental PIHL. We believe that personalized treatment based on the presentation of the disease led to the high incidence of good late results in this series.
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Affiliation(s)
- Paulo Herman
- Department of Gastroenterology, University of São Paulo Medical School, R Eneas De Carvale Aguar, 255 São Paulo, Brazil.
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Affiliation(s)
- A S Fulcher
- Department of Radiology, Medical College of Virginia of Virginia Commonwealth University, 401 N 12th St, PO Box 980615, Richmond, VA 23298-0615, USA.
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Totkas S, Hohenberger P. Cholangiocellular carcinoma associated with segmental Caroli's disease. EUROPEAN JOURNAL OF SURGICAL ONCOLOGY 2000; 26:520-1. [PMID: 11016478 DOI: 10.1053/ejso.1999.0936] [Citation(s) in RCA: 30] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
This case report describes the association of cholangiocarcinoma arising in a liver segment with Caroli's disease. We demonstrate the diagnostic difficulties in recognizing the pathogenetic background and outline the indication for surgical treatment.
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Affiliation(s)
- S Totkas
- Division of Surgery and Surgical Oncology, Robert Roessle Hospital and Tumor Institute, Berlin, Germany
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Affiliation(s)
- D M Gold
- Division of Pediatric Gastroenterology and Nutrition, Schneider Children's Hospital, New Hyde Park, New York 11042, USA
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Tarroch X, Tallada N, Castells C, Garcia M. Fine-needle aspiration biopsy of hepatic papillary cystadenocarcinoma in Caroli's disease. Diagn Cytopathol 1992; 8:167-70. [PMID: 1568414 DOI: 10.1002/dc.2840080215] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/27/2022]
Abstract
A case of papillary adenocarcinoma arising in Caroli's disease (CD) in a 25-yr-old woman is reported. The diagnosis of malignancy was made by ultrasound-guided, fine-needle aspiration biopsy (FNAB). As there were no metastasis, a liver transplant was performed and the diagnosis was histologically confirmed. Only 22 cases of malignant transformation of CD have been reported to date, all among middle-aged subjects (mean age: 52 yr) of both sexes. Most of these reported cases (83%) were found to be bile duct adenocarcinomas with occasional reports of hepatocellular and undifferentiated carcinomas. Our case is the youngest reported to date and the first to be diagnosed cytologically.
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Affiliation(s)
- X Tarroch
- Department of Pathology, Ciutat Sanitaria Universitaria Vall d'Hebron, Universitat Autònoma de Barcelona, Spain
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Strichartz SD, Abedin MZ, Ippoliti AF, Derezin M, Roslyn JJ. Intrahepatic cholesterol stones: a rationale for dissolution therapy. Gastroenterology 1991; 100:228-32. [PMID: 1983825 DOI: 10.1016/0016-5085(91)90605-k] [Citation(s) in RCA: 22] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
A case of primary cholesterol hepatolithiasis is reported. Stone composition was documented by infrared spectroscopy, and the presence of cholesterol saturated bile was demonstrated using standard biochemical techniques. The patient was treated with operative stone extraction, choledochoscopy, biliary enteric anastomosis, and oral dissolution therapy. The administration of oral dissolution agents has altered the composition of the patient's bile and may prevent further stone formation. We advocate the use of both stone and biliary biochemical analysis for patients with primary hepatolithiasis to facilitate optimal therapy.
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Affiliation(s)
- S D Strichartz
- Department of Medicine, University of California School of Medicine, Los Angeles
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Deasy J, Conway W, Burke G, Delaney P. Caroli's disease. Case report with unusual features and a review of current diagnostic and treatment modalities. Ir J Med Sci 1989; 158:93-4. [PMID: 2753663 DOI: 10.1007/bf02942115] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/02/2023]
Abstract
This is a case report of the simple form of Caroli's disease in a 36 year old Irish woman who has extensive bilobar involvement without congenital hepatic fibrosis or portal hypertension. The difficulty of diagnosis and the intractable nature of the disease are emphasised and recent improvements in diagnosis and management are discussed.
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Nakayama F, Soloway RD, Nakama T, Miyazaki K, Ichimiya H, Sheen PC, Ker CG, Ong GB, Choi TK, Boey J. Hepatolithiasis in East Asia. Retrospective study. Dig Dis Sci 1986; 31:21-26. [PMID: 3940820 DOI: 10.1007/bf01347905] [Citation(s) in RCA: 125] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/08/2023]
Abstract
Hepatolithiasis is a major disease in Asia but differences in operative incidence between countries have not been examined. A retrospective study was conducted in Taiwan, Hong Kong, and Singapore, and the results were compared with those in Japan with the aim of defining factors involved in the etiology of the condition. In order to ensure uniformity of the data collected, the same form was used throughout the study and was completed by the same personnel after reviewing the patient's record and radiographs in each case. The years 1976-1980 were chosen for the study, since the newer methods of diagnosis such as ultrasound, endoscopic retrograde cholangiography, and percutaneous transhepatic cholangiography became available during that period. The most significant finding was the difference in the relative prevalence of hepatolithiasis as a proportion of all gallstone cases in Taiwan, Hong Kong, and Singapore, where the majority of the population consisted of patients of Chinese descent. The highest prevalence, 53.5%, was found in Taiwan, while in Hong Kong it was 3.1% and in Singapore 1.7%. Environmental rather than ethnic factors are implicated in the cause of hepatolithiasis.
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Abstract
This study reports four new cases of Caroli's disease complicated by the development of cholangiocarcinoma. Caroli's disease appears to be a premalignant condition, but early diagnosis of malignancy in the cyst wall is difficult because signs of ductal stenosis develop slowly and treatment is often based on erroneous diagnosis of recurrent cholangitis. When carcinoma is present, operative intervention makes no difference in survival. In patients with Caroli's disease, adequate intraductal and hepatic biopsies should be obtained at the first operation. These patients should be followed carefully, and malignant growth should be suspected when cholangitis occurs despite patent anastomoses. Earlier diagnosis will obviate repeated operations in patients with malignant Caroli's disease.
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Sty JR, Hubbard AM, Starshak RJ. Radionuclide hepatobiliary imaging in congenital biliary tract ectasia (Caroli disease). Pediatr Radiol 1982; 12:111-4. [PMID: 7110761 DOI: 10.1007/bf00971797] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/23/2023]
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