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Berg T, Aehling NF, Bruns T, Welker MW, Weismüller T, Trebicka J, Tacke F, Strnad P, Sterneck M, Settmacher U, Seehofer D, Schott E, Schnitzbauer AA, Schmidt HH, Schlitt HJ, Pratschke J, Pascher A, Neumann U, Manekeller S, Lammert F, Klein I, Kirchner G, Guba M, Glanemann M, Engelmann C, Canbay AE, Braun F, Berg CP, Bechstein WO, Becker T, Trautwein C. S2k-Leitlinie Lebertransplantation der Deutschen Gesellschaft für Gastroenterologie, Verdauungs- und Stoffwechselkrankheiten (DGVS) und der Deutschen Gesellschaft für Allgemein- und Viszeralchirurgie (DGAV). ZEITSCHRIFT FUR GASTROENTEROLOGIE 2024; 62:1397-1573. [PMID: 39250961 DOI: 10.1055/a-2255-7246] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 09/11/2024]
Affiliation(s)
- Thomas Berg
- Bereich Hepatologie, Medizinischen Klinik II, Universitätsklinikum Leipzig, Leipzig, Deutschland
| | - Niklas F Aehling
- Bereich Hepatologie, Medizinischen Klinik II, Universitätsklinikum Leipzig, Leipzig, Deutschland
| | - Tony Bruns
- Medizinische Klinik III, Universitätsklinikum Aachen, Aachen, Deutschland
| | - Martin-Walter Welker
- Medizinische Klinik I Gastroent., Hepat., Pneum., Endokrin. Universitätsklinikum Frankfurt, Frankfurt, Deutschland
| | - Tobias Weismüller
- Klinik für Innere Medizin - Gastroenterologie und Hepatologie, Vivantes Humboldt-Klinikum, Berlin, Deutschland
| | - Jonel Trebicka
- Medizinische Klinik B für Gastroenterologie und Hepatologie, Universitätsklinikum Münster, Münster, Deutschland
| | - Frank Tacke
- Charité - Universitätsmedizin Berlin, Medizinische Klinik m. S. Hepatologie und Gastroenterologie, Campus Virchow-Klinikum (CVK) und Campus Charité Mitte (CCM), Berlin, Deutschland
| | - Pavel Strnad
- Medizinische Klinik III, Universitätsklinikum Aachen, Aachen, Deutschland
| | - Martina Sterneck
- Medizinische Klinik und Poliklinik I, Universitätsklinikum Hamburg, Hamburg, Deutschland
| | - Utz Settmacher
- Klinik für Allgemein-, Viszeral- und Gefäßchirurgie, Universitätsklinikum Jena, Jena, Deutschland
| | - Daniel Seehofer
- Klinik für Viszeral-, Transplantations-, Thorax- und Gefäßchirurgie, Universitätsklinikum Leipzig, Leipzig, Deutschland
| | - Eckart Schott
- Klinik für Innere Medizin II - Gastroenterologie, Hepatologie und Diabetolgie, Helios Klinikum Emil von Behring, Berlin, Deutschland
| | | | - Hartmut H Schmidt
- Klinik für Gastroenterologie und Hepatologie, Universitätsklinikum Essen, Essen, Deutschland
| | - Hans J Schlitt
- Klinik und Poliklinik für Chirurgie, Universitätsklinikum Regensburg, Regensburg, Deutschland
| | - Johann Pratschke
- Chirurgische Klinik, Charité Campus Virchow-Klinikum - Universitätsmedizin Berlin, Berlin, Deutschland
| | - Andreas Pascher
- Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Universitätsklinikum Münster, Münster, Deutschland
| | - Ulf Neumann
- Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Universitätsklinikum Essen, Essen, Deutschland
| | - Steffen Manekeller
- Klinik und Poliklinik für Allgemein-, Viszeral-, Thorax- und Gefäßchirurgie, Universitätsklinikum Bonn, Bonn, Deutschland
| | - Frank Lammert
- Medizinische Hochschule Hannover (MHH), Hannover, Deutschland
| | - Ingo Klein
- Chirurgische Klinik I, Universitätsklinikum Würzburg, Würzburg, Deutschland
| | - Gabriele Kirchner
- Klinik und Poliklinik für Chirurgie, Universitätsklinikum Regensburg und Innere Medizin I, Caritaskrankenhaus St. Josef Regensburg, Regensburg, Deutschland
| | - Markus Guba
- Klinik für Allgemeine, Viszeral-, Transplantations-, Gefäß- und Thoraxchirurgie, Universitätsklinikum München, München, Deutschland
| | - Matthias Glanemann
- Klinik für Allgemeine, Viszeral-, Gefäß- und Kinderchirurgie, Universitätsklinikum des Saarlandes, Homburg, Deutschland
| | - Cornelius Engelmann
- Charité - Universitätsmedizin Berlin, Medizinische Klinik m. S. Hepatologie und Gastroenterologie, Campus Virchow-Klinikum (CVK) und Campus Charité Mitte (CCM), Berlin, Deutschland
| | - Ali E Canbay
- Medizinische Klinik, Universitätsklinikum Knappschaftskrankenhaus Bochum, Bochum, Deutschland
| | - Felix Braun
- Klinik für Allgemeine Chirurgie, Viszeral-, Thorax-, Transplantations- und Kinderchirurgie, Universitätsklinikum Schlewswig-Holstein, Kiel, Deutschland
| | - Christoph P Berg
- Innere Medizin I Gastroenterologie, Hepatologie, Infektiologie, Universitätsklinikum Tübingen, Tübingen, Deutschland
| | - Wolf O Bechstein
- Klinik für Allgemein- und Viszeralchirurgie, Universitätsklinikum Frankfurt, Frankfurt, Deutschland
| | - Thomas Becker
- Klinik für Allgemeine Chirurgie, Viszeral-, Thorax-, Transplantations- und Kinderchirurgie, Universitätsklinikum Schlewswig-Holstein, Kiel, Deutschland
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Gittus M, Moore J, Ong ACM. Liver transplant recipients with polycystic liver disease have longer waiting times but better long-term clinical outcomes than those with liver disease due to other causes: A retrospective cross-sectional study. PLoS One 2024; 19:e0294717. [PMID: 38165905 PMCID: PMC10760649 DOI: 10.1371/journal.pone.0294717] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/12/2023] [Accepted: 11/06/2023] [Indexed: 01/04/2024] Open
Abstract
INTRODUCTION Liver transplantation is the only curative option for patients with polycystic liver disease (PLD). In the United Kingdom, these patients are listed on the variant syndrome list due to their preserved liver function reflected in the United Kingdom End-stage Liver Disease (UKELD) score. The transplantation and survival rates for this patient group in the UK have not been previously reported. METHODS A retrospective cross-sectional analysis of patients receiving liver transplantation between 2010 and 2017 was performed using the NHS blood and transplantation database. This database contains the demographic, clinical parameters, indication for transplantation and follow-up of all patients in UK-based transplant centres. Basic statistics was performed using SPSS version 27. RESULTS 5412 recipients received elective liver allografts in the study period. 1.6% (100) of recipients had PLD as their primary indication for transplantation with 60 receiving liver only allografts and 40 receiving combined liver-kidney allografts. PLD patients had a >3-fold longer mean waiting time for transplantation compared to non-PLD patients, 508 days v 154 days respectively. PLD patients receiving combined liver-kidney allografts had a longer waiting time than those receiving a liver only allograft, 610 days v 438 days respectively. There were comparable patient survival rates for people with PLD and non-PLD primary indications at 30 days (94.0% vs 97.6%) and 1 year (92.0% vs 93.2%) but improved survival rates at 5 years (81.3% vs 76.5%). There were also comparable allograft survival rates for people with PLD and non-PLD primary indications at 30 days (93.9% vs 95.3%) and 1 year (91.9% vs 91.2%) but improved survival rates at 5 years (82.5% vs 77.3%). Transplant centre-level analysis identified variation in the proportion of liver transplantations for people with PLD as their primary listed indication. CONCLUSIONS Patients with PLD wait significantly longer for liver transplantation compared to other indications. However, transplanted PLD patients demonstrate better longer-term patient and liver allograft survival rates compared to transplanted non-PLD patients. The unexpected variation between individual UK centres transplanting for PLD deserves further study.
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Affiliation(s)
- Matt Gittus
- Academic Nephrology Unit, Division of Clinical Medicine, Faculty of Health, Sheffield Teaching Hospitals Trust, University of Sheffield and Sheffield Kidney Institute, Sheffield, United Kingdom
| | - Joanna Moore
- Liver Transplant Unit, Leeds Teaching Hospitals Trust, Leeds, United Kingdom
| | - Albert C. M. Ong
- Academic Nephrology Unit, Division of Clinical Medicine, Faculty of Health, Sheffield Teaching Hospitals Trust, University of Sheffield and Sheffield Kidney Institute, Sheffield, United Kingdom
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Norcia LF, Watanabe EM, Hamamoto Filho PT, Hasimoto CN, Pelafsky L, de Oliveira WK, Sassaki LY. Polycystic Liver Disease: Pathophysiology, Diagnosis and Treatment. Hepat Med 2022; 14:135-161. [PMID: 36200122 PMCID: PMC9528914 DOI: 10.2147/hmer.s377530] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/06/2022] [Accepted: 09/07/2022] [Indexed: 11/25/2022] Open
Abstract
Polycystic liver disease (PLD) is a clinical condition characterized by the presence of more than 10 cysts in the liver. It is a rare disease Of genetic etiology that presents as an isolated disease or assoc\iated with polycystic kidney disease. Ductal plate malformation, ciliary dysfunction, and changes in cell signaling are the main factors involved in its pathogenesis. Most patients with PLD are asymptomatic, but in 2-5% of cases the disease has disabling symptoms and a significant reduction in quality of life. The diagnosis is based on family history of hepatic and/or renal polycystic disease, clinical manifestations, patient age, and polycystic liver phenotype shown on imaging examinations. PLD treatment has evolved considerably in the last decades. Somatostatin analogues hold promise in controlling disease progression, but liver transplantation remains a unique curative treatment modality.
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Affiliation(s)
- Luiz Fernando Norcia
- Department of Surgery, São Paulo State University (Unesp), Medical School, Botucatu, São Paulo, Brazil
| | - Erika Mayumi Watanabe
- Department of Radiology, São Paulo State University (Unesp), Medical School, Botucatu, São Paulo, Brazil
| | - Pedro Tadao Hamamoto Filho
- Department of Neurology, Psychology and Psychiatry, São Paulo State University (Unesp), Medical School, Botucatu, São Paulo, Brazil
| | - Claudia Nishida Hasimoto
- Department of Surgery, São Paulo State University (Unesp), Medical School, Botucatu, São Paulo, Brazil
| | - Leonardo Pelafsky
- Department of Surgery, São Paulo State University (Unesp), Medical School, Botucatu, São Paulo, Brazil
| | - Walmar Kerche de Oliveira
- Department of Surgery, São Paulo State University (Unesp), Medical School, Botucatu, São Paulo, Brazil
| | - Ligia Yukie Sassaki
- Department of Internal Medicine, São Paulo State University (Unesp), Medical School, Botucatu, São Paulo, Brazil
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Liver transplantation in a patient with massive polycystic liver disease: A case report and literature review. INTERNATIONAL JOURNAL OF SURGERY OPEN 2022. [DOI: 10.1016/j.ijso.2022.100517] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
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Bae KT, Tao C, Feldman R, Yu AS, Torres VE, Perrone RD, Chapman AB, Brosnahan G, Steinman TI, Braun WE, Mrug M, Bennett WM, Harris PC, Srivastava A, Landsittel DP, Abebe KZ. Volume Progression and Imaging Classification of Polycystic Liver in Early Autosomal Dominant Polycystic Kidney Disease. Clin J Am Soc Nephrol 2022; 17:374-384. [PMID: 35217526 PMCID: PMC8975034 DOI: 10.2215/cjn.08660621] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/24/2021] [Accepted: 01/18/2022] [Indexed: 11/23/2022]
Abstract
BACKGROUND AND OBJECTIVES The progression of polycystic liver disease is not well understood. The purpose of the study is to evaluate the associations of polycystic liver progression with other disease progression variables and classify liver progression on the basis of patient's age, height-adjusted liver cystic volume, and height-adjusted liver volume. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS Prospective longitudinal magnetic resonance images from 670 patients with early autosomal dominant polycystic kidney disease for up to 14 years of follow-up were evaluated to measure height-adjusted liver cystic volume and height-adjusted liver volume. Among them, 245 patients with liver cyst volume >50 ml at baseline were included in the longitudinal analysis. Linear mixed models on log-transformed height-adjusted liver cystic volume and height-adjusted liver volume were fitted to approximate mean annual rate of change for each outcome. The association of sex, body mass index, genotype, baseline height-adjusted total kidney volume, and Mayo imaging class was assessed. We calculated height-adjusted liver cystic volume ranges for each specific age and divided them into five classes on the basis of annual percentage increase in height-adjusted liver cystic volume. RESULTS The mean annual growth rate of height-adjusted liver cystic volume was 12% (95% confidence interval, 11.1% to 13.1%; P<0.001), whereas that for height-adjusted liver volume was 2% (95% confidence interval, 1.9% to 2.6%; P<0.001). Women had higher baseline height-adjusted liver cystic volume than men, but men had higher height-adjusted liver cystic volume growth rate than women by 2% (95% confidence interval, 0.4% to 4.5%; P=0.02). Whereas the height-adjusted liver cystic volume growth rate decreased in women after menopause, no decrease was observed in men at any age. Body mass index, genotype, and baseline height-adjusted total kidney volume were not associated with the growth rate of height-adjusted liver cystic volume or height-adjusted liver volume. According to the height-adjusted liver cystic volume growth rate, patients were classified into five classes (number of women, men in each class): A (24, six); B (44, 13); C (43, 48); D (28, 17); and E (13, nine). CONCLUSIONS Compared with height-adjusted liver volume, the use of height-adjusted liver cystic volume showed greater separations in volumetric progression of polycystic liver disease. Similar to the Mayo imaging classification for the kidney, the progression of polycystic liver disease may be categorized on the basis of patient's age and height-adjusted liver cystic volume.
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Affiliation(s)
- Kyongtae T. Bae
- Department of Radiology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania
| | - Cheng Tao
- Department of Radiology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania
| | - Robert Feldman
- Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania
| | - Alan S.L. Yu
- Division of Nephrology and Hypertension, Department of Internal Medicine, Kansas University Medical Center, Kansas City, Kansas,Jared Grantham Kidney Institute, Kansas University Medical Center, Kansas City, Kansas
| | - Vicente E. Torres
- Division of Nephrology and Hypertension, Mayo Clinic College of Medicine, Rochester, Minnesota
| | | | - Arlene B. Chapman
- Section of Nephrology, University of Chicago School of Medicine, Chicago, Illinois
| | - Godela Brosnahan
- Division of Diseases and Hypertension, University of Colorado Denver Anschutz Medical Campus, Aurora, Colorado
| | | | - William E. Braun
- Department of Nephrology and Hypertension, Cleveland Clinic, Cleveland, Ohio
| | - Michal Mrug
- Department of Medicine, The University of Alabama at Birmingham, Birmingham, Alabama,Department of Veterans Affairs Medical Center, Birmingham, Alabama
| | | | - Peter C. Harris
- Division of Nephrology and Hypertension, Mayo Clinic College of Medicine, Rochester, Minnesota
| | - Avantika Srivastava
- Department of Biomedical Informatics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania
| | - Douglas P. Landsittel
- Department of Biomedical Informatics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania
| | - Kaleab Z. Abebe
- Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania
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Morelli MC, Rendina M, La Manna G, Alessandria C, Pasulo L, Lenci I, Bhoori S, Messa P, Biancone L, Gesualdo L, Russo FP, Petta S, Burra P. Position paper on liver and kidney diseases from the Italian Association for the Study of Liver (AISF), in collaboration with the Italian Society of Nephrology (SIN). Dig Liver Dis 2021; 53 Suppl 2:S49-S86. [PMID: 34074490 DOI: 10.1016/j.dld.2021.03.035] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/16/2021] [Revised: 03/31/2021] [Accepted: 03/31/2021] [Indexed: 02/07/2023]
Abstract
Liver and kidney are strictly connected in a reciprocal manner, in both the physiological and pathological condition. The Italian Association for the Study of Liver, in collaboration with the Italian Society of Nephrology, with this position paper aims to provide an up-to-date overview on the principal relationships between these two important organs. A panel of well-recognized international expert hepatologists and nephrologists identified five relevant topics: 1) The diagnosis of kidney damage in patients with chronic liver disease; 2) Acute kidney injury in liver cirrhosis; 3) Association between chronic liver disease and chronic kidney disease; 4) Kidney damage according to different etiology of liver disease; 5) Polycystic kidney and liver disease. The discussion process started with a review of the literature relating to each of the five major topics and clinical questions and related statements were subsequently formulated. The quality of evidence and strength of recommendations were graded according to the GRADE system. The statements presented here highlight the importance of strong collaboration between hepatologists and nephrologists for the management of critically ill patients, such as those with combined liver and kidney impairment.
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Affiliation(s)
- Maria Cristina Morelli
- Internal Medicine Unit for the treatment of Severe Organ Failure, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Policlinico di S.Orsola, Bologna, Italy, Via Albertoni 15, 40138, Bologna, Italy
| | - Maria Rendina
- Gastroenterology Unit, Department of Emergency and Organ Transplantation, University of Bari, Policlinic Hospital, Piazza G. Cesare 11, 70124, Bari, Italy
| | - Gaetano La Manna
- Department of Experimental Diagnostic and Specialty Medicine (DIMES), Nephrology, Dialysis and Renal Transplant Unit, St. Orsola Hospital, University of Bologna, Via Massarenti 9, 40138, Bologna, Italy
| | - Carlo Alessandria
- Division of Gastroenterology and Hepatology, Città della Salute e della Scienza Hospital, University of Torino, Corso Bramante 88, 10126, Torino, Italy
| | - Luisa Pasulo
- Gastroenterology and Transplant Hepatology, "Papa Giovanni XXIII" Hospital, Piazza OMS 1, 24127, Bergamo, Italy
| | - Ilaria Lenci
- Department of Internal Medicine, Hepatology Unit, Tor Vergata University, Rome Viale Oxford 81, 00133, Rome, Italy
| | - Sherrie Bhoori
- Hepatology and Hepato-Pancreatic-Biliary Surgery and Liver Transplantation, Fondazione IRCCS, Istituto Nazionale Tumori, Via Giacomo Venezian, 1, 20133, Milan, Italy
| | - Piergiorgio Messa
- Unit of Nephrology, Università degli Studi di Milano, Via Commenda 15, 20122, Milano, Italy; Nephrology, Dialysis and Renal Transplant Unit-Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Via Commenda 15, 20122 Milano, Italy
| | - Luigi Biancone
- Division of Nephrology Dialysis and Transplantation, Department of Medical Sciences, Città Della Salute e della Scienza Hospital, University of Turin, Corso Bramante, 88-10126, Turin, Italy
| | - Loreto Gesualdo
- Nephrology Dialysis and Transplantation Unit, Department of Emergency and Organ Transplantation, Università degli Studi di Bari "Aldo Moro", Piazza G. Cesare 11, 70124, Bari, Italy
| | - Francesco Paolo Russo
- Multivisceral Transplant Unit, Gastroenterology, Department of Surgery, Oncology and Gastroenterology, University Hospital of Padua, Via Giustiniani 2, 35128, Padua, Italy
| | - Salvatore Petta
- Section of Gastroenterology and Hepatology, PROMISE, University of Palermo, Piazza delle Cliniche, 2 90127, Palermo, Italy
| | - Patrizia Burra
- Multivisceral Transplant Unit, Gastroenterology, Department of Surgery, Oncology and Gastroenterology, University Hospital of Padua, Via Giustiniani 2, 35128, Padua, Italy.
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Boillot O, Cayot B, Guillaud O, Crozet-Chaussin J, Hervieu V, Valette PJ, Dumortier J. Partial major hepatectomy with cyst fenestration for polycystic liver disease: Indications, short and long-term outcomes. Clin Res Hepatol Gastroenterol 2021; 45:101670. [PMID: 33722781 DOI: 10.1016/j.clinre.2021.101670] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/09/2020] [Revised: 02/18/2021] [Accepted: 02/26/2021] [Indexed: 02/04/2023]
Abstract
BACKGROUND AND OBJECTIVE Symptomatic polycystic liver disease (PLD) with massive hepatomegaly represents a challenging surgical issue. In this work, we focused on early and long term outcomes after partial hepatectomy with cyst fenestration (PHCF) in selected patients. METHODS All patients who had PHCF for treatment of PLD between January 2003 and December 2019 in our center were included in this study. PHCF was undertaken if at least one hepatic section was relatively spared from PLD, afferent and efferent hepatic vasculature was patent, and liver function was maintained. RESULTS Twenty nine patients (25 women) with a mean age of 54.6 ± 9 years underwent PHCF. Major hepatectomy was performed in all cases with 4.3 ± 0.8 resected segments. Overall perioperative morbidity (Clavien ≥ II) and mortality rates were 41.4.6% and 13.8% respectively. Significant postoperative liver volume reduction was 52.8% within the first year and 55.5% thereafter. From preoperative evaluation, performance status (PS) normalized or improved in 84% of patients. After a mean follow-up time of 70.8 ± 65 months, overall patient survival was 82.7%. In univariate analysis, PS, initial liver volume, operative time and transfusion were associated with post-operative complications and PS, preoperative cyst infection, portal hypertension, transfusion, postoperative sepsis and persistent ascites were associated with mortality. CONCLUSIONS Our study confirms that in spite of significant morbidity rate, PHCF allows a massive reduction of liver volume in selected patients with symptomatic PLD and is highly and durably effective for the reduction of liver volume and improvement of quality of life.
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Affiliation(s)
- Olivier Boillot
- Fédération des Spécialités Digestives, Hôpital Edouard Herriot, Hospices Civils de Lyon, Pavillon D, 69437 Lyon Cedex 03, France; Université Claude Bernard Lyon 1, France.
| | - Bénédicte Cayot
- Fédération des Spécialités Digestives, Hôpital Edouard Herriot, Hospices Civils de Lyon, Pavillon D, 69437 Lyon Cedex 03, France
| | - Olivier Guillaud
- Fédération des Spécialités Digestives, Hôpital Edouard Herriot, Hospices Civils de Lyon, Pavillon D, 69437 Lyon Cedex 03, France; Ramsay Générale de Santé, Clinique de la Sauvegarde, Lyon, France
| | - Jessica Crozet-Chaussin
- Fédération des Spécialités Digestives, Hôpital Edouard Herriot, Hospices Civils de Lyon, Pavillon D, 69437 Lyon Cedex 03, France
| | - Valérie Hervieu
- Service d'anatomopathologie, Hôpital Femme Mère Enfant, Bron, France
| | - Pierre-Jean Valette
- Fédération des Spécialités Digestives, Hôpital Edouard Herriot, Hospices Civils de Lyon, Pavillon D, 69437 Lyon Cedex 03, France; Université Claude Bernard Lyon 1, France
| | - Jérôme Dumortier
- Fédération des Spécialités Digestives, Hôpital Edouard Herriot, Hospices Civils de Lyon, Pavillon D, 69437 Lyon Cedex 03, France; Université Claude Bernard Lyon 1, France
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Ding F, Tang H, Zhao H, Feng X, Yang Y, Chen GH, Chen WJ, Xu C. Long-term results of liver transplantation for polycystic liver disease: Single-center experience in China. Exp Ther Med 2019; 17:4183-4189. [PMID: 31007749 DOI: 10.3892/etm.2019.7449] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/29/2018] [Accepted: 02/15/2019] [Indexed: 11/06/2022] Open
Abstract
The aim of the present study was to explore the indications for and safety of orthotopic liver transplantation for polycystic liver disease (PLD). Orthotopic liver transplantation in 11 patients with PLD between May 2004 and September 2013 was retrospectively analyzed. Patient epidemiological, clinical and follow-up data were collected. The survival rate was calculated using the Kaplan-Meier method. Over the 10-year period, 11 patients received orthotopic liver transplantation (n=9) and combined liver-kidney transplantation (n=2) for PLD. The recipients' median age was 56 years [(interquartile range (IQR), 52-57 years], and 7 of the patients (63.6%) were classified as having Gigot type II PLD and 4 (36.4%) as having Gigot type III. A total of 8 (72.7%) patients had a severely decreased quality of life (Eastern Cooperative Oncology Group performance status score, ≥3). Only 3 cases (27.3%) were of Class C stage. The mean hospitalization duration was 45.4±15.3 days and the mean length of stay at the intensive care unit was 4.1±1.9 days. The peri-operative mortality was 18.2% and the morbidity was 54.5%. The median follow-up period was 111 months (IQR, 33-132 months). A total of 2 patients died of severe complications after combined liver-kidney transplantation. Furthermore, 1 patient died of ischemia cholangitis during the follow-up period. The actuarial 1-, 5- and 10-year survival rate during the follow-up period was 81.8, 81.8 and 65.5%, respectively. The mean physical component summary score was 87.1±6.9 and the mean mental component summary score was 81.5±6.4. In conclusion, liver transplantation is the only curative procedure for PLD, and the present study indicated that it is relatively and safe and leads to good long-term prognosis and high quality of life. Based on our experience and results, liver transplantation is a primary option for cases of PLD with progressive or advanced symptomatic disease where previous other forms of therapy to palliate symptoms have been insufficient.
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Affiliation(s)
- Fan Ding
- Department of Hepatic Surgery and Liver Transplantation Center, The Third Affiliated Hospital, Sun Yat-Sen University, Guangzhou, Guangdong 510630, P.R. China.,Organ Transplantation Institute, Sun Yat-Sen University, Guangzhou, Guangdong 510080, P.R. China
| | - Hui Tang
- Department of Hepatic Surgery and Liver Transplantation Center, The Third Affiliated Hospital, Sun Yat-Sen University, Guangzhou, Guangdong 510630, P.R. China.,Organ Transplantation Institute, Sun Yat-Sen University, Guangzhou, Guangdong 510080, P.R. China
| | - Hui Zhao
- Department of Hepatic Surgery and Liver Transplantation Center, The Third Affiliated Hospital, Sun Yat-Sen University, Guangzhou, Guangdong 510630, P.R. China.,Organ Transplantation Institute, Sun Yat-Sen University, Guangzhou, Guangdong 510080, P.R. China
| | - Xiao Feng
- Department of Hepatic Surgery and Liver Transplantation Center, The Third Affiliated Hospital, Sun Yat-Sen University, Guangzhou, Guangdong 510630, P.R. China.,Organ Transplantation Institute, Sun Yat-Sen University, Guangzhou, Guangdong 510080, P.R. China
| | - Yang Yang
- Department of Hepatic Surgery and Liver Transplantation Center, The Third Affiliated Hospital, Sun Yat-Sen University, Guangzhou, Guangdong 510630, P.R. China.,Organ Transplantation Institute, Sun Yat-Sen University, Guangzhou, Guangdong 510080, P.R. China
| | - Gui-Hua Chen
- Department of Hepatic Surgery and Liver Transplantation Center, The Third Affiliated Hospital, Sun Yat-Sen University, Guangzhou, Guangdong 510630, P.R. China.,Organ Transplantation Institute, Sun Yat-Sen University, Guangzhou, Guangdong 510080, P.R. China
| | - Wen-Jie Chen
- Department of Biological Treatment Center, The Third Affiliated Hospital, Sun Yat-Sen University, Guangzhou, Guangdong 510630, P.R. China
| | - Chi Xu
- Department of Hepatic Surgery and Liver Transplantation Center, The Third Affiliated Hospital, Sun Yat-Sen University, Guangzhou, Guangdong 510630, P.R. China.,Organ Transplantation Institute, Sun Yat-Sen University, Guangzhou, Guangdong 510080, P.R. China
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Aussilhou B, Dokmak S, Dondero F, Joly D, Durand F, Soubrane O, Belghiti J. Treatment of polycystic liver disease. Update on the management. J Visc Surg 2018; 155:471-481. [DOI: 10.1016/j.jviscsurg.2018.07.004] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
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Hadžić N, Strazzabosco M. Fibropolycystic Liver Diseases and Congenital Biliary Abnormalities. SHERLOCK'S DISEASES OF THE LIVER AND BILIARY SYSTEM 2018:308-327. [DOI: 10.1002/9781119237662.ch16] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 09/02/2023]
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11
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Rajoriya N, Tripathi D, Leithead JA, Gunson BK, Lord S, Ferguson JW, Hirschfield GM. Portal hypertension in polycystic liver disease patients does not affect wait-list or immediate post-liver transplantation outcomes. World J Gastroenterol 2016; 22:9966-9973. [PMID: 28018103 PMCID: PMC5143763 DOI: 10.3748/wjg.v22.i45.9966] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/30/2016] [Revised: 09/28/2016] [Accepted: 11/16/2016] [Indexed: 02/06/2023] Open
Abstract
AIM To establish the impact of portal hypertension (PH) on wait-list/post-transplant outcomes in patients with polycystic liver disease (PCLD) listed for liver transplantation.
METHODS A retrospective single-centre case controlled study of consecutive patients listed for liver transplantation over 12 years was performed from our centre. PH in the PCLD cohort was defined by the one or more of following parameters: (1) presence of radiological or endoscopic documented varices from our own centre or the referral centre; (2) splenomegaly (> 11 cm) on radiology in absence of splenic cysts accounting for increased imaging size; (3) thrombocytopenia (platelets < 150 × 109/L); or (4) ascites without radiological evidence of hepatic venous outflow obstruction from a single cyst.
RESULTS Forty-seven PCLD patients (F: M = 42: 5) were listed for liver transplantation (LT) (single organ, n = 35; combined liver-kidney transplantation, n = 12) with 19 patients (40.4%) having PH. When comparing the PH group with non-PH group, the mean listing age (PH group, 50.6 (6.4); non-PH group, 47.1 (7.4) years; P = 0.101), median listing MELD (PH group, 12; non-PH group, 11; P = 0.422) median listing UKELD score (PH group, 48; non-PH group, 46; P = 0.344) and need for renal replacement therapy (P = 0.317) were similar. In the patients who underwent LT alone, there was no difference in the duration of ICU stay (PH, 3 d; non-PH, 2 d; P = 0.188), hospital stay length (PH, 9 d; non-PH, 10 d; P = 0.973), or frequency of renal replacement therapy (PH, 2/8; non-PH, 1/14; P = 0.121) in the immediate post-transplantation period.
CONCLUSION Clinically apparent portal hypertension in patients with PCLD listed for liver transplantation does not appear to have a major impact on wait-list or peri-transplant morbidity.
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Gu JY, Lu TF, Li QG, Zhang JJ, Xia L, Xia Q. Adult polycystic liver disease concomitant with severe anemia due to intracystic bleeding is an indication for liver transplantation: case report and review of literature. J Dig Dis 2016; 17:408-4. [PMID: 26945526 DOI: 10.1111/1751-2980.12335] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/18/2015] [Revised: 02/18/2016] [Accepted: 03/02/2016] [Indexed: 12/11/2022]
Affiliation(s)
- Jin Yang Gu
- Department of Liver Surgery, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China
| | - Tian Fei Lu
- Department of Liver Surgery, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China
| | - Qi Gen Li
- Department of Liver Surgery, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China
| | - Jian Jun Zhang
- Department of Liver Surgery, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China
| | - Lei Xia
- Department of Liver Surgery, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China
| | - Qiang Xia
- Department of Liver Surgery, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China
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Reddy SS, Civan JM. From Child-Pugh to Model for End-Stage Liver Disease: Deciding Who Needs a Liver Transplant. Med Clin North Am 2016; 100:449-64. [PMID: 27095638 DOI: 10.1016/j.mcna.2015.12.002] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
This article reviews the historical evolution of the liver transplant organ allocation policy and the indications/contraindications for liver transplant, and provides an overview of the liver transplant evaluation process. The article is intended to help internists determine whether and when referral to a liver transplant center is indicated, and to help internists to counsel patients whose initial evaluation at a transplant center is pending.
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Affiliation(s)
- Sheela S Reddy
- Division of Gastroenterology & Hepatology, Department of Medicine, Thomas Jefferson University, Suite 480 Main Building, 132 South 10th Street, Philadelphia, PA 19107, USA
| | - Jesse M Civan
- Division of Gastroenterology & Hepatology, Department of Medicine, Thomas Jefferson University, Suite 480 Main Building, 132 South 10th Street, Philadelphia, PA 19107, USA.
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14
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15
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Temmerman F, Dobbels F, Ho TA, Pirson Y, Vanslembrouck R, Coudyzer W, Bammens B, van Pelt J, Pirenne J, Nevens F. Development and validation of a polycystic liver disease complaint-specific assessment (POLCA). J Hepatol 2014; 61:1143-50. [PMID: 24996047 DOI: 10.1016/j.jhep.2014.06.024] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/17/2013] [Revised: 06/24/2014] [Accepted: 06/24/2014] [Indexed: 02/07/2023]
Abstract
BACKGROUND & AIMS Polycystic liver disease (PCLD) may lead to extensive hepatomegaly and invalidating complaints. Therapeutic decisions, including somatostatin-analogues (SAs) and (non)-transplant surgery are besides the existence of hepatomegaly, also guided by the severity of complaints. We developed and validated a self-report instrument to capture the presence and severity of disease specific complaints for PCLD. METHODS The study population consisted of 129 patients. Items for the PCLD-complaint-specific assessment (POLCA) were developed based on the chart review of symptomatic PCLD patients (n=68) and literature, and discussed during expert-consensus-meetings. 61 patients who needed therapy were asked to complete the POLCA and the short form health survey version 2 (SF36V2) at baseline and after 6 months of SA-treatment. CT-scans were used to calculate liver volumes (LV). Factor analysis was conducted to identify subscales and remove suboptimal items. Reliability was assessed by Cronbach's alpha. Convergent, criterion validity and responsiveness were tested using prespecified hypotheses. RESULTS In the validation group (n=61), 47 received lanreotide (LAN) and 14 were offered LAN as bridge to liver transplantation (LTx). Factor analysis identified four subscales, which correlated with the physical component summary (PCS). Baseline POLCA scores were significantly higher in LTx-listed patients. In contrast to SF36V2, POLCA-paired observations in 47 patients demonstrated that 2 subscales were lowered significantly and 2 borderline. LV reduction of ⩾ 120 ml resulted in a numerical, more pronounced relative decrease of all scores. CONCLUSIONS In contrast to SF36V2, the POLCA shows good validity and responsiveness to measure complaint severity in PCLD.
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Affiliation(s)
- Frederik Temmerman
- Division and Laboratory of Hepatology, University Hospitals, KU Leuven, Belgium.
| | - Fabienne Dobbels
- Division of Public Health and Primary Care, University Hospitals, KU Leuven, Belgium
| | - Thien Anh Ho
- Division of Nephrology, Université Catholique de Louvain, Brussels, Belgium
| | - Yves Pirson
- Division of Nephrology, Université Catholique de Louvain, Brussels, Belgium
| | | | - Walter Coudyzer
- Division of Radiology, University Hospitals, KU Leuven, Belgium
| | - Bert Bammens
- Division of Nephrology, University Hospitals, KU Leuven, Belgium
| | - Jos van Pelt
- Division and Laboratory of Hepatology, University Hospitals, KU Leuven, Belgium
| | - Jacques Pirenne
- Division of Abdominal Transplant Surgery, University Hospitals, KU Leuven, Belgium
| | - Frederik Nevens
- Division and Laboratory of Hepatology, University Hospitals, KU Leuven, Belgium
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16
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Baber JT, Hiatt JR, Busuttil RW, Agopian VG. A 20-Year Experience with Liver Transplantation for Polycystic Liver Disease: Does Previous Palliative Surgical Intervention Affect Outcomes? J Am Coll Surg 2014; 219:695-703. [DOI: 10.1016/j.jamcollsurg.2014.03.058] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/07/2014] [Revised: 03/22/2014] [Accepted: 03/31/2014] [Indexed: 01/02/2023]
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Ramírez de la Piscina P, Duca I, Estrada S, Calderón R, Ganchegui I, Campos A, Spicakova K, Urtasun L, Salvador M, Delgado E, Bengoa R, García-Campos F. Combined liver and kidney transplant in a patient with budd-Chiari syndrome secondary to autosomal dominant polycystic kidney disease associated with polycystic liver disease: report of a case with a 9-year follow-up. Case Rep Gastrointest Med 2014; 2014:585291. [PMID: 24987537 PMCID: PMC4058590 DOI: 10.1155/2014/585291] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/23/2014] [Revised: 05/03/2014] [Accepted: 05/15/2014] [Indexed: 12/31/2022] Open
Abstract
Polycystic liver disease (PLD) is a hereditary disease inherited by autosomal dominant trait that occurs as a frequent extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). We report a case of a 59-year-old woman diagnosed with ADPKD associated with PLD. End-stage chronic renal failure with a secondary Budd-Chiari syndrome developed during the patient's clinical course. She underwent combined liver and kidney transplantation, with a successful response over a 9-year follow-up period.
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Affiliation(s)
- Patricia Ramírez de la Piscina
- Department of Gastroenterology, Hospital Txagorritxu, Universidad del País Vasco, C/ José Achotegui s/n, Vitoria-Gasteiz, 01009 Álava, Spain
| | - Ileana Duca
- Department of Gastroenterology, Hospital Txagorritxu, Universidad del País Vasco, C/ José Achotegui s/n, Vitoria-Gasteiz, 01009 Álava, Spain
| | - Silvia Estrada
- Department of Gastroenterology, Hospital Txagorritxu, Universidad del País Vasco, C/ José Achotegui s/n, Vitoria-Gasteiz, 01009 Álava, Spain
| | - Rosario Calderón
- Department of Gastroenterology, Hospital Txagorritxu, Universidad del País Vasco, C/ José Achotegui s/n, Vitoria-Gasteiz, 01009 Álava, Spain
| | - Idoia Ganchegui
- Department of Gastroenterology, Hospital Txagorritxu, Universidad del País Vasco, C/ José Achotegui s/n, Vitoria-Gasteiz, 01009 Álava, Spain
| | - Amaia Campos
- Department of Gastroenterology, Hospital Txagorritxu, Universidad del País Vasco, C/ José Achotegui s/n, Vitoria-Gasteiz, 01009 Álava, Spain
| | - Katerina Spicakova
- Department of Gastroenterology, Hospital Txagorritxu, Universidad del País Vasco, C/ José Achotegui s/n, Vitoria-Gasteiz, 01009 Álava, Spain
| | - Leire Urtasun
- Department of Gastroenterology, Hospital Txagorritxu, Universidad del País Vasco, C/ José Achotegui s/n, Vitoria-Gasteiz, 01009 Álava, Spain
| | - Marta Salvador
- Department of Gastroenterology, Hospital Txagorritxu, Universidad del País Vasco, C/ José Achotegui s/n, Vitoria-Gasteiz, 01009 Álava, Spain
| | - Elvira Delgado
- Department of Gastroenterology, Hospital Txagorritxu, Universidad del País Vasco, C/ José Achotegui s/n, Vitoria-Gasteiz, 01009 Álava, Spain
| | - Raquel Bengoa
- Department of Gastroenterology, Hospital Txagorritxu, Universidad del País Vasco, C/ José Achotegui s/n, Vitoria-Gasteiz, 01009 Álava, Spain
| | - Francisco García-Campos
- Department of Gastroenterology, Hospital Txagorritxu, Universidad del País Vasco, C/ José Achotegui s/n, Vitoria-Gasteiz, 01009 Álava, Spain
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Hoshino J, Ubara Y, Suwabe T, Sumida K, Hayami N, Mise K, Hiramatsu R, Hasegawa E, Yamanouchi M, Sawa N, Takei R, Takaichi K. Intravascular embolization therapy in patients with enlarged polycystic liver. Am J Kidney Dis 2014; 63:937-44. [PMID: 24602778 DOI: 10.1053/j.ajkd.2014.01.422] [Citation(s) in RCA: 28] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/14/2013] [Accepted: 01/16/2014] [Indexed: 12/13/2022]
Abstract
BACKGROUND Hepatic transcatheter arterial embolization (TAE) has become an accepted treatment option for patients with symptomatic autosomal dominant polycystic kidney disease (ADPKD) who also have polycystic liver disease and who are not good candidates for surgery. However, indications for TAE and long-term outcome with it are still unclear. STUDY DESIGN Retrospective cohort study. SETTING & PARTICIPANTS Symptomatic patients with ADPKD with polycystic liver disease who underwent hepatic TAE, June 2001 to December 2012, at Toranomon Hospital and whose liver volume data were available were studied (N=244; 56% on dialysis therapy, none with kidney transplants). Mean age was 55 ± 9 (SD) years, and mean liver volumes were 8,353 ± 2,807 and 6,626 ± 2,485 cm(3) in men and women, respectively. Target arteries were embolized from the periphery using platinum microcoils. PREDICTORS Sex-specific quartiles (6,433, 8,142, and 9,574 cm(3) in men and 4,638, 6,078, and 8,181 cm(3) in women) of total liver volume pretreatment. OUTCOMES All causes of mortality were obtained from medical records, followed up until July 31, 2013. MEASUREMENTS Laboratory values were measured before TAE and 1, 3, 6, and 12 months after. Organ volumes were measured pretreatment, then 6 and 12 months after, by summing the products of the organ areas traced in each computed tomographic image. RESULTS Liver/cyst volume decreased to 94.7% (95% CI, 93.5%-95.8%) at 6 months and 90.8% (95% CI, 88.7%-92.9%) at 12 months of pretreatment volumes. Serum protein and hematocrit values improved significantly without liver damage. Survival was significantly better for patients with liver volume ≤ 9,574 cm(3) (men) and ≤ 8,181 cm(3) (women) than for those with larger livers (5-year survival, 69% and 48%; P=0.02). Infection and liver failure caused most deaths, especially in patients with larger livers. LIMITATIONS Referral bias and lack of control group. CONCLUSIONS Hepatic TAE appears to be a safe and less invasive option for patients with symptomatic polycystic liver, especially those contraindicated for surgical treatment (eg, with malnutrition or on dialysis therapy), improving both hepatic volume and nutrition.
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Affiliation(s)
| | - Yoshifumi Ubara
- Nephrology Center, Toranomon Hospital, Tokyo, Japan; Okinaka Memorial Institute, Tokyo, Japan
| | | | | | | | - Koki Mise
- Nephrology Center, Toranomon Hospital, Tokyo, Japan
| | | | | | | | - Naoki Sawa
- Nephrology Center, Toranomon Hospital, Tokyo, Japan
| | - Ryoji Takei
- Department of Radiology, Touzan Hospital, Tokyo, Japan
| | - Kenmei Takaichi
- Nephrology Center, Toranomon Hospital, Tokyo, Japan; Okinaka Memorial Institute, Tokyo, Japan
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Arredondo J, Rotellar F, Herrero I, Pedano N, Martí P, Zozaya G, Bellver M, Pardo F. Trasplante ortotópico de hígado en la poliquistosis hepática. Cir Esp 2013; 91:659-63. [PMID: 23453022 DOI: 10.1016/j.ciresp.2012.11.010] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/14/2012] [Revised: 11/06/2012] [Accepted: 11/22/2012] [Indexed: 02/08/2023]
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Abu-Wasel B, Walsh C, Keough V, Molinari M. Pathophysiology, epidemiology, classification and treatment options for polycystic liver diseases. World J Gastroenterol 2013; 19:5775-5786. [PMID: 24124322 PMCID: PMC3793132 DOI: 10.3748/wjg.v19.i35.5775] [Citation(s) in RCA: 52] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/05/2013] [Revised: 05/21/2013] [Accepted: 07/11/2013] [Indexed: 02/06/2023] Open
Abstract
Polycystic liver diseases (PLD) represent a group of genetic disorders in which cysts occur in the liver (autosomal dominant polycystic liver disease) or in combination with cysts in the kidneys (autosomal dominant polycystic kidney disease). Regardless of the genetic mutations, the natural history of these disorders is alike. The natural history of PLD is characterized by a continuous increase in the volume and the number of cysts. Both genders are affected; however, women have a higher prevalence. Most patients with PLD are asymptomatic and can be managed conservatively. Severe symptoms can affect 20% of patients who develop massive hepatomegaly with compression of the surrounding organs. Rrarely, patients with PLD suffer from acute complications caused by the torsion of hepatic cysts, intraluminal cystic hemorrhage and infections. The most common methods for the diagnosis of PLD are cross sectional imaging studies. Abdominal ultrasound and computerized tomography are the two most frequently used investigations. Magnetic resonance imaging is more sensitive and specific, and it is a valuable test for patients with intravenous contrast allergies or renal dysfunction. Different treatment modalities are available to physicians caring for these patients. Medical treatment has been ineffective. Percutaneous sclerotherapy, trans-arterial embolization, cyst fenestration, hepatic resection and liver transplantation are indicated to specific groups of patients and have to be tailored according to the extent of disease. This review outlines the current knowledge of the pathophysiology, clinical course, diagnosis and treatment strategies of PLD.
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21
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Garcea G, Rajesh A, Dennison AR. Surgical management of cystic lesions in the liver. ANZ J Surg 2013; 83:516-22. [PMID: 23316726 DOI: 10.1111/ans.12049] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/16/2012] [Indexed: 12/14/2022]
Abstract
BACKGROUND Liver cysts are common, occurring in up to 5% of the population. For many types of cysts, a variety of different treatment options exist and the preferred management is unclear. METHODS A PubMed and Medline literature review was undertaken and articles pertaining to the diagnosis and management of cystic lesions within the liver were retrieved. RESULTS Therapy for symptomatic cysts may incorporate aspiration with sclerotherapy or de-roofing. Polycystic liver disease presents a unique management problem because of the high morbidity and mortality rates from intervention and high rates of recurrence. Careful patient counselling and assessment of symptom index is essential before embarking on any treatment. New medical treatments may ameliorate symptoms. Acquired cystic lesions in the liver require a thorough work-up to fully characterize the abnormality and direct appropriate treatment. Hydatid cysts are best treated by chemotherapy, followed by some form of surgical intervention (either aspiration and sclerotherapy or surgery). Liver abscesses can be effectively treated by aspiration or drainage. CONCLUSION All patients with cystic lesions in the liver require discussion at multidisciplinary meetings to confirm the diagnosis and determine the most appropriate method of treatment.
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Affiliation(s)
- Giuseppe Garcea
- Department of Hepatobiliary and Pancreatic Surgery, University Hospitals of Leicester, Leicester, UK.
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22
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Abstract
BACKGROUND Liver cysts are common, occurring in up to 5% of the population. For many types of cysts, a variety of different treatment options exist and the preferred management is unclear. METHODS A Pubmed and Medline literature review using key words non-parasitic hepatic cysts, polycystic liver disease, echinococcus, hydatid cysts parasitic cysts, Caroli's disease, cystadenoma; liver abscess, surgery, aspiration and treatment was undertaken and papers pertaining to the diagnosis and management of cystic lesions within the liver were retrieved. RESULTS Asymptomatic simple cysts in the liver require no treatment. Therapy for symptomatic cysts may incorporate aspiration with sclerotherapy or de-roofing. At present, insufficient evidence exists to recommend one over the other. Polycystic liver disease presents a unique management problem because of high morbidity and mortality rates from intervention and high rates of recurrence. Careful patient counselling and assessment of symptom index is essential before embarking on any treatment. New medical treatments may ameliorate symptoms. Acquired cystic lesions in the liver require a thorough work-up to fully characterize the abnormality and direct appropriate treatment. Hydatid cysts are best treated by chemotherapy followed by some form of surgical intervention (either aspiration and sclerotherapy or surgery). Liver abscesses can effectively be treated by aspiration or drainage. With improved antimicrobial efficacy, prolonged treatment with antibiotics may also be considered. CONCLUSION All patients with cystic lesions in the liver require discussion at multi-disciplinary meetings to confirm and the diagnosis and determine the most appropriate method of treatment.
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Affiliation(s)
- Giuseppe Garcea
- Department of Hepatobiliary and Pancreatic Surgery, University Hospitals of Leicester, Leicester, UK.
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23
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Ramia JM, de La Plaza R, Figueras J, García-Parreño J. [Benign non-parasitic hepatic cystic tumours]. Cir Esp 2011; 89:565-73. [PMID: 21723544 DOI: 10.1016/j.ciresp.2011.05.002] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/09/2011] [Revised: 05/03/2011] [Accepted: 05/03/2011] [Indexed: 02/07/2023]
Abstract
Hepatic cystic tumours are a heterogeneous group of diseases with different aetiology and incidence, and with similar clinical signs and symptoms. They are classified as congenital, traumatic, parasitic, or neoplastic cysts. The congenital cystic tumours are the most prevalent, and include the simple cyst and polycystic hepatic disease. Other less common lesions are, hepatic cystadenoma, ciliated embryonic cyst, and a miscellaneous group. We have carried out a review of all benign non-parasitic hepatic cystic tumours, placing special emphasis on therapeutic strategies.
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Affiliation(s)
- José Manuel Ramia
- Unidad de Cirugía Hepatobiliopancreática, Servicio de Cirugía General y Aparato Digestivo, Hospital Universitario de Guadalajara, Guadalajara, Spain.
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24
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van Keimpema L, Nevens F, Adam R, Porte RJ, Fikatas P, Becker T, Kirkegaard P, Metselaar HJ, Drenth JPH. Excellent survival after liver transplantation for isolated polycystic liver disease: an European Liver Transplant Registry study. Transpl Int 2011; 24:1239-45. [PMID: 21955068 DOI: 10.1111/j.1432-2277.2011.01360.x] [Citation(s) in RCA: 48] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
Patients with end-stage isolated polycystic liver disease (PCLD) suffer from incapacitating symptoms because of very large liver volumes. Liver transplantation (LT) is the only curative option. This study assesses the feasibility of LT in PCLD. We used the European Liver Transplant Registry (ELTR) database to extract demographics and outcomes of 58 PCLD patients. We used Kaplan-Meier survival analysis for survival rates. Severe abdominal pain (75%) was the most prominent symptom, while portal hypertension (35%) was the most common complication in PCLD. The explantation of the polycystic liver was extremely difficult in 38% of patients, because of presence of adhesions from prior therapy (17%). Karnofsky score following LT was 90%. The 1- and 5-year graft survival rate was 94.3% and 87.5%, while patient survival rate was 94.8% and 92.3%, respectively. Survival rates after LT for PCLD are good.
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Affiliation(s)
- Loes van Keimpema
- Department of Gastroenterology and Hepatology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands
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Aussilhou B, Douflé G, Hubert C, Francoz C, Paugam C, Paradis V, Farges O, Vilgrain V, Durand F, Belghiti J. Extended Liver Resection for Polycystic Liver Disease Can Challenge Liver Transplantation. Ann Surg 2010; 252:735-43. [DOI: 10.1097/sla.0b013e3181fb8dc4] [Citation(s) in RCA: 49] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
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Taner B, Willingham DL, Hewitt WR, Grewal HP, Nguyen JH, Hughes CB. Polycystic liver disease and liver transplantation: single-institution experience. Transplant Proc 2010; 41:3769-71. [PMID: 19917384 DOI: 10.1016/j.transproceed.2009.05.043] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/30/2008] [Accepted: 05/04/2009] [Indexed: 12/17/2022]
Abstract
Adult polycystic liver disease (PLD) can cause massive hepatomegaly leading to pain, caval obstruction, and hemorrhage. Many surgical techniques including aspiration, fenestration, and resection have been used to treat PLD. In addition to substantial morbidity and mortality, conservative surgery may have limited success, and palliation may be temporary. With improved results of liver transplantation, it has become the definitive treatment for PLD. We retrospectively reviewed our experience in patients with PLD between 1998 and 2007. Thirteen patients underwent liver only or liver-kidney transplantation. All surgical procedures were performed with preservation of the recipient inferior vena cava and without venovenous bypass (piggyback technique). Our patients experienced a high rate of perioperative morbidity. However, long-term patient and graft survival were excellent.
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Affiliation(s)
- B Taner
- Department of Transplantation Mayo Clinic Florida, Jacksonville, FL, USA.
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27
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Torres OJM, Farias AMS, Costa MHA, Matias MM, Moreira PCO, Cordeiro GM. [Laparoscopic treatment of liver cysts]. Rev Col Bras Cir 2010; 36:493-7. [PMID: 20140392 DOI: 10.1590/s0100-69912009000600006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/09/2008] [Accepted: 02/18/2009] [Indexed: 11/22/2022] Open
Abstract
OBJECTIVE The authors present their experience with the laparoscopic management of non-parasitic liver cysts. METHODS During the period from May 2003 to August 2006, thirteen patients with non-parasitic liver cysts underwent laparoscopic fenestration. Eleven patients were female (84.6%) and two (15.4%) were male. The average age at diagnosis was 48.3 years (range 35-72). The majority of patients presented with pain, discomfort, dyspepsia, or early satiety at diagnosis. Computed tomography scans were performed in all cases. The cysts were solitary in eleven patients (84.6%) and polycystic liver disease in two patients (15.4%). The average size of the solitary cysts was 11.3 cm (range 9.5-17 cm), and the polycystic liver disease was 10.6 cm (range 9.2-12.1 cm). Operation was indicated due to symptoms in all patients. The technique performed was laparoscopic cyst fenestration. RESULTS Laparoscopic fenestration was completed in all patients. Mean operative time was 85 minutes (range 53-110). Intraoperative complications were not detected and there was no death. Postoperative complications were observed in two patients (15.4%). Bile leakage was present in one patient, and ascites in another patient. Both cases were treated conservatively. The average hospital stay was 3.5 days (range 2-9 days). Mean follow-up was 36 months. There was one asymptomatic recurrence (7.6%) in a patient with polycystic liver disease after two years of follow-up. CONCLUSION Laparoscopic fenestration is the preferred method of treatment for non-parasitic liver cyst. This operation causes low morbidity and the recurrence is uncommon. Adequate selection of patients and the laparoscopic technique are important for the treatment.
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Schwartz ME, Roayaie S, Konstadoulakis MM, Gomatos IP, Miller CM. The Mount Sinai experience with orthotopic liver transplantation for benign tumors: brief report and literature review: case reports. Transplant Proc 2008; 40:1759-62. [PMID: 18589189 DOI: 10.1016/j.transproceed.2008.02.076] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/03/2007] [Accepted: 02/26/2008] [Indexed: 02/09/2023]
Abstract
Orthotopic liver transplantation (OLT) is performed for benign hepatic lesions that are symptomatic, too large to be resected, have a malignant transformation potential, cause debilitating/life-threatening manifestations, or in patients experiencing posthepatectomy acute liver failure. Among benign tumors, polycystic liver disease (PLD) is the most common indication for OLT alone, or combined liver-kidney transplantation. Our 10-year experience with OLT for benign tumors includes two patients with PLD and one with a benign giant fibrous tumor. In this report, we present our experience with OLT for benign liver tumors, commenting on relevant published studies.
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Affiliation(s)
- M E Schwartz
- Recanati-Miller Transplantation Institute, Mount Sinai School of Medicine, New York, NY, USA
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Li TJ, Zhang HB, Lu JH, Zhao J, Yang N, Yang GS. Treatment of polycystic liver disease with resection-fenestration and a new classification. World J Gastroenterol 2008; 14:5066-72. [PMID: 18763291 PMCID: PMC2742936 DOI: 10.3748/wjg.14.5066] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
AIM: To evaluate outcomes in patients with autosomal dominant polycyst liver disease (APLD) treated by combined hepatic resection and fenestration. A new classification was recommended to presume postoperative complications and long outcome of patients.
METHODS: Twenty-one patients with APLD were treated by a combined hepatic resection and fenestration technique. All patients were reviewed retrospectively, and clinical symptoms, performance status and morbidity were recorded. A new classification of APLD is recommended here.
RESULTS: All patients were discharged when free of symptoms. The mean follow-up time was 55.7 mo and three patients had a recurrence of symptoms at 81, 68 and 43 mo after operation, respectively. The overall morbidity rate was 76.2%. Two patients with Type B-IIand Type B-I developed biliary leakage. Four patients had severe ascites, including three with Type B-III and one with Type B-II. Nine patients had pleural effusion, including one with Type A-I; one with Type B-I; five with Type B-II; one with Type A-III and one with Type B-III. Three patients with Type B had recurrence of symptoms, while none with Type A had severe complications.
CONCLUSION: Combined hepatic resection and fenestration is an acceptable procedure for treatment of APLD. According to our classification, postoperative complications and long outcome can be predicted before surgery.
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30
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Everson GT, Helmke SM, Doctor B. Advances in management of polycystic liver disease. Expert Rev Gastroenterol Hepatol 2008; 2:563-76. [PMID: 19072404 DOI: 10.1586/17474124.2.4.563] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
The focus of this review is polycystic liver disease, a genetic disorder characterized by multiple macroscopic liver cysts that initially bud from biliary epithelium but subsequently lack communication with the biliary tree. There are two main clinical presentations: polycystic liver associated with autosomal dominant polycystic kidney disease and isolated polycystic liver disease. Both of these forms of polycystic liver disease exhibit an autosomal dominant pattern of inheritance. Clinical manifestations of polycystic liver disease are related to either mass effect of the volume of hepatic cysts or to complications arising within the cysts. Polycystic liver disease rarely progresses to hepatic failure or clinical complications of portal hypertension. Management is directed at counseling patients and families, treating complications and reducing cyst load by surgical techniques: cyst fenestration, hepatic resection or, rarely, hepatic transplantation. Recent research suggests that blockade of cyst secretion or inhibition of epithelial cells might be useful in halting progression of disease--these observations are discussed in this review.
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Affiliation(s)
- Gregory T Everson
- Division of Gastroenterology and Hepatology, Department of Medicine, University of Colorado Health Sciences Center, UCH AOP, PO Box 6510, 1635 N Ursula, B-154, Aurora, CO 80045, USA.
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31
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Moya Herráiz Á, Torres-Quevedo R, Mir Pallardó J. Trasplante hepático en pacientes con lesiones hepáticas benignas. Cir Esp 2008; 84:60-6. [DOI: 10.1016/s0009-739x(08)72135-3] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
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32
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Rossi M, Spoletini G, Bussotti A, Lai Q, Travaglia D, Ferretti S, Poli L, Ginanni Corradini S, Merli M, Novelli G, Mennini G, Pugliese F, Berloco PB. Combined liver-kidney transplantation in polycystic disease: case reports. Transplant Proc 2008; 40:2075-2076. [PMID: 18675135 DOI: 10.1016/j.transproceed.2008.05.058] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
Abstract
Polycystic disease causes a progressive decrease in renal function and liver degeneration. The progression of the disease evolves separately between organs and transplantation options vary: simultaneous or sequential liver-kidney transplantation or single-organ transplantation. From September 2006 to June 2007 3 combined liver kidney transplantations (CLKT) were performed for polycystic disease with end-stage renal disease: 2 with polycystic liver disease, and 1 with hepatic failure due to congenital hepatic fibrosis. The widest dimensions of the polycystic liver of 50 and 60 cm diameter were due to extensive cystic degeneration. We performed 1 simultaneous CLKT and 2 sequential transplantations: 1 liver after kidney, and 1 kidney after liver. At present all patients are alive with 100% graft function. Median creatinine level at discharge was 0.9 mg/dL (ranges, +/-0.2). Good liver graft function was reported in all 3 cases. Transplant benefit in polycystic liver-kidney disease has been already demonstrated; conservative surgical options may result in a high incidence of complications in highly involved polycystic livers. Delaying transplantation results in a more difficult surgical technique, a higher rate of postoperative complications, and a disturbance of optimal graft retrieval because of the worse preoperative condition of the patients.
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Affiliation(s)
- M Rossi
- Chirurgia Generale e Trapianti d'Organo, La Sapienza Università di Roma, Umberto I Policlinico di Roma, Rome, Italy.
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Choice of Transplantation Techniques and Indications for Liver Transplantation in Polycystic Liver Disease in Patients With No Signs of End-Stage Liver Disease. Transplant Proc 2008; 40:1536-8. [DOI: 10.1016/j.transproceed.2008.02.080] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/18/2007] [Accepted: 02/26/2008] [Indexed: 11/23/2022]
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34
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Mekeel KL, Moss AA, Reddy KS, Douglas DD, Vargas HE, Carey EJ, Byrne TJ, Harrison ME, Rakela J, Mulligan DC. Living donor liver transplantation in polycystic liver disease. Liver Transpl 2008; 14:680-3. [PMID: 18433036 DOI: 10.1002/lt.21423] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
In the current Model for End-Stage Liver Disease system, patients with polycystic liver disease (PCLD) who have a poor quality of life secondary to their massive hepatomegaly are no longer competitive for a deceased donor liver transplant if their liver function is well preserved. Traditionally, a caval resection has been advocated in these patients because of the difficulty of the hepatectomy with hepatomegaly, which makes living donation impossible. This series looks at 3 patients who underwent a caval sparing hepatectomy and subsequent living donor liver transplantation (LDLT) for PCLD. Graft and patient survival was 100%, and there were few complications in either donors or recipients. LDLT is an ideal option for patients with PCLD and preserved liver function but poor quality of life.
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Affiliation(s)
- Kristin L Mekeel
- Division of Transplantation, Department of Surgery, Mayo Clinic Arizona, Phoenix, AZ 85054, USA.
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35
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Abstract
The adult forms of polycystic liver disease are characterized by autosomal dominant inheritance and numerous hepatic cysts, with or without renal involvement. Mutations in two distinct genes predispose to renal and liver cysts (PKD1 and PKD2), and mutations in two different genes yield isolated liver cysts (PRKCSH and SEC63). Mutations at certain loci of PKD1 may predispose to more severe renal cystic disease or cerebral aneurysms. Risk factors for severe hepatic cystic disease include aging, female sex, pregnancy, use of exogenous female steroid hormones, degree of renal cystic disease, or severity of renal dysfunction (in patients with mutations in PKD1 or PKD2). Although liver failure or complications of advanced liver disease is rare, some patients develop massive hepatic cystic disease and become clinically symptomatic. There is no effective medical therapy. Treatment options include cyst aspiration and sclerosis, open or laparoscopic cyst fenestration, hepatic resection, and liver transplantation.
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Affiliation(s)
- Gregory T Everson
- Division of Gastroenterology and Hepatology, University of Colorado School of Medicine, 4200 East Ninth Avenue, B-154, Denver, CO 80262, USA.
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36
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Krohn PS, Hillingsø JG, Kirkegaard P. Liver transplantation in polycystic liver disease: a relevant treatment modality for adults? Scand J Gastroenterol 2008; 43:89-94. [PMID: 18938751 DOI: 10.1080/00365520701529360] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
OBJECTIVE Polycystic liver disease (PLD) is a rare, hereditary, benign disorder. Hepatic failure is uncommon and symptoms are caused by mass effects leading to abdominal distension and pain. Liver transplantation (LTX) offers fully curative treatment, but there is still some controversy about whether it is a relevant modality considering the absence of liver failure, relative organ shortage, perioperative risks and lifelong immunosuppression. The purpose of this study was to review our experience of LTX for PLD and to compare the survival with the overall survival of patients who underwent LTX from 1992 to 2005. MATERIAL AND METHODS A retrospective study of the journals of 440 patients, who underwent 506 LTXs between 1992 and 2005, showed that 14 patients underwent LTX for PLD. All patients had normal liver function. Three were receiving haemodialysis and thus underwent combined liver/kidney transplantation. One patient had undergone kidney transplantation 10 years earlier. RESULTS Median follow-up was 55 months. One patient who underwent combined transplantation died after 5.4 months because of multiorgan failure after re-LTX, and one patient, with well-functioning grafts, died of lymphoma after 7 months. At present 12 patients are alive, relieved of symptoms and with good graft function. CONCLUSIONS We conclude that patients treated for PLD by LTX have a good long-term prognosis and excellent relief of symptoms and that LTX might be considered in severe cases of PLD, where conventional surgery is not an option.
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Affiliation(s)
- Paul S Krohn
- Department of Surgery and Transplantation, Rigshospitalet, University of Copenhagen, Denmark
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37
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Abstract
Adult polycystic liver disease (PCLD) is an autosomal dominant condition commonly associated with autosomal dominant polycystic kidney disease (ADPKD). However in the last decade, it has been recognized that there is a distinct form of autosomal dominant PCLD that arises without concomitant ADPKD. Early knowledge of the pathogenesis was gained from the study of hepatic cysts in patients with ADPKD. Bile duct overgrowth after embryogenesis results in cystic hepatic dilatations that are known as biliary microhamartomas or von Meyenburg complexes. Further dilatation arises from cellular proliferation and fluid secretion into these cysts. There is a variable, broad spectrum of manifestations of PCLD. Although PCLD is most often asymptomatic, massive hepatomegaly can lead to disabling symptoms of abdominal pain, early satiety, persistent nausea, dyspnea, ascites, biliary obstruction, and lower body edema. Complications of PCLD include cyst rupture and cyst infection. Also, there are associated medical problems, especially intracranial aneurysms and valvular heart disease, which clinicians need to be aware of and evaluate in patients with PCLD. In asymptomatic patients, no treatment is indicated for PCLD. In the symptomatic patient, surgical therapy is the mainstay of treatment tailored to the extent of disease for each patient. Management options include cyst aspiration and sclerosis, open or laparoscopic fenestration, liver resection with fenestration, and liver transplantation. The surgical literature discussing treatment of PCLD, including techniques, outcomes, and complication rates, are summarized in this review.
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Affiliation(s)
- Robert T Russell
- Vanderbilt University Medical Center, Department of Hepatobiliary Surgery and Liver Transplantation, 1301 22nd Avenue South, Nashville, TN 37232-5545, United States
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38
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Grams J, Teh SH, Torres VE, Andrews JC, Nagorney DM. Inferior vena cava stenting: a safe and effective treatment for intractable ascites in patients with polycystic liver disease. J Gastrointest Surg 2007; 11:985-90. [PMID: 17508255 DOI: 10.1007/s11605-007-0182-3] [Citation(s) in RCA: 22] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
Abstract
We performed a retrospective study of seven patients with polycystic liver disease who underwent stenting of the inferior vena cava for intractable ascites. All patients had symptomatic ascites and inferior vena cava stenosis demonstrable by venography. The mean pressure gradient across the inferior vena cava stenosis before stenting was 14.5 mm Hg (range 6-25 mm Hg) and significantly decreased to a mean pressure gradient of 2.8 mm Hg (range 0-6 mm Hg, p = 0.008) after stenting. Two patients also had stenting of hepatic venous stenoses after unsuccessful inferior vena cava stenting. After a mean follow-up of 12.2 months (range 0.5-39.1 months), five of the seven patients have had maintained clinical improvement, defined as decreased symptoms, diuretic requirements, and frequency of paracentesis. Four patients have required no further intervention. The other patient was lost in follow-up. Patients with clinical improvement had an overall larger mean pressure gradient before stenting (19.2 vs. 9.8 mm Hg) and a larger Delta pressure gradient (15.8 vs. 7.8 mm Hg) compared to those in whom stenting was unsuccessful. These results suggest inferior vena cava stenting is safe and effective and should be considered as a first-line intervention in the treatment of medically intractable ascites in select patients with polycystic liver disease.
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Affiliation(s)
- Jayleen Grams
- Department of Surgery, Mayo Clinic College of Medicine, 200 1st Street SW, Rochester, MN 55905, USA
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39
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Ubara Y. New therapeutic option for autosomal dominant polycystic kidney disease patients with enlarged kidney and liver. Ther Apher Dial 2007; 10:333-41. [PMID: 16911186 DOI: 10.1111/j.1744-9987.2006.00386.x] [Citation(s) in RCA: 31] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
The kidneys of patients with autosomal dominant polycystic kidney disease (ADPKD) usually continue to increase in size even after patients begin dialysis, and mass effects can lead to severe complications. Thus far, renal manifestations of this disorder have been discussed only from the viewpoint of cyst formation or cytogenesis, not from that of vascular abnormality. Because kidneys in ADPKD patients are usually supplied by well-developed arteries, we attempted renal contraction therapy in ADPKD patients with renal transcatheter arterial embolization (TAE) using intravascular coils. Mainly peripheral branches of renal arteries encircling the cysts were embolized. Our treatment has been confirmed to be effective in 266 patients until 2005. Renal size continued to decrease to 53% of the pre-TAE after 1 year. Almost all patients have had improved quality of life and nutritional status. We next tried TAE in 76 intractable patients with symptomatic polycystic liver. We tried to embolize only the hepatic segments replaced by cystic lesions in which the hepatic arteries were well-developed but the intrahepatic portal vein was obstructed. Sixty-four patients have had a good clinical course with this method. Based on our observation of ADPKD through treatment with TAE, we speculate that cyst growth in both the kidney and the liver progresses via the mechanism of 'arteriogenesis' of large vessels as well as 'angiogenesis' of small vessels.
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Affiliation(s)
- Yoshifumi Ubara
- Nephrology Center, Toranomon Hospital, Kajigaya, Kanagawa, Japan.
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40
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NAGORNEY D, SARMIENTO J. Surgical Management of Cystic Disease of the Liver. SURGERY OF THE LIVER, BILIARY TRACT AND PANCREAS 2007:1021-1031. [DOI: 10.1016/b978-1-4160-3256-4.50077-6] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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41
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Arrazola L, Moonka D, Gish RG, Everson GT. Model for end-stage liver disease (MELD) exception for polycystic liver disease. Liver Transpl 2006; 12:S110-1. [PMID: 17123287 DOI: 10.1002/lt.20974] [Citation(s) in RCA: 46] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
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42
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Kirchner GI, Rifai K, Cantz T, Nashan B, Terkamp C, Becker T, Strassburg C, Barg-Hock H, Wagner S, Lück R, Klempnauer J, Manns MP. Outcome and quality of life in patients with polycystic liver disease after liver or combined liver-kidney transplantation. Liver Transpl 2006; 12:1268-77. [PMID: 16741930 DOI: 10.1002/lt.20780] [Citation(s) in RCA: 76] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
In advanced stages of polycystic liver disease, often associated with polycystic kidney disease, a curative therapy is liver or combined liver-kidney transplantation. However, little is known about long-term outcome and quality of life. Between 1990 and 2003, 36 patients (32 female, 4 male) with polycystic liver or combined liver-kidney disease underwent liver (n = 21) or liver-kidney (n = 15) transplantation at our center. Main indications for liver transplantation were cachexia, muscle atrophy, loss of weight, recurrent cyst infections, portal hypertension, and ascites. Apart from clinical parameters, 2 anonymous questionnaires (standard short form 36 and self-designed) addressing quality of life and social status were evaluated. Five patients (14 %) died due to sepsis or myocardial infarction with pneumonia, all within 61 days after transplantation. The follow-up time of the remaining 31 patients ranged from 5 to 156 months, with a mean of 62 months. Of the 23 (74%) answered the questionnaires, 91% of patients felt "much better" or "better," only 9% felt "worse" than before, and 52% of patients participated in sports regularly. Fatigue, physical fitness, loss of appetite, and vomiting improved significantly after transplantation. Physical attractiveness and interest in sex increased as well. Professional occupation did not change for 71% of patients. Family situation before and after transplantation changed in 1 case only. Finally, 78% of patients said they would opt for transplantation again, while 17% were undecided; 1 patient would not repeat transplantation. In conclusion, patients with advanced polycystic liver or polycystic liver-kidney disease have an excellent survival rate and an improved quality of life after liver or combined liver-kidney transplantation.
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Affiliation(s)
- Gabriele I Kirchner
- Gastroenterology, Hepatology, and Endocrinology, Medical School of Hannover, Hannover, Germany.
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43
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Varona JF, Usandizaga I, Pérez Maestu R, Marcos Y Robles J, Lozano F. [77 year old man with jaundice and pruritus]. Rev Clin Esp 2006; 206:197-8. [PMID: 16750093 DOI: 10.1157/13086803] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/25/2023]
Affiliation(s)
- J F Varona
- Servicios de Medicina Interna, Clínica La Luz, Madrid, España
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44
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Neri V, Ambrosi A, Fersini A, Pio Valentino T. Laparoscopic treatment of biliary hepatic cysts: short- and medium-term results. HPB (Oxford) 2006; 8:306-10. [PMID: 18333141 PMCID: PMC2023901 DOI: 10.1080/13651820500465766] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
BACKGROUND The aim of this study was to evaluate the postoperative morbidity and, in the medium-term results, the incidence of relapses in the laparoscopic treatment of non-parasitic hepatic cysts (NPHC) and polycystic liver disease (PCLD). PATIENTS AND METHODS From 1999 to 2003, 12 patients with NPHC and 3 patients with PCLD with few large cysts in the anterior hepatic segments underwent laparoscopic fenestration and deroofing. RESULTS There were no conversions and no mortality; the mean operative time was 55 min for NPHC and 120 min for PCLD. Postoperative morbidity comprised two patients with bronchopneumonic infiltrations and in one patient with PCLD ascites resolved spontaneously. All the patients experienced resolution of the symptomatology. Follow-up was carried out from 3 to 38 months (mean 18 months). There was no relapse of the disease. DISCUSSION The preoperative selection of patients is fundamental to program the surgical treatment. Laparoscopy can be considered a safe and efficacious treatment for NPHC and PCLD.
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Affiliation(s)
- Vincenzo Neri
- Department of Surgical Sciences, Division of General Surgery, Polyclinic of Foggia, University of FoggiaFoggiaItaly
| | - Antonio Ambrosi
- Department of Surgical Sciences, Division of General Surgery, Polyclinic of Foggia, University of FoggiaFoggiaItaly
| | - Alberto Fersini
- Department of Surgical Sciences, Division of General Surgery, Polyclinic of Foggia, University of FoggiaFoggiaItaly
| | - Tiziano Pio Valentino
- Department of Surgical Sciences, Division of General Surgery, Polyclinic of Foggia, University of FoggiaFoggiaItaly
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45
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Abstract
Adult polycystic liver disease (APLD) is an autosomal dominant condition most commonly associated with polycystic kidney disease. However, over the last decade it has come to be recognized that APLD is a genetically heterogeneous disorder involving derangements on at least three different chromosomes. Mutations involving chromosomes 16 and 4 accounting for autosomal dominant polycystic kidney disease (ADPKD) type 1 and type 2 have been well described as have their gene products, polycystin-1 and polycystin-2. These have since been joined by a more recently recognized mutation in the short arm of chromosome 19 thought to be responsible for a much rarer form of autosomal dominant polycystic liver disease without any associated renal involvement. Despite the sometimes impressive physical and radiologic findings, only a minority of patients will progress to advanced liver disease or develop complications as a result of massive hepatomegaly. In these patients, medical management alone has proved ineffectual. Therefore, in the symptomatic APLD patient, surgical therapy remains the mainstay of therapy and includes cyst aspiration and sclerosis, fenestration with and without hepatic resection and orthotopic liver transplantation. The surgical literature on treatment of APLD, to include outcome measurements and complication rates are summarized. Additionally, we review other potential organ involvement and resultant complications.
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Affiliation(s)
- Hays L Arnold
- Gastroenterology Service, Department of Medicine, Brooke Army Medical Center, Fort Sam, Houston, Texas 78234-6200, USA
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46
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Abstract
OBJECTIVES This study describes the natural history of patients with polycystic liver disease, a rare disorder characterized by multiple hepatic cysts. METHODS Cases were identified through review of charts from a hepatology practice, a hepatobiliary surgery practice and a retrospective chart review of inpatient charts from 1990 to 2002. All patients had greater than four simple liver cysts without infectious etiology. Medical records were reviewed for history, physical examination, imaging, and laboratory data. Patients' family practitioners provided follow-up. RESULTS Fifty-three cases (62.3% female, 37.7% male) were identified. The mean age at diagnosis was 56.4 yr. Thirty-eight cases (71.7%) had associated polycystic kidney disease. The minority of patients were symptomatic at diagnosis (pain in 19 (36.5%), dyspnea in 5 (9.6%), and restricted mobility in 5 (9.6%) with hepatomegaly in 23 (45.1%). Follow-up information was attainable for 40 patients with a mean follow-up duration of 4.69 yr (range 0.1-15 yr). Within this subgroup, 9 patients (22.5%) had cyst bleeding, 5 (12.5%) had cyst rupture, 5 (12.5%) had cyst infection, 12 (30%) required an intervention. One patient (2.5%) developed portal hypertension, and two (5%) received a liver transplant. One patient (2.5%) died due to complications from liver cysts. CONCLUSIONS Most patients in this highly selected cohort were asymptomatic with normal hepatic function. Pain was the most common symptom. The natural history is variable however, with some patients developing complications including portal hypertension. Minimally invasive interventions are appropriate initially, with hepatic resection and liver transplantation reserved for those with severe symptoms or life-threatening complications.
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Affiliation(s)
- Lana Bistritz
- Division of Gastroenterology, University of Alberta, Edmonton, Alberta, Canada
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47
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Lerut J, Ciccarelli O, Rutgers M, Orlando G, Mathijs J, Danse E, Goffin E, Gigot JF, Goffette P. Liver transplantation with preservation of the inferior vena cava in case of symptomatic adult polycystic disease. Transpl Int 2005; 18:513-8. [PMID: 15819798 DOI: 10.1111/j.1432-2277.2005.00061.x] [Citation(s) in RCA: 17] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Adult polycystic liver disease (APLD) is a rare disorder of the liver parenchyma, the treatment of which is still controversial. Conservative surgery may have a significant morbidity and is often ineffective in the long run. Liver replacement may be indicated in case of incapacitating hepatomegaly. Patients (one male, five females) undergoing liver transplantation for symptomatic APLD is presented in this study. The particular nature of this series is the fact that successful transplantation was performed in all cases with preservation of the recipient's inferior vena cava and without use of veno-venous bypass despite massive hepatomegaly and previous extensive liver surgery (in three cases). There was minimal morbidity and no mortality. All patients have excellent quality of life with a median follow-up of 41 months (range: 12-58) as testified by a median Karnofsky score of 90% (range: 80-100%).
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Affiliation(s)
- Jan Lerut
- Department of Abdominal transplantation, hepatobiliary and endocrine surgery, Liver Transplant Program, Cliniques Universitaires Saint-Luc, Université catholique de Louvain, Brussels, Belgium.
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48
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Abstract
Congenital cystic lesions of bile ducts may affect intra or extrahepatic bile ducts. Intrahepatic lesions include five entities: congenital hepatic fibrosis, Caroli's syndrome, von Meyenburg complexes, simple cyst of the liver and polycystic liver disease. Congenital hepatic fibrosis and von Meyenburg complexes are secondary to ductal plate malformation affecting the smallest intrahepatic bile ducts. Cystic dilatations are of small size and only detected at histological examination of the liver. They have few clinical consequences. In congenital hepatic fibrosis, the main manifestations result from portal hypertension. Caroli's syndrome is secondary to ductal plate malformation affecting the largest intrahepatic bile ducts. Cystic dilatations are macroscopic and responsible for cholangitis and may lead to biliary stones and carcinoma which develop within cystic dilatations. Caroli's syndrome may be or not associated with congenital hepatic fibrosis. In case of associated congenital hepatic fibrosis, portal hypertension is present. Simple cyst of the liver and polycystic liver disease are characterized by cystic dilatations which, by contrast to the preceding entities, do not communicate with the rest of biliary tree. As a result, they have only few clinical consequences. In congenital hepatic fibrosis and polycystic liver disease, renal abnormalities are frequently observed. They correspond to renal malformations associated with biliary malformations. In congenital hepatic fibrosis, renal lesions are characterized by ectatic collecting tubules which are present in two thirds of the cases and transmitted as an autosomal recessive trait. In polycystic liver disease, renal lesions are characterized by polycystic disease which is present in half of the cases and transmitted as an autosomal dominant trait. Congenital cystic lesions of extrahepatic bile ducts consist of choledochal cyst, which is secondary to malformation of the pancreato-biliary ductal junction. The major risk of choledochal cyst is the development of intracystic cancer, the prevention of which is total surgical resection of the cyst.
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Affiliation(s)
- Daniel Dhumeaux
- Service d'Hépatologie et de Gastroentérologie, Hôpital Henri Mondor, Assistance Publique-Hôpitaux de Paris, Université Paris XII, Créteil.
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49
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Robinson TN, Stiegmann GV, Everson GT. Laparoscopic palliation of polycystic liver disease. Surg Endosc 2004; 19:130-2. [PMID: 15531969 DOI: 10.1007/s00464-004-8813-1] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/09/2004] [Accepted: 06/23/2004] [Indexed: 12/17/2022]
Abstract
The role of laparoscopic surgery in the management of polycystic liver disease (PCLD) is not well defined. The authors hypothesized that laparoscopic fenestration for PCLD relieves symptoms caused by polycystic liver disease. In this study, 11 patients underwent 20 laparoscopic cyst fenestration operations as treatment for symptoms of their PCLD. Symptoms leading to surgery were pain and pressure in 15 (75%) and early satiety in 12 (60%) patients. The median hospital stay was 1 day. The symptoms resolved postoperatively in all the patients. An additional laparoscopic fenestration was required in six (55%) patients for recurrent symptoms. The average time to reoperation was 22 +/- 16 months. Two patients required hepatic transplantation. Initial symptom resolution occurred in all the patients undergoing redo fenestration. The authors conclude that laparoscopic fenestration for PCLD is safe, results in minimal "down" time and relieves the symptoms caused by PCLD. Symptomatic relief usually is temporary, and repeat surgery is required for recurring symptoms in half of the patients.
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Affiliation(s)
- T N Robinson
- Department of Surgery, University of Colorado Health Sciences Center, 4200 East Ninth Avenue, C313, Denver, CO 80262, USA
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50
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Abstract
Autosomal dominant polycystic disease is genetically heterogeneous with mutations in two distinct genes predisposing to the combination of renal and liver cysts (AD-PKD1 and AD-PKD2) and mutations in a third gene yielding isolated liver cysts (the polycystic liver disease gene). Transcription and translation of the PKD1 gene produces polycystin-1, an integral membrane protein that may serve as an extracellular receptor. Mutations occur throughout the PKD1 gene, but more severe disease is associated with N-terminal mutations. The PKD2 gene product, polycystin-2, is an integral membrane protein with molecular characteristics of a calcium-permeant cation channel. Mutations occur throughout the PKD2 gene, and severity of disease may vary with site of mutation in PKD2 and the functional consequence on the resultant polycystin-2 protein. Polycystic liver disease is genetically linked to protein kinase C substrate 80K-H (PRKCSH). The PRKCSH gene encodes hepatocystin, a protein that moderates glycosylation and fibroblast growth factor receptor signaling. More prominent in women, hepatic cysts emerge after the onset of puberty and dramatically increase in number and size through the child-bearing years of early and middle adult life. Although liver failure or complications of advanced liver disease are rare, some patients develop massive hepatic cystic disease and become clinically symptomatic. There is no effective medical therapy. Interventional and surgical options include cyst aspiration and sclerosis, open or laparoscopic cyst fenestration, hepatic resection, and liver transplantation.
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Affiliation(s)
- Gregory T Everson
- Division of Gastroenterology & Hepatology, University of Colorado School of Medicine, Denver, CO 80262, USA.
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