Liver transplantation for adult polycystic liver disease

Kim Swenson, Philip Seu, Milan Kinkhabwala, Melinda Maggard, Paul Martin, John Goss, Ronald Busuttil

Research output: Contribution to journalArticle

86 Citations (Scopus)

Abstract

Polycystic liver disease, commonly associated with polycystic kidney disease, can result in massive hepatomegaly and debilitating symptoms. Surgical intervention for symptomatic polycystic liver disease has been associated with significant morbidity and inconsistent long-term palliation; it is more appropriate in patients with a single dominant cyst or cysts which is/are confined to one lobe. At our institution, nine patients have undergone orthotopic liver transplantation for symptomatic hepatic cysts with excellent long-term results and minimal morbidity and mortality. Surgical candidates were selected based on severe limitations in daily activities and on sequelae of hepatic cystic involvement. Other factors considered were the extent and pattern of hepatic cystic disease, the degree of hepatic and renal dysfunction, and prior surgical intervention. Three patients (33%) required combined liver and kidney transplantation because of renal cystic involvement with renal insufficiency. The one-year survival rate was 89% with excellent symptomatic relief and improved quality of life in all the surviving patients. One death occurred in a significantly malnourished 6-year-old female. Complications included one case each of hepatic artery thrombosis requiring retransplantation, biliary leak necessitating biliary reconstruction, and postoperative bleeding requiring re-exploration. The mean hospital stay was 3 days and the mean intraoperative blood transfusion requirement was 18 units. Our experience demonstrates that appropriately selected patients with extensive hepatic involvement with adult polycystic liver disease can have an excellent outcome with transplantation, with morbidity comparable with other surgical options. palliation (Table 1); those attempts are limited primarily to patients with a single dominant cyst(s) confined to one lobe. In view of the unsatisfactory results with palliative measures and of the encouraging preliminary reports of successful orthotopic liver transplantation (OLT7) for APLD11,12 we have elected to offer OLT for this condition for the past 5 years. We now report our experience involving nine patients with highly symptomatic APLD treated with OLT.

Original languageEnglish (US)
Pages (from-to)412-415
Number of pages4
JournalHepatology
Volume28
Issue number2
DOIs
StatePublished - 1998
Externally publishedYes

Fingerprint

Liver Transplantation
Cysts
Liver
Morbidity
Kidney
Polycystic Kidney Diseases
Hepatomegaly
Hepatic Artery
Polycystic liver disease
Blood Transfusion
Kidney Transplantation
Renal Insufficiency
Length of Stay
Thrombosis
Survival Rate
Transplantation
Quality of Life
Hemorrhage
Mortality

ASJC Scopus subject areas

  • Hepatology

Cite this

Swenson, K., Seu, P., Kinkhabwala, M., Maggard, M., Martin, P., Goss, J., & Busuttil, R. (1998). Liver transplantation for adult polycystic liver disease. Hepatology, 28(2), 412-415. https://doi.org/10.1002/hep.510280218

Liver transplantation for adult polycystic liver disease. / Swenson, Kim; Seu, Philip; Kinkhabwala, Milan; Maggard, Melinda; Martin, Paul; Goss, John; Busuttil, Ronald.

In: Hepatology, Vol. 28, No. 2, 1998, p. 412-415.

Research output: Contribution to journalArticle

Swenson, K, Seu, P, Kinkhabwala, M, Maggard, M, Martin, P, Goss, J & Busuttil, R 1998, 'Liver transplantation for adult polycystic liver disease', Hepatology, vol. 28, no. 2, pp. 412-415. https://doi.org/10.1002/hep.510280218
Swenson, Kim ; Seu, Philip ; Kinkhabwala, Milan ; Maggard, Melinda ; Martin, Paul ; Goss, John ; Busuttil, Ronald. / Liver transplantation for adult polycystic liver disease. In: Hepatology. 1998 ; Vol. 28, No. 2. pp. 412-415.
@article{28043096dc5743ac888ea27963ab5738,
title = "Liver transplantation for adult polycystic liver disease",
abstract = "Polycystic liver disease, commonly associated with polycystic kidney disease, can result in massive hepatomegaly and debilitating symptoms. Surgical intervention for symptomatic polycystic liver disease has been associated with significant morbidity and inconsistent long-term palliation; it is more appropriate in patients with a single dominant cyst or cysts which is/are confined to one lobe. At our institution, nine patients have undergone orthotopic liver transplantation for symptomatic hepatic cysts with excellent long-term results and minimal morbidity and mortality. Surgical candidates were selected based on severe limitations in daily activities and on sequelae of hepatic cystic involvement. Other factors considered were the extent and pattern of hepatic cystic disease, the degree of hepatic and renal dysfunction, and prior surgical intervention. Three patients (33{\%}) required combined liver and kidney transplantation because of renal cystic involvement with renal insufficiency. The one-year survival rate was 89{\%} with excellent symptomatic relief and improved quality of life in all the surviving patients. One death occurred in a significantly malnourished 6-year-old female. Complications included one case each of hepatic artery thrombosis requiring retransplantation, biliary leak necessitating biliary reconstruction, and postoperative bleeding requiring re-exploration. The mean hospital stay was 3 days and the mean intraoperative blood transfusion requirement was 18 units. Our experience demonstrates that appropriately selected patients with extensive hepatic involvement with adult polycystic liver disease can have an excellent outcome with transplantation, with morbidity comparable with other surgical options. palliation (Table 1); those attempts are limited primarily to patients with a single dominant cyst(s) confined to one lobe. In view of the unsatisfactory results with palliative measures and of the encouraging preliminary reports of successful orthotopic liver transplantation (OLT7) for APLD11,12 we have elected to offer OLT for this condition for the past 5 years. We now report our experience involving nine patients with highly symptomatic APLD treated with OLT.",
author = "Kim Swenson and Philip Seu and Milan Kinkhabwala and Melinda Maggard and Paul Martin and John Goss and Ronald Busuttil",
year = "1998",
doi = "10.1002/hep.510280218",
language = "English (US)",
volume = "28",
pages = "412--415",
journal = "Hepatology",
issn = "0270-9139",
publisher = "John Wiley and Sons Ltd",
number = "2",

}

TY - JOUR

T1 - Liver transplantation for adult polycystic liver disease

AU - Swenson, Kim

AU - Seu, Philip

AU - Kinkhabwala, Milan

AU - Maggard, Melinda

AU - Martin, Paul

AU - Goss, John

AU - Busuttil, Ronald

PY - 1998

Y1 - 1998

N2 - Polycystic liver disease, commonly associated with polycystic kidney disease, can result in massive hepatomegaly and debilitating symptoms. Surgical intervention for symptomatic polycystic liver disease has been associated with significant morbidity and inconsistent long-term palliation; it is more appropriate in patients with a single dominant cyst or cysts which is/are confined to one lobe. At our institution, nine patients have undergone orthotopic liver transplantation for symptomatic hepatic cysts with excellent long-term results and minimal morbidity and mortality. Surgical candidates were selected based on severe limitations in daily activities and on sequelae of hepatic cystic involvement. Other factors considered were the extent and pattern of hepatic cystic disease, the degree of hepatic and renal dysfunction, and prior surgical intervention. Three patients (33%) required combined liver and kidney transplantation because of renal cystic involvement with renal insufficiency. The one-year survival rate was 89% with excellent symptomatic relief and improved quality of life in all the surviving patients. One death occurred in a significantly malnourished 6-year-old female. Complications included one case each of hepatic artery thrombosis requiring retransplantation, biliary leak necessitating biliary reconstruction, and postoperative bleeding requiring re-exploration. The mean hospital stay was 3 days and the mean intraoperative blood transfusion requirement was 18 units. Our experience demonstrates that appropriately selected patients with extensive hepatic involvement with adult polycystic liver disease can have an excellent outcome with transplantation, with morbidity comparable with other surgical options. palliation (Table 1); those attempts are limited primarily to patients with a single dominant cyst(s) confined to one lobe. In view of the unsatisfactory results with palliative measures and of the encouraging preliminary reports of successful orthotopic liver transplantation (OLT7) for APLD11,12 we have elected to offer OLT for this condition for the past 5 years. We now report our experience involving nine patients with highly symptomatic APLD treated with OLT.

AB - Polycystic liver disease, commonly associated with polycystic kidney disease, can result in massive hepatomegaly and debilitating symptoms. Surgical intervention for symptomatic polycystic liver disease has been associated with significant morbidity and inconsistent long-term palliation; it is more appropriate in patients with a single dominant cyst or cysts which is/are confined to one lobe. At our institution, nine patients have undergone orthotopic liver transplantation for symptomatic hepatic cysts with excellent long-term results and minimal morbidity and mortality. Surgical candidates were selected based on severe limitations in daily activities and on sequelae of hepatic cystic involvement. Other factors considered were the extent and pattern of hepatic cystic disease, the degree of hepatic and renal dysfunction, and prior surgical intervention. Three patients (33%) required combined liver and kidney transplantation because of renal cystic involvement with renal insufficiency. The one-year survival rate was 89% with excellent symptomatic relief and improved quality of life in all the surviving patients. One death occurred in a significantly malnourished 6-year-old female. Complications included one case each of hepatic artery thrombosis requiring retransplantation, biliary leak necessitating biliary reconstruction, and postoperative bleeding requiring re-exploration. The mean hospital stay was 3 days and the mean intraoperative blood transfusion requirement was 18 units. Our experience demonstrates that appropriately selected patients with extensive hepatic involvement with adult polycystic liver disease can have an excellent outcome with transplantation, with morbidity comparable with other surgical options. palliation (Table 1); those attempts are limited primarily to patients with a single dominant cyst(s) confined to one lobe. In view of the unsatisfactory results with palliative measures and of the encouraging preliminary reports of successful orthotopic liver transplantation (OLT7) for APLD11,12 we have elected to offer OLT for this condition for the past 5 years. We now report our experience involving nine patients with highly symptomatic APLD treated with OLT.

UR - http://www.scopus.com/inward/record.url?scp=0031902105&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0031902105&partnerID=8YFLogxK

U2 - 10.1002/hep.510280218

DO - 10.1002/hep.510280218

M3 - Article

C2 - 9696005

AN - SCOPUS:0031902105

VL - 28

SP - 412

EP - 415

JO - Hepatology

JF - Hepatology

SN - 0270-9139

IS - 2

ER -