Highly symptomatic adult polycystic disease of the liver

Erpecum, Karel J. van; Janssens, A. R.; Terpstra, J. L.; Tham, Richard

doi:10.1016/s0168-8278(87)80068-5

Cited by 73 publications

(30 citation statements)

References 26 publications

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“…Careful assessment of renal function is necessary to determine whether simultaneous cadaveric renal transplantation 8 is also required and to assess the likelihood that renal dysfunction, if present, is likely to result in end-stage renal disease should isolated hepatic transplant be attempted. In our experience, combined OLT/CRT offers both excellent results with symptomatic hepatic involvement and restoration of renal function.…”

Section: Discussionmentioning

confidence: 99%

“…13 Generally, no blood loss was encountered during cyst puncture, but dense adhesions from prior palliative procedures contributed to the difficulty of the dissection and added to the blood loss. The average intraoperative transfusion requirement was 18 U of packed red blood cells, 8 with a range of 5 to 44 U. The mean transfusion requirement for those without a history of prior surgical procedures was 12.6 U, whereas those who underwent operative palliation received an average of 24.5 U. Postoperative maintenance immunosuppression consisted of cyclosporine, azathioprine, and prednisone, or tacrolimus and prednisone.…”

Section: Methodsmentioning

confidence: 99%

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Liver transplantation for adult polycystic liver disease

Seu²,

et al. 1998

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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 62-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 23 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. (HEPATOLOGY 1998;28:412-415.)

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Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Liver transplantation for adult polycystic liver disease

Seu²,

et al. 1998

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“…Fenestration of the cysts yielded a prompt decrease in serum bilirubin from 42 to <5 mg/ml within 10 days postoperatively. Van Erpecum et al [10]reported that the patients had relief of their symptoms, including disappearance of jaundice, as a result, but admitted that the size of the liver was not reduced by fenestration. Farges and Bismuth [5]reported clinical relapse after a few years in 3 of 13 patients who underwent fenestration.…”

Section: Discussionmentioning

confidence: 99%

Successful Left Trisegmentectomy for Polycystic Liver Disease Accompanied by Jaundice

Endo

Tanabe²,

Tanaka³

et al. 2001

Dig Surg

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Background: We report a case of severe polycystic liver disease (PLD) with jaundice in a 57-year-old woman who underwent successful left trisegmentectomy. Method: She was admitted for the first time in February 1992 to our hospital with a 7-year history of PLD, and became jaundiced in June 1995. Because normal liver parenchyma was confirmed mainly to the posterior segment, left trisegmentectomy was performed. Results: No postoperative complication occurred. The serum bilirubin level decreased promptly after the operation, but postoperative endoscopic retrograde cholangiography showed that the root of the posterior hepatic duct remained thin. Thus, the elimination of jaundice was presumed to have been caused by a decrease of intra-abdominal and peripheral biliary pressure, since a large volume of tissue had been removed from the peritoneal cavity. She has since remained well without any symptoms. Conclusion: This procedure is useful for severe PLD, because it can be performed safely and the symptoms disappear dramatically. However, further follow-up is needed to determine the long-term effects of this procedure, because the remaining liver has shown some increase in size.

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“…In patients with long-standing disease, the most frequent symptoms are abdominal pain, early satiety, nausea and vomiting, supine shortness of breath, lower body edema, biliary obstruction, and ascites. These symptoms are usually indicative of significant hepatic enlargement or compression of adjacent structures, but rarely can be from infection or malignant transformation [21,22] . Laboratory studies including bilirubin, hepatic transaminases (AST and ALT), alkaline phosphatase, and gamma glutamyl transferase (GGT) are usually normal in asymptomatic patients.…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Laboratory studies including bilirubin, hepatic transaminases (AST and ALT), alkaline phosphatase, and gamma glutamyl transferase (GGT) are usually normal in asymptomatic patients. In symptomatic patients undergoing evaluation, alkaline phosphatase and GGT may be elevated in up to 47% and 70% of patients respectively [21,[23][24][25] . AST may be elevated in up to 27% of patients and bilirubin levels may be elevated in up to 15% [21,23] .…”

Section: Clinical Presentationmentioning

confidence: 99%