Skip to main content
PMC home page
Annals of Surgery logoLink to Annals of Surgery
. 1997 Mar;225(3):286–294. doi: 10.1097/00000658-199703000-00008

Adult polycystic liver disease: is fenestration the most adequate operation for long-term management?

J F Gigot 1, P Jadoul 1, F Que 1, B E Van Beers 1, J Etienne 1, Y Horsmans 1, A Collard 1, A Geubel 1, J Pringot 1, P J Kestens 1
PMCID: PMC1190679  PMID: 9060585

Abstract

OBJECTIVE: The aim of this study was to evaluate the immediate and long-term results in a retrospective series of patients with highly symptomatic adult polycystic liver disease (APLD) treated by extensive fenestration techniques. A classification of APLD was developed as a stratification scheme to help surgeons conceptualize which operation to offer to patients with APLD. SUMMARY BACKGROUND DATA: Treatment options for APLD remain controversial, with partisans of fenestration techniques or combined liver resection-fenestration. METHODS: Clinical symptoms, performance status, liver volume measurement by computed tomography (CT), and morbidity were recorded before surgery and after surgery. Adult polycystic liver disease was classified according to the number, size, and location of liver cysts and the amount of remaining liver parenchyma. Follow-up was obtained by clinical and CT examinations in all patients. RESULTS: Ten patients with highly symptomatic APLD were operated on using an extensive fenestration technique (by laparotomy in 8 patients and by laparoscopy in 2 patients, 1 of whom conversion to laparotomy was required). The mean preoperative liver volume was 7761 cm3. There was no mortality. Postoperative morbidity occurred in 50%, mainly from biliary complications, requiring reintervention in two cases. Massive intraoperative hemorrhage occurred in one patient. During a mean follow-up time of 71 months (range, 17 to 239 months), all patients were improved clinically according to their estimated performance status. The mean postoperative liver volume was 4596 cm3, which represents a mean liver volume reduction rate of 43%. However, in type III APLD, despite absence of clinical symptoms, a significant increase in liver volume was observed in 40% of the patients. CONCLUSIONS: Extensive fenestration is effective in relieving symptoms in patients with APLD. Hemorrhage and biliary complications are possible consequences of such an aggressive attempt to reduce liver volume. The procedure can be performed laparoscopically in type I APLD. A longer follow-up period is mandatory in type II APLD, to confirm the usefulness of the fenestration procedure. In type III APLD, significant disease progression was observed in 40% of the patients during long-term follow-up. Fenestration may not be the most appropriate operation for long-term management of all types of APLD.

Full text

PDF
286

Images in this article

288Figure 1.
on p.288
290Figure 2.
on p.290

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Abascal J., Moya M., Martin F. Infection of hepatic cysts in polycystic disease. World J Surg. 1984 Jun;8(3):424–425. doi: 10.1007/BF01655097. [DOI] [PubMed] [Google Scholar]
  2. Ambrosetti P., Widmann J. J., Robert J., Rohner A. Syndrome aigu de Budd-Chiari après traitement chirurgical d'une polykystose hépatique. Gastroenterol Clin Biol. 1992;16(11):894–896. [PubMed] [Google Scholar]
  3. Armitage N. C., Blumgart L. H. Partial resection and fenestration in the treatment of polycystic liver disease. Br J Surg. 1984 Mar;71(3):242–244. doi: 10.1002/bjs.1800710331. [DOI] [PubMed] [Google Scholar]
  4. Azizah N., Paradinas F. J. Cholangiocarcinoma coexisting with developmental liver cysts: a distinct entity different from liver cystadenocarcinoma. Histopathology. 1980 Jul;4(4):391–400. doi: 10.1111/j.1365-2559.1980.tb02934.x. [DOI] [PubMed] [Google Scholar]
  5. Bensa P., Haas O., Rat P., Dia A., Favre J. P. Polykystose hépatique. L'intervention de Lin est-elle encore justifiée? Ann Chir. 1989;43(9):720–723. [PubMed] [Google Scholar]
  6. Bhupalan A., Talbot K., Forbes A., Owen M., Samson D., Murray-Lyon I. M. Budd-Chiari syndrome in association with polycystic disease of the liver and kidneys. J R Soc Med. 1992 May;85(5):296–297. doi: 10.1177/014107689208500519. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Bourgeois N., Kinnaert P., Vereerstraeten P., Schoutens A., Toussaint C. Infection of hepatic cysts following kidney transplantation in polycystic disease. World J Surg. 1983 Sep;7(5):629–631. doi: 10.1007/BF01655343. [DOI] [PubMed] [Google Scholar]
  8. CAMPBELL G. S., BICK H. D., VARCO R. L., PAULSEN E. P., LOBER P. H., WATSON C. J. Bleeding esophageal varices with polycystic liver; report of three cases. N Engl J Med. 1958 Nov 6;259(19):904–910. doi: 10.1056/NEJM195811062591903. [DOI] [PubMed] [Google Scholar]
  9. COMFORT M. W., GRAY H. K., DAHLIN D. C., WHITESELL F. B., Jr Polycystic disease of the liver: a study of 24 cases. Gastroenterology. 1952 Jan;20(1):60–78. [PubMed] [Google Scholar]
  10. DelGuercio E., Greco J., Kim K. E., Chinitz J., Swartz C. Esophageal varices in adult patients with polycystic kidney and liver disease. N Engl J Med. 1973 Sep 27;289(13):678–679. doi: 10.1056/NEJM197309272891307. [DOI] [PubMed] [Google Scholar]
  11. Ergün H., Wolf B. H., Hissong S. L. Obstructive jaundice caused by polycystic liver disease. Radiology. 1980 Aug;136(2):435–436. doi: 10.1148/radiology.136.2.7403520. [DOI] [PubMed] [Google Scholar]
  12. Everson G. T., Scherzinger A., Berger-Leff N., Reichen J., Lezotte D., Manco-Johnson M., Gabow P. Polycystic liver disease: quantitation of parenchymal and cyst volumes from computed tomography images and clinical correlates of hepatic cysts. Hepatology. 1988 Nov-Dec;8(6):1627–1634. doi: 10.1002/hep.1840080626. [DOI] [PubMed] [Google Scholar]
  13. FELDMAN M. Polycystic disease of the liver. Am J Gastroenterol. 1958 Jan;29(1):83–86. [PubMed] [Google Scholar]
  14. Farges O., Bismuth H. Fenestration in the management of polycystic liver disease. World J Surg. 1995 Jan-Feb;19(1):25–30. doi: 10.1007/BF00316975. [DOI] [PubMed] [Google Scholar]
  15. Gesundheit N., Kent D. L., Fawcett H. D., Effron M. K., Maffly R. H. Infected liver cyst in a patient with polycystic kidney disease. West J Med. 1982 Mar;136(3):246–249. [PMC free article] [PubMed] [Google Scholar]
  16. Gigot J. F., Legrand M., Hubens G., de Canniere L., Wibin E., Deweer F., Druart M. L., Bertrand C., Devriendt H., Droissart R. Laparoscopic treatment of nonparasitic liver cysts: adequate selection of patients and surgical technique. World J Surg. 1996 Jun;20(5):556–561. doi: 10.1007/s002689900086. [DOI] [PubMed] [Google Scholar]
  17. HENSON S. W., Jr, GRAY H. K., DOCKERTY M. B. Benign tumors of the liver. IV. Polycystic disease of surgical significance. Surg Gynecol Obstet. 1957 Jan;104(1):63–67. [PubMed] [Google Scholar]
  18. Hammerman M. R., Miller S. B. Therapeutic use of growth factors in renal failure. J Am Soc Nephrol. 1994 Jul;5(1):1–11. doi: 10.1681/ASN.V511. [DOI] [PubMed] [Google Scholar]
  19. Heather B., Ashby B. S. Choledochoscopic appearance of hepatic ducts in polycystic disease of the liver. J R Soc Med. 1978 Jul;71(7):526–529. doi: 10.1177/014107687807100714. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Henne-Bruns D., Klomp H. J., Kremer B. Non-parasitic liver cysts and polycystic liver disease: results of surgical treatment. Hepatogastroenterology. 1993 Feb;40(1):1–5. [PubMed] [Google Scholar]
  21. Howard R. J., Hanson R. F., Delaney J. P. Jaundice associated with polycystic liver disease. Relief by surgical decompression of the cysts. Arch Surg. 1976 Jul;111(7):816–817. doi: 10.1001/archsurg.1976.01360250092019. [DOI] [PubMed] [Google Scholar]
  22. Huguet C., Hecht Y., Ricordeau P., Caroli J. Les énormes polykystoses hépatiques. Une indication chirurgicale? Med Chir Dig. 1973;2(4):227–230. [PubMed] [Google Scholar]
  23. Huston J., 3rd, Torres V. E., Sulivan P. P., Offord K. P., Wiebers D. O. Value of magnetic resonance angiography for the detection of intracranial aneurysms in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 1993 Jun;3(12):1871–1877. doi: 10.1681/ASN.V3121871. [DOI] [PubMed] [Google Scholar]
  24. Imamura M., Miyashita T., Tani T., Naito A., Tobe T., Takahashi K. Cholangiocellular carcinoma associated with multiple liver cysts. Am J Gastroenterol. 1984 Oct;79(10):790–795. [PubMed] [Google Scholar]
  25. Iwatsuki S., Starzl T. E. Personal experience with 411 hepatic resections. Ann Surg. 1988 Oct;208(4):421–434. doi: 10.1097/00000658-198810000-00004. [DOI] [PMC free article] [PubMed] [Google Scholar]
  26. Kairaluoma M. I., Leinonen A., Ståhlberg M., Päivänsalo M., Kiviniemi H., Siniluoto T. Percutaneous aspiration and alcohol sclerotherapy for symptomatic hepatic cysts. An alternative to surgical intervention. Ann Surg. 1989 Aug;210(2):208–215. doi: 10.1097/00000658-198908000-00012. [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. Kwok M. K., Lewin K. J. Massive hepatomegaly in adult polycystic liver disease. Am J Surg Pathol. 1988 Apr;12(4):321–324. doi: 10.1097/00000478-198804000-00010. [DOI] [PubMed] [Google Scholar]
  28. Lin T. Y., Chen C. C., Wang S. M. Treatment of non-parasitic cystic disease of the liver: a new approach to therapy with polycystic liver. Ann Surg. 1968 Nov;168(5):921–927. doi: 10.1097/00000658-196811000-00022. [DOI] [PMC free article] [PubMed] [Google Scholar]
  29. Longmire W. P., Jr, Mandiola S. A., Gordon H. E. Congenital cystic disease of the liver and biliary system. Ann Surg. 1971 Oct;174(4):711–726. doi: 10.1097/00000658-197110000-00014. [DOI] [PMC free article] [PubMed] [Google Scholar]
  30. MELNICK P. J. Polycystic liver; analysis of seventy cases. AMA Arch Pathol. 1955 Feb;59(2):162–172. [PubMed] [Google Scholar]
  31. Montorsi M., Torzilli G., Fumagalli U., Bona S., Rostai R., De Simone M., Rovati V., Mosca F., Filice C. Percutaneous alcohol sclerotherapy of simple hepatic cysts. Results from a multicentre survey in Italy. HPB Surg. 1994;8(2):89–94. doi: 10.1155/1994/10372. [DOI] [PMC free article] [PubMed] [Google Scholar]
  32. Morino M., De Giuli M., Festa V., Garrone C. Laparoscopic management of symptomatic nonparasitic cysts of the liver. Indications and results. Ann Surg. 1994 Feb;219(2):157–164. doi: 10.1097/00000658-199402000-00007. [DOI] [PMC free article] [PubMed] [Google Scholar]
  33. Newman K. D., Torres V. E., Rakela J., Nagorney D. M. Treatment of highly symptomatic polycystic liver disease. Preliminary experience with a combined hepatic resection-fenestration procedure. Ann Surg. 1990 Jul;212(1):30–37. doi: 10.1097/00000658-199007000-00005. [DOI] [PMC free article] [PubMed] [Google Scholar]
  34. Obstructive jaundice in a patient with polycystic disease. Am J Med. 1977 Apr;62(4):616–626. doi: 10.1016/0002-9343(77)90425-9. [DOI] [PubMed] [Google Scholar]
  35. Obstructive jaundice in a patient with polycystic disease. Am J Med. 1977 Apr;62(4):616–626. doi: 10.1016/0002-9343(77)90425-9. [DOI] [PubMed] [Google Scholar]
  36. Paliard P., Partensky C. Traitement par fenestration itérative d'une forme douloureuse, puis cholestatique de polykystose hépatique. Intérêt possible de la cimétidine dans la réduction de la sécrétion kystique. Gastroenterol Clin Biol. 1980 Dec;4(12):854–857. [PubMed] [Google Scholar]
  37. Parneix M., Lotte P., Barandon E., Amouretti M. La polykystose hépatique. A propos de 3 formes compliquées. Chirurgie. 1978;104(3):284–294. [PubMed] [Google Scholar]
  38. Peltokallio V. Non-parasitic cysts of the liver. A clinical study of 117 cases. Ann Chir Gynaecol Fenn Suppl. 1970;174:1–63. [PubMed] [Google Scholar]
  39. Que F., Nagorney D. M., Gross J. B., Jr, Torres V. E. Liver resection and cyst fenestration in the treatment of severe polycystic liver disease. Gastroenterology. 1995 Feb;108(2):487–494. doi: 10.1016/0016-5085(95)90078-0. [DOI] [PubMed] [Google Scholar]
  40. ROBSON G. B., FENSTER L. F. FATAL LIVER ABSCESS DEVELOPING IN A POLYCYSTIC LIVER. Gastroenterology. 1964 Jul;47:82–84. [PubMed] [Google Scholar]
  41. Ratcliffe P. J., Reeders S., Theaker J. M. Bleeding oesophageal varices and hepatic dysfunction in adult polycystic kidney disease. Br Med J (Clin Res Ed) 1984 May 5;288(6427):1330–1331. doi: 10.1136/bmj.288.6427.1330. [DOI] [PMC free article] [PubMed] [Google Scholar]
  42. Saini S., Mueller P. R., Ferrucci J. T., Jr, Simeone J. F., Wittenberg J., Butch R. J. Percutaneous aspiration of hepatic cysts does not provide definitive therapy. AJR Am J Roentgenol. 1983 Sep;141(3):559–560. doi: 10.2214/ajr.141.3.559. [DOI] [PubMed] [Google Scholar]
  43. Sanchez H., Gagner M., Rossi R. L., Jenkins R. L., Lewis W. D., Munson J. L., Braasch J. W. Surgical management of nonparasitic cystic liver disease. Am J Surg. 1991 Jan;161(1):113–119. doi: 10.1016/0002-9610(91)90370-s. [DOI] [PubMed] [Google Scholar]
  44. Soravia C., Mentha G., Giostra E., Morel P., Rohner A. Surgery for adult polycystic liver disease. Surgery. 1995 Mar;117(3):272–275. doi: 10.1016/s0039-6060(05)80201-6. [DOI] [PubMed] [Google Scholar]
  45. Starzl T. E., Reyes J., Tzakis A., Mieles L., Todo S., Gordon R. Liver transplantation for polycystic liver disease. Arch Surg. 1990 May;125(5):575–577. doi: 10.1001/archsurg.1990.01410170021003. [DOI] [PubMed] [Google Scholar]
  46. Summerfield J. A., Nagafuchi Y., Sherlock S., Cadafalch J., Scheuer P. J. Hepatobiliary fibropolycystic diseases. A clinical and histological review of 51 patients. J Hepatol. 1986;2(2):141–156. doi: 10.1016/s0168-8278(86)80073-3. [DOI] [PubMed] [Google Scholar]
  47. Taylor J. E., Calne R. Y., Stewart W. K. Massive cystic hepatomegaly in a female patient with polycystic kidney disease treated by combined hepatic and renal transplantation. Q J Med. 1991 Sep;80(293):771–775. [PubMed] [Google Scholar]
  48. Telenti A., Torres V. E., Gross J. B., Jr, Van Scoy R. E., Brown M. L., Hattery R. R. Hepatic cyst infection in autosomal dominant polycystic kidney disease. Mayo Clin Proc. 1990 Jul;65(7):933–942. doi: 10.1016/s0025-6196(12)65154-4. [DOI] [PubMed] [Google Scholar]
  49. Torres V. E., Wiebers D. O., Forbes G. S. Cranial computed tomography and magnetic resonance imaging in autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 1990 Jul;1(1):84–90. [PubMed] [Google Scholar]
  50. Trentalance A., Petroski D. Surgical resection in symptomatic polycystic liver disease. J Med Soc N J. 1983 Jan;80(1):44–46. [PubMed] [Google Scholar]
  51. Turnage R. H., Eckhauser F. E., Knol J. A., Thompson N. W. Therapeutic dilemmas in patients with symptomatic polycystic liver disease. Am Surg. 1988 Jun;54(6):365–372. [PubMed] [Google Scholar]
  52. Vauthey J. N., Maddern G. J., Blumgart L. H. Adult polycystic disease of the liver. Br J Surg. 1991 May;78(5):524–527. doi: 10.1002/bjs.1800780505. [DOI] [PubMed] [Google Scholar]
  53. Vauthey J. N., Maddern G. J., Kolbinger P., Baer H. U., Blumgart L. H. Clinical experience with adult polycystic liver disease. Br J Surg. 1992 Jun;79(6):562–565. doi: 10.1002/bjs.1800790629. [DOI] [PubMed] [Google Scholar]
  54. Wittig J. H., Burns R., Longmire W. P., Jr Jaundice associated with polycystic liver disease. Am J Surg. 1978 Sep;136(3):383–386. doi: 10.1016/0002-9610(78)90300-8. [DOI] [PubMed] [Google Scholar]
  55. Yaqoob M., Saffman C., Finn R., Carty A. T. Inferior vena caval compression by hepatic cysts: an unusual complication of adult polycystic kidney disease. Nephron. 1990;54(1):89–91. doi: 10.1159/000185816. [DOI] [PubMed] [Google Scholar]
  56. van Erpecum K. J., Janssens A. R., Terpstra J. L., Tjon A Tham R. T. Highly symptomatic adult polycystic disease of the liver. A report of fifteen cases. J Hepatol. 1987 Aug;5(1):109–117. doi: 10.1016/s0168-8278(87)80068-5. [DOI] [PubMed] [Google Scholar]

Articles from Annals of Surgery are provided here courtesy of Lippincott, Williams, and Wilkins

RESOURCES