AVANCES EN EL MANEJO DE LA POLIQUISTOSIS HEPÁTICA.

Calixto Duarte-Chang

Submitted November 1, 2024

Published 2026-03-02

Artículos de Investigación

Vol. 25 No. 2 (2012): Revista Médico Científica

PROGRESS IN MANAGEMENT OF POLICYSTIC LIVER DISEASE.

Calixto Duarte-Chang⁺⁻

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DOI: ND

Published: 2026-03-02

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Abstract

Polycystic liver disease is defined by the presence of more than 20 cysts in the hepatic parenchyma. It is an autosomal dominant congenital disorder, which is presented either isolated or as an extrarenal manifestation of autosomal dominant polycystic kidney disease, as it is in nearly 80% of the cases. Clinically, patients have an asymptomatic course and it is diagnosed incidentally, either by abdominal ultrasound or abdominal computerized tomography, which represents the gold standard for diagnosis.The management of this condition has varied over the years. Generally, it is intended for patients who develop self-limited symptoms. Treatment varies from conservative management, consisting of somatostatin analogues; to invasive management, such as radiological procedures, including surgery and liver transplantation.In this review, we propose a summary of the options that we have today in the management of this relatively common condition.

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References

Torres VE, Harris PC. Autosomal dominant polycystic kidney disease: the last 3 years. Kidney Int. 2009; 76: 149–68.
Hoevenaren IA, Wester R, Schrier RW, MacFann K, Doctor RB, DrenthJP, et al. Polycystic liver: clinical characteristics of patients with isolated polycystic liver disease compared with polycystic liver and autosomal dominant polycystic kidney disease. Liver Int. 2007; 28: 264.
Ecder T,Schrier RW. Cardiovascular abnormalities in autosomal-dominant polycystic kidney disease. Nat Rev Nephrol 2009; 5(4): 221-8.
Xu, HW, Yu SQ, Mei CL, Li MH. Screening for intracranial aneurysm in 355 patients with autosomal-dominant polycystic kidney disease. Stroke 2011; 42: 204-6.
van Keimpema L, Nevens F, Vanslembrouck R, van Oijen MG, Hoffmann AL, Dekker HM, de Man RA, Drenth JP. Lanreotide reduces the volume of polycystic liver: a randomized, double-blind, placebo-controlled trial. Gastroenterology 2009; 137(5): 1661–8.
Bistritz L, Tamboli C, Bigam D, Bain VG. Polycystic liver disease: experience at a teaching hospital. Am J Gastroenterol. 2005 Oct; 100(10): 2212-7.
Qian Q, Li A, King BF, Kamath PS, Lager DJ, Huston J 3rd, Shub C, Davila S, Somlo S, Torres VE. Clinical profile of autosomal dominant polycystic liver disease. Hepatology 2003 Jan; 37(1): 164-71.
Torres VE, Harris PC, Pirson Y. Autosomal dominant polycystic kidney disease. Lancet. 2007;369:1287–1301.
Hwang SH, Yu JS, Chung JJ, Kim JH, Kim KW. Diagnosing small hepatic cysts on multidetector CT: an additional merit of thinner coronal reformations. Korean J Radiol. 2011;12:341–350.
Drenth, J. P., Chrispijn, M., Nagorney, D. M., Kamath, P. S. & Torres, V. E. Medical and surgical treatment options for polycystic liver disease. Hepatology 2010; 52(6): 2223-30.
Rusell RT, Pinson CW. Surgical magement of polycystic liver disease. World JGastroenterol. 2007; 13: 5052-9.
van Keimpema L, Ruurda JP, Ernst MF, van Geffen HJ, Drenth JP.Laparoscopic fenestration of liver cysts in polycystic liver disease results in a median volume reduction of 12.5%. JGastrointest. Surg.2008; 12(3): 477-82.
Koperna T, Vogl S, Satzinger U, Schulz F. Nonparasitic Cysts of the Liver: Results and Options of Surgical Treatment. World J Surg.1997; 21: 850-54.
Martin IJ, McKinley, AJ, Currie EJ, Holmes P, Garden OJ. Tailoring the management of nonparasitic liver cysts. AnnSurg. 1998; 228:167–72.
Coelho-Prabhu N, Nagorney DM, Baron TH. ERCP for the treatment of bile leak after partial hepatectomy and fenestration for symptomatic polycystic liver disease. World JGastroenterol. 2012; 18(28): 3705-9.
Temmerman F, Missiaen L, Bammens B, Laleman W, Cassiman D, Verslype C, van Pelt J, Nevens F. Systematic review: the pathophysiology and management of polycystic liver disease. Aliment Pharmacol Ther. 2011; 34:702-13.
van KeimpemaL, Nevens F, Adam R, Porte RJ, Fikatas P, Becker T, Kirkegaard P, Metselaar HJ, Drenth JP.Excellent survival after liver transplantation for isolated polycystic liver disease: an European Liver Transplant Registry study. TransplInt. 2011; 24(12): 1239-45.
van Keimpema L, De Koning DB, Van Hoek B, Van Den Berg AP, Van Oijen MG, De Man RA, Nevens F, Drenth JP. Patients with isolated polycystic liver disease referred to liver centres: clinical characterization of 137 cases. Liver Int. 2011;31(1):92-8.
Takei R, Ubara Y, Hoshino J, Higa Y, Suwabe T, Sogawa Y, Nomura K, Nakanish S, Sawa N, Katori H, etal. Percutaneous transcatheter hepatic artery embolization for liver cysts in autosomal dominant polycystic kidney disease. Am. J. Kidney Dis. 2007; 49(6): 744–52.
Caroli A, Antiga L, Cafaro M, Fasolini G, Remuzzi A, Remuzzi G, Ruggenenti P. Reducing polycystic liver volume in ADPKD: effects of somatostatin analogue octreotide. Clin J Am SocNephrol.2010;5(5): 783–9.
HoganMC, Masyuk TV, Page LJ, Kubly VJ, Bergstralh EJ, Li X, Kim B, King BF, Glockner J, Holmes DR 3rd,et al. Randomized clinical trial of long-acting somatostatin for autosomal dominant polycystic kidney and liver disease. J AmSocNephrol. 2010; 21(6): 1052–61.
Van Keimpema L, Nevens F, Vanslembrouck R, van Oijen MG, Hoffmann AL, Dekker HM, de Man RA, Drenth JP. Lanreotide reduces the volume of polycystic liver: a randomized, double-blind, placebo-controlled trial. Gastroenterology. 2009; 137(5): 1661–8.
Temmerman F, Vanslembrouck R, CoudyzerW, Bammens B, Laleman W, Cassiman D, van Der Merwe S, Verslype C, van Steenbergen W, van Pelt J, Pirson Y, et al. The reduction in liver volume in polycystic liver disease with lanreotide is dose dependent and is most pronounced in patients with the highest liver volume. JHepatol. 2012; 56: suppl 2: S547.
Chrispijn M, Nevens F, Gevers TJG, et al. The long-term outcome of patient with polycystic liver disease treated with lanreotide. Aliment Pharmacol Ther 2012;35:266–274.
Weckbecker G, Briner U, Lewis I,Bruns C. SOM230: A New Somatostatin Peptidomimetic with Potent Inhibitory Effects on the Growth Hormone/Insulin-Like Growth Factor-I Axis in Rats, Primates, and Dogs. Endocrinology. 2002;143(10): 4123–30.

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[1] Torres VE, Harris PC. Autosomal dominant polycystic kidney disease: the last 3 years. Kidney Int. 2009; 76: 149–68.

[2] Hoevenaren IA, Wester R, Schrier RW, MacFann K, Doctor RB, DrenthJP, et al. Polycystic liver: clinical characteristics of patients with isolated polycystic liver disease compared with polycystic liver and autosomal dominant polycystic kidney disease. Liver Int. 2007; 28: 264.

[3] Ecder T,Schrier RW. Cardiovascular abnormalities in autosomal-dominant polycystic kidney disease. Nat Rev Nephrol 2009; 5(4): 221-8.

[4] Xu, HW, Yu SQ, Mei CL, Li MH. Screening for intracranial aneurysm in 355 patients with autosomal-dominant polycystic kidney disease. Stroke 2011; 42: 204-6.

[5] van Keimpema L, Nevens F, Vanslembrouck R, van Oijen MG, Hoffmann AL, Dekker HM, de Man RA, Drenth JP. Lanreotide reduces the volume of polycystic liver: a randomized, double-blind, placebo-controlled trial. Gastroenterology 2009; 137(5): 1661–8.

[6] Bistritz L, Tamboli C, Bigam D, Bain VG. Polycystic liver disease: experience at a teaching hospital. Am J Gastroenterol. 2005 Oct; 100(10): 2212-7.

[7] Qian Q, Li A, King BF, Kamath PS, Lager DJ, Huston J 3rd, Shub C, Davila S, Somlo S, Torres VE. Clinical profile of autosomal dominant polycystic liver disease. Hepatology 2003 Jan; 37(1): 164-71.

[8] Torres VE, Harris PC, Pirson Y. Autosomal dominant polycystic kidney disease. Lancet. 2007;369:1287–1301.

[9] Hwang SH, Yu JS, Chung JJ, Kim JH, Kim KW. Diagnosing small hepatic cysts on multidetector CT: an additional merit of thinner coronal reformations. Korean J Radiol. 2011;12:341–350.

[10] Drenth, J. P., Chrispijn, M., Nagorney, D. M., Kamath, P. S. & Torres, V. E. Medical and surgical treatment options for polycystic liver disease. Hepatology 2010; 52(6): 2223-30.

[11] Rusell RT, Pinson CW. Surgical magement of polycystic liver disease. World JGastroenterol. 2007; 13: 5052-9.

[12] van Keimpema L, Ruurda JP, Ernst MF, van Geffen HJ, Drenth JP.Laparoscopic fenestration of liver cysts in polycystic liver disease results in a median volume reduction of 12.5%. JGastrointest. Surg.2008; 12(3): 477-82.

[13] Koperna T, Vogl S, Satzinger U, Schulz F. Nonparasitic Cysts of the Liver: Results and Options of Surgical Treatment. World J Surg.1997; 21: 850-54.

[14] Martin IJ, McKinley, AJ, Currie EJ, Holmes P, Garden OJ. Tailoring the management of nonparasitic liver cysts. AnnSurg. 1998; 228:167–72.

[15] Coelho-Prabhu N, Nagorney DM, Baron TH. ERCP for the treatment of bile leak after partial hepatectomy and fenestration for symptomatic polycystic liver disease. World JGastroenterol. 2012; 18(28): 3705-9.

[16] Temmerman F, Missiaen L, Bammens B, Laleman W, Cassiman D, Verslype C, van Pelt J, Nevens F. Systematic review: the pathophysiology and management of polycystic liver disease. Aliment Pharmacol Ther. 2011; 34:702-13.

[17] van KeimpemaL, Nevens F, Adam R, Porte RJ, Fikatas P, Becker T, Kirkegaard P, Metselaar HJ, Drenth JP.Excellent survival after liver transplantation for isolated polycystic liver disease: an European Liver Transplant Registry study. TransplInt. 2011; 24(12): 1239-45.

[18] van Keimpema L, De Koning DB, Van Hoek B, Van Den Berg AP, Van Oijen MG, De Man RA, Nevens F, Drenth JP. Patients with isolated polycystic liver disease referred to liver centres: clinical characterization of 137 cases. Liver Int. 2011;31(1):92-8.

[19] Takei R, Ubara Y, Hoshino J, Higa Y, Suwabe T, Sogawa Y, Nomura K, Nakanish S, Sawa N, Katori H, etal. Percutaneous transcatheter hepatic artery embolization for liver cysts in autosomal dominant polycystic kidney disease. Am. J. Kidney Dis. 2007; 49(6): 744–52.

[20] Caroli A, Antiga L, Cafaro M, Fasolini G, Remuzzi A, Remuzzi G, Ruggenenti P. Reducing polycystic liver volume in ADPKD: effects of somatostatin analogue octreotide. Clin J Am SocNephrol.2010;5(5): 783–9.

[21] HoganMC, Masyuk TV, Page LJ, Kubly VJ, Bergstralh EJ, Li X, Kim B, King BF, Glockner J, Holmes DR 3rd,et al. Randomized clinical trial of long-acting somatostatin for autosomal dominant polycystic kidney and liver disease. J AmSocNephrol. 2010; 21(6): 1052–61.

[22] Van Keimpema L, Nevens F, Vanslembrouck R, van Oijen MG, Hoffmann AL, Dekker HM, de Man RA, Drenth JP. Lanreotide reduces the volume of polycystic liver: a randomized, double-blind, placebo-controlled trial. Gastroenterology. 2009; 137(5): 1661–8.

[23] Temmerman F, Vanslembrouck R, CoudyzerW, Bammens B, Laleman W, Cassiman D, van Der Merwe S, Verslype C, van Steenbergen W, van Pelt J, Pirson Y, et al. The reduction in liver volume in polycystic liver disease with lanreotide is dose dependent and is most pronounced in patients with the highest liver volume. JHepatol. 2012; 56: suppl 2: S547.

[24] Chrispijn M, Nevens F, Gevers TJG, et al. The long-term outcome of patient with polycystic liver disease treated with lanreotide. Aliment Pharmacol Ther 2012;35:266–274.

[25] Weckbecker G, Briner U, Lewis I,Bruns C. SOM230: A New Somatostatin Peptidomimetic with Potent Inhibitory Effects on the Growth Hormone/Insulin-Like Growth Factor-I Axis in Rats, Primates, and Dogs. Endocrinology. 2002;143(10): 4123–30.

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