Tratamiento quirúrgico de la enfermedad poliquística hepática autosómica dominante, serie de casos
DOI:
https://doi.org/10.30944/20117582.330Palabras clave:
hígado, poliquístico, riñón poliquístico autosómico dominante, hepatectomíaResumen
En la actualidad, la cirugía hepática y la de la vía biliar son unos de los procedimientos más practicados a nivel mundial. El refinamiento en las técnicas quirúrgicas, los cuidados anestésicos y el trasplante hepático han revolucionado el tratamiento de enfermedades hepáticas que inicialmente eran incurables. La enfermedad poliquística hepática autosómica dominante es rara y puede asociarse a poliquistosis renal o presentarse de forma aislada. Su sintomatología se presenta en los estados avanzados de la enfermedad. A pesar de las múltiples modalidades de tratamiento para esta condición, el manejo quirúrgico con hepatectomía y fenestración es el que ha mostrado mejores resultados en los pacientes con saciedad temprana y hepatomegalia masiva. Se hace una revisión bibliográfica y se presenta una serie de casos atendidos con esta enfermedad en el Hospital Metropolitano de Quito, Ecuador.
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Referencias bibliográficas
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https://doi.org/10.1097/00000478-198804000-00010
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https://doi.org/10.1053/jhep.2003.50006
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https://doi.org/10.1016/S0140-6736(07)60601-1
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https://doi.org/10.1159/000421651
7. Heggo O. A microdissection study of cystic disease of the kidneys in adults. J Pathol Bacteriol. 1966;91:311-5.
https://doi.org/10.1002/path.1700910204
8. Verani R, Silva F. Histogenesis of the renal cysts in adult (autosomal dominant) polycystic kidney disease: A histochemical study. Mod Pathol. 1988;1:457-63.
9. Grantham J. Clinical practice: Autosomal dominant polycystic kidney disease. N Engl J Med. 2008;359:1477-85.
https://doi.org/10.1056/NEJMcp0804458
10. Bae K, Zhu F, Chapman A, Torres V, Grantham J, Guay-Woodford L, et al. Magnetic resonance imaging evaluation of hepatic cysts in early autosomal dominant polycystic kidney disease: The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease cohort. Clin J Am Soc Nephrol. 2006;1:64-9.
https://doi.org/10.2215/CJN.00080605
11. Grünfeld J,Albouze G,Jungers P, Landais P, Dana A, Droz D, et al. Liver changes and complications in adult polycystic kidney disease. Adv Nephrol Necker Hosp. 1985;14:1-20.
12. Gabow P, Johnson A, Kaehny W, Manco-Johnson M, Duley I, Everson G. Risk factors for the development of hepatic cysts in autosomal dominant polycystic kidney disease. Hepatology. 1990;11:1033-7.
https://doi.org/10.1002/hep.1840110619
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https://doi.org/10.1086/368295
17. Feldman M. Polycystic disease of the liver. Am J Gastroenterol. 1958;29:83-6.
https://doi.org/10.1093/ajcp/29.2.160
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https://doi.org/10.1002/hep.1840080626
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22. Kida T, Nakanuma Y, Terada T. Cystic dilatation of peribiliary glands in livers with adult polycystic disease and livers with solitary nonparasitic cysts: An autopsy study. Hepatology. 1992;16:334-40.
https://doi.org/10.1002/hep.1840160209
23. Lazaridis K, Strazzabosco M, Larusso N. The cholangiopathies: Disorders of biliary epithelial. Gastroenterology. 2004;127:1565-77.
https://doi.org/10.1053/j.gastro.2004.08.006
24. Gaines P, Sampson M. The prevalence and characterization of simple hepatic cysts by ultrasound examination. Br J Radiol. 1989;62:335-7.
https://doi.org/10.1259/0007-1285-62-736-335
25. Caremani M, Vincenti A, Benci A, Sassoli S, Tacconi D. Ecographic epidemiology of non-parasitic hepatic cysts. J Clin Ultrasound. 1993;21:115-8.
https://doi.org/10.1002/jcu.1870210207
26. Carrim Z, Murchison J. The prevalence of simple renal and hepatic cysts detected by spiral computed tomography. Clin Radiol. 2003;58:626-9.
https://doi.org/10.1016/S0009-9260(03)00165-X
27. van Keimpema L, de Koning D, Strijk S, Drenth J. Aspirationsclerotherapy results in effective control of liver volume in patients with liver cysts. Dig Dis Sci. 2008;53:2251-7.
https://doi.org/10.1007/s10620-007-0121-x
28. van Erpecum K, Janssens A, Terpstra J, Tjon A Tham RT. Highly symptomatic adult polycystic disease of the liver. A report of fifteen cases. J Hepatol. 1987;5:109-17.
https://doi.org/10.1016/S0168-8278(87)80068-5
29. Que F, Nagorney D, Gross J, Torres V. Liver resection and cyst fenestration in the treatment of severe polycystic liver disease. Gastroenterology. 1995;108:487-94.
https://doi.org/10.1016/0016-5085(95)90078-0
30. Kabbej M, Sauvanet A, Chauveau D, Farges O, Belghiti J. Laparoscopic fenestration in polycystic liver disease. Br J Surg. 1996;83:1697-701.
https://doi.org/10.1002/bjs.1800831211
31. Gigot J, Jadoul P, Que F, van Beers B, Etienne J, Horsmans Y, et al. Adult polycystic liver disease: Is fenestration the most adequate operation for long-term management? Ann Surg. 1997;225:286-94.
https://doi.org/10.1097/00000658-199703000-00008
32. Qian Q. Isolated polycystic liver disease. Adv Chronic Kidney Dis. 2010;17:181-9.
https://doi.org/10.1053/j.ackd.2009.12.005
33. Russell R, Pinson W. Surgical management of polycystic liver disease. World J Gastroenterol. 2007;13:5052-9.
https://doi.org/10.3748/wjg.v13.i38.5052
34. Lin T, Chen C, Wang S. Treatment of non-parasitic cystic disease of the liver: A new approach to therapy with polycystic liver. Ann Surg. 1968;168:921-7.
https://doi.org/10.1097/00000658-196811000-00022
35. Farges O, Bismuth H. Fenestration in the management of polycystic liver disease. World J Surg. 1995;19:25-30.
https://doi.org/10.1007/BF00316975
36. Konstadoulakis M, Gomatos I, Albanopoulos K, Alexakis N, Leandros E. Laparoscopic fenestration for the treatment of patients with severe adult polycystic liver disease. Am J Surg. 2005;189:71-5.
https://doi.org/10.1016/j.amjsurg.2004.03.011
37. Martin I, McKinley A, Currie E, Holmes P, Garden O. Tailoring the management of nonparasitic liver cysts. Ann Surg. 1998;228:167-72.
https://doi.org/10.1097/00000658-199808000-00004
38. Schnelldorfer T, Torres V, Zakaria S, Rosen C, Nagorney D. Polycystic liver disease: A critical appraisal of hepatic resection, cyst fenestration, and liver transplantation. Ann Surg. 2009;250:112-8.
https://doi.org/10.1097/SLA.0b013e3181ad83dc
39. Manterola C, Pineda V, Vial M. Efectividad del tratamiento laparoscópico de quistes y tumores hepáticos. Revisión global de la evidencia. Revista Chilena de Cirugía. 2007;59:264-71.
https://doi.org/10.4067/S0718-40262007000400004
40. Robinson T, Stiegmann G, Everson G. Laparoscopic palliation of polycystic liver disease. Surg Endosc. 2005;19:130-2.
https://doi.org/10.1007/s00464-004-8813-1
41. Hazbón H. Enfermedad poliquística del hígado. Rev Colomb Cir. 2008;23:168-73.
42. US Scientific Registry of Transplant: 2004 OPTN / SRTR Annual Report. US Organ Procurement and Transplantation Network and the Scientific Registry of Transplant Recipients. U.S. Government Sponsored Work, 2004. Fecha de consulta: 23 de septiembre de 2014. Disponible en: www.ustransplant.org
43. Takei R, Ubara Y, Hoshino J, Higa Y, Suwabe T, Sogawa Y, et al. Percutaneous transcatheter hepatic artery embolization for liver cysts in autosomal dominant polycystic kidney disease. Am J Kidney Dis. 2007;49:744-52.
https://doi.org/10.1053/j.ajkd.2007.03.018
44. Ubara Y, Takei R, Hoshino J, Tagami T, Sawa N, Yokota M, et al. Intravascular embolization therapy in a patient with an enlarged polycystic liver. Am J Kidney Dis. 2004;43:733-8.
https://doi.org/10.1053/j.ajkd.2003.12.035
45. Masyuk T, Masyuk A, Torres V, Harris P, Larusso N. Octreotide inhibits hepatic cystogenesis in a rodent model of polycystic liver disease by reducing cholangiocyte adenosine 3',5'-cyclic monophosphate. Gastroenterology. 2007;132:1104-16.
https://doi.org/10.1053/j.gastro.2006.12.039
46. van Keimpema L, de Man R, Drenth J. Somatostatin analogues reduce liver volume in polycystic liver disease. Gut. 2008;57:1338-9.
https://doi.org/10.1136/gut.2008.155721
47. van Keimpema L, Drenth J. Effect of octreotide on polycystic liver volume. Liver Int. 2010;30:633-34.
https://doi.org/10.1111/j.1478-3231.2009.02123.x
48. van Keimpema L, Nevens F, Vanslembrouck R, van Oijen M, Hoffmann A, Dekker H, et al. Lanreotide reduces the volume of polycystic liver: A randomized, double-blind, placebocontrolled trial. Gastroenterology. 2009;137:1661-8.
https://doi.org/10.1053/j.gastro.2009.07.052
49. Hogan M, Masyuk T, Page L, Kubly V, Bergstralh E, Li X, et al. Randomized clinical trial of long-acting somatostatin for autosomal dominant polycystic kidney and liver disease. J Am Soc Nephrol. 2010;21:1052-61.
https://doi.org/10.1681/ASN.2009121291
50. Caroli A, Antiga L, Cafaro M, Fasolini G, Remuzzi A, Remuzzi G, et al. Reducing polycystic liver volume in ADPKD: Effects of somatostatin analogue octreotide. Clin J Am Soc Nephrol. 2010;5:783-9.
https://doi.org/10.2215/CJN.05380709
51. Shillingford J, Murcia N, Larson C, Low S, Hedgepeth R, Brown N, et al. The mTOR pathway is regulated by polycystin-1, and its inhibition reverses renal cystogenesis in polycystic kidney disease. Proc Natl Acad Sci USA. 2006;103:5466-71.
https://doi.org/10.1073/pnas.0509694103
52. Wahl P, Serra A, Le H, Molle K, Hall M, Wuthrich R. Inhibition of mTOR with sirolimus slows disease progression inHan:SPRD rats with autosomal dominant polycystic kidney disease (ADPKD). Nephrol Dial Transplant. 2006;21:598-604.
https://doi.org/10.1093/ndt/gfi181
53. Tao Y, Kim J, Schrier R, Edelstein C. Rapamycin markedly slows disease progression in a rat model of polycystic kidney disease. J Am Soc Nephrol. 2005;16:46-51.
https://doi.org/10.1681/ASN.2004080660
54. Wu M, Wahl P, Le H, Wackerle-Men Y, Wuthrich R, Serra A. Everolimus retards cyst growth and preserves kidney function in a rodent model for polycystic kidney disease. Kidney Blood Press Res. 2007;30:253-9.
https://doi.org/10.1159/000104818
55. Wu M, Arcaro A, Varga Z, Vogetseder A, Le HM, Wüthrich R, et al: Pulse mTOR inhibitor treatment effectively controls cyst growth but leads to severe parenchymal and glomerular hypertrophy in rat polycystic kidney disease. Am J Physiol Renal Physiol. 2009;297:1597-1605.
https://doi.org/10.1152/ajprenal.00430.2009
2. Kwok M, Lewin K. Massive hepatomegaly in adult polycystic liver disease. Am J Surg Pathol. 1988;12:321-4.
https://doi.org/10.1097/00000478-198804000-00010
3. Melnick P. Polycystic liver; analysis of seventy cases. Arch Pathol. 1955;59:162-72.
4. Qian Q, Li A, King B, Kamath P, Lager D, Huston J 3rd, et al. Clinical profile of autosomal dominant polycystic liver disease. Hepatology. 2003;37:164-71.
https://doi.org/10.1053/jhep.2003.50006
5. Torres V, Harris P, Pirson Y. Autosomal dominant polycystic kidney disease. Lancet. 2007;369:1287-301.
https://doi.org/10.1016/S0140-6736(07)60601-1
6. Peters D, Sandkuijl L. Genetic heterogeneity of polycystic kidney disease in Europe. Contrib Nephrol. 1992;97:128-39.
https://doi.org/10.1159/000421651
7. Heggo O. A microdissection study of cystic disease of the kidneys in adults. J Pathol Bacteriol. 1966;91:311-5.
https://doi.org/10.1002/path.1700910204
8. Verani R, Silva F. Histogenesis of the renal cysts in adult (autosomal dominant) polycystic kidney disease: A histochemical study. Mod Pathol. 1988;1:457-63.
9. Grantham J. Clinical practice: Autosomal dominant polycystic kidney disease. N Engl J Med. 2008;359:1477-85.
https://doi.org/10.1056/NEJMcp0804458
10. Bae K, Zhu F, Chapman A, Torres V, Grantham J, Guay-Woodford L, et al. Magnetic resonance imaging evaluation of hepatic cysts in early autosomal dominant polycystic kidney disease: The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease cohort. Clin J Am Soc Nephrol. 2006;1:64-9.
https://doi.org/10.2215/CJN.00080605
11. Grünfeld J,Albouze G,Jungers P, Landais P, Dana A, Droz D, et al. Liver changes and complications in adult polycystic kidney disease. Adv Nephrol Necker Hosp. 1985;14:1-20.
12. Gabow P, Johnson A, Kaehny W, Manco-Johnson M, Duley I, Everson G. Risk factors for the development of hepatic cysts in autosomal dominant polycystic kidney disease. Hepatology. 1990;11:1033-7.
https://doi.org/10.1002/hep.1840110619
13. Comfort M, Gray H, Dahlin D, Whitesell F Jr. Polycystic disease of the liver: A study of 24 cases. Gastroenterology. 1952;20: 60-78.
https://doi.org/10.1016/S0016-5085(52)80213-6
14. Drenth JP, Morsche RH, Smink R, Bonifacino JS, Jansen JB. Germline mutations in PRKCSH are associated with autosomal dominant polycystic liver disease. Nat.Genet. 2003;33:345-7.
https://doi.org/10.1038/ng1104
15. Dávila S, Furu L, Gharavi A, Tian X, Onoe T, Qian Q, et al. Mutations in SEC63 cause autosomal dominant polycystic liver disease. Nat Genet. 2004;36:575-7.
https://doi.org/10.1038/ng1357
16. Li A, Dávila S, Furu L, Qian Q, Tian X, Kamath P, et al. Mutations in PRKCSH cause isolated autosomal dominant polycysticliver disease. Am J Hum Genet. 2003;72:691-703.
https://doi.org/10.1086/368295
17. Feldman M. Polycystic disease of the liver. Am J Gastroenterol. 1958;29:83-6.
https://doi.org/10.1093/ajcp/29.2.160
18. Karhunen P, Tenhu M. Adult polycystic liver and kidney diseases are separate entities. Clin Genet. 1986;30:29-37. 19. Everson GT, Scherzinger A, Berger-Leff N, Reichen J, Lezotte D, Manco-Johnson M, et al. Polycystic liver disease: Quantitation of parenchymal and cyst volumes from computed tomography images and clinical correlates of hepatic cysts. Hepatology. 1988;8:1627-34.
https://doi.org/10.1002/hep.1840080626
20. Onori P, Franchitto A, Mancinelli R, Carpino G, Alvaro D, Francis H, et al. Polycystic liver diseases. Dig Liver Dis. 2010;42:261-71.
https://doi.org/10.1016/j.dld.2010.01.006
21. Redston M, Wanless I. The hepatic von Meyenburg complex: Prevalence and association with hepatic and renal cysts among 2843 autopsies corrected. Mod Pathol. 1996 9:233-37.
22. Kida T, Nakanuma Y, Terada T. Cystic dilatation of peribiliary glands in livers with adult polycystic disease and livers with solitary nonparasitic cysts: An autopsy study. Hepatology. 1992;16:334-40.
https://doi.org/10.1002/hep.1840160209
23. Lazaridis K, Strazzabosco M, Larusso N. The cholangiopathies: Disorders of biliary epithelial. Gastroenterology. 2004;127:1565-77.
https://doi.org/10.1053/j.gastro.2004.08.006
24. Gaines P, Sampson M. The prevalence and characterization of simple hepatic cysts by ultrasound examination. Br J Radiol. 1989;62:335-7.
https://doi.org/10.1259/0007-1285-62-736-335
25. Caremani M, Vincenti A, Benci A, Sassoli S, Tacconi D. Ecographic epidemiology of non-parasitic hepatic cysts. J Clin Ultrasound. 1993;21:115-8.
https://doi.org/10.1002/jcu.1870210207
26. Carrim Z, Murchison J. The prevalence of simple renal and hepatic cysts detected by spiral computed tomography. Clin Radiol. 2003;58:626-9.
https://doi.org/10.1016/S0009-9260(03)00165-X
27. van Keimpema L, de Koning D, Strijk S, Drenth J. Aspirationsclerotherapy results in effective control of liver volume in patients with liver cysts. Dig Dis Sci. 2008;53:2251-7.
https://doi.org/10.1007/s10620-007-0121-x
28. van Erpecum K, Janssens A, Terpstra J, Tjon A Tham RT. Highly symptomatic adult polycystic disease of the liver. A report of fifteen cases. J Hepatol. 1987;5:109-17.
https://doi.org/10.1016/S0168-8278(87)80068-5
29. Que F, Nagorney D, Gross J, Torres V. Liver resection and cyst fenestration in the treatment of severe polycystic liver disease. Gastroenterology. 1995;108:487-94.
https://doi.org/10.1016/0016-5085(95)90078-0
30. Kabbej M, Sauvanet A, Chauveau D, Farges O, Belghiti J. Laparoscopic fenestration in polycystic liver disease. Br J Surg. 1996;83:1697-701.
https://doi.org/10.1002/bjs.1800831211
31. Gigot J, Jadoul P, Que F, van Beers B, Etienne J, Horsmans Y, et al. Adult polycystic liver disease: Is fenestration the most adequate operation for long-term management? Ann Surg. 1997;225:286-94.
https://doi.org/10.1097/00000658-199703000-00008
32. Qian Q. Isolated polycystic liver disease. Adv Chronic Kidney Dis. 2010;17:181-9.
https://doi.org/10.1053/j.ackd.2009.12.005
33. Russell R, Pinson W. Surgical management of polycystic liver disease. World J Gastroenterol. 2007;13:5052-9.
https://doi.org/10.3748/wjg.v13.i38.5052
34. Lin T, Chen C, Wang S. Treatment of non-parasitic cystic disease of the liver: A new approach to therapy with polycystic liver. Ann Surg. 1968;168:921-7.
https://doi.org/10.1097/00000658-196811000-00022
35. Farges O, Bismuth H. Fenestration in the management of polycystic liver disease. World J Surg. 1995;19:25-30.
https://doi.org/10.1007/BF00316975
36. Konstadoulakis M, Gomatos I, Albanopoulos K, Alexakis N, Leandros E. Laparoscopic fenestration for the treatment of patients with severe adult polycystic liver disease. Am J Surg. 2005;189:71-5.
https://doi.org/10.1016/j.amjsurg.2004.03.011
37. Martin I, McKinley A, Currie E, Holmes P, Garden O. Tailoring the management of nonparasitic liver cysts. Ann Surg. 1998;228:167-72.
https://doi.org/10.1097/00000658-199808000-00004
38. Schnelldorfer T, Torres V, Zakaria S, Rosen C, Nagorney D. Polycystic liver disease: A critical appraisal of hepatic resection, cyst fenestration, and liver transplantation. Ann Surg. 2009;250:112-8.
https://doi.org/10.1097/SLA.0b013e3181ad83dc
39. Manterola C, Pineda V, Vial M. Efectividad del tratamiento laparoscópico de quistes y tumores hepáticos. Revisión global de la evidencia. Revista Chilena de Cirugía. 2007;59:264-71.
https://doi.org/10.4067/S0718-40262007000400004
40. Robinson T, Stiegmann G, Everson G. Laparoscopic palliation of polycystic liver disease. Surg Endosc. 2005;19:130-2.
https://doi.org/10.1007/s00464-004-8813-1
41. Hazbón H. Enfermedad poliquística del hígado. Rev Colomb Cir. 2008;23:168-73.
42. US Scientific Registry of Transplant: 2004 OPTN / SRTR Annual Report. US Organ Procurement and Transplantation Network and the Scientific Registry of Transplant Recipients. U.S. Government Sponsored Work, 2004. Fecha de consulta: 23 de septiembre de 2014. Disponible en: www.ustransplant.org
43. Takei R, Ubara Y, Hoshino J, Higa Y, Suwabe T, Sogawa Y, et al. Percutaneous transcatheter hepatic artery embolization for liver cysts in autosomal dominant polycystic kidney disease. Am J Kidney Dis. 2007;49:744-52.
https://doi.org/10.1053/j.ajkd.2007.03.018
44. Ubara Y, Takei R, Hoshino J, Tagami T, Sawa N, Yokota M, et al. Intravascular embolization therapy in a patient with an enlarged polycystic liver. Am J Kidney Dis. 2004;43:733-8.
https://doi.org/10.1053/j.ajkd.2003.12.035
45. Masyuk T, Masyuk A, Torres V, Harris P, Larusso N. Octreotide inhibits hepatic cystogenesis in a rodent model of polycystic liver disease by reducing cholangiocyte adenosine 3',5'-cyclic monophosphate. Gastroenterology. 2007;132:1104-16.
https://doi.org/10.1053/j.gastro.2006.12.039
46. van Keimpema L, de Man R, Drenth J. Somatostatin analogues reduce liver volume in polycystic liver disease. Gut. 2008;57:1338-9.
https://doi.org/10.1136/gut.2008.155721
47. van Keimpema L, Drenth J. Effect of octreotide on polycystic liver volume. Liver Int. 2010;30:633-34.
https://doi.org/10.1111/j.1478-3231.2009.02123.x
48. van Keimpema L, Nevens F, Vanslembrouck R, van Oijen M, Hoffmann A, Dekker H, et al. Lanreotide reduces the volume of polycystic liver: A randomized, double-blind, placebocontrolled trial. Gastroenterology. 2009;137:1661-8.
https://doi.org/10.1053/j.gastro.2009.07.052
49. Hogan M, Masyuk T, Page L, Kubly V, Bergstralh E, Li X, et al. Randomized clinical trial of long-acting somatostatin for autosomal dominant polycystic kidney and liver disease. J Am Soc Nephrol. 2010;21:1052-61.
https://doi.org/10.1681/ASN.2009121291
50. Caroli A, Antiga L, Cafaro M, Fasolini G, Remuzzi A, Remuzzi G, et al. Reducing polycystic liver volume in ADPKD: Effects of somatostatin analogue octreotide. Clin J Am Soc Nephrol. 2010;5:783-9.
https://doi.org/10.2215/CJN.05380709
51. Shillingford J, Murcia N, Larson C, Low S, Hedgepeth R, Brown N, et al. The mTOR pathway is regulated by polycystin-1, and its inhibition reverses renal cystogenesis in polycystic kidney disease. Proc Natl Acad Sci USA. 2006;103:5466-71.
https://doi.org/10.1073/pnas.0509694103
52. Wahl P, Serra A, Le H, Molle K, Hall M, Wuthrich R. Inhibition of mTOR with sirolimus slows disease progression inHan:SPRD rats with autosomal dominant polycystic kidney disease (ADPKD). Nephrol Dial Transplant. 2006;21:598-604.
https://doi.org/10.1093/ndt/gfi181
53. Tao Y, Kim J, Schrier R, Edelstein C. Rapamycin markedly slows disease progression in a rat model of polycystic kidney disease. J Am Soc Nephrol. 2005;16:46-51.
https://doi.org/10.1681/ASN.2004080660
54. Wu M, Wahl P, Le H, Wackerle-Men Y, Wuthrich R, Serra A. Everolimus retards cyst growth and preserves kidney function in a rodent model for polycystic kidney disease. Kidney Blood Press Res. 2007;30:253-9.
https://doi.org/10.1159/000104818
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2015-01-01
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Ayala, A.; Aguilar, J. C.; Jiménez, F.; Serpa, F. Tratamiento Quirúrgico De La Enfermedad poliquística hepática autosómica Dominante, Serie De Casos. Rev Colomb Cir 2015, 30, 139-145.
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