Four female patients with severe complications of polycystic liver disease were treated with liver replacement; two patients were also given kidneys from their liver donors. All four of the patients were suffering from extreme fatigue. Three of the recipients have survived for 8, 11, and 60 months with normal liver function and present good health. The fourth patient recovered from a liver-kidney transplantation, but 5 months later, fulminant hepatic failure developed in this patient due to hepatitis B virus, and she died despite emergency hepatic retransplantation. © 1990, American Medical Association. All rights reserved

Gordon, R

Mieles, L

Reyes, J

Starzl, TE

Todo, S

Tzakis, A

English

Name not available

Liver transplantation for polycystic liver disease

Thomas E. Starzl

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Archives of Surgery

Liver Transplantation for Polycystic Liver Disease

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" no matter What he or she \\;ll ~ facing the medical have professors it, Itors, or physician practiced medicin~ It the practice of )gram, undertaken ion, can do nothing I broken image. It before entering a n the time of tho~ Ictice of medicine s where they fusi t that the products ,ed physicians whu n at the medical "EN, JR, MD, PHD a impact on special-::ation is in danger nority. Therefore, ,ew proposals that .re physician from ms introduced bv ! College, Dnive;. :re Dr Griffin that posals to improve medical education mingo o MD, MPH, MSC Education iation IL_ ~_~f~K-_____________________ _ Jriginal Articles -Liver Transplantation for Polycystic Liver Disease Thon', s E. Starzl, MD, PhD; Jorge Reyes, MD; Andreas Tzakis, MD; Luis Mieles, MD; 5atoru Thdo, MD; Robert Gordon, lID • Four fema~e pat~ents with severe complications of polycys· tie liver d~sease were treated with liver replacement; two pat~ents were a~so given kidneys from the~r liver donors. All four of the petlents were suffer~ng from extreme fatigue. Three of the rec~p~· enls have survived for 8, 11, and 60 months with normal liver funct~on and present good health. The fourth pat~ent recovered trom a ffver-k~dney transp~antationI but 5 months ~UterI fu~mlnUnt hepat:c failure deve~oped ~n this patient due to hepatlt~s B virus, . and ~:e d~ed d .. p~te emergency hepatic retrUnsp~antatlonK (Arch Surg. 1(,90;125:575-577) MOSt patients with polycystic liver disease bear a heavy but nonlethal burden. Hepatic failure is unusual.1,2 Be-tween half' and two thirds2 of patients with the adult autoso-mal dominant disorder also have polycystic disease of the kidney. Before the availability of tlialysis and kidney trans-plantation, renal failure was a common cause of death. Now that renal disease can be treated effectively, more patients can be expected to survive long enough to experience hepatic rom( ;cations.' Th, nature and seriousness of the hepatic complications, ..-hich are largely caused by mass effects, have been empha-sized increasingly in recent reports. U Although many pallia-tivp procedures have been proposed,· these operations are of questionable value and are excessively dangerous unless there are "dominant" or regional cysts. U We report here the first use, to our knowledge, of total hepatectomy and liver transplantation for the treatment of polycystic liver disease in four patients who also had polycystic kidney disease. Con-tIlmitant renal transplantation was performed in two of the four F· 'er recipients. PATIENTS AND METHODS Case Material The four female recipients were aged 37 to 57 years (Table). 4';11ldice, hypoalbuminemia, clotting disorders, and other laboratory fll~ence of hepatic dysfunction "'ere not present. However, one !Qtient had esophageal varices, and two patients had ascites. Three of the four patients had pain from the huge liver masses (Figure), and t\\'o patients were addicted to narcotics (Table). , Significant symptoms had been present for 3 to 13 years (Table). ~K four patients complained of shortness of breath and/or extreme tlgu. 0 The exhaustion was so profound that the four patients had !'.oPPE.; professional or domestic 'Ili"Ork and were confined to bed most -----~-------------------------------------ACCepted for publication October 25, 1989. l' From the Department of Surgery, l"ni\'ersity Health Center of Pittsburgh, . Illversity of Pittsburgh (Pa) School ~ Medicine, and the Veterans Admini8-'tltion Medical Center, Pittsburgh. PL h Reprint requests to Department ~ Surgery, FaIk Clinic. 3601 Fifth Aft, ttsburgh. PA 15213 (Dr StarzI). of the time. Patient 4 <Table) whose main complaint was that she was dying of exhaustion had respiratory insufficiency, and she eventually required ventilatory support until a liver could be found. This same patient had undergone a left transverse colostomy and cecostomy 1 year previously because of colonic obstruction and toxic megacolon. Liver volume was estimated with computed tomographic scans'in two patients to be 7120 and 12 790 mL (Table ~ It was too inconvenient and uncomfortable to attempt these measurements in the other two patients. Patient 3 (Table) had undergone a right hepatic lobectomy and cyst fenestration in 1980, "ith a consequent reduction in the hepatomega-ly and symptomatic relief. However, the liver slowly enlarged back to and beyond its original size (Figure, at right). The cysts were present throughout the liver (Figure) in all of the patients. All four of the patients had polycystic renal disease. Two of the four patients had slightly eltvated serum creatinine concentrations and subnormal creatinine clearances of 0.50 to 0.58 mLls (Table). Surgical Procedures The orthotopic liv~r transplantations were performed by using standard technjques,' with an estimated blood loss of 4 to 45 L. The most serious bleeding was in patient 3 (Table) who had undergone a right hepatic lobectomy plus cyst fenestration 8 years previously. The liver graft did not function well, necessitating retransplantation 11 days later. In patient 4 (Table), the previously placed colostomy and cecos-tomy were closed at the time of transp~antationK Numerous ulcer· ations of the stretched and necrotic anterior abdominal wall in this patient were eliminated by excising a ""ide strip of skin and subcuta-neous tissue from the xiphoid process to the pubis. Patients 1 and 4 (Table) were given a kidney from their liver donor. The degree of renal failure (Table) would not have justified kidney transplantation under nonnal circumstances. However, it was feared that the subnormal renal function would preclude giving a full thera· peutic dose of cyclosporine because of this drug's nephrotoxicity. The liver was transplanted first in both patients, followed by the kidney transplantations only after the conditions of the patients were stable. Aftercare The four patients required ventilatory support postoperatively for 9, 2, IS, and 13 days. Baseline immunosuppression was with cyclo-sporine and steroids. Two patients also had supplemental therapy with OIIT3 and/or azathioprine. RESULTS Liver Size The excised livers weighed 4000 to 12 900 g (Table). This weight range was a gross underestimate of the actual weights since a large amount of fluid was lost when the cysts were ruptured during the hepatectomies or when the specimen was cut afterward. The heaviest surgical pathology specimen (from patient 1, Table) weighed 12900 g. The histopathologic Commentary ~Ch purg-so~ 125, May 1990 Polycystic Liver Disease - ptarz~ el aI 575 -----.,-r,' 1---, I . I .' .. -, r J: C Clinical Findings in Four Patients With Polycystic Liver Disease' Petlent 'ing:-~a-:K€---------------------------------~---------------------------------3 10131188, 11/11188 57 White F 62 x 4 11/21/88 42 White F 60 x X-i~ ';ear, ::ely ',r, ~K K:Ka~::D~ . 71. ' .:.-eC h( .. ~:iyc J -. X X I. =::==~----------K:K:KK--------K-K:~--------~:-:----------~~-----fI~~~ ~~::~------------------------~----------------~--------------------~------------------------~--~D---- ; ~<~ :: ~~~----------------------------------------------------------------------------------~----__ i :ierK~ X : "~ pn:' X -: _:::tKpO~ ~~=---------------------------------~-----------------------------------------K K~fz 12 13 I Cyst drainage Cyst aspiration (1960), cyst colostomy and " ~;Iu-~: drainage (1974), cecostomy! :':''"Ea::: RHl and F ! ',;"r.:.: 1-________________________ KKK:E~19=UM:!KF ____________ I :lg-:.: 12 790 ~Iyilb-r-~~~~~~~~ _____ ~~ _____ ~~ _________ 4~1~ll~ __________ ~~~ __ f~~pI 576 61 X :K""D~ 144.9 ;'.-15. F ;::;.:r,! : 150 058 I · ...... lK~ .. =-~--~--------------~~--~~------------~~----------------~K~------------------~~K~----- -, ~ Arch Surg - Vol 125. May 1990 0.56 0.83' ~e::Ks -~"DfDK Polycystic Liver [FiserapU-~""KI :-~-;Kj c. :!.-2:!' :-:~~b (' ;;:: k:::---:.c..-..;,.: ~K ~ .. a:.--: K~K: !J. ~~:K:~ .\:. to) ";'';: t ~~: :- 1.: ~ ':':<k; .. .:...."t' a: ~~~ -J:,t;r :....:&., --' K_-~--.... ---.-r-----.. ----:J 11121188 42 'Nhite F 60 x x x findings in the specimens were typical of polycystic liver • I disease. Survival Three of the four recipients have been well from 8 months to ,vears postoperatively. Patient 4 (Table) recovered com-~l~tely from a liver-kidney transplantation, but 5 months 13ter. she experienced fulminant hepatic failure caused by heps: ; tis B virus. She died 2 days after emergency liver ~transplantationK The transplant hospitalization was for 35, ~I 71, and 99 days. The longest hospitalizations were re-quired because of pneumonitis (patient 3, Table) and a postop-erative psychosis (patient 1, Table). Function of Grafts x I The liver function has been normal in the surviving recipi-x "I ' ents and was normal for 5 months in patient 4 (Table) until the ----_-1 onset of acute hepatitis. The subnormal renal function in _____ -1 patients 1 and 4 was improved with the renal grafts (Table). ___ x___ .J..J· The ;'-eviously normal renal function was decreased in pa-___ x ___ J : tient: 2 and 3 (Table) who were given a liver only; their loss of J renal function was ascribed to cyclosporine nephrotoxicity.· ____ 1_3 ___ ~g COMMENT Cyst aspiration colostomy and Surgical complications of polycystic liver disease and ways cecostomy of treating these have been reported from clinics with special 12790 1 ~ 0.58 0.83 s.p#. f :ally. At lepatic expertise in hepatic disease. I ,'.ID-a Such complications were thought to be rare until recently. The study by Grunfeld et all provided a more realistic assessment of the hepatic risks to these patients now that death from renal failure could be fores'. ~Klled so effectively. In patients with renal polycystic diseai~ who were undergoing dialysis at the Hopital Necker, Paris, France,' a hepatic origin was the leading cause of death. I Findings from subsequent reports from other cen-terss, have confirmed that there is a small subset of high-risk patients. Initially, it was difficult for us to recommend such a drastic solution as liver transplantation for patients with polycystic liver disease who typically have normal liver function. Liver transplantation was not even considered until 1984, when another patient with polycystic disease was brought in des-perat,:, condition to the operating room for a combined liver and l>dney transplantation, only to die of a fatal pulmonary I embolus before either organ could be transplanted. Instead, , ~e have tried in past years to reduce the hepatic mass in such patients with a partial hepatectomy and with the fenestration procedure, as originally described by Lin and colleagues. II Although these operations can relieve symptoms temporar-ily, usually there is little reduction in the hepatomegaly. I A right hepatic lobectomy and fenestration operation in our patient 3 (Table) left so many adhesions that liver replace-ment nearly 8 years later was extraordinarily difficult. An exceptionally conservative attitude is still warranted about liver transplantation or, for that matter, any other operatIOn for polycystic liver disease. The most common rea-SOn to go forv.'aTd could be a syndrome of lethal exhaustion. The diagnosis of this condition may depend more on common ~nse and longitudinal clinical observations than on sophisti-cated tests. All four of our patients had reached an end to their functional lives because they could no longer carry the weight of~heir slowly enlarging livers. Three of them had intractable ilam, and two patients were narcotic addicts. Fatigue had closed off almost all activity, except that required to breath ~d carry out other basic bodily functions. Peltokallio" may I nave s"en patients 'with this kind of disability, and one patient III GrJnfeld and colleagues' series died of cachexia. The com-Plaints in our four patients were eliminated with the removal of the mass and transplantation of a normal-sized organ. ease-Starzfet J A.rct,Surg-vol125, May 1990 A » Weaning from ventilator support was possible in 2 to 15 days. The two recipients who had become narcotic addicts never again asked for pain medications, even during the first post-operative days. The patients all regained a vigorous life-style, and all remained well, except for the liver-kidney recipient who e..-.;perienced fulminant hepatic failure from hepatitis B virus after 5 months and died despite emergency liver retransplantation. Whether to provide a kidney at the same time as liver replacement in patients with polycystic disease of both organs is a matter of judgment. Because the nephrotoxicity of cyclo-sporine is the limiting factor in the dose of this drug,· subnor-mal function of native kidneys that still may be life supporting could make it impossible to give enough cyclosporine to pro-tect the new liver. It was this consideration that prompted the decision in two of our four patients to transplant a kidney from the donor who also provided the liver. Under normal circUl,1-stances, it would have been too early in the course of the renal failure to have warranted kidney grafting. If a decision is taken to transplant a kidney, there are good reasons to obtain the kidney from the liver donor, especially if the patient's serum contains the wide-ranging cytotoxic anti-bodies that can promptly destroy renal homografts. The liver is peculiarly resistant to this kind of antibody attack," and once the hepatic graft is in place, it can remove or neutralize the donor-specific antibodies, thereby allowing a kidney from the same donor ro survive in the immunologically hostile environment. '6 Fbr a patient who is highly sensitized, this may be the only opportunity to transplant a kidney with a reasonable expectation of success. This study was supported by research grantll from the US Department of Veterans A1fairs, Washington, DC, and by projeet grant OK 29961 from the Nstionallmtitutes of Health , Bethesda, Md. References 1. Dalgaard OA. Bilateral polycystic disease of the kidneys. Acta Med &and. 195i:8(suppl328):1-255. 2. Griin!eld JP, AIbouze G, Jungers P, et aI. Liver changes and complica-tions in aduh polycystic kidney disease. Adv Nephrol. 1985;14:1-20. 3. van Erpecurn KJ, Janssens AR, ThrpstraJL, Tjon A, Tham RTO. Highly symptomatic adult polycystic disease of the liver: a report of fifteen cases. J HepaWl. 198i;5:109-117. 4. Turnage RH, Eckhauser FE, Knol JA, Thompson NW. Therapeutic dilellllllllS in patients ..nth syptomatic polycystic liver disease. Am Surg. 1988;4:365-372. 5. Cappell MS. Obstructive jaundice from benign, nonparasitic hepatic cystll: identification of risk factors and percutaneous aspiration for diagnosis and treatment. Am J Gastroenterol. 1988;83:93-96. 6. Van Truel DH, Hagler NG, Schade RR, et aI. In vivo hepatic volume detennination using sonography and computed tomography. G<utroenlerol-ogy. June 1. 1985;88:1812-1817. 7. StarzI TE, Iwatsuki S, Esquivel CO, et aI. Refinementll in the surgical ~que oflivertranspiantation. Semin Liver Dis. 1985;5:349-356. . Kwok HK,Lewin KJ. Massive hepatomegaly in adult polycystic liver b . Am J Surg lbtJwl. 1988;12:321-324. 9. Myers B, Newwn L, Boshkos C, et aI. Chronic injury of human renal microvessels with low-dose cyc\osporine therapy. 7ranspwnta/ion. 1988; 46:694-703. 10, Henson SW, Gray HK, Dockerty MB. Benign tumors of the liver, IV: polycystic disease of surgical significance. Surg Gymcol Obate!. 1957;104: ~TK 11. LloylWones W, Mountain Je, Warren KW. Symptomatic non·psrasitic eyats of the 1Wer. Br J Surg. 1974;61:118-123. 12. Longmire WP, Mandiola SA,Gordon HE. Congenital cystic disease of the liver and biliary system. Ann Surg.1971;174:7U-726. 13. Lin T'i, Chen ce, Wang SM. Treatment of non-parasitic cystic disease of the liver: a new approach to therapy "D~th polycystic liver. Ann Surg. 1968;168:921-927. 14. Peltokallio V. Non-parasitic cystll of the liver: a clinical study of 117 cases. Ann CltirGyntUrol. 1970;59:1-58. 15. StarzI TE, Demetris AJ, 'lbdo S, et aI. Evidence for hyperacute rejection of human Ii'"l!r grafts: the case of the canary kidneys. Clin 7raMpwnt. 1989;3:37-48. 16. Fung JJ, Griffin M, Duquesnoy R, Shaw B, Starzl TE. Succeasful sequential Iner-kidney transplantation in a patient with prefonned lymphocy· toxic antibodies. 7Tamplant Proc. 1987;19:767-768. Polycystic Liver Disease-Starzl at aI sn 

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Liver Transplantation for Polycystic Liver Disease

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