Liver Transplantation for Budd-Chiari Syndrome

Charles W. Putnam. MD; Kendrick A. Porter. MD; Richard Well III. MD; H. A. S. Reid. MRC Path; Thomas E. StarzI. MD, PhD

• Orthotopic liver tran.plantatlon ... accompllahed In a 22-year-old transplantation. Two episodes of fever woman dying of the Budd-Chlarl syndrome. She I. well and has normal liver sociated with elevated liver enzymes functton 16 month. po.toperatively. In ¥lew of the good earty mult, it will be thought to represent cholangitis and appropriate to consider liver repiacement for this dlseaaeln further well-.. easily controlled with antibiotics. lected ca.... ently, she has normal liver function (JAIfA 236:1142-1143, 1976) is completely well sixteen months afW transplantation.

PATIENTS with many kinds of end­ which eventually could not be controlled by Histopathologic Changes . if; stage liver disease may be candidates diuretics. Despite a low-protein diet, epi­ The hepatic veins, particularly thOlle of for hepatic transplantation.'" We lOdes of hepatic encephalopathy requiring medium and large size, were occluded by record here the first use of liver re­ neomycin sulfate became more frequent. organized and recanalized thrombus (Fig She had demonstrable esophageal varices placement for the treatment of the 2, left). These changes were associated ~, but did not bleed from these. The serum Budd-Chiari syndrome. with I08S of hepatocytes from the centnl.J bilirubin concentration increased to about and middle zones of the lobules, with only a '~; Report of a Cue 2.9 mg/lOO mi, and the serum albumin con­ rim of liver cells surviving at the periphery .~: centration remained below 3.0 gm/lOO ml, A 22-year-old woman was in good health of the lobules (Fig 2, middle). There was ." even with intermittent infusions of con­ until one year prior to transplantation, increased reticulin and fibrous tissue in the,~ .'; centrated albumin. The prothrombin time when she noticed fatigue associated with a centers of the lobules, and fibrous septa , averaged 18 to 22 aemnds while she was re­ voluntary weight-reduction diet. In No­ linked adjacent central zones. Some retieD- : ceiving vitamin K (Aquamephyton). vember and December 1974 she received lin strands also extended from the central ~ . Two months prior to transplantation, re­ two fHiay courses of medroxyprogesterone to the portal areas and were accompanjed· nal fUDCtion began to deteriorate. At the acetate (Provera), in an effort to regulate by regeneration nodules (Fig 2, right). Bile ; time of operation, the blood urea nitrogen her menstrual cycle. Two months later, ductules were present in the centrilobular.* was 56 mg/l00 ml; serum creatinine. 2.9 while living in Germany, she complained of connective tissue. The sinusoids in the ceu- .~. mg/l00 ml, and urine sodium excretion, right upper quadrant pain, nausea, vomit­ tral and middle parts of the lobules ware· Iesa than 1 mEq/liter. ing, and abdominal distention. A needle dilated. and some had formed large, thin- After obtaining informed consent, he­ biopsy of the liver was suggestive of the walled, bypass channels. . patic transplantation was performed on Budd-Chiari syndrome. The diagnosis was Nov 28. 1974. The dauor was a 21-vear-old established at Walter Reed Army Hospital woman who hacfsuffered • lethal head in­ Comment in March 1974 by a venac:avogram (Fig 1) jury in an automobile accident. The donor that showed inferior vena caval narrowing Parker as well as recent' revieww and recipient had mismatches of all four and occlusion of the hepatic veins. Repeat have demonstrated the poor outlook­ antigens of the HLA-A and HLA-B loci. venac:avography at Letterman Army Med­ At exploration, the liver was enlarged, with the Budd-Chiari syndrome. In ical Center confirmed the diagnosis. weighing 2,035 gIn. Large, soft nodules the Royal Free Hospital series of During the next six months, her liver protruded from its surface. The spleen was Tavill et al: 17 of their 19 patienta function deteriorated. There was progres­ also enlarged. Dense fibrosis involved the died of hepatic failure, with or with­ sive enlargement of the liver and spleen, retrohepatic inferior vena cava, as well as out gastrointestinal bleeding, from and spider angiomata developed. She bad six weeks to 3~ years after the increasing ascites and peripheral edema, the right and left he!-tic veins. The latter 0_ were nearly occluded by scarring. The do­ of symptoms. However, some patiellW. nor liver was revacularized orthotop­ can recover with supportive therapy'"! ically.I,' Biliary drainage was established From the Veterans AdministratiOn HoePtat and others may benefit from # with a Roux-en-Y choIecystojejunostomy.' ana the Department of Surgery. Univeraity of talsystemic shunt operations.'" For. Postoperatively, the patient received Colorado Medical Center. Denver (Drs Putnam. this reason, a decision to recomm_ Weil. and Stanl); ana the Department of Pathol­ azathioprine, prednisone, and a three-week ogy. St Mary'S Hospital Medical Schoo. london course of heterologous antilymphocyte transplantation could not be justl­ (Drs Porter ena Reia). fiably reached by the referring physi­ f'leprint requests to Department of Surgery, globulin. I., Liver rejection did not occur. University of Coloraao Medical Center. 4200 E Renal function improved rapidly and was cians until late in the course of tRi Ninth Ave. Denver. CO 80220 (Or Putnam). entirely normal within three weeks of patient's illness, and only after tbIt:

1142 JAMA, Sept 6, 1976-Vol 236, NO.10 Liver Transplantation-Putnam ------

,f f en'l ,,­ :ymes \, r" sand \' rt­ )tics. P ,. ··S· mction and mths alter

FIg 1.-lnferior venacavogram showing ta­ Rg 2.-Features of diseased native liver. Left, Medium-sized hepatic vein (HV) occluded I*in9 and stenosis (larger arrow) of inte­ by organized and recanatized thrombus. Middle, Uver lobule with occlusion of central 1ge. lionena cava below site of entry of right vein (arrow), loss of all hepatocytes except those adjacent to portal tracts, and dilation 'Iy thOSE' of lid left hepatic veins. Small arrows indi- of sinusoids. Right, Regeneration nodule (RN) adjacent to portal tract; central vein (ar­ lCcluded by 0IIIl approximate levels of eventual upper row) is occluded (Silver stain for reticulin counterstained with neutral red, x 20). 1mbus (T-il! .IIId Jewer vena caval anastomoses. ,;.:..: .... associa cd ,.11;;,,- , the cen' 'al with on: . a e periphery jrI¥e prognostic signs of encephalop­ the venacavogram (Fig 1). assuring ReIereI .... There was ,..., and the hepatorenal syndrome an adequate venous outflow for the L Starzl TE. Porter KA, Putnam CW, et al: ,issue in the ...·.ppeared. Orthotopic liver traDllptantation in 93 patients. Jrous septa homograft. Surg Gyr&«d Otm.t 142:487-005,1976. ,ome reticn· Parker" and Tavill et al' could find 2. Starzl TEo Putnam CW: ~ ill He­ .. explanation for the hepatic vein patie Tm,...,1&IGtioa. Philadelphia, WB Saun­ the centrai This work was BIIPported by I"IIIIIW'Cb projeeta den! Co, 19619. ceompanied iIIromboses in 70% and 50%, respec­ MRIS 8118-01 and 722'1~1 from the Veterans 3. Parker RGF: Oc:clusion of the bepatic veins right), Bile iiIely. of their cases. Nor could we in Administration; by grants AM-I7260 and AM- in man. Medici ... 38:369-402, 1959. 07772 from the National Institutes of Health; entrilobu; a r oar patient, either at operation or by 4. Tavill AS, Wood EJ, Kreel L, et al: Tbe and by grants RR-00051 and RR-00069 from the Budd-Cbiari syndrome: Correlation between b~ ; in the c· n­ pathologic analysis of the liver. The General Clinical Reaean:b Centers Program of patie scintigraphy and the clinical, radiological. )bules w r~ NIIIOVal of tbe diseased native liver the Division of Reaean:b Reaeurces, National In­ and pathologicallindings in nineteen cases of be­ large, tr n· stitutes of Health. patie venoua outftow obstruction. &UtTDBlltn-· .. made difficult by dense fibrosis LTC George Bunlidt, MC, USA, MAJ William olow 68:509-618. 1975. :lnIImd the suprabepatic and retro­ PetenlOD, MC, USA; and MAJ Robert 1.. Myers, 5. Langer B, Stone RH, Colapinto RF, et al: Iw.patic inferior vena cava, but the Me, USA; Letterman Army Medical Cenber; and Clinical apectrum of the Budd-Chiari syndrome Rodi Sehmid, MD, Univenlity of California, San and its surgical management. Am J Su'1l iepateetomy was facilitated by a re­ Franeiaco, referred this patient. 129:137-140, 1975. t· reviews CetJy -described retrograde teeh­ 6. Sdlramek A. Better OS, Brook JG, et a1: New Ot.ervatioDll in the clinical apeetrmn of the or outlook ~ that allowed the dissection of Budd-Chiari syndrome. Aft" Surg 18&.368-372, drome. In _~ suprahepatic vena cava to be 1974. NanproprteWy ...... 7. Prandi D, Rueff B, Beuhamou JP: Side-to­ ~ries of ~JiII!I*poned until just before removal MdT...... oIDnig Bide portaI:aval mWlt in the treatment of Budd­ 9 patients ;~ specimen. This dissection went Cbiari syndrome. Gat~ 68:137-141, h or wi d 1- .~ above the constrietion seen on 1975. :ing, fro m ~~f;;.· ~ the on ct -~'if/ )·k~. Ie patie .. t5 .. ~, ~ therapy" from por­ :JOS.'·' For 'ecommend be justi­ ring phy5i­ lrse of (lUr after: elf .lAMA, Sept 6. 1976-Vol 236. No. 10 Liver Transplantation-Putnam et al 1143 'utnam.:- 3'