Postgraduate Medical Journal (August 1982) 58, 511-514

Treatment of the Budd-Chiari syndrome in polycythemia vera by repeatedpercutaneous transluminal angioplasty of a hepatic vein stenosis

M. NISHIKAWA K. SEKIM.D. M.D.

S. MIYOSHIM.D.

Y. IMAIM.D.

S. TARUIM.D.

Y. MINAMIM.D.

S. KAWATAM.D.

H. NAKAMURA*M.D.

The Second Department of Internal Medicine, and *Department of Radiology, Osaka University Medical School,Osaka, Japan

SummaryThis report is of a 63-year-old man with polycythemiavera who developed the Budd-Chiari syndrome due toright hepatic vein stenosis. Diagnosis was made bylaparoscopy and liver biopsy, and confirmed byhepatic venography. The patient was treated bypercutaneous transluminal angioplasty, and recov-ered completely from ascites, leg oedema and venousstasis. No pulmonary embolism was observed. Onemonth after angioplasty, a second laparoscopy andliver biopsy showed a marked improvement in hepaticcongestion and haemorrhagic necrosis, thereby con-firming the effectiveness of this technique in treatingthe Budd-Chiari syndrome. Further treatments withpercutaneous transluminal angioplasty were requiredwith a good clinical outcome.

Introduction

Occlusion of the hepatic veins, or the Budd-Chiarisyndrome, is a rare condition which is fatal in mostcases. Characteristic symptoms are abdominal pain,enlarged liver, ascites and leg oedema. These are dueto congestion of the liver and portal hypertension.Therapeutic measures are therefore directed towardsrelieving the congestion and lowering the portalhypertension. This paper reports a case of the Budd-Chiari syndrome in which successful treatment was

Correspondence: Masahiro Nishikawa, M.D. The Second Depart-ment of Internal Medicine, Osaka University Medical School,Fukushima, Fukushima-ku, Osaka, Japan, 553

achieved by repeated percutaneous transluminalangioplasty (PTA) of the stenosed hepatic vein.

Case reportA 63-year-old man was admitted in August 1980,

because of leg oedema. He had been in good healthuntil April 1980, when he noted a weight-gain ofapproximately 5 kg. A doctor noticed an enlargedliver, ascites, leg oedema and polycythaemia. Thepatient was transferred to our hospital for furtherexamination.

Physical examination on admission revealed di-lated abdominal veins in the epigastric region, anenlarged liver 4 cm below the right costal margin, thespleen 1-5 cm below the left, and bilateral legoedema.

Laboratory investigations showed haemoglobin19-2 g/dl haematocrit 0-56, white cell count, plateletsand erythrocyte sedimentation rate normal. Total redcell volume 57-7 ml/kg (normal 26-33), leucocytealkaline phosphatase score 432 (normal 15-100),glutamic oxaloacetatic transaminase (GOT) 20 u./litre (normal 0-25), alkaline phosphatase 1110 u./litre (normal 33-164), bilirubin 2 mg/dl, albumin 36g/litre, plasma ammonia (NH3) 1500 /g/litre (normal180-480), indocyanine green retention rate (0-5mg/kg) at 15 min (ICGR,5) 29% (normal 0-10),prothrombin time 67%, fibrin degradation productsnormal. Serum creatinine, urinalysis, blood electro-lytes, serological tests, and chest X-ray were all

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512 Clinical reports

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FIG. 1. Liver biopsy, showing intense congestion and centrilobular haemorrhagic necrosis. The sinusoids are dilated. (HE, x 100).

W~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~.. ......... ..... .........49>R w wC* f ~~~~~~~~~~.. ... .. . ....... 44ITE-

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FIG. 2. Hepatic venogram, showing the dilated right hepatic vein.The catheter passed through a stenosed segment.

normal. Electrocardiogram showed an old anterosep-tal myocardial infarction. Computed tomography(CT) liver scan showed a hypertrophic caudate lobe,but liver scintiscan showed no central uptake. Atlaparoscopy, congestion in the right lobe of the liverwas noticed, and histology of the liver biopsyspecimen showed centrilobular haemorrhagic necro-sis (Fig. 1). Hepatic venogram revealed stenosis oftheright hepatic vein near the inferior vena cava (IVC)(Fig. 2). IVC-gram showed no obstruction of the IVCexcept for a narrowing of the hepatic segment due toan enlarged caudate lobe.

Figure 3 illustrates the course of the patient'sillness. Percutaneous transluminal angioplasty (PTA)as described by Gruntzig (1978) was performed usinga balloon of diameter 6 mm. Leg oedema anddilatation of the abdominal veins disappeared after afew days of diuresis, and the liver diminished in sizefrom 4 cm to 1-5 cm below the right costal margin.Plasma ammonia fell to the normal range. Alkalinephosphatase also fell to 550 u./litre. ICGRI5 improvedto 12%. One month later, a second laparoscopy andliver biopsy was performed. Neither hepatic conges-tion nor haemorrhagic necrosis was observed (Fig. 4).However, 21 months after PTA, the patient againdeveloped hepatomegaly and leg oedema. Plasmaammonia, alkaline phosphatase and ICGRI5 againrose. A second PTA was performed 3 months later,followed by venesections, but the effect wasevanescent. One month later, a third PTA

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Clinical reports 513Liver

T Oedemot

Ht(%)M J(IPTA(2)s2) PTAI3) PTA 4

601 V e

501Al-_p 401x(uA/I.) ICGR1529% 29 1 5 12 24 30 30 25 33 1 5 19 18 1 5

NHNH3 \-p

(jg/I)1.000-

1.500

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FIG. 3. Clinical course. Ht, haematocrit; PTA, percutaneous transluminal angioplasty; AL-P, alkaline phosphatase; GOT, glutamic oxalacetictransaminase; ICGR5s, indocyanine green retention time at 15 min.

4 A~~~~~~~~~~~~~~~~~~~~~4.~~~~~-

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FIG. 4. Liver biopsy one month after the first PTA. Parenchyma in the central area has partially replaced fibrous tissues, but the haemorrhageshave disappeared. (HE, x 100).

was performed with anticoagulant therapy (heparin10 u./kg/hr). A fourth PTA was performed using alarger balloon (diameter 8 mm) after a furtherinterval of one month. After which, the pressuregradient between the right hepatic vein and the IVCfell to 4-1 cmH20. The patient fully recovered, theplasma ammonia remains normal, the alkaline phos-

phatase ranges from 500 to 600 u./litre, and ICGRI5 is15% 4 months after the final PTA.

DiscussionThe Budd-Chiari syndrome, caused by hepatic

vein thrombosis or IVC obstruction, is characterized

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by hepatosplenomegaly, ascites, leg oedema, mildjaundice and oesophageal varices due to congestionof the liver and portal hypertension. If decompres-sion is not performed, the portal hypertension symp-toms become more prominent and eventually thepatient dies of gastrointestinal haemorrhage or ofhepatic coma (Reynolds and Peters, 1975). TheBudd-Chiari syndrome following membranous ob-struction of the IVC can be successfully treatedsurgically using transcardiac membranotomy (Ki-mura et al., 1963; Takeuchi et al., 1971). Parker(1959), reviewing 149 reported cases plus 14 cases ofhis own, found polycythemia vera was the mostcommon cause of hepatic vein thrombosis whenportacaval shunt (Orloff and Johansen, 1978) andportopulmonary shunt (Akita and Sakoda, 1980)have been common choices for treatment. Theseprocedures show favourable results in decreasingportal hypertension, but also have a high incidence ofpostoperative encephalopathy.The efficacy of PTA by the technique described by

Gruntzig (1978) has been attested to in variousobstructive diseases (Gruntzig et al., 1978; Rankin etal., 1981). In this patient, congestion of the liver andportal hypertension markedly diminished after PTA.Efficacy was also confirmed by laparoscopy and liverbiopsy. The patient, however, developed hepatome-galy, leg oedema and venous stasis again 21 monthsafter the first PTA, and required further PTAtreatment. Although PTA was effective in the reliefof the congestion, venesection after the second PTAwas not effective in preventing a recurrence of thestenosis. Anticoagulant therapy (Langer et al., 1975)after the third PTA, on the other hand, had abeneficial effect not only in prevention of therecurrence but also in the relief of the congestion.This suggests that a state of hypercoagulability was

present in the liver due to blood stasis, especiallywhen FDP is in the normal range, as was the case inthis patient.PTA is relatively easy to perform, repeatable, and

is more effective than normal operative procedures.This study also indicated the efficacy of applyinganticoagulant therapy in conjunction with PTA incases of the type described in this paper.

ReferencesAKITA, H. & SAKODA, K. (1980) Portopulmonary shunt bysplenopneumopexy as a surgical treatment of Budd-Chiari syn-drome. Surgery, 87, 85.

GRUNTZIG, A. (1978) Transluminal dilatation of coronary-arterystenosis. Lancet, 1, 263.

GRUNTZIG, A., KUHLMANN, U., VETTER, W., LUTOLF, U., MEIER,B. & SIEGENTHALER, W. (1978) Treatment of renovascularhypertension with percutaneus transluminal dilatation of a renalartery stenosis. Lancet, i, 264.

KIMURA, T., SHIROTANI, H., TSUNEKAWA, K., HAYASHI, K.,MATSUDA, S., HIROOKA, H., TERADA, M., IWAHASHI, K.,MAETANI, S. & IKADA, H. (1963) Membranous obliteration ofinferior vena cava in the hepatic part. Nihonrinsho, 21, 125.

LANGER, B., STONE, R.M., COLAPINTO, R.F., MEINDOK, H.,PHILIPS, M.J. & FISHER, M.M. (1975) Clinical spectrum of theBudd-Chiari syndrome and its surgical management. AmericanJournal of Surgery, 129, 137.

ORLOFF, M.J. & JOHANSEN, K.H. (1978) Treatment of Budd-Chiarisyndrome by side-to-side portacaval shunt: Experimental andclinical results. Annals of Surgery, 18, 494.

PARKER, R.G.F. (1959) Occlusion of the hepatic vein in man.Medicine, 38, 369.

RANKIN, R.N., KEOWEN, P.A., ULAN, R.A. & STILLER, C.R. (1981)Percutaneus transluminal dilatation of transplant renal arterystenosis. Postgraduate Medical Journal, 57, 300.

REYNOLDS, T.B., & PETERS, R.L. (1975) Budd-Chiarisyndrome. (Edby Schiff, L.), 4th edn, p. 1402. J. B. Lippincott, Philadelphia.

TAKEUCHI, J., TAKADA, A., HASUMURA, Y., MATSUDA, Y. &IKEGAMI, F. (1971) Budd-Chiari syndrome associated withobstruction of inferior vena cava. American Journal of Medicine,51, 11.

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