Hepatic vein webs and resistant ascites

Het)atic

vein webs and resistact

Diagnosis, management

Webs of the hepatic veins have ken

rarefy described.

ascites

and implications

Two patiwts

presented with intractable ascites in whom

hepatic venography dsmonstrated this unusual hnding. Liver bicpsies from both patients showed variable patterns of perivenular fibrosis. Ttansvenous balloon dilatation of the web was effective in abolishing the pressure gradient across the obstruction and improving hepatic venous outflow. Both patients showed imprwemtnt

a dramatic

clinical

following the procedure. with complete resolution of ascites in one patient and considerable im-

provement in the other with symptoms not recutting over 18 months of follow-up. The histological features that help to dktinguish bepatic venous outflcw obstruction from other caases of petivemdar fibrosis are desctibed.

ed improvement of ascites using translumin~! balloon angioplasly of the stenosis [4,6-a]. Diuretic

resistant ascites is usualiy a sip

of ad-

Gther measures

to co.,;ru! ascites such as peritoneovenous,

meswa-

vanced chronic liver disease and may also occur with

val or mesoatrial shunts, or definitive

the Budd-Chiari

have numercus mmplications and mottality in excess

syndrome. Transplantation

of the

liver in selected patients is the final treatment option available once alternative measures such as paractntesis have failed to control symptoms. Membranous webs of the upp~ inferior vena cava have been widely reported from Japan and South Africa [l-S]

as a cause of &s.tructioa

surgery all

or 30% [14,15]. We report two patients with diuretic-resistant cites who were referred for liver traesplantation.

asThe

histological features of the liver from both patients had suggested a diagnosis of alcoholic liver disease

of hepatic venous

and chronic hepatitis. In both patients a web obstrue-

outflow; similar webs of the hepatic veins have been

tion of a lmge hepatic vein was noted at venography.

rarely described !6,7]. A mlnlber of reports have cit-

Balloon dilatation was rapidly effective in abolishing

CR.

288

et al.

“KXERS

li, RA

7mm

5mm

mg. 1. Hepatic vein web (arrowed) ol the right hspatic vein (patient I). or reducing ascites formation and improving the general health of the patients. thus avoiding the foreseeable need for transplantation.

Rapid development of gross ascites complicated the

Case histories

liver biopsy and this has controlled

with diuretic

treatment.

Patient I

Eighteen An asymptomatic 42-year-oic’ nait: was iouiid io

months following

tions. the patient developed

the initial recurrent

investipascitrs and

have hepatomegaiy at a routine clinical examination

dyspnoea. A large right pleura! effusion was dig

2 years prior to referral. He had an alcohol consurnp-

nosed which contained

lion of up to 80 g/day and no previous history of liver

(18-23 g/l) to the ascitic fluid. Repeated thoracocen-

a similar

protein

contenl

disease. Serum liver enzymes showed the serum p

teses of up to 5 litres per week were necessary to pre-

glutamyl transpeptidase to be 192 U/I (normal, 6-25

vent dyspnoea; lar~$ doses of diuretics (spironolac-

WI),

tone MO mg and frusemide 120 mg daily) failing to

alkaline phosphatase 132 U/I (normal. 70-350

Uii) and aspartate aminotransferase 28 U/l (normal,

control fluid reaccumulation.

5-30 WI). The serum albumin and prothrombin time

ferred subsequently for liver transplantation.

were normal. He ceased consumption of alcohoi and liver eozymes reverted to the normal range. Hepato-

and had muscle wasting. Liver enzymes were within

megaly

the normal range although hypoalbuminaemia,

persisted,

however,

and

a percutaneous

The

On admission to the Liver Unit

patient was rehe felt lethargic 23 fl

biopsy of the righ! !obe of the liver showed extensive

(normal, 33-48 g/l), was present. thought to be a re-

perivenular hepatocyte loss and fibrosis: bridging fi-

sult of repeated thoracccenteses.

brosis was present, but there was no evidence of an established cirrhosis. These changes were thought to be consistent with previous alcoholic liver injury.

Radiological invesdgarionr Hepatic hydrothorsx was confirmed by an intrape-

HEPATK

VEIN WERS

289

right lobes of ale liver. Histology of the right lobe showed progr: scion of fibrosis with severe scarring now present. in contrast, hiitoiogy of the left lobe was nearly normsi, with only a minor degree of sinusoidal fibrosis. On review, both biopsies, and the biopsy taken 2 years earlier, showed areas of sinusoidal dilatation and the histological features were thcughs :rr 5c consistent with chronic veno’us outflow sbstrucrion alone.

The patient bad a remarkable and rapid improvement in symptoms following the diiatatioxt. The ascites and pleural effusion did not reaccumu:ate and all diuretic treatment was discontinued. The serum zlbumin subsequently rose to 32 g!l and ‘he gained weight. Progress hepatic venogmphy performed at 6 and 12 months did not show redersk+ncnt of the stenosis and the pressure gradient remained absent. The patient remains clinicaNy well 1.9months after thedilatation.

ritoneal radionuclide injection of technetirrm-99 sulphur colloid. Two hours post injection, high levek of isotope activity were detected in the right thoracic cavity. Ultrasonography of the liver showed considerable dilatation of the right hepatic vein with second-order branches clearly shown, a finding usually associated with raised inferior vena cava pressures. Hepatie vein catheterisation, from a jugular approach, showed a web-like obstruction of the right hepatic vein (Fig. 1) with pressures as shown (Fig. 2). A gradient of 18 mmHg was present across the stenosis. The catheter was exchanged for a 10 mm angioplasty balloon and the web was dilated (Fig. 3). Pressure gradients were abolished immediately, with a good improvement in venous return. A mesenteric aneriographic subtfaction study demonstrated normal portal vascular anatomy. Further biopshs were taken of both the left and

This Z-year-old female was referred for liver transp!antatioa bewnse of resistant ascites, gross peripheral oedema and cirrhosis. She had originally presented at age 9 with sudden onSet of ascites. Liver biopsy was reported to show chronic active hepatitis and she was treated with corticosteroids. Diuretics controlled the formation of ascites until the age of 15 when a LeVeen peritoneovenous shunt was inszrted. Progress liver biopsy at that time had shown cirrhosis. TheLeVeenshuntcontroiiedsymptomsfor4yycars, at which time the ascites recurred and the shunt was found to be outgrown md dislodged from the subclavian vein. The shunt was removed and was replaced with a Denver sham This shunt later thrombosed and was also removed. Diuretics (spironolactone 800 mg and frosemide 180 mg daily) were ieeffective at controlling ascites formation and paracenteses of up to 10 litres per fortnight were required to keep the pa:bnr comfonable. Liver enzymes were normal although hypoalbuminaemia of 17 ~$1, presumably

290

CR. VKKEPS

1 al.

291

HEPATIC “EIN WEBS front the paracenteses, was presen:. On review of the liver biopsies, the overall pattern WASthought to be consistent with chronic vcxu:: outflow obstruction. A striking feature m the later biopsy was the presence of sex’ere perivctiu!ar sud bridging fibrosis alternating wth anti uI ruir?ix;l fibmsis; occasional areas of sinusoidal dilatation and congested vascular channels were also present (Fig. 4). Radiological

invesdgotiom

A technetium-99 radionuclide liver scan showed a small liver with increased isotope activity in an mlarged caudate lobe, suggestive of a chronic BbddChiari syndrome. Ultrasound showed an echobright liver with no dilatation of the hepatic veins. Hepatic venography showed a membranous-type web obstruction acrass thr right hepatic vein near its junction with the inferior vena cava. A gradient of I mmHg was present nous cclfateral ‘spider’s

acres’s the stenosis.

formati‘m

web’ p?ttcm

was F-esent

seen

in chronic

Extensive

v;-

typical of the Budd-Chiari

syndrome. Using a similar technique as in the first case, a 10 mm balloon dilatation catheter was pushed through the stenosis from the right innominate vein and the web was dilated. An inferior vena cavagram showed no other web obstructions, but a fibrin cast of the Denver shunt was preseut. A repea! venogrnphy study after 1 week rhowed that the pressure gradient had been abolished, The ascites and leg oedema became much less of a clinical problem folIowing the dilatation and diuretic treatment was reduced. The serum albumin subsequently increased from I7 g/I to 41 g/l. Follow-up The patient underwent repeat dilatation of the web at 3 and 6 months because of residual ascites. The gradient remained abolished. She remaks welt afic; 18 months, but with residual mild ascites controlled on diuretic treatment. The legoedema has resolved.

These two cases illustrate the usefulness of exclud-

ing hepatic venous outflow obstruction in patients with resistan! ascites. Both patients responded well to hallonn dilatation of their hepatic vein web whiih has prevented or delayed the need for liver trans$mta?ion.

Tbe aetiology of hepatic vein webs and their relationship to membranous ?bstrurtion of the inferior vena cava is of great interest. Zahn (1889) j16] and Simson [5] have considereti the iatter to be of congenital origin. based on the presence of other anatomical abnormalities of the hepatic vein ostia and intrahepatic portion of the inferior vena cava noted in post-mortem specimens. A high incidence of primary hepatocellular carcinoma has also been noted in associaticn with the inferior vena cava webs in South Africa 151. At least ten anatomic variations have been described for the location of membranous webs in the intrahepatic segment of the inferior vena cava [l]. Our two cases confirm that there is an eleventh site, this being in a major hepatic vein. Okuda [I:] has alternatively proposed that the webs aCitthe end result of a recanalised thrombus. Intraluminal webs or fibrous bands are a relatively common finding in other vessels involve3 in chronic thromboembolic disease, particularly the pulmonary ar:eries. where they form fibrous strands, bulbs, networks or webs. Histological examination of these lesions shows that they coasist predominaotly of collatn. fibroblasts, e!astic tissue and an eudothelial lining [18], similar to that from webs ofthe inferior vena cava [5,19]. Our second case is probably an example of a web resulting from recanalised thmmbus; the juvenile presentation with ascites being a consequence of the Budd-Chiati syndrome. We have also seen a membranous-type defect continuous with an organising thromhus within a large hepatic vein in a case of chronic Budd-Chiafi syndrome coming to transplantation. It is difficult to be certain of the aetiology of the web in our first case, A pre-existing occult lesion, possibly from a subclinical venous thrombosis, must have been present to explain the histology found on his i;wt liver biopsy. The rapid development of ascites following the biopsy was likely the result of puncture of a dilated surface lymphatic. The initial histological interpretation of the liver

biopsies from both patients were misleading, being

liver may also assist in determining the presence of a

said to show, respectively, alcoholic liver disease and

diffuse or lobar hepatic fibrosis.

a chronic active hepatitis with progression to cirrhosis. Chronic venous outflow

obstruction is charac-

These cases emphasize the need for hepatic venography and careful review of histology in patients

terised by fibrosis around central veins. This pattern

with resistant ascites. Balloon dilatation of the hep

of damage may resemMe other conditiorrs such as al-

atic vein web is effective in relieving ascites forma-

coholic perivenular

fibrosis and central byaline ne-

crosis 1201; it may also progrw

to cirrhosis [19]. The

tion. Repeat procedures may be needed to prevent restcnosis. Chamsangawj

has recwtly

reported pre-

presence, however, of sinusoidal dilatation aad con-

liminary

gestion should be a clue to the underlying aetiology.

metal Sent to reduce the risk of vascular restenosis

These features are often not prominent

resu!ts using the Gianturco

expandable

in cases of

following balloon dilatation [21]. This technique may

chronic venous congestion (ii] comparisoo to acute

be of potential benefit to lesions of the hepatic veins.

c:,es).

and may therefore be missed tiii:s.n specifi-

cally looked for. An additional feature in both of OUT cases was a marked variability

in the degree of peri-

venular fibrosis in different parts of a single biopsy. This

Acknowledgemeni

feature may be of relevance not only 8s a diag-

nostic indicator, but also in determining a potentially

The authors acknowledge

the valuabie contribu-

favoorable outcome if the vennw obstroction is re-

tion of Professor R.N.M.

lieved. Biopsies from both left and right lobes of the

the liver hhpsics from the first case.

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drome: apropros of three case% J Radio1 1985: 66: 725-729. Work KB. Bo:ia A. Wslkin DFC. Treatment 01 BuddChimi syndrome by percutaneous rrsnrluminal angiopiw ty. Lawet 1988: ii: 158-159. 1~ Yamada R, Sara M. Kawabmz %. Xakatsuka H. Nakamure K. Kobayarhu N. Sqmental obstruction ofrh? hepmic inferior vena cava treated by rransluminal angioplasty. Radiology 1983: 149: 91-96. 13 Ida M. Ami K. Yoshikawa J, et al. Therapeutic hepatic vein angioplrsty for BuddChiari syndrome. Cardiovasc Intervem Radiol l9ti 9: 187-190. 14 Kimurn C. Shirotani H, Kuma T. et al. Transcardiac mem. branotomy for obliteration of Lc inferior vrna cave in *he hepmic pardon. J Cardiovasc Surg 1972: 3: 393-404. 15 Rubinstein D, Mclsnes I, Dudley F. Morbidity and mortality after perhoneovenous shunt surgery tar refractory as. cilcs. Gut 1985: 26: 1070-1073. 16 Zahn W. Ueher ein eigenrhwdichrs congenitslts Newwcrk in der linken Puhnonalarterie. Virch Arch Path Amo ,889: 1 t5: 58. 17 Okuda K. Membranous obstruction of Ihe iaie%r \‘eena cave: etiology and relation to hepatwellulnr carcinoma. Clastroentcmlo~y 1982; 82: 377-379 18 Wagenvoort CA. Wogenvwrt N. Pathology of Pulmonary Hypertension. New York: Wiley, 1977: 150-162. 19 Simson IW Rmld.Chiari Syndrome and Vcno-Occlusive Disease. In: Peren RI_. Craig JR. eds. Liver Pathology. New York: Churchill Livingscone. 1986.299-314. 20 Nakzmo M. Warner TM. Lieber CS. Perivcnulrr fibrosis in

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WEBS

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