budd chiari syndrome and coarctation of inferior vena cava

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iiiiiiiii 3iiiiiiiiii REVIEW ARTICLE

Budd Chiari Syndrome and Coarctation of Inferior Vena Cava

S o l o m o n Victor, V Jayan th i , N M a d a n a g o p a l a n

B u d d Chlarl S y n d r o m e e n c o m p a s s e s s evera l ae t to loglcal factors. Coarctat ton o f I n f e r io r veda cava is charac ter t sed b y an h o u r glass constrIct ioD o f t he ~ava wi th o r w i t h o u t occ lus ion o f the terml- h a l h e p a t i c velns. The la t ter m a y b e oc- c l u d e d wi th m l n l m a l or n o changes In the In t e r lor veda cars. Thls e n t l ( y has b e e n r e p o r t e d wor ldwide , b u t c o m m o n l y f r o m Africa, China, I nd ia a n d Japan . C h a r a c t e r i s t i c f i n d i n g s o n u l t r a s o n o g r a p h y , a n d f u n c t i o n a l h e p a t o g r a p h y are I l lustrated. Surg lca l p r o c e d u r e s f o r c a v a l d e c o m p r e s s i o n ( Inc lud ing opera t i ve fea tures In authors" ser ies o f 24 pa t ien ts ) , p o r t a l decompres - slon, hepa t i c v enous decompres s ion , a n d r e l l e f o f ascl tes are out l ined. A r g u m e n t s f o r o r agalDat c o n g e n i t a l h y p o t h e s i s , p r l m a r y t h r o m b o t l c theory, o r p r i m a r y I n f l a m m a t o r y cause are listed. The pos - s lb l l l (y o f Hlarlal ae t lo logy Is discussed.

(Based on the Hunterian Lecture Delivered by Professor Solomon Victor at the Royal College of Surgeons of England, London, in 1991)

Address for correspondence: Dr Solomon Victor, 15, East Street, Kilpauk Garden Colony, Madras- 600 010, INDIA

In troduc t ion

Blood vessels had fascinated John Hunter. He was fond of vascular surgery. He had ligated the carotid artery of the deer in Richmond Park to study the effect on the growth of the antlers. His observations, that this resulted in formation of collaterals led him to treat popliteal artery aneu- rysm by ligation of the femoral artery, a method of treatment still relevant for patients with ad- vanced neglected popliteal aneurysms in India. He was aware of occlusive arterial disease in his own coronaries." My life is in the hand of any rascal who provokes me", he had observed. His collections, exhibited in the Hunterian museum, include specimens with thrombotic lesions in the veins. The topic of this lecture on obstruction of the inferior vena cava (IVC) around the level of hepatic veins would have been of interest to Hunter, espe- cially since it emanates from India which must have fascinated him. His wife's brother worked in Lucknow. A hookah from India was among his many prize collections.

N i n e t e e n t h Century

Let us for a moment recall London in the nine- teenth century. Hunter was practicing in Leicester Square initially assisting his brother in anatomy classes, in the Great Windmill Street. Resurrectionists were frequenting the Soho Square. Hunter partici- pated in the meetings of the Royal Society in Young Slaughter's coffee house. Among his friends were H~tydr~, for some of whose compositions Anne Hunter wrote the verses, Reynolds, Johnson and

Indian Journal of Thoracic and Cardiovascular Surgery

Goldsmith. The Endeavor set sail with request from John Humer to bring him specimens from all over the world. In the same century, Reynaud in 1827, Lambroan in 1942, Budd in 1857, Frerich in 1861, Lam in 1861 and Chiari in 1899 described patients with hepatic venous outflow obstruction (HVOO) 1.

Budd in 1857 examined three liver specimens. The first was that of a sailor sent to him by his brother. There was suppurative inflammation of a branch of hepatic vein (HV). This was secondary to a small abscess in the liver. He observed that 'lymphatics effuse within the vein and canalisation of the vein becomes closed'. All the branches that fed the HV as far as their capillary division were described as being choked with fibrin and coagulat- ed blood. The second specimen was sent to him by Mr. Busk in 1843 taken from a man who died due to phlebitis and a third case was referred by Dr. James Russell in 1836 again following phlebitis.

Chiari described three autopsy cases of obliter- ating phlebitis. The first case with acute symptoms, seen in 1885 was presumed to be due to syphilitic phlebitis. There was complete occlusion of HVs by fresh thrombi. The second case was autopsied in 1893. Scarred tissue occluded the HVs. The third case in 1895 revealed thickened HVs with occlusion of ostia and narrowing of the lumen.

T w e n t i e t h C e n t u r y

Unfortunately, subsequently in the twentieth century any patient presenting with HVOO due to var ied pa tho log ica l causes ex tend ing f rom cavoatrial junction to the hepatic venules were labelled as having Budd Chiari Syndrome (BCS). This practice led to many diverse aetiological factors being c lubbed toge ther . The k n o w aetiological causes of BCS can be classified as follows :

I H a e m a t o l o g i c a l

A. Myeloproliferative disorders

B. Hypercoagulable states

II I n f e c t i o n s

A. Hepatic

B. Intra-abdominal

C. Phlebitis

III Cysts o f Liver

A. Congenital

B. Acquired

IV T u m o u r s o f l i ver

A. Benign

B. Primary malignant

C. Secondary turnouts

V T u m o u r s o f IVC and Hepat i c Ve in

A. Benign

B. Primary malignant

C. Secondary

VI T r a u m a

A. Postoperative/Iatrogenic

B. Post-traumatic

C. Radiation

D. Miscellaneous

VII I m m u n o l o g i c a l D i s o r d e r s

VIII V e n o - o c c l u s l v e D i s e a s e

A. Drugs

B. Plant toxins

IX E x t r i n s i c C o m p r e s s i o n

A. Liver

B. Retroperitoneal lesions

C. Tense ascites, raised intra-abdominal pressure

D. Pericarditis

X I n t r a l u m i n a l O c c l u s i o n o f IVC/Hepat lc Ve ins

A. Primary thrombosis of IVC

B. Secondary thrombosis

C. Intraluminal extension of thrombus

XI Cardiac L e s i o n s

Coarctation o f lVC (CIVC) a n d Idiopathic Ob- . struction o f Terminal Hepatic Veins (IOHV)

When known acquired aetiological factors are excluded, there is a group of patients having localised occlusion of IVC with or without involvement of HVs. Though such cases have been reported world- wide, this entity appears to be more prevalent in Africa, 2 China, 3 Japan, 4 and India,5L It has been

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VICTOR et al Budd Chiari Syndrome

variously labelled as :

Coarctation of IVC

Congenital stenosis

Congenital obstruction of IVC

Congenital membranous obstruction of IVC

Membranous obstruction of IVC (MOIVC)

Membranous obstruction with CIVC in its diaphragmatic portion

Membranous stenosis of IVC

Stenosing malformation and thrombosis of superior hepatic vein-vena cava junction

Fibrotic construction of IVC

Idiopathic obstruction of IVC

Partial Budd-Chiari syndrome (segmental obstruction of hepatic vein)

Proliferative thrombophlebitis

Endophlebitis hepatica obliterans

Obliterative hepatica vena cavitis

Hepatophlebo vena caval occlusive syndrome

Liver cava vein obstruction

Hepatic cava vein obstruction syndrome

Hepatic cava syndrome

The term membranous obstruction is commonly used. However this term, like the label BCS, has also been used in various clinical situations. 8 Mem- brane obst ruct ion has been associated with myeloproliferative disorders, oral contraceptives, lupus anticoagulant, systemic lupus erythematosus, Behcet's syndrome, Sjogren's syndrome, pericaval filariasis and visceral thrombophlebitis migrans. When membranous obstruction is the sequel of any specific aetiological factor, it should be included in the appropriate group under known causes of BCS. Only those with a 'membrane ' of unknown or disputed aetiology would be considered in our discussion.

Clinical Presenta t ion

Patients' present with symptoms due to IVC occlusion, hepatic venous occlusion and portal hypertension. 6 Commonest presentation is tense ascites with prominent veins in the lower extremities and abdomen. Splenomegaly is not as common as in portal hypertension due to hepatic or prehepatic

causes. Jugular venous pressure is often elevated in these patients presumably due to the entire venous return reaching the heart through the superior vena cava.

Biochemical tests usually reveal only mild de- rangements in liver function. Liver biopsy shows evidence of hepatic venous outf low obstruction. This entity is a precancerous lesion outflow obstruction. This entity is a precancerous lesion. Incidence of hepatoma in association with CIVC is as high as 44.1%.

Ultrasonography

D i a g n o s i s is e a s i l y e s t a b l i s h e d by ul t rasonography which demonstrates the site of caval constriction, dilated cava below it, dilated HVs (Fig 1), and comma shaped intrahepatic collaterals.9 Stasis thrombi may be seen within the obstructed cava and HVs. Changes in liver parenchyma, ascites and thickening of gall bladder wall can be seen. Evidence of portal obstructionn can be picked up. Dilated intra-abdominal collateral veins are visualised. Doppler ultrasonography adds information regarding direction and velocity of blood flow in the IVC and HVs.

Fig. 1. Ultrasonogram showing dilated retrobepatic IVC (black triangle), site of coarctatton (black arrow), suprahepatic IVC (white triangle) and dilated intrahepattc collateral veins (white arrow).

Venacavogram

Transfemoral inferior vena cavogram outlines the obstructed vena cava and the collaterals. The

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Fig: 2, Inferior venacavogram sbowtng (a) 'dolphin's nose', (b) 'gfraffe's neck'(c) 'shrimp" and (d) 'bird's beak' appearances of the upper end of obstructed IVC.

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VICTOR et al BuddChiariSyndrome

appearance may resemble dolphin's nose (Fig.2a), giraffe's neck (Fig.2b), shrimp (Fig.2c), and bird's beak (Fig.2d). Shelf like (Fig.3a), conical (Fig.3b), dome-shaped (Fig.3c), curvilinear (Fig.3d), wine glass (Fig.3e) or hourglass (Fig.3f) appearances have been seen. The configuration of the obstructed IVC may resemble a carrot (Fig.4a), a cucumber (Fig.4b) or a christmas tree (Fig.4c). The upper end may be irregular due to stasis thrombus below the obstruction (Fig.5a). These appearances are deter- mined by the initial pathology including configuration of stasis thrombi be low the obstruction.

The contrast should be injected as close to the obstruction as possible; otherwise due to reversal of caval blood flow it will get diverted into retroperi-

toneal collaterals giving an erroneous impression about the level of caval obstruction (Fig.5b). At- tempts are made to catheterise the superior HVs selectively and obtain hepatic venogram. Wedged hepatic venogram can also be obtained. When the caval obstruction is complete, IVC should also be catheterised through the right atrium to visualise the cava above the obstruction and identify hepatic or collateral veins entering this segment. Ideally transfemoral and transatrial injections should be combined (Fig.6).

Hepatogram and Hepatic Venogram

Usually it is possible to outline the right HV and sometimes middle and left HVs through selective

Fig. 3. Infertor vena cavograms showlng (a) 'shelJ" ltke (b) 'conical' (c) 'dome sbaped', (d) 'curvtlinear' (e) 'wineglass'and(f) 'hourglass 'appearances of the obstructed cava

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Fig. 4. The obstructed IVC is like a (a) 'carrot' (b) 'cucumber' and (c) 'christmas tree'.

Fig. 5(a). Tbe upper end of lVC sbows irregular filltng defect due to stasfs tbrombi below the obstruction; (b) Entry of contrast into retroperttoneal collaterals is seen, due to distal injection and reversal of f low in tbe lVC.

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VICTOR et al Budd ChiariSyndrome

Fig. 6. Combined transfemoral and transatrtal vtsualtsatton oflVC

catheterisation during venacavogram. If this is not feasible, percutaneous functional hepatogram has been found useful. This is done by injecting a bolus of contrast into the parenchyma of right lobe. The contrast can also be directly injected into a tributary of the HV. Usually patent right HV is seen draining into the IVC below the obstruction with reversal of flow in the retrohepatic cava (Fig.7a). Sometimes the entry of right HV or other collateral channels and drain into the IVC lower down (Fig.7b). Occa- sionally linear narrowing of right HV my be seen (Fig.7c). Contrast may drain into perihepatic collateral veins (Fig.8a). The portal vein can occasionally

serve as an outflow tract (Fig.8b). The contrast may also visualise the lymphatics flowing towards the porta hepatis. The thoracic duct can be outlined occasionally (Fig.8c). It is of interest that the Hunterian brothers have the credit of attributing for the first time, absorption function to the lymphatic system.

Computerised Tomography and Nuclear Mag- netic Resonance Imaging

These are adjuvants for diagnosis if available and affordable and would reveal the changes in the cava, HVs, liver parenchyma, portal venous system, spleen, ascites and collateral channels. 3 Contrast media enhance the information obtained by computerised tomography. Magnetic resonance imaging can pick up blood vessels without the use of contrast. However contrast media are being as- sessed for improving magnetic resonance imaging of the liver.

Classif icat ion

Based on the above investigations patients fall into 3 groups :

Group I : Occlusion of Only IVC

A. Cavoatrial Junction

B. Supradiaphragmatic Cava

C. Cava at Level of Caval Hiatus and Supe-

Fig. 7. Functtonal hepatograms showing (a) patent dilated right hepatic vein draining into lVC, (b) obstruction of right hepatic, close to IVC with collaterals entering the inferior right hepatic vein and (c) linear narrowing of right hepatic vein

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Fig. 8. Functional hepatograms showing (a) entry of contrast into gnfrahepatic collaterals (b) entry of contrast retrograde into portal vein (c) and centrifugal flow in the lymphatics which drain into the thoracic duct.

rior HVs

D. Retrohepatic Cava

The caval obstruction may be due to a transverse shelf of varying thickness, or affect short or long segments of IVC.

Group II : Occlusion of only superior HVs Left, Middle, Right or Combinations

Group III: Combined Occlusion of HVs and IVC

Aetiologieal Considerations

The debate over the aetiopathogenesis 8 of these idiopathic group of cases has revolved around the following :

A. Congenital hypothesis

B. Primary thrombotic theory

C. Infection/Inflammation

Phlebitis

Endophlebitis

Periphlebitis

A. Congenital Hypothesis

The earliest description of possible congenital occlusion of IVC was by Betz in 1877,1 in a male

aged 21, where the IVC terminated as a blind sac close to the heart. Subsequently Osler, Warren, Turner, Griffith and others reported similar lesions. Scudder reported a 17 year o l d male who had collaterals since birth. The arguments relating to congenital aetiology are as follows :

1. Embryological Basis

Congenital obstructions in the aorta, gastrointes- tinal tract, biliary tract, vagina and tracheobronchial tree usually occur at sites of embryonic union. 3 The complex development of IVC and HVs provides ample opportunit ies for various developmental anomalies such as agenesis, hypoplasia, nonfusion or malfusion of the concerned embryonic veins consisting of hepatocardiac channel and its junction with sinus venosus, primitive HVs derived from vitelline and umbilical veins, and subcardinal vein. It is of interest that the site of anomaly has varied in different series. Rossal and CaldwelP ~ found anomalies of the Eustachian valve in a 19 year old boy. Similar caval obstructions at this level have been reported by Kilman et al and Kibel and Marsden. 12 Simson from Africa 2 reported a high incidence of obstruction at the cavoatrial junction with absence of supradiaphragmatic segment of IVC. In India,Japan and China the obstructive lesion is usually at the level of the caval hiatus or just

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VICTOR et al BuddChiariSyndrome

below it, commonly associated with occlusion of terminal left and middle HVs:

2. Associated Congenital Anomal ies

Occurrence of associated congenital anomalies adds credence to congenital hypothesis. Meyer et al 1 reported combined obstruction of IVC and superior vena cava. Johansson et al, 13 recorded obstruction of both brachiocephalic veins. Victor et al, 14 have docu- mented a case of situs inversus totalis with coarctat ion of left sided IVC.

3. Ductus Venosus

Closure of ductus arteriosus has been incriminated in the pathogenes is of coarctat ion of aorta and pulmonary artery. Likewise the postpar tum closure of ductus venosus has been incriminated in pathogenesis of occlusion of left HV and adjacent IVC. This theory however does not explain the other variations encoun te r ed in this entity.

4. Primary Defect in the Diaphragmat ic Hiatus

In the majority of the cases of CIVC, the obstruction is at the level of diaphragmatic hiatus. The possibility of a primary deve lopmenta l defect in the caval hiatus of diaphragm causing obstruction of IVC cannot be totally excluded. The lesion may be analogous to congenital occlusion of aorta at the diaphragm by abnormal formation at diaphragmatic crux. During surgery found fibrotic changes at the caval hiatus of the diaphragm. There was difficulty in dissection of the IVC at this site, there being no plain of cleavage. It is specula ted whether the hiatal narrowing is primary and has led to secondary changes in IVC or whe the r poor formation of IVC resulted irl 'closing in' of the developing diaphragmatic hiatus. Burnt out periphlebit is also needs to

be excluded.

5. Membrane

In coarctat ion of aorta there is a shelf or fold of intimal tissue complet ing the obstruction. In CIVC, such a shelf has attracted more at tention than the constriction in inferior vena cava. The components of the membrane are histologically similar to the wall of the vena cava. Usually it is a round the level of the superior HVs. Occasionally the membrane may occlude the orifice of one or more HVs. Victor et al, r e p o r t e d a m e m b r a n e in the

supradiaphragmatic segment of IVC. is The varied locations of" the membrane at potential sites of embryological dysunion points to a congenital aetiology. The thickness of the membrane has varied from a few millimeter to several centimeter. The membrane is thin when there is failure of fusion be tween otherwise well deve loped embryological segments and thick when segments of the concerned veins are not fully developed. The extent of stasis thrombi on the surface of a congenital membrane would also alter its thickness. Its occurrence would depend upon the blood flow pattern through the HVs and collateral veins.

6. Liver Tunnel

Retrohepatic occlusion of IVC has been attributed to narrowing of the furrow of the liver.

7. Familial

Familial incidence of ostial stenosis of HVs with dilated intrahepatic collaterals have been repor ted in two sisters.

8. Age o f Detection a n d Onset o f Symptoms

The entity has been repor ted in utero, infancy and early chi ldhood. Howeve r late age of onset of symptoms which is usual, has been held as an argument against congeni ta l aetiology. It is not uncommon to find congenital anomalies manifest- ing with symptoms for the first time in adul thood. In the third world, due to illiteracy, poor school health programmes and inadequate diagnostic facil- ities, many congenital malformations may remain unde tec ted for years. Aggravation of obstruct ion due to supervening thrombus at a later age may be responsible for presenta t ion at an older age. Com- plete occlusion by an acquired thrombus of a per- forated membrane can also cause late onset of

symptoms.

9. Age o f Onset o f HCC

Both CIVC and IOHV are precancerous lesions with a high incidence of hepat ic malignancy. The early age of onset of hepatoceIlular carcinoma is in favour of long standing, possibly congenital ob-

struction.

10. Teratogens

Teratogens acting in utero, maternal alcohol-

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ism, use of oral contraceptives and drugs have been incriminated in its pathogenesis, speculating that the 'congenital ' lesion may be 'acquired in utero'.

B. Primary Thrombot ic Theory

Proponents of thrombotic theory assume that the lesion is primarily a thrombus at the hepatic outflow region which later gets organised into a membrane. Localisation of thrombosis to this region has been attributed to movement of the heavy liver around a transverse axis, during breathing, cough- ing or trauma, causing injury to the intima of the vena cava. Eddying currents at this site due to intermingling of blood streams from HVs and IVC have been held responsible for thrombus formation. Preexisting congenital intimal irregularity has been observed at the level of the membrane. Slight constriction and angulation of IVC has been noted in the site where MOVC occurs in a study of foetal casts by Kimura et al. 16 Retardation or reversal of blood flow consequent to increase in pressure within the thorax at the diaphragmatic opening of IVC has been claimed to predispose to thrombus formation. The theories are speculative and not proven. None of these theories can explain the const't'Icting lesions in the wall of IVC and terminal HVs.

C. I n f e c t i o n / I n f l a m m a t i o n

Ever since the days of Hunter, phlebitis and thrombosis have dominated aetiological considerations in occlusive venous disorders. BCS is no exception. Phlebitis could start as periphlebitis, phlebitis or endophlebitis.

John Hunter had a keen interest in the study of veins surrounded by inflammation. He had observed that 'the coats of large veins passing through the inflamed part became so consider- ably inflamed that their inner surface takes on the adhesive, suppurative and ulcerative inflammation'. The cases described by Budd were examples of periphlebitis causing HVOO. We reported pericaval filariasis causing formation of a membrane and obstruction of the IVC and terminal HVs. 17 In recent years there have been no reports of active periphlebitis associated with BCS. It remains speculative whether antecedent burnt out periphlebitis is a cause for IOH~r and CIVC. It is of interest that in China,

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combined obstructions of the IVC and superior vena cava have been reported. Periphlebitis has been demonstrated around the superior vena cava. In- flammation of unknown aetiology leading to con- stricting lesions in the aorta, oesophagus, pulmonary artery, superior vena cava and innominate veins are not uncommon in India,Japan, China and Africa.

Chiari considered phlebitis as a cause for hepatic venous outf low obstruction. It has been suggested that bacterial infection from the gut carried into the IVC would impinge on the wall of the vena cava and promote infection. This process could also occur in septicaemia. Turbulence at the site of entry of ductus venosus has been suggested as a factor predisposing to infection.The high incidenceof this lesion in developing countries has been attributed to proneness for infection and dehydration. However this theory does not explain the world- wide occurrence of the disease.

Medical and Surgical Management

Medical treatment is directed towards allevia- tion of symptoms. Ascites is controlled by diuretics and paracentesis. Autotransfusion of ascitic fluid has been found helpful. Symptoms due to nervous congestion in the lower limbs and scrotum are usually not controlled with conservative management.

Surgical management by invasive dilatation was introduced by Kimura in 1960) 8 Current operative procedures can be classified as follows :

I Caval D e c o m p r e s s i o n

L 1 Invas ive Di la ta t ion

This has been attempted through the right atrium using the finger, various types of dilatory, balloon catheter and knife. Bimanual technique approaching the obstruction through the atrium and IVC have also been tried. Lack of visual control and pulmonary embolism due to dislodgment of stasis thrombi below the obstruction are major concerns. Residual and recurrent stenosis have been encoun- tered.

1.2 Non Invas ive Di la ta t ion

Introduction of sophisticated balloon catheters

VICTOR et a l BuddChiariSyndrome

and imaging techniques have kindled interest in non invasive dilatation using single or multiple balloon catheters. Imperforate membrane has been initially punctured using Brockenborough catheter and subsequent ly dilated. Atherectomy catheters, intravascular saw and laser t ipped catheter~ have been used to establ ished a passage through the s tenosed area. Embolisation of stasis thrombi is a constant threat. Anticoagulants have been given before and after dilatation. Increased venous return following such dilatation may result in pulmonary oedema and should be anticipated. Residual and restenosis has been a problem. Expanded metallic stents have been inserted to avoid restenosis. Rup- ture can ocOai" though rare.

1.3 Excis ion • Pa tch Venoplasty

To avoid problems associated with non invasive dilatation, open excision of the obstruct ion tissue has been introduced. The site of obstruct ion has been exposed by thoracoabdominal approach with anterior d i sp lacement of liver, t ransthoracic transdiaphragmatic retrohepatic retroperitoneal approach or by median sternotomy and superior approach. Inflow occlusion technique by clamping of IVC above and below and porta hepatis or descend- ing aorta, ~9, z0 circulatory arrest, partial or total cardiopulmonary bypass have been used to expose the obstructed segment. After veno tomy the obstructing tissue is excised. Wherever possible, the obstructed terminal HVs are recanalised. The IVC is usually repaired using a patch. Supradiaphragmatic CIVC is easily corrected by transthoracic approach. After clamping the IVC above and be low the obstruction, the membrane is excised and IVC repaired with a patch. Similar technique is adopted to relieve obstruct ion at the cavoatrial junction.

I. 4 Do rsal Cavoatr ia l Bypass

Caval obstruction has been bypassed by insert- ing a rigid graft such as r inged e x p a n d e d polytetraflouroethylene graft (ePTFE) be tween the vena cava be low the obstruction and cava above the obstruction or right atrium. 2~ z~ Cava-azygos bypass x2 has also been reported. Dorsal bypass grafts are advantageous because of the shorter length of the graft and more direct flow.

Failure to dilate the lesion in our first patient

using the finger through the transatrial route and desire to define the pathology at t h e site of obstruct ion and relieve the obstruct ion led us to explore the retrohepatic area. "Why think? Try the experiment", John Hunter had advised Edward Jenner. We approached the retrohepatic cava by right posterolateral thoracotomy in the left lateral posit ion traversing the anatomical area familiar to the Hunter brothers and beautifully illustrated by charles Bell. Incidentally Hunter 's contribution to assisted ventilation has been virtually forgotten. He had conceived and deve loped assisted ventilation with positive and negative phases in 1755.

The operat ive findings in all our cases were similar. There was an hour glass constriction of the IVC just be low the diaphragm and above the right hepatic vein. The affected segment was 5mm to I cm in length. The IVC above the diaphragm was normal. The IVC below the obstruction was tense, distended and thick walled. The right hepatic vein was seen draining into IVC just below the obstruction. There was no evidence of extrinsic compression or acute inflammation. There was fibrosis around the obstructed segment with collateral veins, making it difficult to expose this segment clearly. In three patients, we used antibiotic preserved descend- ing thoracic aortic homograft including the terminal arch to drain the retrohepatic cava into right atrium. It is of interest to recollect Hunter's pioneering experiments in tissue transplantation. The diaphragm was anchored to the anterior abdominal wall to relieve the pressure of the liver offthe graft. The aortic homografts, however, failed. One had thromboembolism. Another had clinical occlusion. The third patient had clinical occlusion and radio- logical calcification. In this patient, the calcified homograft was removed and replaced by 22 mm plain ePTFE graft. Gratifying relief of symptoms led us to use ePTFE graft in 7 patients and later 16 m m ringed eP'ITE graft. Gratifying relief of symptoms led us to use ePTFE graft in 8 patients. Follow-up to 12 years has been gratifying (Fig.9).

The patency of the grafts can be studied using noninvasively by ultrasound 9 or magnetic resonance imaging.

L5 Ventral Bypass Graft

These ate no longer popular. Long grafts were used connect the IVC and right atrium using a graft passing

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Fig. 9. Postoperative cavogram showing obstructed end of lVC and functioning dorsal cavoatrial bypass graft.

through the diaphragm behind the sternum. Long length favours thrombosis. Constriction has occurred at the site were the graft passes through the diaphragm. The poor risk patients femoral vein has been connected subcuta- neously to subclavian vein.

One would have anticipated caval decompression to minimise the risk of hepatic malignancy. However caval decompression may result in regeneration, increased liver cell turnover and malignancy. Regeneration of tissues fascinated John Hunter.

II Portal D e c o m p r e s s i o n

In patients with BCS due to idiopathic occlusion of HVs, without caval obstruction, portal system has

been decompressed using mesocaval, portacaval or splenorenal shunt. When there is associated caval obstruction due to primary pathology in IVC or due to secondary compression of IVC by enlarged liver or ascites, mesoatrial, splenoatrial and portoatrial shunts have been performed. A high mortality due to postoperative pericarditis has led to perfor- mance of meso-innominate, meso-extra pericarditis superior vena cava, or meso-jugular shunts. In ear- lier years, splenic vein was anastomosed to pulmonary artery. Procedures such as splenopneumopexy, hepa topneumopexy and omentos ternopexy have been performed to promote collateral blood flow. 24

Ill Direct Hepat ic Venous D e c o m p r e s s i o n

This could be achieved by dilating the obstructed terminal veins using balloon catheters or dilators, by noninvasive or invasive techniques. Coring out of the hepatic tissue around the terminal HVs has been resorted to, until a plane is reached were the proximal HVs are exposed. The right atrium is then sutured around the raw area of the liver enabling the freely bleeding HVs to drain into it. 3

IV D e c o m p r e s s i o n o f t h e A s c i t e s / H e p a t i c Lym- phatic System

Thoracic duct-jugular vein and Le Veen shunts have been tried to alleviate ascites. 3

V C o m b i n e d Procedures

When there is primary occlusion of HV and secondary caval obstruction due to liver or ascites, an initial mesoatrial shunt has been performed and converted later to a mesocaval obstruction and secondary obstruction of HVs by thrombus, open excision of the obstructing tissue and removal of the thrombus combined with cavoplasty is pre- ferred. When there is primary obstruction of both IVC and HVs, the obstructing tissue could be cored out and the distal IVC and the raw area of the liver with patent HVs be anastomosed to the right atrium 3 or procedures to decompress both IVC and portal system such as ilio mesoatrial shunt or excision and patch venacavoplasty combined with mesocaval shunt can be performed. TM-

VI Liver Transp lant

The disease is common in Africa, Japan, China

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VICTOR et al Budd Chiari Syndrome

and Ind ia w h e r e l iver t r a n s p l a n t a t i o n is not avai l - able or p o p u l a r . M o r e o v e r in this d i s ease l iver funct ion is fair ly wel l p r e s e r v e d and r e p a r a t i v e

p r o c e d u r e s l is ted a b o v e are p r e f e r a b l e to l iver transplant.

Conclus ion

If J o h n H u n t e r w a s a l ive t o d a y he w o u l d h a v e gone a r o u n d the w o r l d us ing m e a n s of t ravel fas ter than Capta in Cook ' s ship, s tudy ing the pa tho log ica l s p e c i m e n s w h i c h w o u l d y ie ld the final a n s w e r to the a e t i o l o g y o f the d i sease . 'The d e a d h a v e an a n s w e r that m a y save the l iv ing ' , he had said. He might h a v e i n o c u l a t e d filarial larva in his ve ins to see it it l eads to cava l obs t ruc t ion !

Our task t o d a y is no di f ferent f rom that at which Hunte r l aboured ; it is the rel ief of suf fer ing t h rough surgery . He w o u l d ce r t a in ly h a v e b e e n p l e a s e d at a t t emp t s to r e l i eve s y m p t o m s d u e to o c c l u s i o n o f IVC and HVs by v a r i ous a p p r o a c h e s .

"The a l imigh ty w o u l d h a v e fa l len far o f His a c k n o w l e d g e d m ercy , if He had not f u r n i s h e d us

wi th ( m e a n s o f a l l ev ia t ion) w h e n He g a v e us (suf- fer ing)" J o h n H u n t e r

Acknowledgment

We wish to a c k n o w l e d g e wi th g ra t i t ude the g e n e r o u s s u p p o r t f rom g a s t r o e n t e r o l o g i s t s Prof. P. Rajasambandam, Prof. K. Raghuram, physic ians Prof. Dan te M a t h u r a n a y a g a m , Prof. A. R a t n a s a b a p a t h y , rad io log is t s Prof. Arcot Gajaraj , Dr. I. K a n d a s a m y , Dr. Bhara th i Dha la , Dr. C. S a t h y a b h a m a , p a t h o l o - gists Prof. M. P a n c h a n a n d a m , Dr. S. Chitra, C.S. Vijayalakshmi and anaes thes io logis t , Dr. M. Kabeer . We thank Miss Arul Mangai for secretar ia l ass is tance in p r e p a r i n g the m a n u s c r i p t .

References 1. Pleasant JH. Obstruction of the inferior vena cava with a

report of eighteen cases.John Hopkins Hosp Reports 1911; 16: 363- 548.

2. Simson IW. Membranous obstruction of the inferior vena cava and hepatocellular carcinoma in South Africa. Gastroenterology 1982; 82: 171-8.

3. Vascular Surgery. Proceedings of the First International Symposium on Budd-Chiari Syndrome,Japan, China; 1988. Eds: Wang Z, Mishima Y. Published by Chinese Research Committee on Systemic

Vascular Disorders, Ministry of Health and Welfare, Japan.

4. The Second International Symposium on Budd-Chiari Syn- drome. Abstract Book. Japan Cardiovascular Research Foundation, Kyoto,Japan, 1991.

5. Victor S, Jayanthi V, Madanagopalan N. Coarctation of inferior vena cava. TropicalGastroenterology 1987; 8: 127-42.

6. Madanagopalan N, Victor S, Jayanthi V, Kandasamy I, Raghuram K, Gajaraj A, Balakumar M, Panchanadam M. Clinical spectrum and surgical relief of chronic obstruction of hepatic and suprahepatic segment of inferior vena cava - 10 years survey.JGastroenterol Hepato11986 ; 359-69.

7. Datta DV, Saha S, Singh SAK, Gupta BB, Aikat BK, Chugh KS, Chuttani PN. Chronic Budd-Chiari Syndrome due to obstruction of the intrahepatic portion of the inferior vena cava. Gut1972; 13: 372- 8.

8. Victor S, Jayanthi V, Madanagopalan N. Aetiology of membranous obstruction of inferior vena cava. Letter to the Editor. Gastroenterology1993; 104: 1581-2.

9. Jayanthi V, Victor S, Bharathi Dhala, Gajaraj A, Madanagopalan N. Pre and postoperative ultrasound evalution in patients with Budd- Chiari Syndrome due to coarctation of inferior vena cava. clin Radiol 1988; 39: 154-8.

10. Rossall RE, Caldwell RA. Obstruction of inferior vena cava by a persistent Eustachian Valve in a young adult. JOin Path 1957; 10: 40-5.

11. KilmanJW, Wiliams TE, Kakos GS, Molnar W, RyanJM. Budd Chiari Syndrome due to congenital obstruction of the Eustachian valve of the inferior vena cava.JThoracCardiovascSurg 1971; 62: 226- 30.

12. Kibel MA, Marsden HB. Inferior vena caval and hepatic vein throfl~oosis: The Chiari Syndrome in Childhood. Arch Dis Child 1956; 31: 225-8.

13. Johansson L, Moberg A, Soderlund S, Thornel G. Congenital obstruction of the inferior vena cava. Acta ChirScand 1966; 131: 509-15.

14. Victor S,Jayanthi V, Thillai T Vallal, Madanagopalan N. Coarctation of inferior vena in situs inversus totalis. Indian J Gastroenterol1992; 11: 89.

15. Victor S, RavindranJayanthiV, Kandasamy I,Annapooma S, Kabeer M, Madanagopalan N. Venous isthmusplasty for coarctation of inferior vena cava. IndianJ Thorac Cardiovasc Surg 1983; 2: 55- 8.

16. Kimura C, Shirotani H, Hirooka M, Terada M, Iwahashi K, Maetani S. Membranous obliteration of the inferior vena cava in the hepatic portion. (Review of 6 cases with 3 autopsies). JCardiovasc Surg 1963; 4: 87-98.

17. Solomon Victor, Jayanthi V, Panchanandam M, Chitra S, Vijayalakshmi CS, Madanagopalan N. Budd Chiari syndrome and per/caval filariasis. Tropical Gastroenterology 1994; 15: 161-8.

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18. Kimura C, Shirotani H, Kuma T, Hirooka M, Hayashi K, Tsunekawa K, Matsuda S. Transcardiac membranotomy for obliteration of the inferior vena cava in the hepatic portion.JCardiovasc Surg 1962; 3: 393-404.

19. Victor S, Gajaraj A, Krishnan S, Bharathi Dhala,Jayanthi V, Madanagopalan N. Inflow occlusion technique for correction of coarctation of inferior vena cava causing Budd Chiari Syndrome. Tropical Gastroenterology 1986; 7: 49-53.

20. ViaorS,JayanthiV, Alageson V, Kandasamyl,Madanagopalan N. Dorsal cavoatrial bypass graft for coarctation of interior vena cava: trial of aortic inflow occlusion technique. Tropical Gastroenterology 1991; 12: 148-152.

21. Vi~:tor S,Jayanthi V, Kandasamy I, Ratnasabapathy A,

Madanagopalan N. Retrohepatic cavoatrial bypass for coarctation of inferior vena cava with a polytetraflouroethylene graft. J Tborac Cardiovasc Surg 1986; 91: 99-105.

22. Solomon Victor, Jayanthi V, Madanagopalan N. Surgical management ofcoarctation of inferior vena cava causing chronic Budd Chiari syndrome. Proceedings of the first International Symposium on Budd Chiari syndrome,Jinan, Cbina 1988; 18-22.

23. Yamamoto S, Yokoyama Y, Takeshige K, lwatsuki S. Budd Chiari Syndrome with obstruction of the inferior vena cava. Gastro- enterology1986; 54: 1070-1084.

24. Hirooka M, Kimura C. Membranous obstruction of the hepatic portion of the inferior vena cava. Surgical correction and aetiological study. Arcb Surg 1970; 100: 656-63.

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