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FUSIFORM ANEURYSMAL DILATATIONOF THE PORTAL VEIN

ANTHONY J. LEONSINS, F.R.C.S.E.SHIRLEY SIEW, M.B.(RAND)

University of Witwatersrand, Medical School

A variety of pathologic changes may be as-sociated with Banti's syndrome. These mayinclude cirrhosis of the liver, splenic vein throm-bosis, cavernomatous transformation of the portalvein and aneurysm of the splenic artery. However,a protracted search of the literature revealed nocase of fusiform aneurysm of the portal vein as aresult of an enlarged, congested spleen and livercirrhosis. As the following case is one of unusualinterest we are briefly reporting the clinical courseand the pathological findings.Case ReportMr. P.E.C., a truck driver, aged 52 years, was ad-

mitted to the General Hospital, Johannesburg, SouthAfrica, on July 29, 1951, with the diagnosis of infectivehepatitis. Two days prior to admission he had developedmarked anorexia and extreme weakness. He passeddark urine and noted the onset of jaundice; this wasthe fifth occasion that he had become jaundiced in thepast 32 years.

Past HistoryJaundice first occurred at the age of 19 years but he

was well until 1930, when, aged 31 years, he had a smallhaematemesis. At this time a large spleen was dis-covered and the diagnosis of Banti's syndrome wasmade.

In 1943, whilst in a military hospital in Pretoria, hehad two further severe episodes of haematemesis andhe was discharged-from the services with the diagnosisof Banti's splenomegaly with hepatomegaly. In 1949,he was admitted to the General Hospital, Johannesburg,with sudden severe abdominal pain which subsidedafter two weeks. No operation was advised. He hadan icteric index of 56, and total bilirubin io.8 mg./Iooml. Just over two years later he was re-admitted withthe same complaint. Prior to this admission he was ingood health and was gainfully employed.

Family History; Nil contributory. Habits; 25cigarettes daily. Two brandies and one pint beer per day.Weight; Steady-averaging I40 lb. Central NervousSystem; Excellent vision and no headaches or episodesof drowsiness or uncontrolled social behaviour. Cardio-vascular System; Breathlessness on exertion for thepast four or five years. No ankle oedema.

Physical Examination; Drowsy, deeply jaundiced,middle-aged male; not dyspnoeic and no clubbing ofthe fingers; blood pressure 120/50; pulse Io8; temp.IoI.2°F.; spider naevi on cheeks and palmar erythema.

Chest;. Nil of note. Nipples not enlarged. Abdomen;Easily palpable, enlarged, hard, non-tender spleenextending to the right of the umbilicus. Liver enlarged-about two fingers: surface granular and non-tender;no ascites. There were bilateral indirect inguinalherniae. There were no other positive physical findings.Total bilirubin 10.2 mg./loo ml. Icteric index 60.Albumen 3.0 mg. and globulin 3.7 g./loo ml. ThymolFlocculation Test: +++ +. Takata-Ara: ++ +.Haemoglobin I5.1 g.%. Prothrombin index 48%.Barium Meal showed well-marked oesophageal varices.After five days the jaundice was no longer visible buthe was still very drowsy. On August 9, 1951, he passeda large tarry stool. Papilloedema was present and spon-taneous purpura developed. A 5oo-ml. blood transfusionwas administered and patient rallied. Thymol tur-bidity 7 units; Thymol Flocculation + +; Takata-Ara reaction + + +; Serum Bilirubin (Direct) = 2.08mg./xoo ml.; Total Bilirubin = 4.8 mg./xoo ml.;Alkaline Phosphatase 7 K.A. units; icteric index 30.August 15, 1951. Further melaena stools at which

time Hb. dropped to 6.4 g.% and i,ooo ml. of packedred cells were transfused.August 21, I951. The right parotid gland was

obviously enlarged, rubbery on palpation but non-tender. Patient was very lethargic but non-complaining.August 23, 1951. Another 500 ml. blood transfused.

No jaundice noted.August 24, 195I. Abdomen very distended and

marked fluid thrill present. Further I,ooo ml. of wholeblood transfused and 2,700 ml. of ascitic fluid aspiratedfrom peritoneal cavity.August 28, 1951. Massive melaena. 2,000 ml. blood

transfused. 500 ml. plasma and Vit. K administered.Gross oedema of the genitalia was present, but anklesand legs were non-oedematous.August 30, I951. Another transfusion of I,o00 ml.

blood was necessary. After 1,300 ml. of ascitic fluidhad been drained pre-operatively, Drs. Lee McGregorand Leonsins performed an, emergency splenectomythrough a thoraco-abdomirtnal approach. The liver, wasshrunken to about 6 in, in diameter and was nodular andfibrotic. On the first post-operative daiy patient appearedvery well.

September i, 1951. Slight jaundice was apparent.Heparin 5,000 units was administered every six hours.

Histology of Spleen -

The spleen was a massively enlarged, firm organ,weighing 3,150 g. The histology showed the presenceof well-marked fibrous thickening of the capsule andtrabeculae. There was also extensive fibrosis of the

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September i960 LEONSINS and SIEW: Fusiform Aneurysmal Dilatation of the Portal Vein

FIG. i.-The lower border of the liver is seen in the upper left-hand corner.The hepatic capsule is thickened and the surface shows an irregularnodularity. The liver has been incised and the cut surface presents acoarse irregular cirrhosis with broad bands of fibrous tissue traversing theliver substance in an irregular manner. Coming down from the portahepatis to the head of the pancreas along the right border of the photographis the fusiform aneurysm of the portal vein. The base of the aneurysmalsac projects below the lower edge of the pancreas. The wall of the sac isthickened and it is filled with ante-mortem thrombus. The pancreas isseen along the lower portion of the photograph and, in relation to its tail,there is present the dilated, thrombosed splenic vein.

splenic pulp and dilatation of the sinusoids. In addition,foci of haemorrhage and ' Gamna-Gandy' nodulescould be seen. The histological features were con-sistent with those of a splenomegaly of the Banti'ssyndrome type.The liver biopsy showed a moderate degree of peri-

portal fibrosis with which was associated a round cellinfiltration. Bile thrombi were present in some of thesmaller canaliculi. There was an increase of the pigmentwithin the liver cells.

September i , 195I. All the wound sutures wereremoved but unfortunately 3 in. of the wound gapedopen and this necessitated re-suturing.September 12, I951. The greater portion of the

wound burst open and this too had to be re-sutured.B.P. oo00/8. Pulse IIo. A large left pleural effusiondeveloped.

September 18, 195I. Patient was quite cheerful andmuch improved. The wound was oozing large quantitiesof serous discharge and healing was poor.

September I9, I951. Two melaena stools and a smallhaematemesis. (Hb. II.4 g.%. B.P. 120/70.) ,ooo0ml. blood transfused.

September 23, 195 . Patient very anaemic; 2,000 ml.blood given into the sternum, as most of the accessibleveins were thrombosed. During the period patient wasgiven high doses of Vitamin B complex, methiscol andchloramphenicol.

September 24, I95I. A second operation was per-formed by Mr. Lee McGregor. A vitallium tube wasinserted via the external abdominal ring between theperitoneal cavity and the thigh in order to drain theascitic fluid into the lower limb tissue lymphatics. AfterCompletion of this minor procedure a right subcostal

incision was made and after exposure of the free edgeof the lesser omentum the main hepatic artery was ex-posed and ligated. At the conclusion of this operationthe patient's condition appeared satisfactory.

September 29, 195I. A large melaena stool waspassed and the patient's condition deteriorated rapidly.Despite many blood transfusions and other supportivetherapy the patient died on October 5, I951.Autopsy FindingsThe autopsy was performed by one of us (S.S.) seven

hours after death. The principal findings were:The heart was slightly enlarged (390 g.). All the

valves were competent and healthy. The myocardiumwas pale and flabby and the endocardium of the leftventricle was thickened. The coronary arteries werehealthy. There was fatty streaking in the commence-ment of the aorta and moderate atheroma in the ab-dominal aorta. The oesophagus showed grossly dis-tended varicose veins, extending three quarters of theway up from the cardiac end. The stomach was con-tracted; it contained an abundant quantity of alteredblood and the mucosa showed a chronic hypertrophicgastritis. Blood staining of the intestinal mucosa wasseen throughout the length of the rest of the tract. Theliver was diminished in size and was deeply bile stained.Its surface was grossly irregular due to a coarse nodu-larity. On section, an irregular coarse cirrhosis waspresent. Scattered throughout the liver were small paleareas of necrosis. Thrombosis was present in thehepatic artery. The gall bladder was very flabby owingto gross oedema of its wall. There was no evidence ofextra-hepatic obstruction as a probe could be passed withease from the ampulla up the common bile duct. The

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FIG. 2.-Section of the portal vein, stained by the haematoxylin and eosinmethod. Ante-mortem thrombus is present, intimately adherent to theintima. The intima can be seen to be grossly thickened and to occupy thebulk of the wall. In the more superficial layers there is loosening anddegeneration but in the deeper layers the fibrous tissue becomes moredense. Many vascular clefts (v.c.) and plugs of fibrin (f) are present. Themedia is thinned and atrophic. There is fragmentation of the internalelastic lamina.

pancreas was increased in consistency and on section alarge aneurysmal sac containing ante-mortem thrombuswas found in relation to the head of the pancreas. Ondissection, this proved to be the portal vein which hadundergone aneurysmal dilatation. The sac was 8 cm.long and 4 cm. wide and extended from the portahepatis to the inferior border of the pancreas (Fig. i).The splenic vein was also dilated and thrombosedthroughout its course in relation to the pancreas.

HistopathologySections of the portal vein (Fig. 2) showed well marked

thickening of the intima which formed the greater partof the wall of the vein. The intima showed layers ofdense fibrous tissue, poor in nuclei, separated by spindle-shaped clefts lined by endothelial cells, i.e. vascularspaces, thus indicating a mild degree of cavernomatoustransformation. An ante-mortem thrombus was in-timately adherent to the luminal surface of the intimaand localized foci of fibrin deposit were found within thefibrous tissue of the intima itself. Special staining withthe Weigert-van Gieson technique showed the presenceof some longitudinal muscle fibrils in the intima and theinternal elastic membrane was seen to be thinned andfragmented. Foci of calcification were noticed in theintima. The media was thinner than normal and in

places extreme atrophy was present. There was adiminution in the elastic and muscle fibres and an in-crease in the fibrous tissue (Fig. 3). Sections of thesplenic vein showed the changes of phlebosclerosis andante-mortem thrombosis as described above in the portalvein, but the lesion was not so far advanced in this vessel.Sections of the liver showed the presence of a coarseirregular fibrosis of the portal tracts which had led togreat distortion of the lobular architecture. However,in some areas normal lobules could still be recognized.A large area of lobular necrosis was present and therewere many foci of centri-lobular necrosis, some of whichshowed evidence of organization. Bile staining was wellmarked, particularly in central zone and bile thrombiwere seen in the intercellular canaliculi and in thecholangioles. The bile ducts in the portal tractsshowed no evidence of abnormality. These features arethose of a coarse cirrhosis of the 'post-necrotic scarring'variety, with evidence of acute intra-hepatic obstruction.The areas of recent necrosis have followed ligation of thehepatic artery. Sections of the kidneys showed thepresence of lower nephron damage with bile casts in thedistal convoluted tubules. Sections of the lungsshowed the presence of congestion, some atelectasis andfoci of bronchopneumonia. Sections of the oesophagusshowed well marked distension of the vessels.

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September I960 LEONSINS and SIEW: Fusiform Aneurysmal Dilatation of the Portal Vein

FIG. 3.-Section of the portal vein stained by the Weigert-van Gieson method.The dense fibrosis of the deeper layers of the intima is well shown. Inthe media the fragmentation of the elastic tissue is clearly demonstrated.

DiscussionDouglass, Baggenstoss and Hollinshead7

measured the portal vein in 92 dissections. Theirresults were as follows:Average Diameter .... o.89 cm.Range .. .. .. .. 0.64 to 1.2I cm.Average Length .. .. 6.4 cm.Range .. .. .. .. 4.8 to 8.8. cm.

Purcell14 found the average diameter to beI.o9 cm. Child6 states that in cases of hepaticcirrhosis a portal vein with a diameter of 2 cm. hasbeen found. It can thus be seen that the portalvein in this case was grossly dilated, reachinganeurysmal proportions as the sac measured 4 cm.in diameter. The aneurysm was of the fusiformmorphology and involved the entire length of theportal vein.

Careful searching through the literaturel, 11, 17failed to disclose a similar case and, on personalquestioning, Prof. Sheila Sherlock, Dr. RobertLinton, Dr. Paul Steiner, Dr. Bax and Dr.Schlamm all declared that in their experience theyhad never encountered a case of an aneurysm of theportal vein. Barzilai and Kleckner3 reported asaccular aneurysm of the right branch of the portalvein which ruptured into the common bile ductproducing a haemocholecyst in a case of post-necrotic cirrhosis with thrombosis of the portalvein.

Histologically the portal vein showed well

marked phlebosclerosis and ante-mortem throm-bosis.

Banti2, 9 described a ' Sclerosing Endophle-bitis' affecting especially the splenic, less fre-quently the portal and infrequently the mesentericveins, in association with the Banti spleen (I898and 191o).Simmonds18 found thickening of the intima of

the portal vein, with calcification and hyalinechange, in association with cirrhosis of the liverand syphilis. Since then portal phleboscleroticchanges have been reported repeatedly in cases ofportal hypertension. In I942 Moschowitz13 wrote' Sclerosis of the veins of the portal system isperhaps the most commonly reported example ofphlebosclerosis. It is nearly always associatedwith conditions that may cause obstructions of theportal veins, such as hepatic cirrhosis and portalthrombosis.'

Li10 gave a detailed description of the changesin the portal vein in cases of portal hypertension.Of his series of 26 patients changes were demon-strated in 20, i.e. 76 per cent.-the lesions con-sisting of muscular hypertrophy of the media andintimal thickening with the development of longi-tudinal muscle fibres subintimally. In the stage ofdecompensation this medial myohypertrophywould fail and the muscle and elastic tissue wouldthen be replaced by fibrous tissue and phlebo-sclerosis would result. In this stage AdamiX

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noticed an increase of connective tissue in all thelayers with degeneration of the elastic tissue andremarked upon the fact that the connective tissuein the intima was poor in nuclei. He describedthis vein lesion as being due to a strain fibrosis.

Benda4 had mentioned fatty changes in theintima of the portal vein in hepatic cirrhosis andatheroma of the portal vein with ante-mortemthrombosis was described by Whiteley 19 in the caseof a 64-year-old woman who had a coarselylobulated cirrhosis of the liver.The possibility of sclerosis being secondary to

hepatitis which spread into the portal vein isdiscussed by Magovern and Muehsam.12 Primarysclerosis of the portal vein was reported by Reich,16Buday,5 Hart,8 Winkler,20 and Redo andO'Sullivan.15The case reported by Reich16 was that of an

Italian women aged 21, who died with multiplethrombosis of the portal, superior mesenteric, leftcommon iliac, external iliac, femoral, hypogastricand pulmonary veins, the submucosal veins ofthe small intestine and the veins of the lymphnodes. There was marked fibrous tissue prolifera-tion and diffuse round-cell infiltration surroundingthe thrombi with fraying, thickening and inter-ruption of the internal elastic membrane. Thepossibility that the phlebosclerosis is not actuallyprimary but is secondary to the thrombosis cannotbe excluded in such a case as this.Redo and O'Sullivan15 suggested that the

etiology of the sclerosis of the portal vein in thetwo cases reported by them was not the portalhypertension, but rather that the sclerosis mighthave been the probable cause of the hypertension.This suggestion could be considered only in thesecond case discussed by them who showed noevidence of abnormality on biopsy of the liver buthad sclerosis, calcification and probable throm-bosis of the portal vein, a portal pressure of200-210 mm. saline and oesophageal varices; butis hardly applicable to their first patient who hadevidence of hepatic cirrhosis and splenomegaly 14years before they operated on him and foundcalcification of the portal vein which prevented itsuse in a porto-caval shunt. At the present state ofour knowledge one cannot minimise the finding ofa definite cirrhosis in cases of portal hypertensionand must accept it as being the primary cause ofthe hypertension which leads to compensatorymedial myohypertrophy of the portal vein whichwould be followed by decompensation andphlebosclerosis.The histopathology of the portal vein in our

case showed it to be in an advanced stage of de-compensation sclerosis with the subintimal longi-tudinal muscle fibrils being evidence of previouscompensatory hypertrophy. This extreme fibrosis

of the wall of the veins had caused gross weakeningand resulted in progressive dilatation of the portalvein with the formation of an aneurysm. Ante-mortem thrombosis had taken place within theaneurysmal sac. The vascular clefts and thedeposits of fibrin seen in the intima indicated re-canalization and organization of these luminalthrombi.The following sequence of events must be

postulated: coarse atrophic cirrhosis of the liver(post-necrotic scarring) causing progressive portalhypertension which led to (a) rupture of theoesophageal varices (b) splenomegaly (c) phlebo-sclerosis of the portal vein leading to aneurysmaldilatation and ante-mortem thrombosis.

SummaryA case of a fusiform aneurysm of the portal vein

is described in a male aged 52 years who presentedclinically with a Banti's syndrome and who had asplenectomy. At autopsy a coarse irregularcirrhosis of the liver was found with evidence ofportal hypertension-phlebosclerosis and aneurys-mal dilatation of the portal vein.

AcknowledgmentWe are indebted to Mrs. A. Vorster and Mr. R.

Holloway for the microscopic sections and we alsowish to thank Mr. R. Holloway for photographingthe specimen and Mr. A. Shevitz for the printing.Our gratitude is extended to Mrs. J. E. Bailey

for secretarial assistance and for typing this articleand we thank Miss E. G. Lahner for transcribingthe numerous articles of reference.

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'System of Medicine.'2. BANTI, G. (I9Io), Folio Haemat., o0, 33, Translated from

Italian by Robert Tissot.3. BARZILAI, R., and KLECKNER, M. S. (I956), A.M.A. Arch

Surg., 72, 725.4. BENDA, C. (1924), 'Phlebosklerose Henke und Lubansch's

Handbuch der speziellen pathologischen Anatomie undHistologie,' vol. ii, p. 831, Berlin.

5. BUDAY, K. (I903), Zbl. allg. Path. path. Anat., 14, I6i.6. CHILD, C. G. (I954), 'The Hepatic Circulation and Portal

Hypertension.' Philadelphia: Saunders.7. DOUGLASS, B. E., BAGGENSTOSS, A. H., and HOLLINS-

HEAD, W. H. (I950), Surg. Gynec. Obstet., 91, 562.8. HART, C. (1913), Berl. klin. Wschr., 50, 2231.9. HOLMAN, S., 'Banti's Syndrome,' ' Thesis presented to the

University of Witwaterstrand,' February, I954.Io. LI, P.L. (I94o), J. Path. Bact., 50, I2I.II. LICHTMAN, S. S. (1949), ' Diseases of the Liver, Gall Blad-

der and Bile Ducts,' Ed. 2. Philadelphia: Lea and Febiger.12. MAGOVERN, G. J., and MUEHSAM, G. E. (I954), Amer. J.

Roentgenol, 7I, 84.I3. MOSCHOWITZ, E. (1942), 'Vascular Sclerosis,' p. 82.

London, New York, Toronto: Oxford University Press.I4 PURCELL, H. K., CONNOR, J. J., ALEXANDER, W. F.,

and SCULLY, N. M. (I95I), A.M.A. Arch. Surg., 62, 670.I5. REDO, S. F., and O'SULLIVAN, W. D. (I956), A.M.A. Arch.

Surg., 73, 532.I6. REICH, N. E. (I942), Arch. intern. Med., 69, I 7.I7. SHERLOCK, S. (1955), 'Diseases of the Liver and Biliary

System.' Oxford: Blackwell Scientific Publications.I8. SIMMONDS, M. (1912), Arch. Path. Anat., 207, 360.19. WHITELEY, H. J. (I953), J. Path. Bact., 66, 563.20. WINKLER, H. (1915), Frankfurt, Z. Path,:I7t 377.

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