Gut, 1972, 13, 982-985

Persistent ductus venosus without portal hypertensionin a young alcoholic manP. BARJON, J.-L. LAMARQUE, H. MICHEL, J. FOURCADE, A. MIMRAN,AND J.-F. GINESTIE

From the Departments of Medical Pathology, Clinical Medicine, and Radiology, Faculty of Medicine,University of Montpellier, France

SUMMARY The discovery of a large patent ductus venosus resulted from radiological investigationsin a 34-year-old man, a chronic alcoholic of low mental status. Splenoportal and inferior cavalvenograms were performed because of recent exacerbation of the neurological symptoms andelectroencephalographic criteria of portacaval encephalopathy. Portal pressure was 8 mm Hg. Aliver scan, a laparoscopy, and a liver biopsy were performed. They showed that the gland was atrophicwith a microscopic appearance of alcoholic fibrosis, but without any nodular regeneration. Therelationship between the fistula, the mental state, and the atrophic liver is discussed. Such a

malformation appears to be very uncommon.

Radiological investigations in the diagnosis of liverdysfunction, especially cirrhosis, lead commonly tothe discovery of portacaval shunts. Most of theseconnexions are explained by portal hypertensionitself. Yet, in some cases, the anastomotic veins havean unusual topography, either splenorenal or umbil-ical, and it is reasonable to assume the preexistenceof a spontaneous portacaval derivation.We recently observed a case of a large portacaval

fistula with the radiological configuration of apersistent ductus venosus.

Case Report

A 34-year-old man, a contruction worker of Italianorigin, was admitted to the Montpellier UniversityHospital in March 1970 because of psychiatricsymptoms of a few weeks' duration. He was aknown alcoholic and these symptoms were at firstrelated to his drinking. However, initial examinationrevealed a flapping tremor. The patient exhibitedalternately indifference and agitation, and he becamesomnolent for a few days; normal consciousnessreappeared rapidly and objective examination con-firmed mental subnormality which probably pre-ceded the recent psychiatric disturbance. Physicalexamination revealed no ascites or collateral circu-

Please address requests for reprints to: Dr Paul Barjon, CliniquesSaint-Charles, 34059 Montpellier Cedex, France.

Received for publication 4 October 1972.

lation and the liver and spleen were not enlarged;there was neither jaundice nor oedema and noevidence of abdominal bruit or thrill could beelicited.

Biological results showed: a low bromsulphaleinclearance (6%); prothrombin (53%); bilirubin 0.8mg per 100 ml. The serum protein level was 6 g per100 ml (serum albumin 48% and gamma globulins29%); SGOT and SGPT were normal. The venousblood ammonia level was 63 gg per 100 ml. Bloodurea nitrogen and blood sugar levels were normal,but an oral glucose tolerance test resulted in anexcessive rise in blood sugar, followed by a pro-longed and exaggerated plasma insulin response.

Fig. 1 Reproduction of the liver scan.

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Persistent ductus venosus without portal hypertension in young alcoholic man

Fig. 2 Splenoportal venogram. This figure shows clearly Fig. 3 Inferior caval venogram. After catheterization ofthe division ofthe portal vein: the right branch is atrophic, the other extremity of the portacaval shunt, dye wasthe left branch is greatly enlarged and probably has a high injected against the flow. Partial injection suggests theblood flow, as indicated by the dilution of the contrast existence of a high flow through the fistula, and confirmssubstance. It continues into a large tortuous vessel which the features seen on the splenoportal venogram.seems to be directed towards the spinal column. There areno visible derivative collaterals. The topography of theshunt provides consistent evidence ofa persistent ductusvenosus.

Fig. 4 Schematic representation of the radiographs. (1)Inferior vena cava: (2) left and (3) right branches of theportal vein; (4) opening of the left branch into theinferior vena cava; this appearance was seen on the cavalvenogram and also on the splenoportogram. (5) Repre-sentation of the right hepatic vein which did notcommunicate with thefistula.

Radiological investigation showed no oesophagealvarices. A liver scan (Fig. 1) showed atrophy of thegland. A laparoscopy was performed which showedthat the liver was atrophic, with a smooth surfaceand no regenerative nodules. A liver biopsy revealedsevere dissecting fibrosis with Mallory bodies, peri-portal inflammation, and diffuse fat. A right hepaticvein was catheterized and appeared normal; thewedge hepatic venous pressure was 2.5 mm Hg aboveT.V.C. A first EEG recording, performed during the

Fig. 5 Inferior caval venogram. Direct injection of theright hepatic vein, which appeared to be normal in itsshape and position.

period of severe neurological disturbance, showedslow cerebral activity with diphasic and trapezoidalwaves compatible with a portacaval encephalopathy.Recordings were later done during the patient'srecovery and from time to time demonstrated thesame abnormalities, although not so clearly.

Because of the abnormal liver and of the presenceof the neurological syndrome, with no evidence ofportal hypertension, a portal venogram was requested(Fig. 2). Intrasplenic pressure was 8 mm Hg; the

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P. Barjon, J.-L. Lamarque, H. Michel, J. Fourcade, A. Mimran, and J.-F. Ginestie

spleen, splenic and portal veins were found to benormal, but the right portal vein branch was verynarrow, with an atrophic distribution; the left branchhad increased dimensions but no collateral vesselsto the left lobe could be seen. In fact, the left branchseemed to anastomose directly with the inferior venacava through a wide channel. A cavogram (Figs. 3 and4) showed the same formation and confirmed theportacaval fistula. The right hepatic vein (Fig. 5)seemed to be normal. A hepatic arteriogram did notreveal any other abnormality.Ammonia blood concentration was measured in

the right hepatic vein (30 ,ug/100 ml), in the inferiorvena cava (96 ,g/100 ml), and in the right atrium(80 ,ug/100 ml). Unfortunately, no ammonia levelcould be measured inside the fistula itself, as theblood flow was very high and prevented catheteriza-tion. Removal of ammonia from the blood was alsostudied following an intravenous infusion of 800 mgof NH4CI. The disappearance curve was within thenormal range; however, the EEG recorded duringNH4Cl loading showed transient trapezoidal slowwaves.A blood volume determination was performed by

the 51Cr red cell labelling method. The result was7 610 ml, a 37% increase according to weight andsurface; the cardiac index (Cardiogreen method)was 4.16 1/min/m2. Plasma renin activity was 12ng/1/min in basal conditions, a low value accordingto our method. Tetra-hydro-aldosterone urinaryexcretion was less than 4 ,ug/day.The patient was discharged on a low protein diet

and abstinence from alcohol was recommended.Unfortunately, no follow up is available.

Discussion

Although a full demonstration was not obtained inthe absence of direct catheterization, there is strongradiological evidence that the portacaval shunt foundin this patient represents a patent ductus venosus.However, the relationship between this abnormalityand the patient's other disorders seems complex.Most of the unusual portacaval anastomoses have

been found in patients with both raised portal press-ure and liver cirrhosis. This apparent relationshipcould be explained by the fact that radiological vas-cular investigations are performed on these particularpatients. However, this finding means alse that thepresence of a direct shunt of congenital originbetween the portal and the caval systems is rarely, ifat all, sufficient to prevent the development of portalhypertension. Also, it has become reasonable toassume that these anastomoses are small and non-functional at first (or, at least, that their blood flowis insignificant) and that their anatomical extension

is an expression of portal hypertension itself. Forthis reason, during the simultaneous aggravation ofthe causal liver cirrhosis (usually in the alcoholicform), their subsequent role was never importantenough to keep portal pressure levels down to normal.

In some cases, portal pressure was found to becloser to normal in the presence of a congenitalshunt rather than is common in cases of portalhypertension (Detrie and Martini, 1960) but therewas no clear relationship between the diameter ofthe shunt and the portal pressure. Anatomicalpeculiarities, such as tortuousness of the shunt, werepostulated to explain this fact (Detrie and Martini,1960; Paris, Gerard, Toison et al, 1969).

Splenorenal and umbilical derivations (Cruveilhier-Baumgarten syndrome) have often been observed.Other anatomical types have rarely been found; theyinclude observations of splenocaval fistulae (Detrieand Martini, 1960) and venous formations havingthe characteristics of a persistent-or recanalized-ductus venosus (Detrie and Martini, 1960; Boquien,Morin, Leger, Roy-Camille and Graveleau, 1961;Coppo and Agnolucci, 1968; Neidballa, 1966).The opposite concept has been suggested by the

demonstration of the existence of similar congenitalmalformations in normal people, ie, without portalhypertension or liver cirrhosis. This has been shownby casual observations of patients with variousdiseases (Caroli, Paraf, and Schwarzmann, 1951;Piccone, Lentino, and Le Veen, 1967) and by groupnecropsies (Caroli et al, 1951). This finding indicatesthat such a shunt could develop in the absence ofportal hypertension. Conflicting reports on whatbecomes of the ductus venosus point either to theconstant early obliteration of this channel (Fontan,1911; Ostroverkhov and Nikolsky, 1968, Asuncionand Silva, 1971) or to its persistence, with onlyinterruption of its function (Doviner, 1962). Theexistence of a fully functioning ductus venosus hasrarely been reported (Caroli et al, 1951).A stimulating problem is created by the recent

observation of several cases of congenital portacavalfistulae with coexistent liver disease, but with strictlynormal portal pressure (Darnis, Mosse, Gutmann,Khalifat, Amigues, and Gillot, 1966; Mahoudeau,Dubrisay, and Ruff, 1965; Price, Voorhees, andBlakemore, 1963; Raskin, Price, and Fishman, 1964).The usual initial symptom is a recurrent encephalo-pathy with hyper ammonaemia, without any clinicalevidence of portal hypertension (Darnis et al, 1966;Raskin et al, 1964). We observed this fact in thepresent case, and it was also found in patients withother types of congenital anastomoses (Price et al,1963; Raskin et al, 1964). Thus, there is a strongrelationship between portacaval encephalopathy andconcomitant liver dysfunction. Years ago, experi-

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Persistent ductus venosus without portal hypertension in a young alcoholic man 985

mental studies (Eck's fistulae) and partial hepa-tectomies gave identical findings, mainly that neuro-logical disorders do not occur until hepatic functionbecomes severely impaired (Islami, Pack, Miller,Vanamee, Randall, and Roberts 1956; Liebowitz,1959). These facts seem to imply that the neurologicalsymptoms, which are presumably due to ammoniaintoxication, are related to a decrease in the hepaticclearance of ammonia rather than to the direct con-sequence of the portacaval shunt (Coppo andAgnolucci, 1968; Leger and Henriot, 1971). However,our patient still demonstrated a certain capacity forurea synthesis, because the hepatic vein level ofammonia was lower than found elsewhere in thebody, and removal of infused ammonia was stillpossible.An interesting question is the pathogenesis of the

liver alteration that we observed. Although themicroscopic studies of the liver showed lesionssuggestive of an alcoholic origin, we were reluctantto ascribe them to this aetiology only. Several facts-such as the macroscopic aspect of the liver, theabsence of nodular formations, and the age of thepatient-support the concept that chronic portalbypass ofthe liver may have a functional significance.Most of the blood appeared in fact to be divertedthrough the fistula. Perhaps our patient's subnormalmentality itself resulted from chronic cerebralammonia intoxication due to the congenital fullyfunctioning shunt. Severe hepatic fibrosis has beenobserved with types of shunt other than Arantii'sduct (Price et al, 1963); the absence of any ethanolintoxication could be verified in some cases (Raskinet al, 1964; Darnis et al, 1966). Experimental studiessupport the conclusion that chronic portal bypass ofthe liver leads to fibrosis and atrophy of the gland.This fact is presumably related to the diversion ofhepatotrophic substances from portal blood originor to the decrease of blood flow itself through theliver (Fisher, Fisher, and Lee, 1967).

Lastly, we should like to emphasize the existenceof systemic haemodynamic abnormalities. A hyper-dynamic condition was demonstrated in our patient,with an increased blood volume and a high cardiacoutput. These changes appeared to be related to thehuge, longstanding fistula, although it was a veno-venous shunt. Predominance of the dynamic con-dition upon the hepatic alteration was indicated bydecreased aldosterone secretion.

Conclusion

Our patient seems to represent an exceptional case ofpatent ductus venosus. The vascular malformationappears to have had an important role from the verybeginning of the patient's life, with possible con-sequences for his mental state. As a clinical demon-stration of an Eck's fistula, it is postulated thatchronic diversion of the portal blood from the livercan lead to atrophy of the gland and sensitize it tosecondary alcoholic damage.

References

Asuncion, Z. G., and Silva, Y. J. (1971). Surgical significance of theductus venosus Arantii. Amer. J. Surg., 122, 109-111.

Boquien, Y., Morin, M., Leger, L., Roy-Camille R., and Graveleau,J. (1961). Hypertension portale et hypertension cave inf6rieureassoci6e. Presse med., 69 2249-2252.

Caroli, J., Paraf, A., and Schwarzmann, V. (1951). Traitements nou-veaux ou soi-disant tels de I'ascite cirrhotique et leurs basesphysio-pathologiques. Rev. int. HOpat., 1, 61-217.

Coppo, M., and Agnolucci, M. T. (1968). Quelques problemes pos6spar les anastomoses porto-caves spontanees. Actual. hepato-gastroent., 4, B 103-112.

Darnis, F., Mosse, A., Gutmann, A., Khalifat, S. R., Amigues, N., andGillot, C. (1966). Contribution a 1'6tude des consequences desanastomoses porto-caves spontan6es. Sem. Hop. Paris, 42,3108-3114.

D6trie, P., and Martini, R. (1960). Les anastomoses porto-cavesspontan6es. J. Chir. (Paris), 80, 452-475.

Doviner, D. G. (1962). Arantius's duct in adult man. (Russian) Arkh.Anat., 42, (1), 93-102.

Fisher, B., Fisher, E. R., and Lee, S. (1967). Experimental evaluationof liver atrophy and portacaval shunt. Surg. Gynec. Obstet.125, 1253-1258.

Fontan, C. L. M. J. (1911). Le canal veineux d'Arantius. (Thesis(Lille), no. 26), Lille.

Islami, A. H., Pack, G. T., Miller, :T. R., Vanamee, P., Randall, H.T., and Roberts, K. E. (1956). Postoperative course followingtotal right hepatic lobectomy. Surgery, 39, 551-556.

L6ger, L., and Lenriot, J-P. (1971). Retentissement fonctionnel etcons6quences m6taboliques des d6rivations porto-caves chez lecirrhotique. Presse mid., 79, 699-700.

Liebowitz, H. R. (1959). Bleeding Esophageal Varices: Portal Hyper-tension, p. 347. Thomas, Springfield, Illinois.

Mahoudeau, D., Dubrisay, J. and Ruff, F. (1965). Enc6phalopathiem6tabolique, anastomose porto-cave spontan6e et p6ricarditeconstrictive. Bull. Soc. Med. Paris, 116, 959-969.

Neidballa, R. G. (1966). A large spontaneous portacaval shunt. Arch.intern. Med., 117, 54-56.

Ostroverkhov, G. E., and Nikolsky, A. D. (1968). Pre-and post-natalanatomy of Aranti's duct. Arkh. Anat., 55 (11), 6-10.

Paris, J., Gerard, A., Toison, G., et al (1969). etude des anastomosesspontan6es et fonctionnelles entre les syst6mes porte et caveinf6rieur au cours des cirrhoses du foie. Ass. mid. lang. franC.,5, 117-127.

Piccone, V. A., Lentino, W., and Le Veen, H. H. (1967). Radiologicdemonstration of a patent ductus venosus in an adult. Gastro-enterology, 52, 263-266.

Price, J. B., Jr., Voorhees, A. B., Jr., and Blakemore, A. H. (1963).Spontaneous hemodynamically effective portasystemic shunts.Ann. Surg., 158, 189-194.

Raskin, N. H., Price, J. B., and Fishman, R. A. (1964). Portal-systemicencephalopathy due to congenital intrahepatic shunts. NewEngl. J. Med., 270, 225-229.

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