Etiologic Factors and Consequences ofSplenic Vein Obstruction

FRANK R. JOHNSTON, M.D., RICHARD T. MYERS, M.D.

SPLENIC VEIN obstruction is an infrequently recognizedand infrequently reported intra-abdominal vascular

problem. It is usually grouped with portal hypertension,rarely being considered a separate entity. Thus, Sutton,4 in1968, found only 53 cases of isolated splenic vein obstruc-tion in the English literature. No cases of primary splenicvein obstruction have been reported; it is always sec-ondary to or a complication of localized upper abdominaldisease or a generalized systemic process. The course ofthat primary disease may be profoundly altered, how-ever, by this complication, which often is manifest bygastrointestinal bleeding. When bleeding occurs, sple-nectomy is the procedure of choice, combined with theappropriate treatment of the primary disease. In thisreport we will present a summary of eight cases ofsplenic vein obstruction.

Clinical MaterialBetween 1955 and 1971, eight patients were seen at

the North Carolina Baptist Hospital with surgical, patho-logic, or angiographic evidence of isolated splenic veinobstruction. Six of these cases were observed within thelast 2 years. The data from the eight cases are presented inTable 1.

Clinical Histories: Of the three patients presentingwith massive hematemesis, melena or both, two hadundergone, at another hospital, an operative procedurethat had unsuccessfully controlled the bleeding, and hadbeen transferred immediately to our hospital for furthertreatment.

Laboratory Findings: Anemia was present only in thepatients with hematemesis and melena and could beaccounted for by the blood loss. Bone marrow studies

Presented at the Annual Meeting of the Southern Surgical Asso-ciation, December 4-6, 1972, Boca Raton, Florida.

Please address reprint requests to Dr. Johnston.

From the Department of Surgery, The Bowman Gray Schoolof Medicine of the Wake Forest University,

Winston-Salem, North Carolina 27103

were done on two patients: the marrow was slightly hyper-plastic in one and hypoplastic in the other. None of theeight patients had clearcut evidence of hypersplenism.

Angiography: Celiac axis angiography was done in thesix patients seen after angiography had become a com-mon diagnostic procedure. Splenoportography was donein one of the six as well. Representative angiograms areshown in Figures 1 and 2.

Diagnoses: Disease of the pancreas was primary in allof these cases (Table 1). The diagnosis was clearcutin six; in the two cases diagnosed as nonspecific pancre-atitis, a mass containing multiple small cystic spaces wasfound at operation. The mass incorporated the pancreasand the splenic vein, and there were huge, dilated, tor-tuous and thin-walled veins about the stomach, omentumand splenic pedicle. The pathologist did not believethat the material submitted for diagnosis was clearly acystadenoma.

Results: One patient died. The remaining seven pa-tients were discharged and have remained well, withthe exception of the two with carcinoma.

DiscussionSplenic vein obstruction may be caused by thrombosis,

benign or malignant tumors, inflammation, or fibrosis.5The course of the splenic vein along the superior-posteriorsurface of the pancreas, where it often forms a groovein the pancreas makes it susceptible to entrapment by anydisease involving the pancreatic body and tail. Obstruc-tion of this vein becomes life-threatening only when bleed-ing into the gastrointestinal tract or the peritoneal cavityoccurs. Rupture of the subserosal venous collateral cir-culation with intraperitoneal hemorrhage is a particu-

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larly treacherous complication, and anyone observing,during celiotomy, how these vessels bleed at the slightesttouch must wonder why this complication does not occurmore often. The stomach is the most common site ofbleeding, however, since there the thin-walled, dilated,submucosal collateral vessels are subject to corrosion byacidic peptic juice. When such hemorrhage is the mani-festation of splenic vein obstruction, we believe thatsplenectomy alone is the procedure of choice and that itcan be expected to control both intragastric and intraperi-toneal bleeding. Any other surgical maneuver is directedat the primary disease causing the obstruction.When splenic vein obstruction is present without com-

plicating hemorrhage, the therapy should be directedtoward the primary disease, and splenectomy should bedone or not, depending on the surgeon's assessment of thecase. It is of interest that Arner,1 using follow-up or serialsplenoportography observed re-opening of the splenicvein in one patient 17 months after the initial roentgeno-graphic diagnosis had been made. Whether this is aregular phenomenon is not known.The development of abdominal angiography has

greatly enhanced our understanding of this complicationof upper abdominal disease. The detection of six casesin the last two years, all diagnosed by angiography, con-trasts sharply with the two cases seen in the previous 15years when diagnosis depended on observation at opera-tion or autopsy. The two angiographic methods of observ-ing the splenic venous circulation are complementary.Celiac axis injection is the more versatile, and allows studyof other arterial beds; the venous phase must be includedin the study if all information is to be obtained. Althoughsplenoportography yields better definition of the splenicvenous bed when clarification of detail is essential to theproposed treatment, the hazard to the patient is increasedby splenic puncture.The development of angiography as a diagnostic tool

has allowed clarification of the course of the collateralcirculation in isolated splenic vein obstruction. In the sixpatients in this series in whom angiography was done,the blood draining from the spleen returned to the portalvein by one of three routes: 1) retrograde flow throughthe short gastric veins, across the fundus of the stomachto the left and right gastric or coronary vein, and thenceto the portal vein; 2) retrograde flow through the left gas-troepiploic vein, across omental branches to the branchesof the right gastroepiploic vein, and thence to the superiormesenteric or portal vein; 3) less commonly, retrogradeflow through the left gastroepiploic vein to omentalbranches or branches of the left colic vein, andthence to the inferior mesenteric vein. Leger2 has demon-strated a fourth route, via the diaphragmatic and inter-costal branches back to the caval system. If the left

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JOHNSTON AND MYERS

FIG. 1. Celiac axis angio-gram (left) and spleno-portogram (right) fromCase 5. On the left, thearterial phase clearly de-lineates the tortuous sple-nic artery and the en-

larged spleen. The splenicvein did not fill on any

of the films in this se-quence. On the right, thisframe clearly outlines thedilated tortuous collater-als over the fundus of thestomach filling the coro-nary and portal veins.The left and right gas-troepiploic veins also actas collaterals.

gastric or coronary venous system is open and availablefor drainage, the esophageal plexus is apparently rarelyinvolved, and esophageal varices rarely develop. Thatthere may be other pathways is certainly probable sincethe upper abdomen is more generously supplied withcollateral arterial and venous circulation than any otherpart of the body.3

It is of interest that none of these patients had clearcutevidence of hypersplenism.

Summary

Eight patients with isolated splenic vein obstructionhave been seen and treated at the North Carolina Baptist

Hospital over the past 17 years. From a review of thesepatients, the following conclusions are suggested.

Splenic vein obstruction may be caused by a variety ofpathologic conditions. All in these patients were relatedto the pancreas. Splenic vein obstruction is rarely ifever a primary disease.

Splenic vein obstruction may produce intragastric orintraperitoneal bleeding that may be life-threatening.Splenectomy is then the procedure of choice.

Splenic vein obstruction may produce no signs or

symptoms.Splenic vein obstruction usually results in the develop-

ment of collateral pathways to the portal vein through thegastric or gastroepiploic veins.

FIG. 2. Two frames inthe series of celiac axisangiograms from Case 6.On the right, the arterialphase shows the largespleen and on the left,the venous phase delin-eates dilated tortuousveins filling retrogradelyto the epiploics, omental,left colic, and then to theinferior mesenteric vein.The portal vein is faintlyoutlined in the right up-per quadrant above theright renal pelvis.

738 Ann. Surg. - June 1973

Vol. 177 * No. 6 SPLENIC VEIN OBSTRUCTION 739

Celiac axis arteriogram and splenoportography are theonly reliable means of diagnosing this complicationother than operation or autopsy.

References1. Arner, 0. and Fernstr6m, I.: Obstruction of the Splenic Vein,

A Splenoportographic Study of the Clinical Features of"Thrombosis of the Splenic Vein" with Notes on Its Treat-ment. Acta Chir. Scand., 122:66-74, 1961.

2. Leger, L.: Splenoportography. Charles C Thomas, Springfield,1966.

3. Michels, N. A.: Blood Supply and Anatomy of the Upper Ab-dominal Organs; with a Descriptive Atlas. J. B. LippincottCompany, Philadelphia, 1954.

4. Sutton, J. P., Yarborough, D. Y. and Richard, J. T.: IsolatedSplenic Vein Occlusion. Review of Literature and Report ofan Additional Case. Arch. Surg., 100:623, 1970.

5. Yale, C. E. and Crummy, A. B.: Splenic Vein Thrombosis andBleeding Esophageal Varices: JAMA, 217:317, 1971.

DISCUSSIONPROFESSOR J. PHILIP SANDBLONI (Lausanne, Switzerland): I

was very interested in commenting on this beautifully presentedseries of a disorder that has seemed to be very rare. I agreewith the authors that it might be occurring more often than hasbeen thought.The reason for my opinion is that 10 years ago we went

through our material of portal hypertension in over 200 patients.By way of splenoportography we found four patients with splenicvein obstruction.

Three of them bled from ruptured esophageal varices. Onlvone of the four had pancreatic disease as primary cause; thethree others were unexplained. They might have been of a dif-ferent kind than the ones presented. Their symptoms of pain,hemorrhage, and short-lasting ascites recurred several times.

All were treated by splenectomy, and none rebled. One patientdied because of recurrent thrombosis that continued up into themesenteric vein, and a large intestinal resection was performed.Another point of interest is the source of hemorrhage in portal

hypertension. It has been thought that these patients generallybleed from ruptured varices in the esophagus or the fundus of thestomach. If the bleeding source is hemorrhagic gastritis, this hasnot been thought to be related directly to their portal hypertension.Patients may bleed from many sources in portal hypertension, in-cluding the esophagus, the gastric mucosa, and even the biliarytract. Twice I found hemorrhage into the gallbladder.

I think the primary cause is the portal hypertension, throughthe congestion of the mucosa which secondarily succumbs to thedeleterious effects of the gastric juice in hyperacidity and drugabuse. The series that the authors here reported so beautifullyis also an example of the fact that the hemorrhage in portalhemorrhage is not necessarily due to burst varicosities of theesophagus.

Dli. ATEF SALA_M (Atlanta): During the last 18 months wesaw five patients with splenic vein thrombosis, three of whom pre-sented with upper GI bleeding. All of them had previous vagect-omy and pyloroplasty, although the cause of bleeding was notrecognized at the time of surgery.

[Slide] The workup of these patients should include livercatheterization, direct or indirect splenoportography and superiormesenteric angiography. This slide demonstrates the most char-acteristic angiographic feature of this disease, namely a tortuous,dilated gastroepiploic vein.The diagnosis may not be made preoperatively if it is not

suspected or if the studies could not be done because of severebleeding. The correct diagnosis can be made at the time ofoperation if the surgeon is familiar wvith the operative findingscharacteristic of this disease. Dilated gastroepiploic veins andgastric varices in absence of any evidence of portal hypertensionin the superior mesenteric venous bed should raise the possibilityof splenic vein thrombosis. The diagnosis can be confirmed by

threading a catheter through one of the branches of the superiormesenteric vein into the portal vein. [Slide] In contrast to patientswith generalized portal hypertension, portal pressure and liverperfusion with portal blood remain normal in patients with iso-lated thrombosis of the splenic vein. Contrast material injecteddirectly into the portal vein would not opacify the coronary veinof the variceal plexus since the direction of blood flow in thesevessels is toward the portal vein.

Finally, I would like to raise two questions regarding theoperative management of pancreatic pseudocyst associated withsplenic vein thrombosis. Splenectomy in these patients will leavethe cyst in free communication with the rest of the peritonealcavity. I wonder whether Dr. Johnston would agree to stagingthe operation, namely the cyst is internally drained first andsplenectomy is delayed to a later date. The second question is:"WVhat would be the method of choice for internal drainage ofthe pseudocyst in presence of splenic vein thrombosis?" Dr. Zeppaand Dr. Smith in their excellent presentations indicated thatbleeding is sometimes encountered postoperatively following cysto-gastrostomy. The risk of this complication is even greater inpresence of splenic vein thrombosis because of the increasedvascularity of the stomach wall associated with this disease. Forthis reason, we prefer cystojejunostomy for internal drainage ofpancreatic pseudocyst in such patients.

DR. RICHARD T. MYERS (Closing): We are aware of the greatwork Professor Sandblom has done in this area, and thank himfor emphasizing the points that were important to us; namely, sur-prise at the infrequency with which we have encountered it inrecent years is probably due more to the increasing incidence ofpancreatic disease, rather than our diagnostic acumen.We would certainly agree that this is a form of localized portal

hypertension, and that one must consider the possibility of bleed-ing from the gastric mucosa as well as the esophageal varices.

Dr. Salam, we thank you for your comments. It's an interest-ing parallel to the increasing frequency in Winston-Salem andAtlanta. We would agree that the diagnosis cannot be made pre-operatively as to the primary etiologic factors concerned.

So far as your posed question, I think in the absence ofbleeding from the pseudocyst we would certainly agree that theprocedure should be staged.

Finallv, in the interest of time, I would like to simply underlineand emphasize one of the points; namely, the use of angiographyin upper GI bleeding. I think this paper would tend to-at least,to me-indicate the value of looking at the venous phase as wellas the arterial phase. We tend to become very enthralled atwhat is going on within the lumen of the bowel, and not thevenous phase of the angiography. This has been a great boon tothe preoperative diagnosis and management of gastrointestinalbleeding, and I think, properly extended and utilized, it can beof great value in uncovering some of the obscure causes whichwe are now missing.