557 0361-803x/79/1324-0557 $0.00
Portal Vein Aneurysm
HUGH S. VINE,”2 JOSEPH C.SEQUEIRA,2 WARREN C. WIDRICH,3 AND BARRY A....
Fig. 1.-Case 1. A, Transverse view through liver
and porta hepatis. Splenic vein (arrowhead) drains
into enlarged portal v...
Fig. 3.-Case 3. Venous phase of superior mesenteric angiogram.
Large portal vein aneurysm (arrow) lies directly beneath ri...
560
REFERENCES
VINE ET AL.
1 . Harrell G: Ventral portal vein. AJR 121 :369-373, 1974
2. Marks C: Surgical implications of...
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Portal Vein Aneurysm

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Portal Vein Aneurysm

  1. 1. 557 0361-803x/79/1324-0557 $0.00 Portal Vein Aneurysm HUGH S. VINE,”2 JOSEPH C.SEQUEIRA,2 WARREN C. WIDRICH,3 AND BARRY A. SACKS’ While there have been a few references to portal vein aneurysm In the world literature, this is the first report in United States radlologic literature. During a routine evaluation for fever In one patient, an ultrasound examination suggested this unusual entity at the junction of the splenic and superior mesenterlc veIn. It was later confirmed by anglography. Two other patients were being investigated anglographically for gastrointestinal bleeding when portal vein aneurysms were discovered. In contrast to the central location of the first patient’s aneurysm, the latter two were more distal in the portal tree. The literature is reviewed and different etlologic hypotheses discussed. Venous abnormalities of the portal system have been thoroughly investigated [1-4]. However, an aneurysm of the portal vein is uncommon. While investigating a patient (case 1) for a fever of unknown origin, abdominal ultrasound demonstrated a large anechoic area at the junction of the superior mesenteric and splenic veins. Subsequent angiography confirmed the initial impres- sion that this represented a localized dilated venous structure. As this unusual entity was being researched, two similar cases were made known to us. Barzilai and Kleckner [5] were apparently the first to report such an entity. They described a patient who developed postnecrotic cirrhosis, thrombosis of the right portal vein, and an aneurysm proximal to the thrombus with subsequent rupture into the biliary system. Seven other cases have been reported since, but none have used ultrasound evaluation [6-11]. Three more cases of portal vein aneurysm are reported here. Case 1 Case Reports A SO-year-old woman was admitted to the Peter Bent Brigham Hospital for evaluation of fever of unknown origin. Over the previous 10 years, several episodes of hemolytic anemia asso- ciated with elevated cold agglutinins had been successfully treated with steroids. Because of the fever and possible abdom- inal abscess, ultrasound was performed with both real-time phased-array scanning and a commercially available gray scale unit using a standard 2.25 MHz internally focused transducer. A 3.0 cm well circumscribed anechoic structure was noted at the junction of the splenic and portal veins. This was believed to most likely represent a portal vein dilatation or aneurysm (fig. 1A), and the phased array unit seemed to confirm this impres- sion by demonstrating continuation of this structure with the intrahepatic portal venous radicles. Because this rare diagnosis was still uncertain, celiac arteriography was performed. During the arterial phase (fig. 1B), there was bowing and displacement of the gastroduodenal artery laterally. as well as minimal irreg- ularity of the intrahepatic radicles. In the venous phase (fig. 1C). a large portal vein varix was seen at the confluence of the splenic and superior mesenteric veins. In addition, there was slight reflux of contrast material down the superior mesenteric vein. A 99mTc sulfur colloid liver scan demonstrated moderately heterogeneous uptake consistent with mild diffuse hepatic parenchymal disease. No liver biopsy was done. Case 2 A 57-year-old man was admitted to the Boston Veterans Administration Hospital. He was a known chronic alcoholic with esophageal varices and several past episodes of upper gastroin- testinal bleeding. He first underwent a side-to-side splenorenal shunt and liver biopsy, which showed postnecrotic cirrhosis. Postoperatively, he did well for 12 days, when he developed massive upper gastrointestinal bleeding. Splenic arteriography and left renal venography demonstrated that the shunt had occluded. The venous phase of superior mesenteric arteriography showed a patent paraumbilical vein apparently draining from the right portal vein (fig. 2). A 3.5 cm venous aneurysmal dilatation was shown at the anastomosis of the portal vein branch and the paraumbilical vein. The bleeding was controlled with vasopres- sin (Pitressin) infusion. The venous hum of the Cruveilhier- Baumgarten syndrome was heard over the umbilicus. When the Pitressin was infused, the hum stopped, but when the Pitressin was discontinued, the hum returned. Almost 7 weeks after initial surgery, he underwent an end-to- end portacaval shunt. No note was made of intraoperative visualization of the aneurysm. Postoperatively. he went into metabolic acidosis and died 11 days later. No autopsy was obtained. Case 3 An 87-year-old white woman was transferred to Beth Israel Hospital with abdominal pain and hematemesis. She had a history of peptic ulcer disease (after vagotomy in 1962). During the night before admission, she developed sharp epigastric pain and vomited bright red blood. On admission, hematocrit was 29 and nasogastric lavage revealed bright red blood. Endoscopy showed an active bleeding site at the gastroesophageal region. Because of continued bleeding, the patient was referred for angiography. Celiac and superior mesenteric arteriography failed to demonstrate an active bleeding site. The parenchymal phase of hepatic arteriography showed an inhomogenous pat- tern consistent with diffuse hepatic disease (etiology unknown); the venous phase showed a large round intrahepatic collection of contrast believed to represent a portal vein aneurysm (fig. 3). Active bleeding stopped during arteriography, and hematocrit and metabolic status returned to normal. After several days of medical therapy, an upper gastrointestinal examination docu- Received October 31 , 1977; accepted after revision January 3. 1979. ‘Department of Radiology. Beth Israel Hospital. Harvard Medical School, 330 Brookline Avenue, Boston, Massachusetts 02215. Address reprint requests to H. S. Vine. 2Department of Radiology. Peter Bent Brigham Hospital. Harvard Medical School, Boston. Massachusetts 02115. 3Department of Radiology, Veterans Administration Hospital, Tufts University School of Medicine, Boston, Massachusetts 02130. AJR 132:557-560, AprIl 1979 © 1979 American Roentgen Ray Society
  2. 2. Fig. 1.-Case 1. A, Transverse view through liver and porta hepatis. Splenic vein (arrowhead) drains into enlarged portal vein (arrow). B, Arterial phase of celiac arteriogram. Bowing and displacement of gas- troduodenal artery laterally and slight irregularity of hepatic artery radicles. C. Venous phase. Large varix (arrow) of portal vein with reflux down superior mesenteric vein (arrowhead). 558 VINE ET AL. AJR:132, April 1979 mented a greater curvature gastric ulcer. She was discharged on a conservative regimen. Discussion The portal venous system develops from the vitelline and umbilical veins that drain the primitive intestine. Various congenital abnormalities of the portal vein have been described. The portal vein may be duplicate [2], receive anomalous pulmonary venous drainage [2, 3], become atretic [2], develop cavernous transformation [12], assume an abnormally ventral position [1], or man- ifest other anomalies of lesser radiographic importance. The size of the portal vein is somewhat variable. In 92 dissections, Douglass et al. [13] found that the diameter of portal veins in patients without cirrhosis or portal hypertension was 0.64-1 .21 cm (average, 0.89 cm). Pur- cell et al. [14] found the average diameter to be 1 .09 cm. Reynolds [15] noted that although the average diameter is 1 .2 cm, larger portal veins are found in cirrhotics. Doust and Pearce [16] ultrasonically studied both normal and cirrhotic patients, and the maximum anteroposterior diameter did not exceed 1 .5 cm in normals and 1 .9 cm in cirrhotics. Aneurysms of the portal vein may occur either proxi- mally at the junction of the superior mesenteric vein and splenic vein or more distally in the portal vein radicles. These aneurysms are thought to have two main etiolo- gies: (1) congenital or (2) acquired secondary to portal hypertension. Liebowitz and Rousselot [9] reported a case of agnogenic myeloid metaplasia with a portal vein aneurysm, but without evidence for portal hypertension because of normal intrasplenic pulp pressure and ab- sence of collateral venous circulation in the splenopor- togram. Thomas [8] also believed that his two cases of
  3. 3. Fig. 3.-Case 3. Venous phase of superior mesenteric angiogram. Large portal vein aneurysm (arrow) lies directly beneath right hemidia- phragm. vein branch and draining paraumbilical vein (arrowheads). AJR:132, April 1979 PORTAL VEIN ANEURYSM 559 script. portal vein aneurysm were either hereditary or develop- mental because of the absence of gross intra- or extra- hepatic obstruction. Thus, he postulated the portal hy- pertension was secondary to venous aneurysms. In con- trast, Barzilai and Kleckner [5] believed that the hemo- cholecyst in their patient after rupture of a portal vein aneurysm resulted from portal hypertension secondary to postnecrotic cirrhosis. Leonsins and Siew [6] likewise believed that in their patient with Banti’s syndrome and portal vein aneurysm, the most likely progression was cirrhosis causing portal hypertension, which led to esophageal variceal rupture, splenomegaly, and portal vein aneurysmal dilatation. In our three cases, only one (case 2) had unequivocal evidence for hepatocellular disease. However, in the other two cases, the liver scintigram (case 1) and angio- grams (cases 1 and 3) demonstrated evidence for hepatic parenchymal abnormality. Thus, as with all the cases in the literature, liver parenchymal abnormalities were as- sociated with this unusual entity, but their sequence is difficult to ascertain. As discussed above, some authors believe that the portal vein aneurysm is secondary to portal hypertension. Wenz et al. [10] presented the most convincing evidence for a congenital or developmental abnormality. Their 6-year-old patient demonstrated por- tal hypertension, hepatofugal flow, and varices with biopsy-proven normal hepatic parenchyma. Unfortu- nately, our three cases do not clarify the etiologic issue. Case 1 is unique because it is the only reported case with an ultrasonic image of the portal vein aneurysm. This well defined anechoic structure within the liver could be mistaken for a hepatic cyst if the connection with portal venous system were not appreciated. A high- speed scanner makes this characteristic more easily apparent. Once the vascular nature of this lesion is understood, the ultrasonic picture is essentially pathog- nomonic. Previously reported cases were evaluated an- giographically. Most of the cases in the literature were discovered because of investigation for gastrointestinal bleeding. Several of these patients with both portal hypertension and portal vein aneurysms underwent por- tacaval shunts with excellent results [7, 8, 11]. Two other patients died from rupture of their aneurysm. One of Thomas’s [8] patients bled into his lesser sac and retro- peritoneum. The patient of Barzilai and Kleckner [5] ruptured into the biliary system with a resultant hemo- cholecyst. Thus, although the severity of these lesions seems apparent, these patients were all studied for acute gastrointestinal hemorrhage. It is not known what the significance is in patients (as in our case 1) who have neither bled nor have severe portal hypertension. With the more frequent use of noninvasive studies such as ultrasound and computed tomography, both the incidence of portal vein aneurysms and their prognosis may become established. The etiology of these aneu- rysms is still unclear, but may be elucidated by serial studies. ACKNOWLEDGMENT We thank Deirdre Sullivan for help in preparing the manu-
  4. 4. 560 REFERENCES VINE ET AL. 1 . Harrell G: Ventral portal vein. AJR 121 :369-373, 1974 2. Marks C: Surgical implications of portal system malforma- tion . Ann R Coil Surg Eng! 55 : 299-306, 1974 3. Olberg 5, Olsson A: Congenital absence of portal venous system in a 50 year old woman. Acta Med Scand 196:343- 345, 1974 4. Samanek M, Tuma 5, Benesova D, Povysilova V. Prazsky F, Capova E: Atresia of right pulmonary veins and anomalous left pulmonary venous drainage into portal circulation. Thorax 29:446-450, 1974 S. Barzilai A, Kleckner MS: Hemocholecyst following ruptured aneurysm or portal vein. Arch Surg 72:725-727, 1956 6. Leonsins AJ, Siew S: Fusiform aneurysmal dilatation of the portal vein. Postgrad Med J 36:570-574, 1960 7. Hermann RE, Shafer WH: Aneurysm of the portal vein and portal hypertension. Ann Surg 162:1101-1104, 1965 8. Thomas TV: Aneurysm of the portal vein: report of two cases, one resulting in thrombosis and spontaneous rup- ture. Surgery 61:550-555, 1967 AJR:132. April 1979 9. Liebowitz HA, Rousselot LM: Saccular aneurysm of portal vein with agnogenic myeloid metaplasia. NY State J Med 67:1443-1447, 1967 10. Wenz W, Beckenbach H, Daum A: Die kindliche Pfortader bei Erkankugen der Oberbauchorgane. Eur J Pediatr 9: 354-362, 1971 1 1 . Duhmke VE: Aneurysma der vena portae. ROEFO 12:474- 475, 1975 12. Klemperer P: Cavernomatous transformation of the portal vein. Arch Patho! Lab Med 6:353-377, 1928 13. Douglass BE, Baggenstoss AA, Hollingshead WH: The anat- omy of the portal vein and its tributaries. Surg Gyneco! Obstet 91 :562-576, 1950 14. Purcell HK, Connor JJ, Alexander WF, Scully NM: Observa- tions on the extra-hepatic major radicles of the portal systems. Arch Surg 62:670-677, 1951 15. Reynolds TB: Portal hypertension, in Diseases of the Liver, edited by Schiff L, Philadelphia, Lippincott, 1975, p 330 16. Doust B, Pearce J: Gray-scale ultrasonic properties of the normal and inflamed pancreas. Radiology 120:653-657, 1976

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