DOI:10.2214/AJR.05.0843
AJR 2007; 189:W150-W152
© American Roentgen Ray Society
Congenital Absence of the Portal Vein in a Patient with Urolithiasis
Thomas C. Alewine1,
William R. Carter1 and
Michael J. Frew1
1 All authors: Department of Radiology, National Naval Medical Center, 8901
Wisconsin Ave., Bethesda, MD 20889.
Received May 18, 2005;
accepted after revision July 20, 2005.
Address correspondence to T. C. Alewine
(tcalewine{at}bethesda.med.navy.mil).
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Keywords: abdominal imaging • anatomy • developmental anomalies • liver • liver transplantation
Introduction
Congenital absence of the portal vein is a rare anomaly of the portal
venous-mesenteric vascular system. Sixty-one cases, mostly in girls, have been
documented
[1-4].
Most cases have been discovered with sonography or angiography. In many cases,
hepatic nodular lesions have been associated with this abnormality, as have
cardiac defects and hepatic neoplasms, which bring most cases to medical
attention [4]. In this report
we describe this rare anomaly found incidentally in a middle-aged man with
symptomatic ureterolithiasis.
Case Report
A 46-year-old man was seen in the emergency department for left flank pain.
The patient had no relevant medical or surgical history and no previous
cross-sectional images. Physical examination showed left flank tenderness, and
laboratory studies showed only microscopic hematuria. The complete blood cell
count was within normal limits. A supine abdominal anteroposterior radiograph
showed nondilated loops of small and large bowel partially obscuring the renal
fossae. Numerous old bilateral rib fractures were present. No suspicious
calcifications were identified.
Contrast-enhanced abdominal and pelvic CT was performed for evaluation of
the laboratory findings and symptoms. The right kidney and ureter were normal
in radiographic appearance. The left kidney was mildly asymmetrically enlarged
with small hypodensity consistent with a cyst in the midpole region. No
nephroliths were found. The portal vein was absent
(Fig. 1A). The splenic vein and
superior mesenteric vein joined (Figs.
1B and
1C) and continued inferiorly as
an aberrant vessel within the left retroperitoneum lateral to the aorta.
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Fig. 1B —56-year-old man with congenital absence of portal vein and
left ureterolithiasis. Contrast-enhanced CT scan of abdomen caudad in relation
to A shows distended inferior vena cava (asterisk) and splenic
vein (arrows).
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Fig. 1C —56-year-old man with congenital absence of portal vein and
left ureterolithiasis. Contrast-enhanced CT scan of abdomen caudad in relation
to B shows superior mesenteric vein (asterisk) and aberrant
vein (arrows) crossing midline at level of superior mesenteric vein
toward left.
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A 3-mm rounded intraluminal calcification was present in the proximal left
ureter with mild proximal ureteral dilatation (Figs.
1D and
1E). The aberrant vessel
crossed the left ureter at the level where the ureterolith rested
(Fig. 1E). It continued
inferiorly, crossed the midline below the aortic bifurcation, and joined the
right internal iliac vein with shunting of blood flow into the right deep
venous system. The admixture resulted in differential enhancement of the right
common iliac vein compared with the left common iliac vein
(Fig. 1F). No stones were found
in the bladder. There was no adenopathy. The aorta, superior mesenteric
artery, hepatic arteries, celiac axis, and iliac arteries were normal in
opacification.
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Fig. 1D —56-year-old man with congenital absence of portal vein and
left ureterolithiasis. Contrast-enhanced CT scan of abdomen caudad in relation
to C shows aberrant vessel (thin arrow), mildly dilated left
ureter (thick arrow), mildly dilated inferior vena cava
(asterisk), and superior mesenteric vein (arrowhead).
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Fig. 1E —56-year-old man with congenital absence of portal vein and
left ureterolithiasis. Contrast-enhanced CT scan of abdomen caudad in relation
to D shows aberrant vessel (solid arrow) and ureteral stone
(open arrow).
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Fig. 1F —56-year-old man with congenital absence of portal vein and
left ureterolithiasis. Curved planar reformatted contrast-enhanced CT scan of
abdomen and pelvis shows aberrant vessel (asterisks) coursing
inferiorly from left, taking sharp bend (+) (not depicted well on curved
planar reformatted image), crossing midline, and joining right internal iliac
vein (ri). Differential attenuation exists between right internal iliac vein
(ri) and left internal iliac vein (li). ivc = inferior vena cava.
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Discussion
Portal vein development is first noted in the 4- to 10-week embryo. The
vein forms by involution of the periintestinal vitelline venous loop.
Excessive involution can result in congenital absence of the portal vein
[2]. The interrupted
enterohepatic circulation causes a portosystemic venous shunt without
secondary signs of portal hypertension, such as collateral veins, ascites, and
splenomegaly. The most common drainage sites of the portal vein in decreasing
order are the inferior vena cava, renal vein, and left hepatic vein
[5]. In our patient with an
unusual case of this rare anomaly, the drainage site was more distal—the
right internal iliac vein. Several previously described patients had hepatic
tumors, such as hepatoblastoma and, more often, focal nodular hyperplasia
[2-5].
Portal blood is thought to protect hepatocyte function, structure, and
regeneration [5]. No hepatic
lesions were identified in our patient.
Most patients with congenital anomalies of the portal venous system have
agenesis of the right or left portal vein. True congenital absence of the
portal vein is rare, and a classification has been proposed. Knowledge of the
various portal branch variants and anomalies is important for surgical
planning [6]. According to
Morgan and Superina's [1]
classification of congenital absence of the portal vein, the incidental
finding in this case would be classified type 1b: liver not perfused with
portal blood, total shunt, superior mesenteric vein and splenic vein joining
to form confluence. Type 1a is absence of intrahepatic portal vein with
superior mesenteric vein and splenic vein not joining to form the portal vein.
Type 2 is patent intrahepatic veins. Patients with type 1 shunts are typically
young and preponderantly female, unlike in our case.
The level of the ureteral stone may or may not be related to the level of
the crossing aberrant shunt vessel. It is unlikely that the crossing vessel
was a predisposing factor in urolith formation
[7]. The urolith probably
(48-76% chance) will pass spontaneously given its location and size
[8].
References
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Introduction
Case Report
