Selected Arterial and Venous Diseases
Case 7 -
Thrombosis and Calcification of Portal Vein and Intra-hepatic Portal Vein Branches Complicating Central Venous Catheter
Alan G. Rose
The patient was a 3 ½ month old female with Noonan syndrome who was extremely cyanotic from birth and
underwent neonatal repair of the following cardiovascular defects: pulmonary valvar stenosis,
aortico-pulmonary window defect, atrial septal defect, aortic valvar stenosis, patent ductus arteriosus
and coarctation of the aorta. The patient also had a perimembranous ventricular septal defect. The
thoracic duct was ligated due to a chylothorax. Her postoperative recovery was complicated by delayed
chest closure, total body edema and she required significant vasopressor support. She was placed on a
respirator and developed renal failure of uncertain etiology for which peritoneal dialysis was
administered. Prior to death she had increasing need for larger amounts of vasopressors and mechanical
Autopsy revealed the patient to be status post repair of the congenital defects listed above. The
umbilical vein was obliterated and both umbilical artery vestiges were identified on either side of the
obliterated urachus. The liver (310 grams) appeared large relative to the other organs and it extended 6
cm below the right costal margin in the mid-clavicular line. An intra-peritoneal catheter indented the
liver's capsular surface and a small (less than 1 cm diameter) area of hemorrhage was present nearby.
Multiple small, discrete foci of calcification were scattered throughout the liver. The major portal
vein, prior to reaching the hepatic hilum, showed occlusive thrombosis with signs of organization and
early peripheral recanalization. Calcification was evident in the media and adventitia of the thrombosed
The seminar slide shows widespread thrombosis of many intra-hepatic branches of the portal vein. The
situation of these venules within the portal tracts clearly distinguishes them as portal vein branches
and distinguishes them from central, sub-lobular and larger hepatic veins. Some portal venous
tributaries are totally occluded by organized thrombi with minimal recanalization, while others show less
severe luminal occlusion. Calcification, which appears to be dystrophic in nature, is prominent in the
wall of affected portal venules as well as in the organized thrombus to a limited extent. The liver
parenchyma shows fatty change and areas of sclerosis. The key element in this patient was the
trans-umbilical venous cannulation via the ductus venosus into the inferior vena cava and thence into the
right atrium for parenteral nutrition in the initial postoperative period. This led to thrombosis of the
main portal vein with extension of the thrombotic process to involve many of the intra-hepatic branches
of the portal vein in the portal tracts. No calcification was noted in portal venous branches unaffected
by thrombosis. As is commonly noted in children in a variety of pathologic conditions, there was a great
propensity for calcification to occur in relation to the vessels affected by organized thrombi.
Calcification in children occurs commonly in bioprostheses  and is commonly observed in
ischemic myocardial lesions in the young. This propensity for calcification has been attributed to
differences in calcium metabolism in the young, who tend to have an elevated alkaline phosphatase level.
In infants, portal vein thrombosis may result from umbilical cord infection or cannulation of the
umbilical vein. In adults it usually is a complication of liver cirrhosis. Pyelophlebitis (e.g. due to
appendix abscess) may also lead to portal vein thrombosis. Pancreatic tumor, pancreatitis, or diseases
of adjacent lymph nodes may induce extrinsic thrombosis. Intrahepatic obstruction of the portal
circulation may be due to lesions in the portal venules (as in our patient) or within the liver sinusoids
due to reticuloendothelial disease, sarcoidosis, congenital hepatic fibrosis, or bilharziasis. If portal
vein thrombosis occurs rapidly and completely (e.g. due to trauma) it may produce hemorrhagic infarction
of the intestine. In some patients in whom the portal vein thrombosis is not fatal, extensive
recanalization of the vein may occur; which is termed cavernous transformation of the portal
vein.  The causes of portal hypertension are listed in Table 1.
Table 1: Causes of Portal Hypertension
| Extrahepatic obstruction|
| Intrinsic thrombosis of portal vein|
| Neonatal sepsis|
| Increased blood coagulability|
| Extrinsic thrombosis of portal vein|
| Pancreatic tumor|
| Diseases of adjacent lymph nodes|
| Intrahepatic obstruction|
| Lesions in the portal vein branches|
| Reticuloendothelial diseases|
| Congenital hepatic fibrosis|
| Obstruction of hepatic veins|
| Hepatic veno-occlusive disease|
| Webs in suprahepatic inferior vena cava|
|Increased Blood Flow into Portal System|
| Arteriovenous fistula|
A number of papers have reviewed the complications (Table 2) that may arise in neonates from the use
of vascular catheters.
The commonest complication is infection, which is related to birth
weight, male sex and duration of catheter placement.  Other complications include venous
thrombosis (e.g. superior vena cava syndrome), edema, thrombosis, ischemia and catheter
obstruction.  Dislocations and thromboembolic complications may occur.  Cardiac
tamponade (following cardiac rupture due to catheter tip injury), pneumothorax, hydrothorax and
hemothorax have also been reported.  Correct placement of the catheter tip in the superior
vena cava poses little risk of cardiac perforation. [8 ]Transjugular intrahepatic portosystemic
shunt (TIPS) has been used to treat Budd-Chiari syndrome or portal vein thrombosis. 
Table 2: Complications of Transvenous Cannulation in the Young
Acute thrombotic occlusion of an intra-hepatic portal vein radicle does not cause ischemic infarction,
but produces a Zahn infarct that is characterized by sinusoidal distention, hepatocellular atrophy and
Obstruction of Blood Flow Through the Liver May also Occur at the Sinusoidal Level
This is seen in cirrhosis, sickle cell disease, disseminated intravascular coagulopathy (e.g. in
eclampsia) or in diffuse spread of carcinoma.
Miscellaneous Lesions of Veins
Venous Aneurysms / Ectasias
Aneurysms of arteries are common and also occasionally occur in the heart. Owing to the low blood
pressure within veins, aneurysms are seldom encountered. If they occur, they take the form of an
aneurysmal varix (due to a direct communication between an artery and a vein without an intervening false
sac), or a varicose aneurysm (an indirect communication between an artery and a vein via a false sac).
1. Portal vein aneurysm
Venous aneurysms, including portal vein aneurysms are rarely encountered in clinical practice. Portal
vein aneurysms  may be intra-hepatic or extra-hepatic, and their origin may be
congenital  or acquired. Acquired aneurysms are associated with liver disease, trauma or
degenerative changes. Methods of management include observation, resection, thrombectomy, or portal
venous decompression. Extrahepatic portal vein aneurysms can thrombose and lead to portal hypertension,
they may rupture or they may compress the portal vein itself. Portal thromboembolism to the liver from a
thrombosed portal vein aneurysm is a theoretical possibility. The aneurysm may also compress the bile
duct or other organs e.g. the duodenum or inferior vena cava. Rupture of a portal aneurysm may occur,
but is fortunately rare due to the low intra-vascular pressure.
Venous aneurysms are uncommon in clinical practice, but should be included in the differential
diagnosis of a subcutaneous mass.  Venous aneurysms of the popliteal, jugular and saphenous
veins have been described most often. Other sites include intracranial veins, internal jugular
vein, superior vena cava, inferior vena cava, renal vein, forearm veins, femoral
vein and the portal vein. Most of the aneurysms are thought to be congenital in origin and are
asymptomatic. Thus, surgery is usually not indicated. Popliteal aneurysms appear to be an exception to
this rule. Aneurysms of the popliteal vein appear to be more prone to thrombosis and recurrent pulmonary
embolism. Popliteal venous aneurysms should be sought for in patients with pulmonary emboli and no
evidence of lower extremity deep-vein thrombosis. Venography, ultrasonography, angiography or magnetic
resonance imaging may make the diagnosis. Excision of the popliteal venous aneurysm with venous
reconstruction is recommended.
Some confusion exists in the literature between aneurysms and varices of renal veins. 
Varices are usually multiple, smaller, serpiginous and not typically saccular in appearance and
usually have a better blood flow through them than venous aneurysms, which comprise solitary sacs filled
with stagnant blood. Recognition of an aneurysm is important in order to avoid percutaneous biopsy,
which may be complicated by retroperitoneal hemorrhage.
Barzilai and Kleckner  reported a case of persistent gastrointestinal bleeding as a result
of portal vein aneurysmal rupture into the extrahepatic bile duct. The differential diagnosis of the
particular venous aneurysm will depend on the site of the aneurysm. Those situated in veins in the upper
half of the body will enlarge with the Valsalva maneuver. Like a cavernous hemangioma, venous aneurysms
may exhibit a hum on auscultation. It is not known whether the alterations of elastase
activity  that may favor arterial aneurysm development (see case 4 in this seminar) are also
operative in venous aneurysm formation.
2. Aneurysm of inferior vena cava
I have had personal experience with an idiopathic aneurysm of the inferior vena cava which killed a
young girl due to massive, sudden pulmonary thromboembolism by bulky thrombus, which had filled much of
the (probably congenital) aneurysm.  The aneurysm lay below the level of the renal veins and
was lined by endothelial cells resting upon collagen apart from at an area of mural tearing, where
exposure of the underlying tissue had led to intra-aneurysmal massive thrombosis. Local bleeding had
occurred around the aneurysm at the tear site. The aneurysm had the appearance of a 'true' aneurysm with
remnants of the muscle coat of the inferior vena cava being represented in the aneurysm wall.
3. Rupture of the splenic vein in portal hypertension
Abdominal apoplexy is a rare condition characterized by usually idiopathic massive, spontaneous
retroperitoneal or intra-abdominal hemorrhage. A cause may be found in some cases, most being due to
arterial rupture (e.g., dissection, segmental mediolytic arteriopathy, or aneurysmal rupture). Rupture
of the splenic vein is very rare indeed and only about 15 cases have been reported in the literature.
Eight of these have been associated with pregnancy  and the remaining 7 occurred in cirrhotic
patients with portal hypertension.  We have encountered such a case, in a 47-year-old woman
with primary biliary cirrhosis, who had repeated intestinal bleeding. The low venous pressure led to
more protracted fatal retroperitoneal bleeding than that associated with arterial rupture.
Rupture of the splenic vein is probably under-diagnosed. It is an unfamiliar concept and the splenic
vein is often not examined in detail at autopsy. Splenectomy and ablation of the affected portion of the
splenic vein holds the only chance for survival in such patients. The rarity of splenic vein rupture in
portal hypertension suggests that some vascular conditioning factor must be operative in affected
patients. Rupture of the splenic vein has also been reported as a fatal complication of catheterization
via the umbilical vein in which echocardiography was not adequately used to guide the catheter below the
diaphragm.  We have come full circle since we started this case discussion with a
consideration of complications following umbilical vein catheterization.
Venular Thrombosis in Hyperacute Cardiac Rejection
Venular thrombosis appears to be the key event in the pathogenesis of hyperacute and delayed xenograft
rejection.  Understanding of the sequential features of hyperacute rejection has led to a
grading system  for hyperacute rejection that may have clinical application once it becomes
feasible to use xenografts in clinical practice.
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