Portal vein aneurysm: report of six cases and review of the literature

Portal vein aneurysm is very rare, and its relation to portal hypertension has been emphasized. We report six cases of portal vein aneurysm (five extrahepatic and one intrahepatic). All patients were asymptomatic and had no signs suggestive of portal hypertension; the lesion was incidentally detected by ultrasound. Color Doppler sonography showed a constant hepatopetal flow along the aneurysmal wall, which immediately led to the diagnosis. We stress the usefulness of color Doppler sonography for studying the hemodynamics of this vascular anomaly and briefly review the literature. Received: 29 December 1995/Accepted: 14 February 1996     

Intraoperative US diagnosis of pylephlebitis (portal vein thrombosis) as a complication of appendicitis: a case report

We report a case of infectious thrombosis of the superior mesenteric vein (pylephlebitis) that was suspected preoperatively with computed tomography and confirmed at intraoperative ultrasonography as confined to the extrahepatic portal vein and superior mesenteric vein. Intraoperative ultrasonography revealed intraluminal echogenic thrombus material in the dilated superior mesenteric and extrahepatic portal veins, slightly dilated open splenic vein, and numerous venous collaterals in the hepatoduodenal ligament. When preoperative imaging studies are inconclusive, intraoperative sonography can confirm the correct diagnosis of pylephlebitis and may give valuable information about the extent of the thrombosis. Received: 19 December 1995/Accepted: 31 January 1996     

Computed tomography of superior mesenteric vein thrombosis following appendectomy

During a 5-year period, superior mesenteric vein (SMV) thrombosis was detected with computed tomography (CT) in six patients shortly after an appendectomy. No sign of SMV was present at appendectomy, and a period of more than 2 weeks free of clinical symptoms had elapsed between the appendectomy and the onset of the SMV thrombosis. In four cases, the appendicitis was complicated. These patients had nonspecific signs and symptoms, although two of them had elevation of blood hepatic enzyme levels. In all cases, postcontrast CT demonstrated enlargement of the SMV, with well-defined enhancement of the vascular wall and an intraluminal clot. In one case, CT showed extension of the thrombus to the portal vein with the presence of low-attenuation areas in the liver, consistent with hepatic infarcts. Two patients had predisposing diseases: idiopathic hypersplenism in one case and chronic hepatic disease in the other. SMV thrombosis is a possible complication of appendicitis, and early appendectomy in appendicitis can prevent this complication. Moreover, as in any abdominal surgery, early appendectomy may be complicated by thrombosis of the SMV, thus creating problems of postoperative diagnosis. The complication is more frequent when the initial operation is performed under difficult conditions (peritonitis), or when the patient presents with a coagulopathy. CT is useful in the diagnosis of SMV thrombosis, thus leading to early management with anticoagulant therapy, with a view to avoiding complications such as intestinal ischemia, portal vein thrombosis, and hepatic infarction. Received: 19 March 1997/Accepted after revision: 15 July 1997     

Small bowel varices: report of two cases

Two cases of small bowel (S-B) varices associated with portal hypertension, one with liver cirrhosis and one with portal thrombus, are reported. Detection of S-B varices has been a challenging task and several invasive diagnostic techniques have been used for this purpose. However, in our cases, color Doppler sonography revealed the S-B varices supplied by the superior mesenteric vein and draining to the iliac (one case) or ovarian vein (other case), which helped to establish an early appropriate diagnostic and treatment plan. Received: 19 March 1997/Accepted: 14 May 1997     

Portal tumor thrombus due to gastrointestinal cancer

Methods: We studied the clinical data of seven patients with portal tumor thrombus (PTT) due to gastrointestinal (GI) cancer to determine the radiologic patterns and clinical implications of this rare complication. Results: (a) PTT was located along the entire splenic vein in three cases, at the splenomesenteric confluence in one case, and in the superior mesenteric vein in one case. Intrahepatic PTT occurred in two of four cases with liver metastasis. (b) One cirrhotic case was complicated by the occurrence of colon cancer associated with PTT in the splenic vein; the esophageal varices became rapidly enlarged and poorly controlled, and the patient died due to repeated variceal rupture. (c) In all patients, abdominal sonography (US) detected PTT and color Doppler sonography confirmed the US findings. Conclusions: The splenic vein should be meticulously observed by color Doppler sonography to check for PTT in patients with GI cancer to improve patient care. Received: 29 December 1998/Accepted: 24 February 1999     

Portal vein opacification during ERCP in patients with pancreatitis

We report two cases of portal vein visualization during ERCP in patients with pancreatitis, one from inadvertent cannulation of the superior mesenteric vein, and in the other, through a preexisting fistula. Prompt recognition of this potentially significant event will obviate confusion and unnecessary prolongation of the procedure. Received: 9/9/96/Accepted: 10/16/96     

Portal hypertension in children and young adults: sonographic and color Doppler findings

Portal hypertension is a relatively uncommon pathologic condition in children and young adults in contrast with older adults. The aim of this study is to evaluate the utility of sonography and color Doppler sonography in the diagnosis of portal hypertension in children and young patients and to evaluate the sonographic pattern of each disease. We reviewed 25 such patients who were younger than 30 years old and obtained the following sonographic findings: (1) liver cirrhosis: (a) multiple intrahepatic venovenous shunts in patients with primary Budd-Chiari syndrome and (b) intrahepatic vascular narrowing and nodular coarse parenchymal texture, with multiple very-high-echo spots along the portal vein in patients with Wilson disease; (2) congenital hepatic fibrosis: marked and developed collaterals, wide periportal echogenic band, and a heterogeneous parenchymal texture comprised of multiple high echoes but without portal thrombus; and (3) extrahepatic portal thrombosis: invisible portal lumen except as an echogenic band. Sonography and color Doppler sonography are very useful in diagnosing these portal hypertensive diseases. However, there are no specific sonographic findings, and the role of sonography is limited to follow-up observation of associated secondary hepatobiliary changes in patients with congenital biliary atresia. Received: 1 May 1995/Accepted: 24 June 1995     

Portal vein absence and nodular regenerative hyperplasiaof the liver with giant inferior mesenteric vein

We present a patient with nodular regenerative hyperplasia of the liver (NRH) and portal vein absence studied with CT, MR imaging, and MR angiography. The most striking feature was exuberant hemorrhoids due to a giant hepatofugal inferior mesenteric vein. A relationship between unbalanced portal blood flow and nodular regenerative transformation of the liver is suggested in this patient. Received: 28 May 1996/Accepted: 10 July 1996     

Radiologically identifiable intratumoral portal vein in intrahepatic cholangiomas: a diagnostic pitfall

Background: Although intratumoral patent portal vein (ITPV) is one of the characteristic features of benign hepatic lesions, ITPVs can be demonstrated in malignant tumors. We present the spectrum of MR and CT findings of ITPV identified in intrahepatic cholangiomas with pathological correlations. Methods: The ultrasound, CT and/or MRI findings of pathologically-confirmed intrahepatic cholangiomas were reviewed and correlated with surgical specimen or autopsy findings. Results: Intratumoral patent vessels were radiographically-demonstrated in 5 patients with intrahepatic cholangiomas. All intratumoral vessels were secondary or tertiary order portal vein branches. Some wall thickening was identified on pathological examinations. Conclusion: The radiological demonstration of intratumoral portal vein is not a specific sign of benignity. In the case of a hepatic tumor with a patent portal tract, cholangioma should be considered, as well as benign tumors or lymphoma. Received: 28 February 1995/Accepted: 29 March 1995     

A case of extrahepatic portal vein aneurysm accompanying lupoid hepatitis

Portal vein aneurysm, especially that of the extrahepatic portal vein, is a very rare entity. We recently observed a case of aneurysm at the junction of the superior mesenteric vein and the splenic vein which accompanied lupoid hepatitis. Abdominal ultrasonography, color Doppler ultrasonography, abdominal computed tomography (CT), magnetic resonance imaging (MRI), and angiography revealed a portal vein aneurysm that increased in size after a period of about 10 months. © 1995 John Wiley & Sons, Inc.     

Color Doppler sonography for preoperative diagnosis of an aneurysm of the ileal branch of the superior mesenteric artery

Aneurysms of the superior mesenteric artery are uncommon, and aneurysms of its branches occur even less frequently. We report the case of a 60-year-old man with an aneurysm of the ileal branch of the superior mesenteric artery whose initial symptom was abdominal pain. Gray-scale and color Doppler sonography provided noninvasive, accurate preoperative identification of the aneurysm despite its uncommon location, a small branch of the superior mesenteric artery. CT and conventional and magnetic resonance angiography confirmed the sonographic diagnosis. The aneurysm was resected successfully, and the patient's postoperative course was uneventful. Although angiography is required for a definitive diagnosis and for surgical planning, sonography is a useful tool for preoperative evaluation and diagnosis of such aneurysms.     

Preoperative assessment of the portal vein and hepatic artery in children with end-stage liver disease: is selective angiography still necessary?

Background: Selective angiography involves increased risk for children and may cause vascular complications. The aim of this study was to determine whether selective angiography is still necessary for viewing the portal vein and hepatic artery. Methods: Doppler ultrasound, abdominal aortography, and selective angiography were performed and interpreted independently in 62 children (median age = 1 year 3 months), with end-stage liver disease, who were candidates for orthotopic liver transplantation. Results: Selective angiography agreed with the Doppler ultrasound findings of patency, flow direction, and diameter of the extrahepatic portal vein in 84%, 73%, and 79% of the children, respectively. Important additional information was obtained from selective angiography in only five of the 62 children (8%). Selective angiography showed an anomaly of the hepatic artery in 21 of the 62 children (33%). In 18 out of these 21 children (85%), an anomaly of the hepatic artery was already visible on the abdominal aortogram. Conclusion: Selective angiography did not play any significant role in the detection of an anomaly of the hepatic artery and should only be done if the Doppler ultrasound findings of the portal vein are inconclusive or if abdominal aortography cannot provide reliable information about the hepatic artery. For the evaluation of the portal vein and hepatic artery, we recommend Doppler ultrasound and abdominal aortography, a less invasive angiographic procedure. Received: 12 May 1995/Accepted: 26 June 1995     

Regression of splenic vein aneurysm following resolution of splenomegaly

Aneurysms of the portal venous structures have rarely been reported. We present a case of a splenic vein aneurysm that appeared and regressed in parallel with splenic size during the course of a systemic infection in a patient with leukemia. The aneurysm was demonstrated with B-mode and color Doppler sonography and computed tomography. After appropriate therapy, the spleen diminished in size. This decrease was accompanied by regression of the aneurysm. Color Doppler sonography enables the noninvasive detection, diagnosis, and follow-up of splenic vein aneurysms. © 1998 John Wiley & Sons, Inc. J Clin Ultrasound 26 : 98–102, 1998.     

Three-dimensional CT imaging of an isolated dissecting aneurysm of the superior mesenteric artery

A case of an isolated dissecting aneurysm of the superior mesenteric artey is presented with findings of three-dimensional CT imaging. False lumen, intimal flap, entry, and re-entry are clearly identified on threedimensional CT imaging. Received: 27 April 1995/Accepted: 3 June 1995     

Intrahepatic venous collaterals

Background: The aim of this study was to reevaluate the causes and sites of intrahepatic venous collaterals and to determine the role of color Doppler sonography in the diagnosis of this relatively rare vascular abnormality. Methods: Real-time color Doppler sonography was used to study 21 patients with intrahepatic venous collaterals. The cause, distribution, and clinical manifestations of collaterals were determined, and Doppler waveforms obtained from the collaterals were also analyzed. Results: First, the causes of intrahepatic venous collaterals were divided roughly into two groups according to the presence or absence of veno-occlusions. The former group included liver tumors (six cases), primary Budd-Chiari syndrome (five cases), and metastatic adrenal tumors invading the inferior vena cava (two cases). The latter group consisted of diaphragmatic hernia (three cases), Osler-Weber-Rendu disease (two cases), and congestive liver (one case). The cause was not determined in two cases. Second, venous collaterals were distributed throughout the entire liver in primary Budd-Chiari syndrome but localized in the other cases. Third, Doppler waveforms of the collaterals were divided into two patterns: flat flow and multiphasic flow. Flat flow pattern was seen in patients with veno-occlusive diseases, and multiphasic flow pattern was seen in patients without veno-occlusive disease. Conclusion: The relationship between intrahepatic venous collaterals and veno-occlusive diseases has been emphasized in the literature, but the results of our series showed that they occurred under a wide variety of conditions, even without veno-occlusive diseases, including diaphragmatic hernia and Osler-Weber-Rendu disease. The analysis of the Doppler waveforms of the collaterals was useful in differentiating those due to veno-occlusive diseases and those not. Received: 13 December 1996/Accepted: 22 January 1997     

Spontaneous rupture and thrombosis of an intrahepatic portal vein aneurysm

Aneurysm of the portal venous system, a localized fusiform or saccular dilatation, is a rare clinical abnormality. Most aneurysms are located in the extrahepatic segment and rarely in intrahepatic branches. Portal vein aneurysms are usually uncomplicated and asymptomatic and detected incidentally during diagnostic work-up. We describe a case of a bleeding intrahepatic portal vein aneurysm studied with ultrasonography, color Doppler, computed tomography, and percutaneous transhepatic portography in a noncirrhotic patient. Although there are 43 published cases of portal vein aneurysm in the English-language literature, neither rupture and spontaneous thrombosis of an intrahepatic portal vein aneurysm nor its nonsurgical conservative treatment has been reported.     

Portal vein thrombosis: CT features

There are many causative diseases to produced portal vein thrombosis (PVT) with the most common being liver cirrhosis with hepatocellular carcinoma. Visualization of abnormalities associated with PVT is crucial to diagnosis and appropriate intervention. Dynamic contrast enhanced CT is the best means of diagnosis of PVT and evaluation of various causative diseases. The findings of PVT of the dynamic CT are filling defect partially or totally occluding the vessel lumen and rim enhancement of the vessel wall. Signs and symptoms of PVT may be subtle or nonspecific and overshadowed by the underlying illness. Radiologists should be aware of the clinical situations that predispose a patient to portal or mesenteric vein thrombosis.     

Postpartum ovarian vein thrombophlebitis: sonographic diagnosis

Background: We describe our experience with sonographic diagnosis of ovarian vein thrombosis, an uncommon but dangerous postpartum complication. Methods: We retrospectively reviewed the medical records of seven patients in our institution who developed postpartum ovarian vein thrombophlebitis within the past 5 years. Results: In all cases the diagnosis was made by ultrasound, which showed tubular hypoechoic masses lateral to the great abdominal vessels. The postpartum ovarian vein thrombophlebitis was on the right side in six cases and on the left side in one. In five cases, it protruded into the inferior vena cava. The first three cases were referred to computed tomography after the sonographic diagnosis. In the last four cases, the diagnosis relied solely on sonography and no further evaluation was necessary. Conclusion: Sonographic examination can be diagnostic for ovarian vein thrombosis if performed very carefully in symptomatic postpartum patients. Received: 23 February 2001/Accepted: 21 March 2001     

Intestinal malrotation as an incidental finding on CT in adults

Background:Intestinal malrotation in adults is usually an incidental finding on computed tomography (CT). We present the CT findings of 18 adult patients with malrotation and discuss the clinical implications. Methods: Abdominal scans of 18 patients (12 women, six men; age range = 15–79 years) with intestinal malrotation were reviewed. Special attention was directed to the location of the superior mesenteric vessels, the location of the small and large bowels, the size of the uncinate process, the situs definition, and additional anomalies. Results: The malrotation was an incidental finding in all but one patient. The malrotation was type Ia in 17 patients and IIc in the one symptomatic patient. The superior mesenteric vessels were vertically oriented in 10, inverted in two, normally positioned in four, and mirror imaged in two cases with situs ambiguus. All patients had aplasia of the pancreatic uncinate process, five had a short pancreas, and two had a preduodenal portal vein. Fourteen patients had a normal situs and four had heterotaxia. Seven patients had polysplenia, six of which with associated inferior vena cava anomalies. Conclusions: Intestinal malrotation can be diagnosed on CT by the anatomic location of a right-sided small bowel, left-sided colon, an abnormal relationship of the superior mesenteric vessels, and aplasia of the uncinate process. Awareness of these abnormalities is necessary to diagnose this anomaly. It should be sought in patients with a situs problem, inferior vena cava anomalies, polysplenia, or preduodenal portal vein. Although usually an incidental finding, it is important to diagnose such a malrotation because it may cause abdominal symptoms. Also, knowledge of associated vascular anomalies is important when abdominal surgery is planned. Received: 1 October 1998/Revision accepted: 27 January 1999     

Sonographic diagnosis and follow-up of idiopathic hepatic artery aneurysm, an unusual cause of obstructive jaundice.

Hepatic artery aneurysms are rare. We report an idiopathic hepatic artery aneurysm causing obstructive jaundice in a case in which the common hepatic artery arose from the superior mesenteric artery. The diagnosis of hepatic artery aneurysm was suggested by gray-scale sonography, which showed that the common bile duct and intrahepatic biliary radicles were dilated with no obvious intraluminal abnormality and showed no evidence of a mass in the head of the pancreas. A papillotomy of the papilla duodeni major was performed to relieve the cholestasis. Repeat sonography 2 weeks later showed dilatation of the common bile duct and a cyst-like lesion at the porta hepatis impressing the anterior part of the common bile duct. Doppler sonography confirmed pulsatile flow within the cyst-like lesion. Helical CT showed a well-circumscribed lesion with a density similar to that of the abdominal aorta, and mesenteric angiography showed a 3-cm, smooth aneurysm arising from the common hepatic artery, which originated from the superior mesenteric artery. The aneurysm was successfully treated with transcatheter embolization.