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Table of Contents
Year : 2023  |  Volume : 11  |  Issue : 1  |  Page : 60-62

Extrahepatic portal vein occlusion presenting with vascular, biliary, and visceral complications evaluated on multidetector computed tomography

Department of Radio-Diagnosis, Datta Meghe Institute of Medical Sciences, Wardha, Maharashtra, India

Date of Submission22-Apr-2021
Date of Decision18-May-2021
Date of Acceptance07-Jul-2021
Date of Web Publication05-Dec-2022

Correspondence Address:
Dr. Vaishali B Dhawan
Department of Radiodiagnosis, Jawaharlal Nehru Medical College Sawangi (Meghe), Wardha, Maharashtra - 442 001
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ajim.ajim_49_21

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Extrahepatic portal venous obstruction, predominantly a disease of childhood, is a chronic and long-standing disease characterized by thrombosis of the portal vein, leading to multiple collateral formation and other associated complications owing to long-standing nature of the disease. We present the case of a 22-year-old male with symptoms and signs of gastrointestinal bleed and pain in the left upper abdomen with similar episodes in the past.

Keywords: Extrahepatic portal venous obstruction, portal biliopathy, portal cavernoma, portal colopathy

How to cite this article:
Dhawan VB, Gupta R, Varma A. Extrahepatic portal vein occlusion presenting with vascular, biliary, and visceral complications evaluated on multidetector computed tomography. APIK J Int Med 2023;11:60-2

How to cite this URL:
Dhawan VB, Gupta R, Varma A. Extrahepatic portal vein occlusion presenting with vascular, biliary, and visceral complications evaluated on multidetector computed tomography. APIK J Int Med [serial online] 2023 [cited 2023 Feb 6];11:60-2. Available from: https://www.ajim.in/text.asp?2023/11/1/60/362624

  Introduction Top

Extrahepatic portal vein obstruction (EHPVO), primarily a vascular condition, presents secondary to chronic long-standing blockage and cavernous transformation of the portal vein. Intrahepatic portal branches, splenic veins, or superior mesenteric veins may or may not be additionally involved.[1] EHPVO is a disease of childhood and is the predominant cause of pediatric portal hypertension (PHT) in developing condition. The development of portal vein thrombosis is seldom recognized, and patients may remain asymptomatic for a long duration.[1] Patients with EHPVO typically present in the first two decades with symptomatic PHT, most commonly in the form of (well-tolerated) episodes of upper gastrointestinal (GI) bleed.[2] Later, these patients can develop abnormal liver function tests and secondary biliary cirrhosis (2%–4%) and may eventually develop impaired growth and mild neurocognitive dysfunction.[1] In developing countries, EHPVO is the predominant cause of pediatric PHT and GI bleed, whereas in the Western world, it is more common in adults, being the second most common cause of PHT. It is seen in only 11% of pediatric case in western world.[1],[2]

  Case Report Top

A 22-year-old male presented with hematemesis and melena for 2–3 days and pain in the left hypochondrium for 5 days. He had many similar episodes in the past which went uninvestigated. Liver function tests were deranged with raised serum bilirubin, serum alanine aminotransferase, and aspartate aminotransferase levels. Endoscopy showed large esophageal and fundal varices. The blood clotting test was within normal limits. No predisposing factors such as hypercoagulable states, infections, and inflammation were present in this patient, rendering the portal venous occlusion to be of idiopathic etiology.

Imaging findings

The portal vein showed cavernous transformation with evidence of multiple varices in the upper abdomen in the periportal, peripancreatic, and perisplenic region [Figure 1]. Liver shows mild lobulated contour with transient altered enhancement in the right lobe near the porta. Mild dilatation of the right and left intrahepatic biliary radicles was seen with mild dilatation of the CBD in its whole length. Gallbladder (GB) and ducts show no evidence of calculi. Minimal peri-GB varices were present [Figure 2]a and [Figure 2]b. The spleen was enlarged approximately 24 cm and showed upper pole parenchymal infarct in the form of peripheral nonenhancing area. The splenic artery showed postcontrast enhancement with no evidence of luminal thrombosis or aneurysm [Figure 3]. Almost the whole of the large colon showed mild circumferential wall thickening. Small bowel walls appeared normal in thickness [Figure 4].
Figure 1: Portal vein and vascular changes on multidetector computed tomography axial image of portovenous phase showing portal cavernoma formation (white thick arrow) and perigastric, peripancreatic, and splenic varices (black arrows)

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Figure 2: (a and b) Biliary changes on multidetector computed tomography axial image showing mild dilatation of the biliary tree in both the right and left lobes of the liver (black-margined white arrows)

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Figure 3: Splenic changes on multidetector computed tomography axial image (a) and coronal image (b) showing nonenhancing area at the upper pole of the enlarged spleen (white arrows)

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Figure 4: Colon changes on multidetector computed tomography axial image showing circumferential wall thickening of hepatic flexure and proximal transverse colon (white arrows)

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  Discussion Top

EHPVO and noncirrhotic portal fibrosis are the two most common causes of noncirrhotic PHT.[1] Most of the cases are found to be of idiopathic etiology, however, some etiologies such as hypercoagulable states, infections, inflammation, and portal vein anomaly (stenosis, atresia, and agenesis) have been proposed.[1] Noninvasive imaging techniques like multidetector computed tomography (CT) with postprocessing software, and faster image acquisition protocols in magnetic resonance imaging (MRI), along with ultrasonography (USG) play an important role in diagnosis of EHPVO.[1] Imaging can help assess the severity of venous thrombosis, collaterals, and secondary changes in the biliary tree.[1],[2] USG and Doppler are initial investing tools for assessment of portal cavernomas and liver and spleen parenchymal echotextures but can be of limited help for evaluation of the biliary tree. Evaluation with high precision is better displayed by CT/MRI modalities which can depict changes in the splanchnic circulation, allowing exact assessment of the extent of obstruction of the portosplenomesenteric axis.[2] GB walls, peri-GB varices, and dilatation of CBD and intrahepatic biliary radicles due to extrinsic compression by collaterals can also be seen on CT.[1] CT helps in ruling out other causes of portal vein thrombosis and other causes like malignancy or extrinsic compression by lymph nodes causing extra- and intrahepatic biliary dilatation.[1],[2] Owing to chronic PV thrombosis and collaterals, the central liver receives better perfusion from portoportal collaterals as compared to the peripheral liver, which can be seen as transient hepatic attenuation differences on CT. With progression of the disease and compromised liver blood flow, parenchymal atrophy and lobulations may set in, mimicking cirrhotic-like imaging features.[1],[2] Usually, moderate-to-severe grade of splenomegaly is seen in 90%–100% of patients. CT arterial phase images are vital to look for the presence, number, and size of splenic artery aneurysms which form secondary to hyperkinetic splenic circulation.[1] Biliary changes which are known as portal biliopathy or portal cavernoma cholangiopathy have also been seen in cases of EHPVO, which are abnormalities of the bile duct and GB.[2] Biliary abnormalities are thought to be secondary to external compression by collateral veins or portal cavernous transformation and peribiliary fibrosis due to inflammatory or ischemic changes.[2],[3] The spectrum of biliary changes includes extrinsic indentations, luminal narrowing with or without proximal dilatation, bile duct thickening, angulation or displacement of the extrahepatic duct, choledocholithiasis, and hepatic lithiasis (owing to cholestasis).[2],[3] In the present days, MR cholangiography is the modality of choice, replacing endoscopic retrograde cholangiography (ERC) which is mainly reserved for therapeutic interventions.[1],[2] Noninvasive imaging, namely CT and MRI with MRCP, is preferred to evaluate and make a diagnosis. The imaging findings include biliary strictures, biliary dilatation, and collateral veins with wavy appearance of the bile duct. Findings may mimic primary sclerosing cholangitis and cholangiocarcinoma.[4] However, the absence of vascular findings and regional lymphadenopathy can be helpful in differentiating the above lesions from EHPVO.[3] Patients with long-standing EHPVO can develop colopathy. Cases of gastropathy and enteropathy have also been reported.[5] The pathogenesis of portal hypertensive colopathy (PHC) remains poorly understood. Hyperkinetic circulatory state and PHT seem to play an important role. The main pathological change in PHC is colonic mucosal capillary ectasia, and the main diagnostic tool is lower GI endoscopy.[6]

  Conclusion Top

EHPVO characterized by portal vein thrombosis and collateral formation frequently presenting in childhood and being a chronic condition leads to multiple organ involvements. Noninvasive techniques such as MDCT, MR portovenography, and MRCP play very important diagnostic tools in diagnosing whereas invasive techniques like ERC can be used as therapeutic measures.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Pargewar SS, Desai SN, Rajesh S, Singh VP, Arora A, Mukund A. Imaging and radiological interventions in extra-hepatic portal vein obstruction. World J Radiol 2016;8:556-70.  Back to cited text no. 1
Arora A, Sarin SK. Multimodality imaging of primary extrahepatic portal vein obstruction (EHPVO): what every radiologist should know. Br J Radiol 2015;88:20150008. doi: 10.1259/bjr.20150008. Epub 2015 May 29. PMID: 26023944.  Back to cited text no. 2
Kalra N, Shankar S, Khandelwal N. Imaging of portal cavernomacholangiopathy. J ClinExpHepatol 2014;4 Suppl 1:S44-52.  Back to cited text no. 3
Srisajjakul S, Prapaisilp P, Bangchokdee S. Imaging features of vascular compression in abdomen: Fantasy, phenomenon, or true syndrome. Indian J Radiol Imaging 2017;27:216-24.  Back to cited text no. 4
[PUBMED]  [Full text]  
Kumar N, Srivastava A, Kumari N, Mittal S, Yachha SK, Nayez Z, et al. Prevalence, nature, and predictors of colonic changes in children with extrahepatic portal vein obstruction. Gastrointest Endosc 2020;91:849-58.  Back to cited text no. 5
Urrunaga NH, Rockey DC. Portal hypertensive gastropathy and colopathy. Clin Liver Dis 2014;18:389-406.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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