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CASE REPORT Table of Contents  
Ahead of print publication
Extrahepatic portal vein obstruction with portal cavernoma transformation in a child

 Department of Radiology, St. John's Hospital, Chennai, Tamil Nadu, India

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Date of Submission09-Jun-2021
Date of Decision19-Jul-2021
Date of Acceptance20-Jul-2021
Date of Web Publication20-Aug-2021


Extrahepatic portal vein obstruction (EHPVO) refers to the cavernous transformation of the portal vein (also called “ portal cavernoma”) following acute thrombosis of the portal vein in the absence of recanalization. In adults, EHPVO mainly occurs following thrombosis, while in children it may be related to congenital malformations and/or neonatal umbilical venous catheterization. However, 50% of the cases of EHPVO remain idiopathic. This case report demonstrates the ultrasonography appearances of cavernous transformation of the portal vein in a 3-year-old child.

Keywords: Cavernous transformation of the portal vein, child, portal hypertension, portal vein thrombosis, ultrasonography

How to cite this URL:
Reddy R. Extrahepatic portal vein obstruction with portal cavernoma transformation in a child. Apollo Med [Epub ahead of print] [cited 2021 Nov 30]. Available from: https://www.apollomedicine.org/preprintarticle.asp?id=324227

  Introduction Top

Portal cavernoma cholangiopathy has been reported in 6% of children with extrahepatic portal vein obstruction (EHPVO).[1] Children may be free of clinical symptoms but with radiological abnormalities of the biliary tract and/or abnormal liver blood tests. In children, the most common signs are related to portal hypertension, including gastrointestinal bleeding, splenomegaly, and thrombocytopenia. Children with portal cavernoma cholangiopathy always develop liver fibrosis and even cirrhosis.[2] Thus, surgical treatment of portal hypertension should be considered in all children with portal cavernoma cholangiopathy to prevent the development of secondary biliary cirrhosis.

  Case Report Top

A 3-year-old male was admitted to the emergency unit with complaints of upper abdominal pain for the past 3 days. He had also been experiencing mild heaviness and dragging sensation in the left hypochondrium for the past1 month. The history was negative for jaundice. On palpation, the child was tender in the right hypochondrium. The spleen was palpable 5 cm below the left subcostal margin and firm. Laboratory findings were-hemoglobin -10 g/dl, total leukocyte count-1340/mm3 and platelet count 1.1 lakh/mm3, general blood picture was microcytic hypochromic. Liver function tests showed total serum bilirubin 0.3 mg/dl, serum albumin, and liver enzymes were within the normal limits. Viral markers for hepatitis B and C were negative. Ultrasonography abdomen revealed cavernous transformation of the portal vein [Figure 1]a and [Figure 1]b with moderate splenomegaly. Liver biopsy showed normal morphology. Differential diagnoses considered were congenital hepatic fibrosis and schistosomiasis. The child was managed conservatively with intravenous fluid, octreotide, proton pump inhibitors, and two units of whole blood were transfused. As cavernous transformation of the portal vein directs toward a procoagulant state, the child was referred for a detailed coagulation profile workup which revealed prolonged prothrombin time and decreased levels of protein C and protein S. Upper gastrointestinal endoscopy revealed Grade I esophageal varices. After being stabilized, the child was discharged and subsequently referred for bone marrow biopsy and parental counseling regarding portosystemic anastomosis was offered.
Figure 1: (a) High-resolution transverse ultrasonography image demonstrating numerous venous collaterals at the porta hepatis suggesting cavernous transformation of the portal vein around bile duct confluence, central bile ducts, and common hepatic duct. (b) Color Doppler ultrasonography image demonstrating multiple tortuous periportal veins in the hepatic hilum corresponding to cavernous transformation of the portal vein

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  Discussion Top

Several underlying pathologies leading to portal vein thrombosis (PVT) as a consequence have been described, which include schistosomiasis, malignancies, acute pancreatitis, use of oral contraceptives, pregnancy, hyper-homocysteinemia, and coagulopathies.[3] Coagulopathies can either be inherited or acquired: The former include protein C, protein S, and antithrombin deficiency and the latter comprises myeloproliferative disorders, antiphospholipid syndrome, and paroxysmal nocturnal hemoglobinuria.[4] Protein C and protein S are naturally occurring anticoagulants, and their deficiencies have been described as an infrequent yet probable cause of PVT.[5]

In nearly one-third of the cases of PVT, the cirrhotic liver is the main contributory factor and has even accounted for 6%–64% in autopsy studies.[6] The presence of PVT without cirrhosis is rare and is commonly associated with myeloproliferative neoplasms such as polycythemia rubra vera, essential thrombocythemia, and primary myelofibrosis. Protein C and protein S deficiencies were reported as 0%–10% and 0%–30%, respectively, as risk factors for noncirrhotic PVT.[7]

The clinical picture of PVT varies greatly depending on the acute or chronic presentation. The acute symptoms may include abdominal pain and new-onset ascites. During later stages, the portosystemic shunting develops, leading to esophageal varices, rectal bleeding, and hematemesis along with signs of portal hypertension. Long-standing PVT may lead to the development of alternative dilatation of multiple venous channels around the thrombosed PV, thus bypassing the occlusion. This is known as cavernous transformation of the portal vein or portal cavernoma.

Following thrombosis, the portal vein may or may not recanalize. Recanalization is seen more frequently in patients without cirrhosis or disease of the liver leading to inherently increased resistance to portal flow. In patients whose portal vein do not recanalize, or only partially recanalizes, collateral veins (thought to be paracholedochal veins) dilate, and become serpiginous. This process takes a variable amount of time, from as little as a week to a year. These vessels drain variably into the left and right portal veins or more distally into the liver. Additional communications can also be identified with the pericholecystic veins. Cavernous transformation of the portal vein is most of the time inefficient in guaranteeing adequate portal vein inflow to the liver parenchyma far from the hilum and therefore, is associated with an increased hepatic arterial flow to those peripheral liver segments. These changes lead to central liver hypertrophy and peripheral liver atrophy.

In children, portal cavernoma cholangiopathy may be related to congenital malformations and/or neonatal umbilical venous catheterization. However, 50% of the cases of EHPVO remain idiopathic. Longstanding disease results in secondary biliary cirrhosis. Secondary biliary cirrhosis is an unfortunate consequence of a problem where a primary liver disease never existed in the first place. It ultimately leads to end-stage liver disease. Considering the longevity of a child, quality of life in the growing years and gainful living, it is imperative to actively search for asymptomatic biliary changes with serial imaging.

The diagnosis is based on imaging by ultrasound, contrast-enhanced computed tomography, or contrast-enhanced magnetic resonance imaging showing a lack of visualization of the portal vein, which is replaced by collaterals that are enhanced during the portal phase. Liver biopsy is needed to exclude underlying chronic liver disease (in particular cirrhosis and portosinusoidal vascular disease) in patients with altered liver function tests. Liver stiffness <10 kPa may rule out the presence of underlying cirrhosis.

Children may be free of clinical symptoms but with radiological abnormalities of the biliary tract and/or abnormal liver function tests. Children with portal cavernoma cholangiopathy always develop liver fibrosis and even cirrhosis. Thus, surgical treatment of portal hypertension should be considered in all children with portal cavernoma cholangiopathy to prevent the development of secondary biliary cirrhosis.

Endoscopic ultrasound (EUS) can provide information about the vascular changes in the peribiliary area and choledochal varices. Similar to EUS use in adults, it can illustrate dilated choledochal perforators, intracholedochal, and intramural gallbladder varices in children. Children have biliary diameters that are narrower than in adults, more so due to compressive changes in portal cavernoma cholangiopathy. Hence on EUS, the narrow bile duct surrounded by cavernoma is strenuously identified and appears as the “ last log of wood engulfed in flames.”

Mesocaval shunt decompresses the portal cavernoma. However, issues related to the large spleen remain. Central end to side splenorenal shunt decompresses the portal system. Portal cavernoma cholangiopathy reversal may depend on superior mesenteric vein patency. Meso-Rex bypass has been shown to successfully treat various complications of extrahepatic portal vein obstruction, including variceal bleeding, portal cavernoma cholangiopathy, and cardiopulmonary complications. This technique is associated with a 93% of success rate in children with favorable anatomy, in particular with a patent recess of Rex and mesenteric vein.[8] Meso-Rex bypass is the reference surgical treatment because it restores physiological portal blood flow to the liver. Meso-Rex bypass is contraindicated in the presence of thrombosis of the left portal vein or when obliterative portal venopathy is associated with extrahepatic portal vein obstruction.[9] Surgical portosystemic shunt procedures should be considered in patients with either refractory variceal bleeding following endoscopic therapy or portal cavernoma cholangiopathy.[10] However, persistent postprocedural shunting can lead to predictable and severe complications such as hepatic encephalopathy, hepatic nodules, and portopulmonary hypertension. Thus, surgical portosystemic shunting is contraindicated in patients with portopulmonary hypertension or hepatic encephalopathy. Liver transplantation should be considered in these cases.

  Conclusion Top

In children, EHPVO may be related to congenital malformations and/or neonatal umbilical venous catheterization. In conclusion, this case report deserves a special mention as there are only handful cases of cavernous transformation of the portal vein occurring in children reported in the literature. The complications related to EHPVO such as bleeding from gastroesophageal varices and management options in children such as portosystemic anastomosis have also been outlined.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for 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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Moomjian LN, Winks SG. Portal cavernoma cholangiopathy: Diagnosis, imaging, and intervention. Abdom Radiol (NY) 2017;42:57-68.  Back to cited text no. 1
Puri P. Pathogenesis of portal cavernoma cholangiopathy: Is it compression by collaterals or ischemic injury to bile ducts during portal vein thrombosis? J Clin Exp Hepatol 2014;4:S27-33.  Back to cited text no. 2
Costache RS, Dragomirică AS, Dumitraş EA, Mariana J, Căruntu A, Popescu A, et al. Portal vein thrombosis: A concise review (Review). Exp Ther Med 2021;22:759.  Back to cited text no. 3
Gioia S, Nardelli S, Ridola L, Riggio O. Causes and management of non-cirrhotic portal hypertension. Curr Gastroenterol Rep 2020;22:56.  Back to cited text no. 4
Hung HC, Lee JC, Cheng CH, Wang YC, Wu TH, Lee CF, et al. Protein S for portal vein thrombosis in cirrhotic patients waiting for liver transplantation. J Clin Med 2020;9:20.  Back to cited text no. 5
Nadinskaia MY, Kodzoeva KB, Ulyanova KA, Volkova AS, Rogacheva SI, Dekhanov AS, et al. Risk factors associated with portal vein thrombosis in liver cirrhosis: A case-control study. Ter Arkh 2019;91:73-81.  Back to cited text no. 6
Dhiman RK, Saraswat VA, Valla DC, Chawla Y, Behera A, Varma V, et al. Portal cavernoma cholangiopathy: Consensus statement of a working party of the Indian national association for study of the liver. J Clin Exp Hepatol 2014;4:S2-14.  Back to cited text no. 7
Han D, Tang R, Wang L, Li A, Huang X, Shen S, et al. Case report of a modified Meso-Rex bypass as a treatment technique for late-onset portal vein cavernous transformation with portal hypertension after adult deceased-donor liver transplantation. Medicine (Baltimore) 2017;96:e7208.  Back to cited text no. 8
Elkrief L, Houssel-Debry P, Ackermann O, Franchi-Abella S, Branchereau S, Valla D, et al. Portal cavernoma or chronic non cirrhotic extrahepatic portal vein obstruction. Clin Res Hepatol Gastroenterol 2020;44:491-6.  Back to cited text no. 9
Dokmak S, Aussilhou B, Sauvanet A, Lévy P, Plessier A, Ftériche FS, et al. Safety of supramesocolic surgery in patients with portal cavernoma without portal vein decompression. Large single centre experience. HPB (Oxford) 2016;18:623-9.  Back to cited text no. 10

Correspondence Address:
Ravikanth Reddy,
Department of Radiology, St. John's Hospital, Bengaluru 560 034, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/am.am_52_21


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