A Young Male With Non-cirrhotic Cryptogenic Portal Cavernoma: An Authoritative Case Study

Abstract

The growth of several porto-portal collateral veins encircling an existing stenosed or obstructed entry vein is an uncommon condition known as portal cavernoma. It is traditionally shown as the entry vein thrombosis (portal vein thrombosis - PVT) outcome. A male of 25 years with stomach discomfort for three days that was acute, nonprogressive, and was not accompanied by fever, loose stools, or vomiting. After he had undergone abdominal ultrasonography, portal vein thrombosis was discovered, and based on no involvement of suprahepatic veins according to ultrasonography, Budd-Chiari syndrome was ruled out. It was accompanied by dilated periportal tortuous veins and visible mesenteric and peri-splenic collaterals. Moderate splenomegaly was also present. All these features on ultrasound were suggestive of the "portal cavernoma" formation. The patient is not an alcoholic and does not have any chronic, hereditary, or metabolic liver disease. Thrombophilia and cancer screening through tumor markers were also negative. We, with this, present a rare case of non-cirrhotic idiopathic portal cavernoma. This rare case contributes to advancing medical and scientific knowledge that will encourage further dialogue on the topic.

Keywords: idiopathic portal cavernoma; non-cirrhotic; rare cause; thrombophilia and cancer screening; young male.

Figure 1. An axial section of the CECT abdomen showing multiple dilated collateral vessels (red arrow A) causing compression of the distal column of CBD (red arrow B).

Figure 2. This coronal section of the CECT abdomen shows a portal cavernoma formation (red arrow B) with upstream dilatation of distal CBD (red arrow A).

Figure 3. This coronal section of magnetic resonance cholangiopancreatography (MRCP) shows portal cavernoma considered as flow void in Figure 3 with a red circle.

Figure 4. Dilated collaterals as seen with a red arrow in this axial section of MCRP.

Figure 5. Figure depicts a graphical (animated) representation of our patient clearly describing all features, which he had, thus supporting his diagnosis of idiopathic portal cavernoma.

Image credits: Dr. Devansh Khandol