Not all varices mean cirrhosis! What every radiologist should know about Non-Cirrhotic Portal Hypertension (NCPH)

Ankur Arora, S. Rajesh, S.K. Sarin Institute of Liver & Biliary Sciences (ILBS), New Delhi, India

GE varices INTRODUCTION LEARNING OBJECTIVES Cirrhosis is the most common cause of portal hypertension 1. To discuss and (PHT). illustrate the radiologic manifestations of different However, it is crucial to be aware disorders that constitute NCPH that PHT can also be present in the Non-Cirrhotic Liver absence of liver cirrhosis, a condition 2. Appraise their patho- referred to as NCPH. Splenomegaly physiology & clinico-pathological features

Clinico-radiologically NCPH simulates cirrhotic PHT (variceal bleed, splenomegaly, ascites, etc), but the 3. Discuss various radiological interventional management and treatment option differs. options available for management of NCPH

HVOTO Typically, patients present with SOS features of PHT (bleeding varices, splenomegaly, hypersplenism) Sinusoidal obstruction Hepatic venous outflow but without any evidence of liver tract obstruction (HVOTO) dysfunction syndrome (SOS) is an results from occlusion of one * obliterative venulitis of the or more main hepatic veins terminal hepatic venules. (HV) and/or the IVC. A potentially fatal form of Liver is non-cirrhotic until Attenuated hepatic veins, heterogeneous hepatic injury that occurs liver enhancement & ascites in a post- predominantly after drug or late however longstanding chemotherapy patient disease predisposes to toxin exposure. cirrhosis. It can present in an acute, Radiological interventions subacute or chronic form Mosaic liver parenchymal usually with abdominal pain enhancement in a patient with HV include: HV or IVC & IVC occlusion with signs of PHT recanalization/ venoplasty/ and swelling, with evidence (splenomegaly and varices) stent placement and TIPS of PHT .

EHPVO OPV

Extrahepatic portal vein obstruction Obliterative portal venopathy (OPV) is (EHPVO) is characterized by obstruction characterized by 'obliterative’ changes in of the PV +/- SV or SMV. the intrahepatic PV branches leading to presinsusoidal PHT. Pre-hepatic resistance to the splanchnic blood flow results in backpressure Infections in the East and prothrombotic changes leading to NCPH. states in the West have been widely implicated. It is a disorder of childhood and young adults. OPV as a cause of NCPH is being increasingly diagnosed especially in In West, it is responsible for 11% & in the patients with HIV. FDA has issued a Portal cavernoma in a young Attenuated intrahepatic PV patient with a non-cirrhotic liver and developing world 54% cases of childhood branches (pruned-tree appearance) warning about the association of splenomegaly and GE varices PHT. with massive splenomegaly & didanosine use in HIV and OPV. dilated spleno-portal axis

CHF Sinistral PHT

Congenital hepatic fibrosis CONCLUSION Sinistral, or left-sided PHT, is (CHF) is an autosomal a rare cause of upper GI recessive disease that affects Awareness of bleed/ NCPH. the hepatobiliary and renal NCPH is imperative systems. for the radiologist The primary pathology usually arises in the pancreas Characterized by hepatic to avoid an (pancreatitis or carcinoma) Dysmorphic liver with right lobe atrophy & PHT with extensive GE varices in a patient hypertrophied seg-IV (asterisk) fibrosis, PHT and renal cystic erroneous with chronic calcific pancreatitis & SVT and results in thrombosis of disease. diagnosis of the splenic vein (SVT). cirrhotic PHT and Imaging shows a dysmorphic aid the clinical Splenic bed venous pressure liver with preserved/ elevation causes splenic hypertrophied seg-IV (unlike colleagues in enlargement and formation cirrhosis wherein there is planning apposite of gastric varices which lead early seg-IV atrophy). to hematemesis. Renal cystic disease therapy.