ABSTRACTS 21ST ANNUAL CONFERENCE—2013

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RARE VASCULAR NEOPLASM AS A REASONFOR HYPERTENSION: CASE REPORT

Zemfira Babayeva Matthias Lang

Hospital Erlenbach, Germany

A 47-year-old caucasian female was referred to our ED withspontaneously Epistaxis (Nosebleed) in August 2012. Theinitial BP was 200 mm Hg. After using Nitrospray thiswas 158mmHg. She has no significant medical problemsother than being allergic to penicillin and ambroxol.Physical exam (Vital signs, neuro, cardiovascular, Respira-tory, abdominal exam) were in norm. Carotid Doppler,Chest X-ray, ECG, Echocardiogram were without any path-ological changes. Lab studies, which included serum ami-notranspherases, alkalaine phosphatase, bilirubin (totaland direct), BUN and creatinine, were all in the normalrange. Only INR was 0.89, and AFP was 6.8klU/l (Ref.<5.8), otherwise, patient history and physical examinationdid not contribute to a conclusive working diagnosis.Ultrasonography reported about multiple hypoechoicmasses with a hypoechoic halo or rim and with diffuselyheterogeneous regions. The lesion seemed to be hepaticin origin. Intra- and extrahepatic billiary ducts and theportal vein were normal in diameter. The gallbladdershowed normal wall thickness without stone, sludge orspace-occupying lesions. Kidneys, ureters, and the bladderwere normal in ultrasonography. The patient underwentcomputed tomography (CT) scanning of the abdomen –

this reported about disseminated metastatic disease with-out primary tumour. The magnetic resonance imaging(MRI Angio) shows us a lot of cystic lesions in the liverand kidney. So we were discussing about a metastatic dis-ease or even a porphyria. To determine the characterisationof focal liver lesions we decided us for application of con-trast-enhanced ultrasound. CEUS correctly identified ma-lignant liver lesions unknown origin. So transabdominalpunkture was done. Histologically, the tumors were com-prised of spindel and epithelioid cells with lightly fatty livercells. The stroma was fibrous, with myxohyaline areas. Im-munohistochemically, they were positive for at least oneendothelial marker (CD34, and CD31). Proliferation withKi67 was approximately 1%.Diagnosis: Epithelioid hemangioendothelioma of theliver. Malignant epithelioid hemangioendothelioma isa rare hepatic tumor of vascular origin, which occurs atsites such as soft tissues, liver, or lung, and has a highly un-predictable malignant potential. It is an intermediate en-tity between well-differentiated hemangioma andangiosarcoma and most commonly found in young tomiddle aged women. I am presenting a case with this raredisease in which the tumor was detected fortuitously andthe definitive diagnosis was based on histological evidence.Neoplastic cells expressed the CD31 or CD34. Treatment

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was surgical resection - like liver transplant. Conservativetreatment for this disease is not recommended. Our caseshighlight the importance of a ultrasonography for detect-ing liver lesions and value of a histological diagnosis toavoid it being mistaken for another entity. This Patientwas sent for liver transplantation.

Corresponding author: Zemfira Babayeva.E-mail: babayeva_zemfira@yahoo.de

HVOTO: TWENTY FIVE YEARS' EXPERIENCEAT A TERTIARY CARE CENTER

Shalimar, Hanish Sharma, Saurabh Kedia, Ajay Kumar,Shivanand Gamanagatti, Gurpreet Gulati,Siddhartha Dutta Gupta, Renu Saxena,Subrat Kumar Panda, Subrat Kumar Acharya

All India Institute of Medical Science, New Delhi, India

Background and Aims: Hepatic venous outflow tract ob-struction (HVOTO) is an uncommon disorder character-ised by obstruction of the outflow tract of hepatic veinsat any level from small hepatic veins to the junction of In-ferior vena cava (IVC) and right atrium. The literature re-garding this disease is sparse and comprises of case seriesfew in number and limited by small number of patients.We describe a cohort of patients seen over a period oftwenty-five years in a tertiary care center.Methods: All consecutive patients diagnosed as HVOTOwere followed up. Etiological work up was done, includingthat for hypercoagulable states. Therapeutic interventionsincluded angioplasty, stenting and TIPS. The patients werefollowed up for symptom relief and development of imme-diate and late complications (including HCC).Results: Three hundred and forteen cases of HVOTO wereidentified. A total of 305(97%) were symptomatic. Meanage was 32.5 � 12.5 years. The presentation was chronicin most patients -265(87%), and fulminant in none. A spe-cific etiology was found in 63 out of the 183 patients. Pro-tein C deficiency was the most common cause. IsolatedHepatic vein involvement was the most common presenta-tion in 50% cases, followed by combined HV and IVC andisolated IVC involvement in 37% and 13% respectively.Sixty five patients were lost to follow up. Interventionswere carried out in 185 patients -20 patients underwentTransjugular intrahepatic portosystemic shunt (TIPS)and the remaining underwent angioplasty/ stenting inIVC/ hepatic veins. Symptomatic improvement occurredin 83% of patients post stenting. Restenosis was seen in27%, 13% and 10% cases after angioplasty, stenting andTIPS respectively.Conclusions: Most HVOTO patients are young, isolatedhepatic vein involvement is seen most commonly. ProteinC deficiency is the most common etiology. Therapeutic

© 2013, INASL

JOURNAL OF CLINICAL AND EXPERIMENTAL HEPATOLOGY

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interventions result in symptomatic improvement in ma-jority of patients.

Corresponding author. S.K. Acharya.E-mail: subratacharya@gmail.com

INFANTILE BUDD-CHIARI AND RESPONSETO BALLOON DILATATION

Amit Dey, Ira Shah, Sushmita Bhatnagar,Gireesh Walawadkar

BJ Wadia Hospital for Children, Mumbai, India

Abstract: Budd-Chiari Syndrome (BCS) is a rare hepaticdisease caused by occlusion of the hepatic venous outflow.Percutaneous hepatic vein angioplasty (PHVA) may alsoachieve symptomatic relief in patients with BCS causedby short length hepatic vein stenosis or occlusion. How-ever, effectiveness of PHVA in infants with BCS is rarely re-ported. We report two children with infantile Budd-Chiariwho responded to balloon dilatation.Case 1: A 1 year old girl presented in February 2012 withsudden onset ascites. She was born at 7 months of gesta-tion with a birth weight of 1.2 kg and was in NICU for21 days. CT angiography showed non visualization of he-patic veins with diffuse long segment narrowing of inferiorvena cava (IVC) in its distal intrahepatic course for 22 mm.She underwent an angiography which showed 90% and95% stenosis of ostium and proximal segment of middlehepatic vein (MHV). Venoplasty of MHV was done as rightand left hepatic veins could not be cannulated. She is onregular follow-up and in July 2012, her weight was 6.4kg, there is no ascites and she is continued on anticoagula-tion consisting of heparin.Case 2: An 8 months old boy presented with ascitis in May2011. Ultrasound abdomen with colour doppler showedthrombus in middle hepatic vein with patent right andleft hepatic veins with elevated velocity in terminal portionof right hepatic vein (RHV) suggestive of narrowing andterminal narrowing of IVC with moderate ascites. Throm-bophilia workup showed protein C activity of 29% (normal= 70-150%). He underwent right & left hepatic venoplastyas MHV could not be cannulated and there was 70% steno-sis of RHV ostium and 90% stenosis of LHV ostium. He wasstarted on heparin and then shifted to warfarin but INR al-ways remained below 1.5 inspite of increasing doses. InSeptember 2011, he again had abdominal distension andDoppler showed narrowing in proximal portion of RHVthough there was no ascitis. In November 2011, in viewof persistent hepatomegaly, angiography was done thatshowed 90% restenosis of RHV and 95% restenosis ofLHV with multiple intrahepatic collaterals. MHV couldnot be cannulated. Again left and right hepatic venoplastywas done and he was put on lowmolecular weight heparin.

Journal of Clinical and Experimental Hepatology | March 2013 | Vol. 3 | No

Corresponding author. Amit Dey.E-mail: amit4kem@gmail.com

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