Diagnostic Ultrasound - Abdomen and Pelvis
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Diagnostic Ultrasound - Abdomen and Pelvis

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Venoocclusive Disease TERMINOLOGY Abbreviations • Venoocclusive disease (VOD) Synonyms • Hepatic sinusoidal obstruction syndrome Definitions • Hepatic venous outflow obstruction due to occlusion of terminal hepatic venules and sinusoids IMAGING General Features • Best diagnostic clue ○ Acute onset of painful hepatomegaly, jaundice, and ascites within 3 weeks following hematopoietic or stem cell transplantation • Location ○ Diffuse process involving entire liver Ultrasonographic Findings • Grayscale ultrasound ○ Hepatomegaly ○ Splenomegaly ○ Gallbladder wall thickening ○ Ascites ○ Narrowing of hepatic veins due to hepatic edema ○ Rouleaux formation in portal veins due to slow flow • Pulsed Doppler ○ Elevated hepatic arterial velocity > 100 cm/s ○ Abnormal hepatic arterial resistive index < 0.55 or > 0.75 (variably seen) ○ Slow portal venous velocity (< 10 cm/s) or hepatofugal flow ○ Monophasic waveforms in hepatic veins • Color Doppler ○ Slow or reversed flow in portal vein – Flow may be so slow that it is undetectable on color Doppler ○ Prominent hepatic arteries ○ Narrowed hepatic veins CT Findings • Hepatomegaly • Ascites • Pleural effusion • Hepatic vein narrowing • Gallbladder edema and periportal edema • Patchy hepatic parenchymal enhancement MR Findings • Same general findings as CT • Hepatomegaly • Ascites • Pleural effusion • Hepatic vein narrowing • Periportal cuffing and gallbladder edema ○ High signal intensity of periportal area and gallbladder wall on T2WI • Patchy hepatic parenchymal enhancement Imaging Recommendations • Best imaging tool ○ Ultrasound is imaging modality of choice • Protocol advice ○ Doppler evaluation of hepatic vessels critical for appropriate diagnosis DIFFERENTIAL DIAGNOSIS Graft-vs.-Host Disease (GVHD) • Primarily a clinical diagnosis ○ Characterized by acute hepatic injury, skin rash, and gastrointestinal disease • Bowel wall thickening more frequently accompanies GVHD than VOD Budd-Chiari Syndrome • Thrombosis or obstruction at level of main hepatic veins or inferior vena cava (IVC) • Not related to bone marrow transplantation Portal Vein Thrombosis • Bland thrombosis of portal vein ○ Variably echogenic clot ○ May be accompanied by cavernous transformation of portal vein • Tumor thrombus of portal vein ○ Often seen in setting of hepatocellular carcinoma ○ Tumor vessels usually visible within tumor thrombus Portal Hypertension • Although some imaging features may overlap with VOD, typical history of chronic liver disease is key to diagnosis of portal hypertension ○ May see slow flow or reversed direction of flow in portal vein ○ Ascites suggests hepatic decompensation ○ Gallbladder wall thickening often seen Opportunistic Infection • Hematopoietic stem cell transplantation patients are at risk for hepatic infections • Multifocal small hypoechoic lesions may suggest fungal or mycobacterial microabscesses PATHOLOGY General Features • Liver biopsies are most striking for pronounced sinus congestion • Injury to hepatic venous endothelium • Progresses to deposition of fibrinogen + factor VIII within venule and sinusoidal walls • Progressive venular obstruction and centrilobular hemorrhagic necrosis • Sclerosis of venular wall and intense collagen deposition in sinusoids and venules • Chronic VOD ○ Thickened collagen cuffs surrounding central veins Staging, Grading, & Classification • Disease severity (Seattle criteria) ○ Mild disease http://radiologyebook.com/ Diagnoses: Liver 193

Venoocclusive Disease Diagnoses: Liver – No adverse effects of liver disease, and – No medications required for diuresis or hepatic pain, and – All symptoms, signs, and laboratory features reversible ○ Moderate disease (most common form of VOD) – Adverse effects of liver disease present, and – Sodium restriction or diuresis required, or – Medication for hepatic pain required, and – All symptoms, signs, and laboratory features reversible ○ Severe disease – Adverse effects of liver disease present, and – Symptoms, signs, or laboratory features not resolved within 100 days of transplantation, or – Death CLINICAL ISSUES Presentation • Most common signs/symptoms ○ Painful hepatomegaly, jaundice, peripheral edema, unexplained weight gain ○ Elevation of liver function tests • Other signs/symptoms ○ Signs and symptoms of liver failure such as ascites, encephalopathy, etc. Demographics • Gender ○ Women affected more than men • Risk factors ○ Preexisting liver disease ○ Specific types of conditioning therapy prior to transplantation ○ Mismatched source of hematopoietic cells or marrow ○ Use of specific antibiotics during transplantation Natural History & Prognosis • Clinical and laboratory features of VOD usually begin within 3 weeks of transplantation • Occurs most frequently following hematopoietic cell transplantation ○ Affects 50-80% of marrow transplant, stem cell, or umbilical cord blood recipients ○ Responsible for 5-15% of deaths in population with VOD ○ Fatality rate as high as 30% in VOD associated with hematopoietic cell transplantation ○ Severe VOD seen in 15% of patients withhematopoietic cell transplantation • Prognosis depends on extent of hepatic injury and dysfunctions • VOD may occasionally be seen in setting of pyrrolizidine alkaloid ingestion in form of teas Treatment • Antithrombotic and thrombolytic medication • Diuretics and sodium restriction • Analgesia for right upper quadrant pain control DIAGNOSTIC CHECKLIST Consider • Clinical history of recent prior hematopoietic or stem cell transplantation and high dose chemotherapy helpful in diagnosis • Imaging can only suggest VOD;diagnosis based on clinical criteria ± biopsy • Liver biopsy is usually diagnostic but is often hazardous due to coexisting coagulopathy • Clinical diagnosis (modified Seattle criteria) ○ At least 2 of the following occurring within 20 days of transplantation – Serum bilirubin > 34 μmol/L (> 2mg/dL) – Hepatomegaly with right upper quadrant pain – > 2% weight gain from baseline due to fluid retention Image Interpretation Pearls • Ultrasound is imaging modality of choice • Doppler evaluation of hepatic vessels critical in appropriate diagnosis SELECTED REFERENCES 1. Kambham N et al: Hematopoietic stem cell transplantation: graft versus host disease and pathology of gastrointestinal tract, liver, and lung. Adv Anat Pathol. 21(5):301-20, 2014 2. Zhou H et al: Hepatic sinusoidal obstruction syndrome caused by herbal medicine: CT and MRI features. Korean J Radiol. 15(2):218-25, 2014 3. Mahgerefteh SY et al: Radiologic imaging and intervention for gastrointestinal and hepatic complications of hematopoietic stem cell transplantation. Radiology. 258(3):660-71, 2011 4. Coppell JA et al: Hepatic veno-occlusive disease following stem cell transplantation: incidence, clinical course, and outcome. Biol Blood Marrow Transplant. 16(2):157-68, 2010 5. Rubbia-Brandt L: Sinusoidal obstruction syndrome. Clin Liver Dis. 14(4):651- 68, 2010 6. Chung YE et al: Electronic clinical challenges and images in GI. Hepatic venoocclusive disease. Gastroenterology. 135(1):e3-4, 2008 7. Erturk SM et al: CT features of hepatic venoocclusive disease and hepatic graft-versus-host disease in patients after hematopoietic stem cell transplantation. AJR Am J Roentgenol. 186(6):1497-501, 2006 8. Lassau N et al: Prognostic value of doppler-ultrasonography in hepatic venoocclusive disease. Transplantation. 74(1):60-6, 2002 9. McCarville MB et al: Hepatic veno-occlusive disease in children undergoing bone-marrow transplantation: usefulness of sonographic findings. Pediatr Radiol. 31(2):102-5, 2001 10. van den Bosch MA et al: MR imaging findings in two patients with hepatic veno-occlusive disease following bone marrow transplantation. Eur Radiol. 10(8):1290-3, 2000 11. Lassau N et al: Hepatic veno-occlusive disease after myeloablative treatment and bone marrow transplantation: value of gray-scale and Doppler US in 100 patients. Radiology. 204(2):545-52, 1997 12. McDonald GB et al: Veno-occlusive disease of the liver and multiorgan failure after bone marrow transplantation: a cohort study of 355 patients. Ann Intern Med. 118(4):255-67, 1993 194 http://radiologyebook.com/

Venoocclusive Disease<br />

TERMINOLOGY<br />

Abbreviations<br />

• Venoocclusive disease (VOD)<br />

Synonyms<br />

• Hepatic sinusoidal obstruction syndrome<br />

Definitions<br />

• Hepatic venous outflow obstruction due to occlusion of<br />

terminal hepatic venules <strong>and</strong> sinusoids<br />

IMAGING<br />

General Features<br />

• Best diagnostic clue<br />

○ Acute onset of painful hepatomegaly, jaundice, <strong>and</strong><br />

ascites within 3 weeks following hematopoietic or stem<br />

cell transplantation<br />

• Location<br />

○ Diffuse process involving entire liver<br />

Ultrasonographic Findings<br />

• Grayscale ultrasound<br />

○ Hepatomegaly<br />

○ Splenomegaly<br />

○ Gallbladder wall thickening<br />

○ Ascites<br />

○ Narrowing of hepatic veins due to hepatic edema<br />

○ Rouleaux formation in portal veins due to slow flow<br />

• Pulsed Doppler<br />

○ Elevated hepatic arterial velocity > 100 cm/s<br />

○ Abnormal hepatic arterial resistive index < 0.55 or > 0.75<br />

(variably seen)<br />

○ Slow portal venous velocity (< 10 cm/s) or hepatofugal<br />

flow<br />

○ Monophasic waveforms in hepatic veins<br />

• Color Doppler<br />

○ Slow or reversed flow in portal vein<br />

– Flow may be so slow that it is undetectable on color<br />

Doppler<br />

○ Prominent hepatic arteries<br />

○ Narrowed hepatic veins<br />

CT Findings<br />

• Hepatomegaly<br />

• Ascites<br />

• Pleural effusion<br />

• Hepatic vein narrowing<br />

• Gallbladder edema <strong>and</strong> periportal edema<br />

• Patchy hepatic parenchymal enhancement<br />

MR Findings<br />

• Same general findings as CT<br />

• Hepatomegaly<br />

• Ascites<br />

• Pleural effusion<br />

• Hepatic vein narrowing<br />

• Periportal cuffing <strong>and</strong> gallbladder edema<br />

○ High signal intensity of periportal area <strong>and</strong> gallbladder<br />

wall on T2WI<br />

• Patchy hepatic parenchymal enhancement<br />

Imaging Recommendations<br />

• Best imaging tool<br />

○ <strong>Ultrasound</strong> is imaging modality of choice<br />

• Protocol advice<br />

○ Doppler evaluation of hepatic vessels critical for<br />

appropriate diagnosis<br />

DIFFERENTIAL DIAGNOSIS<br />

Graft-vs.-Host Disease (GVHD)<br />

• Primarily a clinical diagnosis<br />

○ Characterized by acute hepatic injury, skin rash, <strong>and</strong><br />

gastrointestinal disease<br />

• Bowel wall thickening more frequently accompanies GVHD<br />

than VOD<br />

Budd-Chiari Syndrome<br />

• Thrombosis or obstruction at level of main hepatic veins or<br />

inferior vena cava (IVC)<br />

• Not related to bone marrow transplantation<br />

Portal Vein Thrombosis<br />

• Bl<strong>and</strong> thrombosis of portal vein<br />

○ Variably echogenic clot<br />

○ May be accompanied by cavernous transformation of<br />

portal vein<br />

• Tumor thrombus of portal vein<br />

○ Often seen in setting of hepatocellular carcinoma<br />

○ Tumor vessels usually visible within tumor thrombus<br />

Portal Hypertension<br />

• Although some imaging features may overlap with VOD,<br />

typical history of chronic liver disease is key to diagnosis of<br />

portal hypertension<br />

○ May see slow flow or reversed direction of flow in portal<br />

vein<br />

○ Ascites suggests hepatic decompensation<br />

○ Gallbladder wall thickening often seen<br />

Opportunistic Infection<br />

• Hematopoietic stem cell transplantation patients are at risk<br />

for hepatic infections<br />

• Multifocal small hypoechoic lesions may suggest fungal or<br />

mycobacterial microabscesses<br />

PATHOLOGY<br />

General Features<br />

• Liver biopsies are most striking for pronounced sinus<br />

congestion<br />

• Injury to hepatic venous endothelium<br />

• Progresses to deposition of fibrinogen + factor VIII within<br />

venule <strong>and</strong> sinusoidal walls<br />

• Progressive venular obstruction <strong>and</strong> centrilobular<br />

hemorrhagic necrosis<br />

• Sclerosis of venular wall <strong>and</strong> intense collagen deposition in<br />

sinusoids <strong>and</strong> venules<br />

• Chronic VOD<br />

○ Thickened collagen cuffs surrounding central veins<br />

Staging, Grading, & Classification<br />

• Disease severity (Seattle criteria)<br />

○ Mild disease<br />

http://radiologyebook.com/<br />

Diagnoses: Liver<br />

193

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