Venoocclusive Disease

Published on 19/07/2015 by admin

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Last modified 22/04/2025

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[level-membership-for-radiology-category] Hematopoietic cell transplantation (HCT)

image Graft vs. host disease (GVHD)

IMAGING

• Sensitivity and specificity of imaging findings are low (overlap those of GVHD)
• Ultrasound provides suggestive evidence of VOD; helps exclude other diagnoses (e.g., opportunistic infection)

image Hepatosplenomegaly; ascites
image Periportal and gallbladder wall edema
image Hepatofugal flow on Doppler; ↑ resistive index (> 0.75)
image Abnormal portal vein wave form
image Small caliber hepatic veins

TOP DIFFERENTIAL DIAGNOSES

• Opportunistic infection, hepatic
• Graft vs. host disease
• Budd-Chiari syndrome

CLINICAL ISSUES

• Acute onset of painful hepatomegaly, jaundice, ascites within 3 weeks following hematopoietic or stem cell transplantation
• Occurs most frequently following HCT (affects 50-80% of recipients)
• Similar disease may occur following exposure to various toxic agents

image Chemotherapy
image Liver transplantation
• Treatment

image Antithrombotic and thrombolytic medication
image
(Left) Complete obliteration of the central vein image accompanied by centrizonal sinusoidal congestion is seen in venoocclusive disease (VOD). (Courtesy S. Kakar, MD.)

image
(Right) Endothelial swelling with subendothelial edema and fibrosis image led to partial occlusion of the lumen of a small hepatic vein in venoocclusive disease. (Courtesy S. Kakar, MD.)
image
(Left) Axial T2WI MR in a 43-year-old woman with VOD following chemotherapy with FOLFOX for appendiceal carcinoma shows ascites image and periportal edema image, which are nonspecific findings. Liver biopsy showed sinusoidal congestion and fibrotic venules.

image
(Right) Axial contrast-enhanced T1WI MR in the same patient shows the ascites image and periportal edema image.

TERMINOLOGY

Abbreviations

• Hepatic venoocclusive disease (VOD)

Synonyms

• Hepatic sinusoidal obstruction syndrome (preferred)

Definitions

• Hepatic venous outflow obstruction due to occlusion of terminal hepatic venules and sinusoids

IMAGING

General Features

• Best diagnostic clue

image  

CT Findings

• Same general findings as on US (hepatomegaly, ascites, etc.)

MR Findings

• Ascites and periportal edema, both bright (hyperintense) on T2WI

Ultrasonographic Findings

• Sensitivity and specificity of imaging findings are low
• Overlap those of graft vs. host disease (GVHD) 

image Hepatosplenomegaly; ascites
image Periportal and gallbladder wall edema
image Hepatofugal flow on Doppler; ↑ resistive index (> 0.75)
image Abnormal portal vein wave form
image Small caliber hepatic veins

Imaging Recommendations

• Best imaging tool

image Ultrasound provides suggestive evidence of VOD; helps exclude other diagnoses (e.g., opportunistic infection)

DIFFERENTIAL DIAGNOSIS

Opportunistic Infection, Hepatic

• Hematopoietic (or stem) cell transplantation (HCT) patients are at risk for hepatic infections
• Multifocal small lesions with enhancing rims suggest fungal (e.g., Candida) or mycobacterial microabscesses

Graft-vs.-Host Disease

• Primarily a clinical diagnosis, characterized by acute hepatic injury (↑ bilirubin + transaminases), skin rash, and gastrointestinal disease (diarrhea, hematochezia)
• No specific hepatic imaging characteristics to distinguish from VOD

Budd-Chiari Syndrome

• Thrombosis or obstruction at level of larger hepatic veins or IVC
• Different etiology (hypercoagulable states); pathogenesis; imaging features
• Not related to bone marrow transplantation

PATHOLOGY

Microscopic Features

• Injury to hepatic venous endothelium
• Progresses to deposition of fibrinogen + factor VIII within venule, sinusoidal walls
• Progressive venular obstruction, centrilobular hemorrhagic necrosis
• Progressive deposition of collagen, fibrosis vascular lumina

CLINICAL ISSUES

Presentation

• Most common signs/symptoms

image Acute onset of painful hepatomegaly, jaundice, ascites within 3 weeks following hematopoietic or stem cell transplantation
image Weight gain
• Other signs/symptoms

image Signs and symptoms of liver failure

– Ascites, encephalopathy, etc.

Demographics

• Gender

image Women affected more than men
• Risk factors

image Preexisting liver disease
image Specific types of conditioning therapy prior to transplant
image Source of hematopoietic cells

– Worse with mismatched source of cells or marrow
image Use of specific antibiotics during transplantation

Natural History & Prognosis

• Occurs most frequently following hematopoietic cell transplantation

image Affects 50-80% of marrow transplant, stem cell, or umbilical cord blood recipients
image Responsible for 5-15% of deaths in this population
• Similar disease may occur following exposure to various toxic agents

image Chemotherapy, alkaloid toxins, radiation therapy, liver transplantation
image  
image  
image  
• Clinical and laboratory features of VOD usually begin within 3 weeks of transplantation

image Timing and severity of symptoms may vary
image Mild disease

– May require no specific therapy
– Mild elevation of liver function tests (LFTs)
– May resolve spontaneously
image Moderate disease

– Diuretics + salt restriction to treat fluid retention
– Medication to relieve pain from hepatomegaly
– Probably most common form of VOD
– Moderate aberration of LFTs
image Severe disease

– May require thrombolytic medication, supportive measures
– May progress to fatal outcome within 100 days of transplantation
– Confusion, renal insufficiency, cardiac failure, bleeding

Treatment

• Antithrombotic and thrombolytic medication

DIAGNOSTIC CHECKLIST

Consider

• Imaging can only suggest VOD; diagnosis based on clinical criteria, ± biopsy
• Most cases are diagnosed presumptively on clinical criteria prior to biopsy

image Based on 2 of the following criteria developing within 20 days of transplantation

– Rise in serum total bilirubin > 2 mg/dL
– Hepatomegaly or right upper quadrant pain
– Sudden weight gain due to fluid retention
• Biopsy can be problematic

image Patients may be coagulopathic, with bleeding risk
image Sampling error may result in equivocal results

Image Interpretation Pearls

• Imaging (usually US) can suggest diagnosis of VOD and help exclude other causes of hepatic dysfunction among HCT recipients

SELECTED REFERENCES

1. Gao, H, et al. Definitive diagnosis of hepatic sinusoidal obstruction syndrome induced by pyrrolizidine alkaloids. J Dig Dis. 2012; 13(1):33–39.

2. Jevtic, D, et al. Veno-occlusive disease in pediatric patients after hematopoietic stem cell transplantation: relevance of activated coagulation and fibrinolysis markers and natural anticoagulants. J Pediatr Hematol Oncol. 2011; 33(3):227–234.

3. Lin, G, et al. Hepatic sinusoidal obstruction syndrome associated with consumption of Gynura segetum. J Hepatol. 2011; 54(4):666–673.

4. Mahgerefteh, SY, et al. Radiologic imaging and intervention for gastrointestinal and hepatic complications of hematopoietic stem cell transplantation. Radiology. 2011; 258(3):660–671.

5. Menke, J. Education and imaging. Hepatobiliary and pancreatic: sinusoidal obstruction syndrome during chemotherapy. J Gastroenterol Hepatol. 2011; 26(6):1083.

6. Sanei, MH, et al. Acute cellular rejection resulting in sinusoidal obstruction syndrome and ascites postliver transplantation. Transplantation. 2011; 92(10):1152–1158.

7. Sebagh, M, et al. Significance of isolated hepatic veno-occlusive disease/sinusoidal obstruction syndrome after liver transplantation. Liver Transpl. 2011; 17(7):798–808.

8. Rubbia-Brandt, L. Sinusoidal obstruction syndrome. Clin Liver Dis. 2010; 14(4):651–668.

 

Song, MK, et al. Hepatic artery resistance index at doppler ultrasonography is a useful parameter of hepatic graft-vs-host disease after allogeneic stem cell transplantation. Transplant Proc. 2010; 42(9):3717–3722.

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