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Hepatic Venoocclusive Disease
Ethan A. Smith, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Sinusoidal obstruction syndrome
            • Definitions

              • Therapy-induced occlusion of hepatic sinusoids → clinical triad of jaundice, ascites, & hepatomegaly
              • Onset is typically within weeks of stem cell transplantation (SCT); later onset can occur in children
                • Secondary to myeloablative chemotherapy &/or irradiation preceding SCT
              • Risk may be specific to different diseases & treatment regimens
                • Wilms, rhabdomyosarcoma, & leukemia are most frequent

            IMAGING

            • General Features

              • Radiographic Findings

                • Ultrasonographic Findings

                  • CT Findings

                    • MR Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    Selected References

                                    1. Mahadeo KM et al: Diagnosis, grading, and treatment recommendations for children, adolescents, and young adults with sinusoidal obstructive syndrome: an international expert position statement. Lancet Haematol. 7(1):e61-72, 2020
                                    2. Albers BK et al: Vascular anomalies of the pediatric liver. Radiographics. 39(3):842-56, 2019
                                    3. Kernan NA et al: Final results from a defibrotide treatment-IND study for patients with hepatic veno-occlusive disease/sinusoidal obstruction syndrome. Br J Haematol. 181(6):816-27, 2018
                                    4. Keating GM: Defibrotide: a review of its use in severe hepatic veno-occlusive disease following haematopoietic stem cell transplantation. Clin Drug Investig. 34(12):895-904, 2014
                                    5. Kotecha RS et al: Hepatic sinusoidal obstruction syndrome during chemotherapy for childhood medulloblastoma: report of a case and review of the literature. J Pediatr Hematol Oncol. 36(1):76-80, 2014
                                    6. Richardson PG et al: Hepatic veno-occlusive disease after hematopoietic stem cell transplantation: novel insights to pathogenesis, current status of treatment, and future directions. Biol Blood Marrow Transplant. 19(1 Suppl):S88-90, 2013
                                    7. Mahgerefteh SY et al: Radiologic imaging and intervention for gastrointestinal and hepatic complications of hematopoietic stem cell transplantation. Radiology. 258(3):660-71, 2011
                                    8. Cefalo MG et al: Hepatic veno-occlusive disease: a chemotherapy-related toxicity in children with malignancies. Paediatr Drugs. 12(5):277-84, 2010
                                    9. Lee SH et al: Hepatic veno-occlusive disease in children after hematopoietic stem cell transplantation: incidence, risk factors, and outcome. Bone Marrow Transplant. 45(8):1287-93, 2010
                                    10. 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
                                    11. Ghersin E et al: Color Doppler demonstration of segmental portal flow reversal: an early sign of hepatic veno-occlusive disease in an infant. J Ultrasound Med. 22(10):1103-6, 2003
                                    12. Lassau N et al: Prognostic value of doppler-ultrasonography in hepatic veno-occlusive disease. Transplantation. 74(1):60-6, 2002
                                    13. 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
                                    14. Deeg KH et al: Diagnosis of veno-occlusive disease of the liver by color-coded Doppler sonography. Pediatr Radiol. 23(2):134-6, 1993
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Top Differential Diagnoses

                                          • Pathology

                                            • Clinical Issues

                                              TERMINOLOGY

                                              • Synonyms

                                                • Sinusoidal obstruction syndrome
                                              • Definitions

                                                • Therapy-induced occlusion of hepatic sinusoids → clinical triad of jaundice, ascites, & hepatomegaly
                                                • Onset is typically within weeks of stem cell transplantation (SCT); later onset can occur in children
                                                  • Secondary to myeloablative chemotherapy &/or irradiation preceding SCT
                                                • Risk may be specific to different diseases & treatment regimens
                                                  • Wilms, rhabdomyosarcoma, & leukemia are most frequent

                                              IMAGING

                                              • General Features

                                                • Radiographic Findings

                                                  • Ultrasonographic Findings

                                                    • CT Findings

                                                      • MR Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      Selected References

                                                                      1. Mahadeo KM et al: Diagnosis, grading, and treatment recommendations for children, adolescents, and young adults with sinusoidal obstructive syndrome: an international expert position statement. Lancet Haematol. 7(1):e61-72, 2020
                                                                      2. Albers BK et al: Vascular anomalies of the pediatric liver. Radiographics. 39(3):842-56, 2019
                                                                      3. Kernan NA et al: Final results from a defibrotide treatment-IND study for patients with hepatic veno-occlusive disease/sinusoidal obstruction syndrome. Br J Haematol. 181(6):816-27, 2018
                                                                      4. Keating GM: Defibrotide: a review of its use in severe hepatic veno-occlusive disease following haematopoietic stem cell transplantation. Clin Drug Investig. 34(12):895-904, 2014
                                                                      5. Kotecha RS et al: Hepatic sinusoidal obstruction syndrome during chemotherapy for childhood medulloblastoma: report of a case and review of the literature. J Pediatr Hematol Oncol. 36(1):76-80, 2014
                                                                      6. Richardson PG et al: Hepatic veno-occlusive disease after hematopoietic stem cell transplantation: novel insights to pathogenesis, current status of treatment, and future directions. Biol Blood Marrow Transplant. 19(1 Suppl):S88-90, 2013
                                                                      7. Mahgerefteh SY et al: Radiologic imaging and intervention for gastrointestinal and hepatic complications of hematopoietic stem cell transplantation. Radiology. 258(3):660-71, 2011
                                                                      8. Cefalo MG et al: Hepatic veno-occlusive disease: a chemotherapy-related toxicity in children with malignancies. Paediatr Drugs. 12(5):277-84, 2010
                                                                      9. Lee SH et al: Hepatic veno-occlusive disease in children after hematopoietic stem cell transplantation: incidence, risk factors, and outcome. Bone Marrow Transplant. 45(8):1287-93, 2010
                                                                      10. 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
                                                                      11. Ghersin E et al: Color Doppler demonstration of segmental portal flow reversal: an early sign of hepatic veno-occlusive disease in an infant. J Ultrasound Med. 22(10):1103-6, 2003
                                                                      12. Lassau N et al: Prognostic value of doppler-ultrasonography in hepatic veno-occlusive disease. Transplantation. 74(1):60-6, 2002
                                                                      13. 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
                                                                      14. Deeg KH et al: Diagnosis of veno-occlusive disease of the liver by color-coded Doppler sonography. Pediatr Radiol. 23(2):134-6, 1993