J Pediatr Intensive Care 2014; 03(03): 183-193
DOI: 10.3233/PIC-14102
Review Article
Georg Thieme Verlag KG Stuttgart – New York

Hepatic veno-occlusive disease in children after hematopoietic stem cell transplantation

Kris M. Mahadeo
a   Pediatric Blood and Marrow Transplantation, Children's Hospital, Montefiore Albert Einstein College of Medicine, New York, NY, USA
Rajinder P.S. Bajwa
b   Department of Hematology/Oncology/BMT, Nationwide Children's Hospital, Columbus, OH, USA
› Author Affiliations

Subject Editor:
Further Information

Publication History

02 August 2014

02 September 2014

Publication Date:
28 July 2015 (online)


Hepatic veno-occlusive disease (VOD), or sinusoidal obstruction syndrome, is a potentially fatal complication, which occurs in 7–27% of children undergoing hematopoietic stem cell transplantation. In this article, we review commonly accepted diagnostic criteria, atypical diagnostic features as well as preventative and treatment measures associated with VOD. Reversal of portal venous flow by Doppler ultrasound is often a late finding; many patients with anicteric VOD may never develop hyperbilirubinemia, and yet perish from severe VOD. Transjugular liver biopsy is usually not available and/or is often avoided. Prophylactic therapies with ursodeoxycholic acid, heparin and defibrotide are discussed. Supportive care with fluid restriction and diuretic therapy, analgesia, blood and platelet transfusions, paracentesis as well as other pulmonary and renal therapies remain imperative to superior outcomes. Specific therapy with thrombolytics such as defibrotide and recombinant tissue plasminogen activator is discussed and a classification of severity of VOD is proposed.