SPR Unknown # 79 -- FINAL

Mary Wyers, M.D.

Lurie Children's Hospital

mwyers@luriechildrens.org

Keywords

VOD, venoocclusive disease, liver, spr unknown 79, ultrasound


Publication Date: 20121207

History

4 year old with history of pre-B ALL, on chemotherapy, presenting with abdominal distention and liver dysfunction.

Findings

Ultrasound of the abdomen with doppler: Ascites, right pleural effusion, gallbladder wall thickening, and reversal of flow within the portal vein

Diagnosis

Hepatic venoocclusive disease

Discussion

Hepatic veno-occlusive disease (HVOD) is related to fibrous obliteration of terminal hepatic venules and small sublobular veins and the pathogenesis is thought to be due to endothelial damage, resulting in activation of the coagulation cascade. It is typically seen as an early complication of bone marrow transplantation, after the conditioning chemotherapy and radiotherapy that these patients undergo in order to obliterate the marrow. It is usually seen in the first 2-3 weeks following bone marrow transplant. In our patient, who had not had a bone marrow transplant, the veno-occlusive disease was thought clinically to be secondary to one of the patient's drugs: thioguanine.

Although different sonographic findings have been reported to be at least associated with a diagnosis of HVOD, such as gallbladder wall thickening, ascites, portal venous flow reversal or decrease in portal venous flow velocity, and elevated hepatic artery resistive index, the results have been conflicting. None of these ultrasound features are sensitive and specific to make the diagnosis reliably in a given patient, and clinical factors must be taken into account.

References

  1. Herbetko J, Grigg A, Buckley A, Phillips G. Venoocclusive Liver DIsease After Bone Marrow Transplantation: Findings at Duplex Sonography, AJR: 158, May 1992, pp 1001-1005.
  2. McCarville M, Hoffer F, Howard S, Goloubeva O, Kauffman W. Hepatic Veno-occlusive disease in children undergoing bone-marrow transplantation: usefulness of sonographic findings. Pediatr Radiol (2001) 31: 102-105.

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