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Partial splenic embolization in a child with Gaucher disease, massive splenomegaly and severe thrombocytopenia

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Abstract

A 13-month-old boy with Gaucher disease presented with severe thrombocytopenia, anemia and massive splenomegaly. In addition he had significant respiratory compromise caused by abdominal compartment syndrome, requiring mechanical ventilation. Because of the degree of respiratory compromise and his existing bone marrow suppression, splenic artery embolization was chosen as an alternative to splenectomy. Splenic artery embolization was performed using 355–500-µm polyvinyl alcohol particles, with 70% ablation achieved. Within 24 h of the procedure the platelet count had risen to greater than 70,000/mm3 and to more than 170,000/mm3 on postoperative day 4. At the 8-month follow-up the splenic size had decreased from 18 cm to 8 cm, with a platelet count of 578,000/mm3. Partial splenic embolization provides a minimally invasive alternative to splenectomy in patients with Gaucher disease with massive splenomegaly and bone marrow suppression.

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Authors and Affiliations

  1. Department of Radiology, The Children’s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA, 19104-4399, USA

    Andres H. Pena, Egor Clevac & Anne Marie Cahill

  2. Division of Metabolic Diseases, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Paige Kaplan & Jaya Ganesh

Authors
  1. Andres H. Pena
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  2. Paige Kaplan
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  3. Jaya Ganesh
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  4. Egor Clevac
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  5. Anne Marie Cahill
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Correspondence to Anne Marie Cahill.

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Pena, A.H., Kaplan, P., Ganesh, J. et al. Partial splenic embolization in a child with Gaucher disease, massive splenomegaly and severe thrombocytopenia. Pediatr Radiol 39, 1006–1009 (2009). https://doi.org/10.1007/s00247-009-1303-9

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  • DOI: https://doi.org/10.1007/s00247-009-1303-9

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