A Case of Splenic Embolization Followed by Splenectomy with Preservation of Accessory Spleen in Gaucher's Disease

ABSTRACT

Gaucher's disease is an autosomal recessive genetic disorder of lipid metabolism. A deficiency of beta-glucocerebrosidase causes an accumulation of glucocerebroside in the reticulo-endothelial system and bone marrow. Total or partial splenectomy has been used in case of massive splenomegaly with hypersplenism and/or mechanical pressure symtoms. Partial splenectomy is preferred to prevent susceptibility to overwhelming postsplenectomy sepsis and to delay the massive deposition of glucocerebroside in the liver and bones. We report the case of a 20-year-old woman with Gaucher's disease and who had a splenic embolization 4 years ago. The spleen cross the midline of the abdomen reached to the true pelvis and elevated the left diaphragm. Angiotensin-converting enzyme, acid phophatase and ESR were increased but beta-glucocerebrosidase was normal. Osteosclerotic changes of the distal femur was observed. Hepatomegaly and splenomegaly with mutiple accessory spleens were seen on abdominal CT. On isotope scan for liver and spleen, multiple accessory spleens had isotope uptake, but spleen did not. We noted severe adhesion of spleen to neighboring structure and no viable splenic tissue for preservation. Total splenectomy with preservation of four accessory spleens was performed. We needed multiple transfusion during dissection and bleeding was continuous for 3 days postoperatively. The patient was discharged without problems on the postoperative 15 th day.