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

Other Title
Gaucher’s Disease에서 비장색전술후 시행한 비장절제술 1예
Authors
류, 기선; 소, 의영; 김, 명욱; 임, 현이; 김, 현주
Citation
Taehan Oekwa Hakhoe, 55(4):598-603, 1998
Journal Title
Taehan Oekwa Hakhoe; 대한외과학회지
ISSN
1226-0053
Abstract
Gaucher’s disease is an autosomal recessive genetic disorder of lipid metabolism. A

deficiency of β-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 β

-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 darts postoperatively. The patient

was discharged without problems on the postoperative 15 th day.
Keywords
Gaucher’s diseaseAccessory spleenSplenic embolization
Appears in Collections:
Journal Papers > School of Medicine / Graduate School of Medicine > Surgery
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