Partial splenectomy for massive splenomegaly secondary to Gaucher's disease.
A 16 year old girl with Type 1 Gaucher's disease presented with massive splenomegaly, hypersplenism and abdominal discomfort. Traditionally hypersplenism has been treated with splenectomy, but this results in a high incidence of overwhelming sepsis and accelerated sphingolipid deposition in both liver and bone. A 90% partial splenectomy was therefore performed leaving a fully vascularized inferior segment of the spleen and resecting 5.8 kg of splenic tissue. The patient made an uneventful recovery with a marked improvement in her haematological parameters and general condition.
Thomas
W E
WE
Department of Surgery, Royal Hallamshire Hospital, Sheffield, UK.
Winfield
D A
DA
D000293:Adolescent; D005260:Female; D005776:Gaucher Disease / Q000150:complications*; D006801:Humans; D006971:Hypersplenism / Q000209:etiology; D012039:Regional Blood Flow; D013154:Spleen / Q000098:blood supply; D013156:Splenectomy / Q000379:methods*; D013163:Splenomegaly / Q000209:etiology / Q000601:surgery*