Gaucher disease: treatment of hypersplenism with splenic embolization.
Hypersplenism is a frequent complication of Gaucher disease requiring splenectomy. A patient with Gaucher disease and severe hypersplenism was treated with partial splenic embolization to avoid the increased risk of serious infectious complications and deterioration of the disease associated with splenectomy. A first embolization (25% ablation) was performed at 4 years. Because of persisting abdominal discomfort, failure to thrive and signs of hypersplenism a second embolization (40-50% ablation) was performed 18 months later. Subsequently, the patient's health improved remarkably and 4 years later he achieved normal growth, maintains normal haematologic parameters, is free of symptoms and has no skeletal abnormalities. No serious infections have occurred. The size of the liver and the spleen has not changed appreciably. It appears that partial splenic embolization may be preferable to splenectomy in patients with Gaucher disease, especially in those of young age.
Paediatric Cardiovascular Laboratory, Nikea General Hospital, Piraeus, Greece.
B D
BD
Thanopoulos
C A
CA
Frimas
S P
SP
Mantagos
N G
NG
Beratis
D002648:Child; D002675:Child, Preschool; D004621:Embolization, Therapeutic; D005500:Follow-Up Studies; D005776:Gaucher Disease / Q000150:complications*; D006128:Growth; D006801:Humans; D006971:Hypersplenism / Q000000981:diagnostic imaging / Q000209:etiology / Q000628:therapy*; D008297:Male; D011859:Radiography