Chiasmatic syndrome due to extramedullary hematopoiesis in beta thalassemia

ABSTRACT

A 21 year-old man with homozygous beta thalassemia noted gradual deterioration of his vision over a period of five years resulting from a chiasmatic syndrome. The difficulty with visual perception did improve with timely blood transfusion as required once every few weeks in these patients. An enhancing tumor on CT of head turned out to be a mass of extramedullary marrow upon trans-sphenoidal exploration. Postoperative low dose radiation resulted in a modest degree of improvement of his visual acuity over a six year period