Scoliosis in β thalassemia

Skeletal abnormalities in patients with homozygous β thalassemia have been noted as early as 1927 by Cooley et al1 and have been the subject of many reviews.2-5 Abnormalities have been observed in the entire skeletal system, especially the skull and the mandible, giving the patients a characteristic appearance. Bone changes cause mainly expansion of the medullary cavity, subperiosteal overgrowth of the bones of the skull, and cortical thinning with porous rarefaction of the long bones. Although most of the bone abnormalities in inadequately transfused thalassemic patients result from the overgrowth of the bone marrow, hypoparathyroidism and vitamin C deficiency occasionally occur and may cause bone deformities.6,7