Incidence of Scoliosis in β-Thalassemia and Follow-Up Evaluation

Abstract

Study Design 

One hundred fifteen of 120 patients with β-thalassemia followed in the thalassemia unit were studied for the presence of scoliosis. Forty-nine of these patients were reevaluated 1 year later.

Objectives 

To determine the frequency and the course of scoliosis in β-thalassemia and to compare the findings with those of patients with idiopathic scoliosis.

Summary and Background Data 

There is only one report indicating increased frequency of scoliosis in a limited number of patients with thalassemia. In this study, the authors assessed the frequency of scoliosis in a large sample of patients and followed the evolution of this spinal deformity.

Methods 

Patients with β-thalassemia aged 3-35 years were examined clinically and radiologically for scoliosis. Forty-nine of them were reexamined 1 year later for determination of the evolution of scoliosis.

Results 

Lateral curves of at least 5° Cobb were found in 77 patients (67%), with a male-to-female ratio of 0.9. Scoliosis of at least 10° was found in 21.7% of the male and 20% of the female patients with thalassemia. The ratio was 1.18 for curves of at least 10° and 0.77 for curves of a smaller magnitude. The most common curve pattern was the left lumbar (35.1%), followed by the double-curve pattern (16.9%). Forty-nine randomly selected patients (42.6%) of the 115 included in the study were reexamined 1 year later. Seven male and 7 female patients (total, 28.6%) showed a progression of at least 5°. Six patients (12.2%) experienced spontaneous improvement of less than 6°. The pattern and the evolution of scoliosis observed in patients with β-thalassemia differ from those found in Greek children with idiopathic scoliosis.

Conclusions 

The findings of this study show that the incidence, evolution, and etiology of scoliosis in β-thalassemia differ from those of idiopathic scoliosis, indicating that the spinal deformities in patients with β-thalassemia represents a distinct type of scoliosis. Longer follow-up is needed to investigate the natural history of this type of scoliosis.