Presentation
Known case of thalassemia major. CT for work up of developmental delay.
Patient Data
Age: 3 years
Gender: Male
From the case:
Thalassemia - skull manifestation
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Diffuse widening of the diploic space of the calvarial bones (except the occipital bone) with typical hair on end appearance, more prominent in the frontal bones.
Thickening of facial bones is also noted.
No pneumatization of the frontal, maxillary, and sphenoid sinuses. Normal pneumatization of the ethmoid air cells.
No gross abnormality in the brain parenchyma.
Case Discussion
The skull manifestations of thalassemia include:
- widening of the diploic space
- thinning of the inner and outer tables
- prominent secondary trabeculae (hair on end appearance)
- the occipital bone is spared (lack of haemopoietic bone marrow)
Facial bones:
- hypopneumatisation of the frontal, maxillary and sphenoid sinuses (filled with marrow containing bone)
- ethmoid sinuses are spared as they contain no red bone marrow
- dental malocclusion
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