Thalassaemia (skull manifestation)

Case contributed by Dr Hidayatullah Hamidi

Presentation

Known case of thalassaemia major. CT for work up of developmental delay.

Patient Data

Age: 3 years
Gender: Male

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 pneumatisation of the frontal, maxillary, and sphenoid sinuses. Normal pneumatisation of the ethmoid air cells.

No gross abnormality in the brain parenchyma.

Case Discussion

The skull manifestations of thalassaemia include:

  • widening of the diploic space
  • thinning of the inner & outer table
  • prominent secondary trabeculae (hair on end appearance)
  • the occipital bone is spared (lack of haemopoietic bone marrow)

Facial bones:

  • hypopneumatisation of the frontal, maxillary & sphenoid sinuses (filled with marrow containing bone)
  • ethmoid sinuses are spared as they contain no red bone marrow
  • dental malocclusion
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Case information

rID: 56548
Case created: 8th Nov 2017
Last edited: 9th Nov 2017
Inclusion in quiz mode: Included
Institution: French Medical Institute for Mothers and Children

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