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The hair-on-end appearance is a classic radiographic sign seen in skull X-rays, characterized by:

Pathophysiology


Etiologies


Cause Explanation
Thalassemia major Most classic and frequent cause due to severe chronic anemia
Sickle cell disease (SCD) Marrow hyperplasia from chronic hemolytic anemia
Hereditary spherocytosis If anemia is severe and prolonged
Congenital dyserythropoietic anemia Rare inherited forms leading to ineffective erythropoiesis
Iron deficiency anemia Very rarely if chronic and severe
Leukemia Occasionally, from leukemic infiltration and marrow expansion

Radiology


https://youtu.be/BZgY9E4USjc

Modality Findings
XR Expanded diploic space, vertical striations projecting outward from calvarial surface
CT Thickened diploic space, coarse vertical trabeculae
MR May show widened diploic space with altered marrow signal

(a) Axial CT of the calvarium in a 17-year-old girl with TDT and a history of delayed onset transfusion therapy shows marked diploic space widening and “hair-on-end” appearance of the frontal and parietal bones. (b) CT topogram of the same patient as in (a) shows sparing of the occipital bone (arrow) as well as sparing of the ethmoid sinuses ( arrowhead). TDT, transfusion dependent thalassaemia. | Gosein, M., Maharaj, P., Balkaransingh, P., Banfield, R., Greene, C., Latchman, S., & Sinanan, A. (2019). Imaging features of thalassaemia. The British journal of radiology, 92(1095), 20180658. https://doi.org/10.1259/bjr.20180658

(a) Axial CT of the calvarium in a 17-year-old girl with TDT and a history of delayed onset transfusion therapy shows marked diploic space widening and “hair-on-end” appearance of the frontal and parietal bones. (b) CT topogram of the same patient as in (a) shows sparing of the occipital bone (arrow) as well as sparing of the ethmoid sinuses ( arrowhead). TDT, transfusion dependent thalassaemia. | Gosein, M., Maharaj, P., Balkaransingh, P., Banfield, R., Greene, C., Latchman, S., & Sinanan, A. (2019). Imaging features of thalassaemia. The British journal of radiology, 92(1095), 20180658. https://doi.org/10.1259/bjr.20180658

Hair‐on‐end appearance: lateral view. | Azam, M., & Bhatti, N. (2006). Hair-on-end appearance. Archives of disease in childhood, 91(9), 735. https://doi.org/10.1136/adc.2006.100032

Hair‐on‐end appearance: lateral view. | Azam, M., & Bhatti, N. (2006). Hair-on-end appearance. Archives of disease in childhood, 91(9), 735. https://doi.org/10.1136/adc.2006.100032

Associated Clinical Features:

Patients with underlying hemoglobinopathies often also have: