The "lamellated" skull in beta-thalassaemia.
The skull in homozygous beta-thalassaemia may present several abnormalities, such as osteopenia, widening of the diploic space, and a "hair-on-end" appearance. In some cases it presents also a particular stratified appearance caused by a variable number of osseous lamellae, parallel with the inner table. This "lamellated skull" was observed in 16 out of 150 patients affected by the disease (10.6%). Possible mechanisms are discussed. The lamellar osseous changes could be due to repeated periosteal osteoblastic reactions to the sinusoidal neovascularization associated with marrow hyperplasia in poorly transfused patients.
Department of Radiology, St. Anna Hospital, Ferrara, Italy.
C
C
Orzincolo
G
G
Castaldi
P N
PN
Scutellari
F
F
Franceschini
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006720:Homozygote; D006801:Humans; D008297:Male; D011859:Radiography; D012886:Skull / Q000000981:diagnostic imaging*; D013789:Thalassemia / Q000000981:diagnostic imaging* / Q000235:genetics