'Hair-on-end' skull changes resembling thalassemia caused by marrow expansion in uncorrected complex cyanotic heart disease.
"Hair-on-end" skull changes resembling thalassemia were rarely described in the 1950s and 1960s in children with cyanotic congenital heart diseases; these changes were described almost entirely in patients with tetralogy of Fallot or D-transposition of the great arteries. As these lesions have become correctable, the osseous changes, never common, seem now only to exist in a small number of patients with uncorrectable complex cyanotic congenital heart disease who survive in a chronic hypoxic state. We present two cases: a case of marked marrow expansion in the skull of a 5-year-old boy with uncorrectable cyanotic heart disease studied by CT, and a second case of an 8-year-old with tetralogy of Fallot and pulmonary atresia studied by plain skull radiographs. The true incidence of these findings is unknown.
Walor
David M
DM
Department of Radiology Children's Hospital of New York, Columbia University Medical Center, 3959 Broadway, New York, NY 10032, USA. Web2@columbia.edu
Berdon
Walter E
WE
Westra
Sjirk J
SJ
D001855:Bone Marrow Diseases / Q000000981:diagnostic imaging*; D002648:Child; D002675:Child, Preschool; D003914:Dextrocardia / Q000150:complications; D004310:Double Outlet Right Ventricle / Q000150:complications; D019066:Facies; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000150:complications*; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000150:complications; D008297:Male; D018633:Pulmonary Atresia / Q000150:complications; D011859:Radiography; D012886:Skull / Q000000981:diagnostic imaging*; D013771:Tetralogy of Fallot / Q000150:complications; D013789:Thalassemia / Q000000981:diagnostic imaging*