Sternum, Premature Obliteration Of Sutures Of

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2019-09-22
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Currarino and Silverman (1958) reported cases in which the sternal sutures were hypoplastic or closed prematurely leading to a characteristic deformity of the sternum which was abnormally short with an acute angulation in the normal position of the angle of Louis and a depression in its lower part. Associated manifestations in some cases included micrognathia, cryptorchidism, and congenital heart malformation. Dorst (1966) observed the sternal anomaly in mother and daughter who were otherwise normal. This sternal deformity is characteristic of Noonan syndrome (163950). It was also seen in brothers with multiple osteochondritis dissecans (165800), originally reported by Hanley et al. (1967) and considered by later writers, probably correctly, to have had Aarskog syndrome (305400).