The form of osteopathia striata familiar to radiologists is illustrated on the left. The disorder consists of longitudinal striations through most of the long bones, and is asymptomatic. The process is centered in the metaphysis.
A much rarer form of the disease, osteopathia striata with cranial stenosis is illustrated on the left. It is felt to be autosomal dominant in inheritance and consists of longitudinal long bone striations with cranial sclerosis. The clinical syndrome consists of multiple congenital anomalies including dysmorphic features with frontal bossing, hypertelorism, cleft palate, macrocephaly, hearing loss and mild mental retardation. There may be an associated cardiac anomaly.
REFERENCES
Gay BB Jr, Elsas LJ, Wyly JB, Pasquali M. Osteopathia striata with cranial sclerosis. Pediatr Radiol. 1994;24(1):56-60.
Greenspan A. Sclerosing bone dysplasias--a target-site approach. Skeletal Radiol. 1991;20(8):561-83.