Osteopathia Striata

Abstract

Voorhoeve (1924) observed linear striations in the long bones and pelvis of a father and his son and daughter, and Fairbank (1925) reported another similar case, employing the term ‘osteopathia striata’ to describe the radiological changes. The condition was initially accepted as a clinically unimportant variant, and this concept still held true when Fairbank (1950) reviewed his material 25 years later. Subsequently, the association between bony striations and cranial sclerosis was noted and it was recognised that deafness or minor spinal problems could be inconsistent components of this syndrome (Hurt 1953; Bloor 1954; Rucker and Alfidi 1964; Jones and Mulcahy 1968; Taybi and Nurock 1969; Walker 1969; de Boer and van Gool 1974; Franklyn and Wilkinson 1978). The autosomal dominant inheritance of the osteopathia striata-cranial sclerosis syndrome was established by Horan and Beighton (1978), following a review of four families with the disorder.