ABSTRACT

In their 1948 discussion of the radiologic features of neurofibromatosis, John Holt and Edwin Wright’ (University of Michigan) emphasized the rib changes that could simulate the rib notching seen in patients with coarctation of the aorta. They studied 127 patients, with von Recklinghausen’s neurofibromatosis, seen during a 13-year period. Credit is given to earlier authors who had described pressure defects 2 , and pits or caves in the ribs 3 . However, it was Holt and Wright who used the ‘twisted-ribbon’ analogy to describe the now classic appearance of the rib abnormalities in neurofibromatosis (Figure 1). They recognized that ‘There probably is nothing precisely characteristic about this type of rib erosion, but as it is so infrequently associated with intrathoracic tumors other than neurofibromata, it must be regarded as having considerable diagnostic significance.’ The twisted-ribbon term appears in the figure legends that accompany the article’s illustrations. ‘Extensive erosion of left side of upper thoracic spine and adjoining ribs due to neurofibroma arising within the spinal canal and extending into the thorax. Note the twisted-ribbon appearance of the fourth and fifth ribs.’ Reprinted from Holt and Wright’. The radiologic features of neurofibromatosis. <italic>Radiology,</italic> 1948, 51, 647–63<bold>,</bold> with permission of the RSNA https://s3-euw1-ap-pe-df-pch-content-public-p.s3.eu-west-1.amazonaws.com/9781003076568/34c29fd5-5662-4e8f-b58c-badd7fa5d724/content/fig68_1_B.jpg"/>