Bone Changes in Neurofibromatosis

Double meningocele.

“…Von Recklinghausen's disease is frequently found with intrathoracic meningoceles, but its rare coexistence with lumbosacral meningoceles and meningomyeloceles is regarded as no more than coincidental (Levene, 1959). Summary A child with both a thoracic meningocele and a lumbar meningomyelocele is described.…”

Intrathoracic meningocele and neurofibromatosis

“…Even when the vertebra becomes wedge-shaped the upper and lower surfaces remain intact and the intervertebral disks are not affected. Quite extensive changes in the spine of a dysplastic nature, such as fusion, cuneate vertebra, hemivertebra, and bifid spines and bodies, may be seen at other levels (Levene, 1959;Hunt and Pugh, 1961;Heard and Payne, 1962). When the chest shadow is large it may be hard to appreciate the vertebral changes, and then tomography becomes important.…”

“…Pathogenesis.-Neurofibromatosis is characterized by lesions of skin and nerve but, as had been known before von Recklinghausen's description in 1882, bony abnormalities are part of the disorder (Thannhauser, 1944;Levene, 1959;Heard and Payne, 1962). There has been controversy as to whether these bony changes were due to neurofibromatous infiltration or due to some developmental error.…”

Neurofibromatosis with Pathological Fractures in the Newborn

“…All endocrine organs appeared normal (though with minor abnormalities of innervation) and no lesion could be detected in the hypothalamus. Discussion Skeletal changes in neurofibromatosis have been recognized for a considerable time and have been well documented (Brooks and Lehman, 1924;Holt and Wright, 1948;Levene, 1959). They have been reported to occur in 7 % of cases, but this estimate may be on the low side as many patients with the disease have not been subjected to radiological examination.…”

“…Two cases of Paget's disease have also been described in association with neurofibromatosis, but Levene (1959), in recording one such case, assumes that there is no definite connexion between the two conditions.…”