Related Experiment Videos
Insights
Sclerosteosis and van Buchem's disease are rare bone disorders causing cranial nerve compression. Radiological features in childhood cases are presented, highlighting similarities in these craniotubular hyperostoses.
Area of Science:
- Medical Genetics
- Radiology
- Orthopedics
Background:
- Craniotubular dysplasias and hyperostoses are rare genetic bone disorders.
- Cranial involvement can lead to significant neurological complications, including facial and auditory nerve compression.
Abstract:
Craniotubular dysplasias and craniotubular hyperostoses are a group of confusing disorders in which the cranial involvement may cause compression of facial and auditory nerves. Two of the hyperostotic conditions that regularly produce cranial nerve compression are sclerosteosis and the recessive form of endosteal hyperostosis (van Buchem's disease). Both have identical radiological features with cranial involvement presenting in childhood. The Dutch and South African cases have come from relatively close-knit communities, those in the latter country being Afrikaners who had their origins in Holland. The radiological features of three such cases of sclerosteosis are briefly presented.