Three cases of achondroplasia with neurological complications.

The role played by congenital malformations of the skull and spine in the production of lesions of the central nervous system has been increasingly appreciated in recent years. In the skull premature synostosis of the sutures of the vault (craniostenosis) can lead to optic atrophy and symptoms of intracranial hypertension. Hydrocephalus and compression of the brain stem and upper cervical cord can result from deformities of the base of the skull, such as platybasia and narrowing of the foramen magnum. In the spine congenital deformities, such as hemivertebrae, absence of cervical segments, scoliosis, and kyphosis, may also result in cord compression in adult life. In this paper attention is drawn to the possible effects on the nervous system of achondroplasia. This condition of foetal chondrodystrophy is well known and is characterized by failure of endochondral ossification. It results in a striking disproportion of the body; a large head, short limbs, and a spine of relatively normal length. The complications of hydrocephalus and compression of the spinal cord and cauda equina are demonstrated in the following case reports.