Neural arch stenosis and spinal cord injury in thanatophoric dysplasia.
Am J Dis Child
; 145(1): 87-9, 1991 Jan.
Article
en En
| MEDLINE
| ID: mdl-1985437
ABSTRACT
Bony abnormalities caused by thanatophoric dysplasia affect the base of the skull and the vertebrae as well as the ribs and appendicular long bones. We present our findings in a full-term infant with thanatophoric dysplasia in whom the posterior fossa, the rostral vertebral column, and the neuraxis at and adjoining the craniovertebral junction were studied by dissection, roentgenography, and histologic examination. In this infant, malformations of the vertebral laminae, most prominent in the basiocciput and atlas vertebra, led to compression of the rostral cervical spinal cord, causing gliosis and focal necrosis. Stenosis of the foramen magnum and spinal canal may contribute to the ventilatory insufficiency that often causes death in patients with thanatophoric dysplasia. We suggest that the causes of death in patients with thanatophoric dysplasia and other severe forms of osteochondrodysplasia should be sought in neuraxial injury rather than attributed solely to pulmonary hypoplasia.
Buscar en Google
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Traumatismos de la Médula Espinal
/
Estenosis Espinal
/
Displasia Tanatofórica
Tipo de estudio:
Etiology_studies
Límite:
Humans
/
Male
/
Newborn
Idioma:
En
Revista:
Am J Dis Child
Año:
1991
Tipo del documento:
Article