Diastematomyelia and structural spinal deformities.

Sixty patients with diastematomyelia were seen over a thirty-year period and congenital scoliosis was found in 60 per cent. All of the patients had associated vertebral abnormalities and most (87 per cent) had a neural deficit. Myelography was helpful in the diagnosis, particularly prior to any procedure that might cause traction on the spinal cord. Laminectomy for removal of the spur was indicated when neural deficits were progressive or before corrective surgery on the spine, and in ten patients the operation alleviated neural sequelae. Observation of patients with diastematomyelia who have no neural deficit or a stable, non-progressing deficit is recommended.

Skeletal growth and development of the achondroplastic dwarf.

Certain patterns in growth and development can be seen in the typical achondroplastic dwarf. Height is approximatley 5 standard deviations below average size for age through the age of 14. Sitting height is within normal limits at all ages. On the average the femur is 8 standard deviations and the tibia 7 standard deviations below normal size. Compared to normal, both femur and tibia exhibit approximately a 50 per cent inhibition in growth. A slight decrease in the deviance from the norm is observed for all parameters during growth until the time of the adolescent growth spurt when an increased deviance is seen. Related to normal children of the same sex and age, females are more severely affected than male achondroplastic dwarfs. The skeletal development of the hand is irregular, often having extra metacarpal epiphyses and a wide variance in skeletal maturation assessment between the carpals and the rest of the hand. The skeletal age is generally below chronological age until the adolescent growth spurt when a rapid maturation pattern occurs. Standard skeletal age assessment techniques cannot be applied to the achondroplastic individual.