The effect on pulmonary function of serial VEPTR expansion thoracoplasty was studied longitudinally in anesthetized children with spondylothoracic dysplasia using a special mobile unit. The median age of 24 children at the start of surgery was 4.6 years (1.8-10.8) and most exhibited a moderate-to-severe restrictive lung defect. After a median of 3.2 years (1.0-6.5), their forced vital capacity (FVC) was found to have increased by an average of 11.1%/year. The rate of increase was greater in children who were younger than 6 years at the start of the study than in older children (14.5% versus 6.5%, p<0.01). The average specific respiratory system compliance (C(rs)) was mildly-to-moderately decreased at the start, and over the study it decreased on average to 56% of the initial value in spite of clinically successful expansion thoracoplasty and lung growth, indicating increasing stiffness of the thorax with growth.