Mini-Symposium: Medical and surgical aspects of chest wall deformities
Thoracic malformation with early-onset scoliosis: Effect of serial VEPTR expansion thoracoplasty on lung growth and function in children

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Summary

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 (Crs) 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.

Introduction

Surgical interventions for infants and children with thoracic and spinal malformations in early-onset scoliosis have focused almost exclusively on the correction of spinal deformity. Such treatments commonly involved spinal fusion, which stunts growth of the spinal column and limits the thoracic volume and lung growth.1, 2 More recently, Campbell et al. have focused their attention on the interrelationship between the spine, thoracic wall and lungs with growth, especially in children with combined malformations of the thorax and spine (spondylothoracic dysplasia).3 They have developed vertical expandable prosthetic titanium ribs (VEPTR) for serial expansion thoracoplasty to control the three-dimensional progression with body growth of both spine and chest wall deformities, and resultant respiratory insufficiency, which they have termed thoracic insufficiency syndrome (TIS).4

Using the VEPTR technique, Campbell et al. have reported growth of the concave side of the thorax along with clinical improvements in the respiratory status of afflicted children.4, 5 It has been assumed that the lung volume would also increase with serial expansion of the concave side of the hemithorax. However, direct measurements of lung volumes and pulmonary function in these patients have been limited to a small number of older children.2 Furthermore, the longitudinal effects of serial VEPTR thoracoplasty on respiratory function have not been reported in the literature.

Previously, we have developed a mobile unit to perform a variety of pulmonary function tests (PFTs), using both passive and forced deflation techniques, in anesthetized or heavily sedated infants and children in the operating room or intensive care unit.6 In our preliminary report, we described the results for 41 serial PFTs in 10 children (aged 2–10 years) in the operating room under general anaesthesia immediately before VEPTR thoracoplasty. The study methods and techniques used are detailed below, and the effects of VEPTR on lung volume and respiratory mechanics described in this preliminary report as well as more recent follow-up study results in a larger patient population are summarized.

Section snippets

Pulmonary function tests

Bedside PFTs were performed following the induction of general anaesthesia and intubation with a cuffed endotracheal (ET) tube by means of passive and forced deflation techniques in patients in a supine position.6

Preliminary results

At the initial PFTs, there were moderate-to-severe restrictive defects in most children, with an average FVC of 69.2 ± 17.4% of predicted value (range 36–101%); as a group, FVC was significantly decreased (p < 0.001).9 At the last PFTs following serial VEPTR thoracoplasties, there were definite signs of lung growth, as indicated by significant increases in FVC at an average annual rate of 19.6 ± 6.0% (p < 0.01), or about 73% of normal growth. On the other hand, in terms of % predicted values, FVC

Growth and development of the spine, thorax and lungs in early childhood

A condition of thoracic wall abnormalities with progressive early-onset scoliosis in infants and young children is a challenging disease both medically and surgically. If untreated, the progression of thoracic deformity would impede the growth of thoracic and lung volumes and would compromise respiratory function and pulmonary gas exchange, leading to respiratory insufficiency, known as TIS.4 More than in any other age group, spinal fusion in young children – which until recently was the

Conclusion

The effect of surgical intervention in children with thoracic and spinal malformation and progressive early-onset scoliosis has been reviewed, with emphasis on the effect of serial expansion thoracoplasty with VEPTR and its longitudinal effect on lung volume and respiratory mechanics, which were studied under general anaesthesia at the time of surgery using a specially constructed mobile PFT device. As expected from clinical impressions and indirect measurements, most children with TIS of

Acknowledgement

The authors thank Rebecca Mutich, RT, Division of Allergy and Pulmonology, for data management and analysis, Joanne Londino, RN, Department of Orthopedic Surgery, for gathering surgical data and David Chasey, Department of Anesthesiology, for editorial assistance.

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