Clinical Issues for Pediatric Pulmonologists Managing Children With Thoracic Insufficiency Syndrome

Abstract

Thoracic insufficiency Syndrome (TIS) is a recently coined phrase to describe children with spine and chest wall deformities, inherited and acquired, who have respiratory impairment, and are skeletally immature. This population has both restrictive and less often obstructive lung disease due to changes in spine and rib configuration which reduce lung volume, stiffen the chest wall, and reduce respiratory muscle strength. Although the population is heterogeneous with regard to age of onset, etiology, severity of deformity, and rate of progression of the deformity, there are common issues that arise which can be addressed by pediatric pulmonologists. These are illustrated in this review by using Early Onset Scoliosis as a common form of TIS. The pulmonary issues pertaining to TIS require collaboration with multi-disciplinary teams, particularly spine surgeons, in order to make decisions about non-surgical and surgical strategies, timing of surgery and medical supportive care over time. Pulmonary input about respiratory function should be used in conjunction with structural features of each deformity in order to determine the impact of the deformity and the response to various treatment options. In those patients with residual lung function impairment as young adults, pediatric pulmonologists must also ensure successful transition to adult care.

Keywords: children; management; pulmonary; scoliosis; spine; thorax.

Figure 1

Measurement of the spine's coronal curve using the Cobb angle: Lines drawn along the edges of the vertebrae that are most angled relative to a horizontal line above and below the apex of the curve. The intersection of those lines is used to derive the Cobb angle.

Figure 2

(A) CT scan of lobar bronchial compression by the spine due to scoliosis and vertebral intrusion into the thorax. (B) Lung ventilation scan correlated with the CT scan in (A) demonstrating loss of ventilation in the right middle and lower lobes.

Figure 3

Correlation between Maximum Inspiratory Pressure (MIP) and Forced Vital Capacity (FVC) among Children with Early Onset Scoliosis without underlying neuromuscular conditions.