The characteristics of thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis

Thoracic insufficiency syndrome is the inability of the thorax to support normal respiration or lung growth. The rare condition of fused ribs and congenital scoliosis may result in a three-dimensional thoracic deformity with adverse effects on thoracic growth and function with development of thoracic insufficiency syndrome. The normal thorax is defined by two characteristics: normal, stable volume and the ability to change that volume. Volume depends on the width and depth of the rib cage, and the thoracic spine provides height. The ability to change volume, termed thoracic function, is provided by the diaphragm and the secondary muscles of respiration. On radiographs, the loss of the vertical height of the lung of the concave, restricted hemithorax is defined by the percentage of space available for the lung. Spine rotation causes a windswept thorax, with both restriction of the volume of the convex hemithorax and restriction of the motion of the involved ribs.. Constrictive three-dimensional deformity of the thorax may cause extrinsic, restrictive lung disease. Progressive thoracic insufficiency syndrome is diagnosed on the basis of clinical signs of respiratory insufficiency, loss of chest wall mobility as demonstrated by the thumb excursion test, worsening indices of three-dimensional thoracic deformity on radiographs and computed tomography scans, or a relative decline in percent predicted vital capacity due to thoracic "failure to thrive," as demonstrated by pulmonary function tests. Treatment of progressive thoracic insufficiency syndrome should provide an acute increase in the thoracic volume with stabilization of any flail chest-wall defects and maintain these improvements as the patient grows, without the need for spine fusion.