The Characteristics of Thoracic Insufficiency Syndrome Associated With Fused Ribs and Congenital Scoliosis

Is the Vertebral Expandable Prosthetic Titanium Rib a Surgical Alternative in Patients with Spina Bifida?

“…The dysplastic anatomy of the vertebral column and truncal weakness in myelodysplasia together often cause progressive spine and chest wall deformity in a paralytic spine leading to thoracic insufficiency. Primary thoracic insufficiency syndrome (TIS) is defined by Campbell and Smith as the inability of the thorax to support normal respiration or lung growth and may be the result of thoracic spine and chest wall deformity adversely affecting lung function and growth [2][3][4][5]. Secondary TIS is commonly encountered in nonambulatory patients with myelodysplasia as a result of decreased space available for the lung and decreased tidal volume as the diaphragm is forced cephalad by a collapsing spinal deformity or short trunk.…”

“…Secondary TIS is commonly encountered in nonambulatory patients with myelodysplasia as a result of decreased space available for the lung and decreased tidal volume as the diaphragm is forced cephalad by a collapsing spinal deformity or short trunk. Crowding of the abdominal or visceral contents resulting from the deformity further inhibits diaphragm effectiveness and affects pulmonary function [4,5,9]. A clinical manifestation is the ''marionette sign'' in which the head and torso bob in synchrony with respiration with the diaphragm doing a push up against body weight [4,5,9].…”

“…Crowding of the abdominal or visceral contents resulting from the deformity further inhibits diaphragm effectiveness and affects pulmonary function [4,5,9]. A clinical manifestation is the ''marionette sign'' in which the head and torso bob in synchrony with respiration with the diaphragm doing a push up against body weight [4,5,9]. The decrease in pulmonary capacity may go unnoticed as a result of the child's limited physical activities.…”

“…The decrease in pulmonary capacity may go unnoticed as a result of the child's limited physical activities. The patients must use their upper extremities to extend their torso for adequate breathing and to maintain sitting balance [5]. The achievement of a functional sitting height is essential in nonambulatory patients with myelodysplasia to allow for efficient pulmonary function and to avoid abdominal crowding.…”

Calculus anuria in a spina bifida patient, who had solitary functioning kidney and recurrent renal calculi

“…4 Campbell et al, 5 in their recent article, give a comprehensive overview of the characteristics of thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis. The normal thorax is defined by two characteristics: normal, stable volume and the ability to change that volume.…”