Ten year follow-up of Jarcho-Levin syndrome with thoracic insufficiency treated by VEPTR and MCGR VEPTR hybrid.

Abstract

Jarcho-Levin syndrome patients commonly suffer from repeated respiratory infections and become ventilator dependent due to an insufficient thoracic volume. Multiple congenital vertebral anomalies are associated with this genetic disorder and results in stunted spine growth. The purpose of this case report is to present the results of a hybrid vertical expandable prosthetic titanium rib (VEPTR) and magnetically controlled growing rod (MCGR) construct for the management of a patient with Jarcho-Levin syndrome. A boy with Jarcho-Levin syndrome undergoes a hybrid VEPTR-MCGR construct to treat his thoracic insufficiency syndrome and spinal deformity. The patient could wean off ventilator and had reduced chest complications with the construct. He was also able to achieve some spine length gain with the distraction device. However, there were limitations in prolonged treatment as no spine height was gained once he reached 12 years old. Once no further growth can be achieved, the MCGR led to progressive kyphosis. Nevertheless, at latest follow-up after the removal of all implants, the patient could maintain satisfactory correction of both thoracic and spine deformities. A hybrid construct is necessary for managing Jarcho-Levin syndrome as the VEPTR deals with the thoracic cage deformity while the MCGR deals with the spine deformity.