A novel approach in the management of Thoracic Insufficiency Syndrome in Jarcho-Levin Syndrome using a magnetic controlled growing rod - vertical expandable prosthetic titanium rib hybrid construct

Abstract

INTRODUCTION: Jarcho-Levin syndrome (JLS) is a rare condition leading to deformity of the spine with a characteristic markedly shortened thorax potentially leading to thoracic insufficiency syndrome (TIS). Use of vertical expandable prosthetic titanium rib (VEPTR) has been reported in cases of JLS. Magnetically controlled growing rods (MCGR) allow gradual distractions at small intervals to increase spinal height. To our knowledge, we are the first to report its use in patients with JLS. MATERIALS AND METHODS: We present our 8-year experience, utilising a MCGR-VEPTR hybrid construct in an 11-year-old patient with JLS, with VEPTR inserted at age of 3 years and MCGR inserted at age of 8 years. Serial radiographs were reviewed for changes in spinal length, thoracic height, abdominal height, chest width, pelvis height, and sagittal balance. Anthropomorphic body measurements were also documented. Computed tomographic scan of lung volumetry was used to assess lung function. RESULTS AND DISCUSSION: Over 3 years, serial VEPTR distraction resulted in a 95.5% increase in the total lung volume, reflecting clinically in a transition from preoperative ventilator dependence to complete respiratory independence. A 48.3% increase in body height measurement with combined VEPTR and serial MCGR distraction were observed over the 8-year period. An increase in spinal height on lateral radiographs since commencement of MCGR distraction was noted. Progressive kyphosis was seen with distraction, highlighting a potential shortcoming of distracting with the posteriorly positioned MCGR in a spine with a rigid anterior column.