Management of thoracic insufficiency syndrome in patients with Jarcho-Levin syndrome using VEPTRs (vertical expandable prosthetic titanium ribs).

BACKGROUND: Jarcho-Levin syndrome represents a spectrum of clinical and radiographic irregularities including abnormal vertebral segmentation or formation defects, rib deformities, and short-trunk dwarfism. These abnormalities cause reduced thoracic capacity for lung development, resulting in thoracic insufficiency syndrome. In the present study, we reviewed outcome measures related to scoliotic curve correction, thoracic growth, and respiratory function following VEPTR treatment in patients with Jarcho-Levin syndrome. METHODS: Twenty-nine patients with Jarcho-Levin syndrome, subclassified as spondylocostal dysostosis (SCD) or spondylothoracic dysplasia (STD), were treated with VEPTR expansion thoracoplasty and followed for at least two years since the initial implantation. Spinal and respiratory measures were collected prior to the initial VEPTR implantation, immediately after implantation, and at the most recent follow-up. RESULTS: VEPTR treatment was associated with improved clinical respiratory function and with increases in thoracic height (by 50% in the SCD group and 42% in the STD group) and thoracic width (by 37% in the SCD group and 28% in the STD group). VEPTR treatment resulted in scoliosis curve correction (improvement in the Cobb angle of 41% [22°] in the SCD group and 26% [3.7°] in the STD group) and in improved thoracic symmetry in patients with SCD. Patients with SCD displayed increased lumbar lordosis, and both groups of patients developed increased thoracic kyphosis approaching normal. CONCLUSIONS: VEPTR treatment improved thoracic symmetry, controlled spinal deformity, and was associated with improved clinical respiratory function.

Diagnosis and management of primary pyogenic spinal infections in intravenous recreational drug users.

OBJECT: Primary spine infection secondary to intravenous drug abuse (IVDA) is a difficult clinical entity encountered by spine surgeons and infectious disease specialists. Patients tend to be noncompliant with the treatment and follow-up, and some continue to use IV recreational drugs even after the diagnosis of spine infection. The authors undertook this study to analyze the presentation, etiology, demographic characteristics, treatment, and outcome of primary pyogenic spinal infection in patients with IVDA as the major risk factor. METHODS: The medical records, radiology imaging, and laboratory results (white blood cell count, inflammatory markers, bacteriology cultures) of all patients with pyogenic spine infection and history of IVDA presenting to a tertiary care center from August 2005 through December 2013 were retrospectively reviewed. The department of neurosurgery database and the hospital electronic medical records of University Hospital in San Antonio were used to identify the cohort for our study. RESULTS: A total of 164 patients with spinal infection were evaluated during the study period; 102 of these patients had a history of IVDA. Their average age was 45.4 years, and only 14 (13.7%) were women. The mean laboratory values at presentation included a white blood cell count of 11.1 × 10(3) cells/µl (range 0.5-32 × 10(3) cells/µl), erythrocyte sedimentation rate (ESR) of 74 mm/hr (range 9.9-140 mm/hr), and C-reactive protein (CRP) level of 67 mg/L (range 0.1-327 mg/L). Twenty-six patients (25.4%) had an associated epidural abscess. The most common organism isolated from cultures of the bone and/or blood was methicillin-sensitive Staphylococcus aureus (MSSA), which was found in 37 cases. A close second was methicillin-resistant S. aureus (MRSA), found in 23 cases. The most commonly involved region was the lumbar spine (24 cases [57.8%]), and most patients (69.6%) had involvement of only a single level. Eighty patients were initially treated with long-term IV antibiotic therapy, and only 22 underwent surgical intervention (24 procedures). Of the latter group, 8 patients underwent laminectomy alone while 16 required some type of instrumented stabilization. Of the patients requiring stabilization procedures, 2 (12.5%) required reoperation with extension of their surgical constructs to other levels. The average follow-up was 29.7 weeks (range 6 weeks to 3 years). CONCLUSIONS: Diagnosis and management of spinal infection in patients with a history of IVDA is challenging. The data from this study show that initial laboratory values are difficult to interpret given that only a minority of these patients present with leukocytosis. Back pain was the only reliable predictor of spine infection. The authors' experience indicates that the majority of patients with spine infection and a history of IVDA can be successfully treated with IV antibiotic therapy alone.