Halo-gravity traction versus surgical release before implantation of expandable spinal devices: a comparison of results and complications in early-onset spinal deformity.

STUDY DESIGN: Between 1997 and 2009, 124 children with severe early-onset spinal deformity were treated with the vertical expandable prosthetic titanium rib or growing rods. Before the implantation of the device, 12 underwent halo-gravity traction (HGT) and 13 had a spinal release (SR). This observational study is based on x-rays obtained before and after surgery and at the last follow-up and on medical records. OBJECTIVE: To describe the indications and effectiveness of HGT and SR in young patients treated with expandable devices. SUMMARY OF BACKGROUND DATA: Several studies have described the short-term effects of HGT and SR, in particular their safety and effectiveness on severe scoliosis, but none before the implantation of expandable spinal devices in severe early-onset spinal deformity. METHODS: We measured the thoracic scoliosis and kyphosis, and the radiographic thoracic height and spinal length on x-rays. We also collected postoperative complications. RESULTS: Postoperatively, SR resulted in a better correction of the scoliosis (46 degrees) than HGT or no release (37 and 27 degrees, respectively) but HGT provided a better correction of the kyphosis (48 degrees) than SR or no-spinal release (27 and 9 degrees, respectively). At follow-up, all groups showed a loss of correction of the kyphosis. The postoperative increase in thoracic spine height, which was initially greater in the HGT group because of the traction, became higher in the SR group at follow-up. Device complications occurred in 54% of the participants in the HGT group, 75% in the SR group, and 28% in the control group, and had a negative impact on the final thoracic spine height. Neurologic complications occurred in 8% of the participants and were more frequent in the HGT group (25%). CONCLUSIONS: The surgical procedures had different short-term impacts on the curve, which did not necessary predict long-term outcomes (loss of correction, loss of height increase). The risk of device loosening was high in the HGT and SR groups, as was the risk of neurologic loss.

Increased hemoglobin levels in patients with early onset scoliosis: prevalence and effect of a treatment with Vertical Expandable Prosthetic Titanium Rib (VEPTR).

STUDY DESIGN: The medical records of 138 subjects with Early Onset Scoliosis (EOS) from 5 US institutions were reviewed to analyze their hemoglobin levels before and after surgery. Eighty-five subjects were operated with the Vertical Expandable Prosthetic Titanium Rib (VEPTR) and 53 with growing rods. OBJECTIVE: To estimate the prevalence of hypoxia and thoracic insufficiency using hemoglobin as a surrogate marker for pulmonary function, and to measure the effect of spine surgery with expandable devices. SUMMARY OF BACKGROUND DATA: An early intervention with expandable devices might improve pulmonary function. This hypothesis is difficult to test in young subjects because the standard pulmonary function tests require that the patient be at least 7-year old. Previous studies demonstrated that hemoglobin levels are correlated with chronic hypoxemia. METHODS: Blood data were collected before and 6 to 24 months after surgery. The hemoglobin and hematocrit levels were converted into Z-scores, using age-adjusted references to perform t test paired comparisons. RESULTS: The prevalence of elevated hemoglobin and hematocrit levels in EOS was 23.2% and 22.5%, respectively. The mean hemoglobin Z-score decreased from 1.26 to 0.92 (P = 0.03) after surgery and the hematocrit Z-score changed from 0.90 to 0.88 (P = 0.90). In the VEPTR group, the mean hemoglobin Z-score decreased from 0.98 to 0.69 (P = 0.20) and in the subgroup of subjects who had an expansion thoracostomy, it decreased from 0.82 to 0.24 (P = 0.04). In the subjects operated with growing rods, the hemoglobin Z-score decreased from 1.75 to 1.32 (P = 0.83). CONCLUSION: Twenty-three percent of the patients with EOS showed signs of chronic hypoxia. The hemoglobin levels decreased significantly 6 to 24 months after surgery. The greatest effect was observed in subjects with congenital scoliosis and rib fusion and who had an expansion thoracostomy with implantation of VEPTR.

Mutations in the MESP2 gene cause spondylothoracic dysostosis/Jarcho-Levin syndrome.

Spondylothoracic dysostosis (STD), also known as Jarcho-Levin syndrome (JLS), is an autosomal-recessive disorder characterized by abnormal vertebral segmentation and defects affecting spine formation, with complete bilateral fusion of the ribs at the costovertebral junction producing a "crab-like" configuration of the thorax. The shortened spine and trunk can severely affect respiratory function during early childhood. The condition is prevalent in the Puerto Rican population, although it is a panethnic disorder. By sequencing a set of candidate genes involved in mouse segmentation, we identified a recessive E103X nonsense mutation in the mesoderm posterior 2 homolog (MESP2) gene in a patient, of Puerto Rican origin and from the Boston area, who had been diagnosed with STD/JLS. We then analyzed 12 Puerto Rican families with STD probands for the MESP2 E103X mutation. Ten patients were homozygous for the E103X mutation, three patients were compound heterozygous for a second nonsense mutation, E230X, or a missense mutation, L125V, which affects a conserved leucine residue within the bHLH region. Thus, all affected probands harbored the E103X mutation. Our findings suggest a founder-effect mutation in the MESP2 gene as a major cause of the classical Puerto Rican form of STD/JLS.

The impact of a suspicious prostate biopsy on patients' psychological, socio-behavioral, and medical care outcomes.

OBJECTIVE: To evaluate the psychological, socio-behavioral, and medical implications of apparently false-positive prostate cancer screening results. METHODS: One hundred and twenty-one men with a benign prostate biopsy performed in response to a suspicious screening test (biopsy group) and 164 men with a normal prostate-specific antigen (PSA) test result (normal PSA group) responded to a questionnaire 6 weeks, 6 and 12 months after their biopsy or PSA test. RESULTS: The mean (+/-SD) age of respondents was 61+/-9 years (range, 41 to 88 years). One year later, 26% (32/121) of men in the biopsy group reported having worried "a lot" or "some of the time" that they may develop prostate cancer, compared with 6% (10/164) in the normal PSA group (P<.001). Forty-six percent of the biopsy group reported thinking their wife or significant other was concerned about prostate cancer, versus 14% in the normal PSA group (P<.001). Medical record review showed that biopsied men were more likely than those in the normal PSA group to have had at least 1 follow-up PSA test over the year (73% vs 42%, P<.001), more likely to have had another biopsy (15% vs 1%, P<.001), and more likely to have visited a urologist (71% vs 13%, P<.001). CONCLUSION: One year later, men who underwent prostate biopsy more often reported worrying about prostate cancer. In addition, there were related psychological, socio-behavioral, and medical care implications. These hidden tolls associated with screening should be considered in the discussion about the benefits and risks of prostate cancer screening.

The treatment of spine and chest wall deformities with fused ribs by expansion thoracostomy and insertion of vertical expandable prosthetic titanium rib: growth of thoracic spine and improvement of lung volumes.

STUDY DESIGN: Prospective clinical trial of vertical expandable prosthetic titanium rib (VEPTR) in patients with combined spine and chest wall deformity with scoliosis and fused ribs. OBJECTIVE: Report the efficacy and safety of expansion thoracostomy and VEPTR surgery in the treatment of thoracic insufficiency syndrome (TIS) associated with fused ribs. SUMMARY OF BACKGROUND DATA: Traditional attitudes toward early-onset combined chest and spine deformity assume that thoracic deformity is best controlled by treatment directed at spine deformity, often involving early spinal arthrodesis. Campbell and others have heightened awareness of the interrelationship between lung, chest, and spine development during growth and characterized TIS as the inability of the thorax to support normal respiration or lung growth. Expansion thoracostomy and VEPTR insertion was developed to directly control both spine and chest wall deformity during growth, while permitting continued vertebral column and chest growth at an early stage. METHODS: Multidisciplinary evaluation of children with combined spine and chest wall deformity included pediatric pulmonologist, thoracic, and orthopedic surgeon evaluations. One or more opening wedge expansion thoracostomies and placement of VEPTR devices were performed as described by Campbell, with repeated device lengthenings during growth. Parameters measured included Cobb angle, length of thoracic spine, CT-derived lung volumes, and in older children pulmonary function tests. RESULTS: Thirty-one patients with fused ribs and TIS were treated, 4 of whom had undergone prior spinal arthrodesis at other institutions with continued progression of deformity. In 30 patients, the spinal deformity was controlled and growth continued in the thoracic spine during treatment at rates similar to normals. Increased volume of the constricted hemithorax and total lung volumes obtained during expansion thoracostomy were maintained at follow-up. Complications included device migration, infection, and brachial plexus palsy. CONCLUSIONS: Expansion thoracostomy and VEPTR insertion with serial lengthening may be the preferred treatment for young children with chest wall deformity and scoliosis associated with fused ribs but requires multidisciplinary care and attention to details of soft tissue management. When indicated, surgical intervention with VEPTR can be considered early in growth, before deformity is severe, since spinal growth will continue with treatment.

Psychological effects of a suspicious prostate cancer screening test followed by a benign biopsy result.

PURPOSE: To evaluate the psychological implications of an apparently false-positive screening result for prostate cancer. METHODS: The sample comprised 167 men with a benign biopsy result in response to a suspicious screening test result (biopsy group) and 233 men with a normal prostate-specific antigen (PSA) test result (control group). The men responded to a questionnaire within about 6 weeks of their biopsy or PSA results. They were asked about demographic characteristics, medical history, psychological effects, biopsy experience, and prostate cancer knowledge. RESULTS: The survey response rate was 85% (400/471). The mean (+/- SD) age of respondents was 60 +/- 9 years (range, 40 to 88 years); 88% (n = 350) were white. Forty-nine percent (81/167) of men in the biopsy group reported having thought about prostate cancer either "a lot" or "some of the time", compared with 18% (42/230) in the control group (P < 0.001). In addition, 40% (67/167) in the biopsy group reported having worried "a lot" or "some of the time" that they may develop prostate cancer, compared with 8% (18/231) in the control group (P < 0.001). CONCLUSION: Men who underwent prostate biopsy more often reported having thought and worried about prostate cancer, despite having received a benign result. This underrecognized human cost of screening should be considered in the debate about the benefits and harms of prostate cancer screening.

Validity of primary care patient self-report of abnormal (but not cancer) prostate biopsy results.

PURPOSE: We assessed the validity of patient self-report of abnormal (but not cancer) prostate biopsy results, ie, atypical cells or prostatic intraepithelial neoplasia. MATERIALS AND METHODS: We compared survey responses and pathology records for 156 men from a multicenter, prospective cohort study, which was designed to evaluate the impact of a suspicious prostate cancer screening test followed by benign prostate biopsy. RESULTS: Of the 156 subjects 45 (29%) reported having had atypical cells or prostatic intraepithelial neoplasia and 38 (24%) had a pathology report with atypia or prostate intraepithelial neoplasia. The sensitivity of the self-report was 87%, specificity was 90%, positive predictive value was 73% and negative predictive value was 95%. The kappa statistic was 0.7. CONCLUSIONS: Men in this study reported this complex result accurately. However, it was concerning that 5 men (13%) appeared unaware of an abnormal prostate biopsy result, especially given the substantial residual risk of prostate cancer conferred by this result.