Thoracic vertebral fractures and hyperkyphosis in elderly patients with end-stage kidney disease; do these patients have different clinical outcomes?

BACKGROUND AND OBJECTIVES: Elderly patients with end-stage kidney disease (ESKD) are at high risk for fractures. However, the prevalence of vertebral fractures and hyperkyphosis is not studied well. This is relevant, because in the general population, both vertebral fractures and hyperkyphosis are associated with poor outcome. Therefore, the primary aim of our study was to assess the prevalence of vertebral fractures and hyperkyphosis in the ESKD population. The secondary aim was to assess if patients with vertebral fractures and/or hyperkyphosis more often have poor outcome after starting dialysis, such as accidental falling, functional decline and mortality compared to the patients without vertebral fractures and/or hyperkyphosis. DESIGN, SETTING, PARTICIPANTS & MEASUREMENTS: This study included patients ≥65 years with ESKD who were enrolled in the Geriatric assessment in Older patients starting Dialysis (GOLD) study of whom a lateral chest radiograph was available. Chest radiographs were scored independently by two observers for vertebral fractures (Genant ≥1) and hyperkyphosis (≥50 degrees). The relation between vertebral fractures and hyperkyphosis with clinical outcomes (falls, decline in ADL and IADL, mortality) was studied using the Chi-square test. RESULTS: Of the 196 enrolled patients, chest radiographs were available for 160 patients. Mean age was 75.3 (SD ±6.9), and 35% were female. The prevalence of vertebral fractures was 43% and of hyperkyphosis 22%. Patients with hyperkyphosis had a higher one-year mortality compared to patients without hyperkyphosis (20% vs. 8%, p = 0.04). No differences were observed between patients with and without hyperkyphosis, vertebral fractures and the remaining outcomes after six months of follow-up. CONCLUSIONS: In patients ≥65 years old with ESKD starting dialysis, vertebral fractures are highly prevalent. In contrast to the general population, patients with vertebral fractures did experience poor outcome as often as patients without vertebral fractures. Remarkably, patients with hyperkyphosis did have a higher one-year mortality. However, these patients did not experience more functional decline or accidental falls.

Overall Similar Infection Rates Reported in the Physician-reported Scoliosis Research Society Database and the Chart-abstracted American College of Surgeons National Surgical Quality Improvement Program Database.

STUDY DESIGN: This is a retrospective cohort study. OBJECTIVE: To compare the infection rates in the Scoliosis Research Society (SRS) morbidity and mortality database to those in the American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP) database. SUMMARY OF BACKGROUND DATA: Surgical databases have demonstrated value in orthopaedic surgery. However, few studies in the literature have used the SRS database relative to other available databases. To validate the surgeon-reported data of the SRS database, we compared the rate of postoperative infection in this database to that in the chart-abstracted ACS-NSQIP database. METHODS: International Classification of Disease, ninth revision (ICD-9) codes were used to group patients from the 2012 and 2013 ACS-NSQIP database into groups corresponding to spinal deformity diagnoses delineated by SRS. Postoperative infection rates after surgical correction of these deformities compared with those reported in the 2012 and 2013 SRS database using χ tests. RESULTS: The overall rate of acute postoperative infection was slightly lower in the SRS database than in the ACS-NSQIP database (1.21% compared to 2.05% in ACS-NSQIP, a difference of only 0.84%, statistically significant, P < 0.001).The infection rates of most diagnoses did not differ; these included scoliosis (idiopathic <10 yr of age/congenital/neuromuscular/other scoliosis), spondylolisthesis (isthmic/dysplastic), and kyphosis (congenital and other). The only differences noted were in Scheuermann kyphosis (difference of 5.49%, P = 0.003), degenerative spondylolithesis (difference of 0.69%, P = 0.003), and idiopathic scoliosis in the age 10 to18 yr and adult age ranges (differences of 1.10% and 1.28%, P < 0.001 for both). CONCLUSION: Although some statistical differences were found in infection rates between the surgeon-reported SRS database and the chart-abstracted ACS-NSQIP database, these small differences likely reflect differences in surgical cases and data collection methods. This finding supports the validity of the surgeon-reported SRS morbidity and morality database. LEVEL OF EVIDENCE: 3.

Does obesity affect surgical outcomes in adolescent idiopathic scoliosis?

STUDY DESIGN: A retrospective review of surgical outcomes in adolescents with idiopathic scoliosis. OBJECTIVE: To determine if an association exists between body mass and surgical outcomes in adolescent idiopathic scoliosis (AIS). SUMMARY OF BACKGROUND DATA: Obesity has reached epidemic proportions globally. In adults, obesity increases the likelihood of developing multiple medical comorbidities and has been associated with an increased incidence of perioperative complications. The effect of obesity on surgical outcomes in the treatment of AIS patients has not been studied previously. METHODS: Radiographic measures, perioperative data, and Scoliosis Research Society Outcomes scores were collected on surgically treated AIS patients. The body mass index (BMI) was calculated for each patient and normalized to sex and age (BMI %). Analysis of variance was used to identify differences between healthy weight (BMI % <85) and overweight patients (BMI % >or=85). The data were checked for normality and equal variances, and the level of significance was set at 0.01. RESULTS: Two hundred forty-one patients (204 women, 37 men; 14.3 +/- 2.0 years) with a minimum of 2-year follow-up met the inclusion criteria for this study. The average BMI (kg/m2) was 20.7 +/- 3.7 (BMI % average: 54.5, range: 1-99). No significant differences were found between the overweight (n = 48) and healthy weight (n = 193) patients with regards to surgical time, estimated blood loss, major Cobb percent correction, maintenance of correction, rate of implant failure, pseudarthrosis, and surgical revision. However, the preoperative thoracic kyphosis was significantly greater in the overweight group (27.0 degrees +/- 12.6 degrees) compared with the healthy weight patients (21.8 degrees +/- 12.5 degrees) (P = 0.004). CONCLUSION: Overweight adolescents (BMI % >or=85) had a greater thoracic kyphosis before surgery compared with their healthy weight peers. Body mass, however, did not affect the ability to achieve coronal or sagittal scoliotic deformity correction, and did not increase perioperative morbidity or mortality. These findings were either influenced by the small sample size of this cohort, or because the comorbidities responsible for increased perioperative complications in adults, had not yet developed in this adolescent population.

Survival of patients with kyphoscoliosis receiving mechanical ventilation or oxygen at home.

BACKGROUND: Home mechanical ventilation (HMV) and long-term oxygen therapy (LTOT) are the two treatment alternatives when treating respiratory insufficiency in patients with kyphoscoliosis. We aimed to study the effect on survival with regard to HMV or LTOT alone in patients with respiratory insufficiency due to kyphoscoliosis. METHODS: Swedish patients with nonparalytic kyphoscoliosis (ie, scoliosis not related to neuromuscular disorders) who started LTOT or HMV between 1996 and 2004 were followed up prospectively until February 14, 2006, with death as the primary outcome. Treatment modality, arterial blood gas levels, the presence of concomitant respiratory diseases, and age were recorded at the onset of treatment. No patient was lost to follow-up. RESULTS: One hundred patients received HMV, and 144 patients received oxygen therapy alone. Patients treated with HMV experienced better survival, even when adjusting for age, gender, concomitant respiratory diseases, and blood gas levels, with a hazard ratio of 0.30 (95% confidence interval, 0.18 to 0.51). CONCLUSION: The survival of patients with kyphoscoliosis receiving HMV was better than that of patients treated with LTOT alone. We suggest HMV and not oxygen therapy alone as the primary therapy for patients with respiratory failure due to kyphoscoliosis, regardless of gender, age, and the occurrence of concomitant respiratory diseases.

[Schimke immuno-osseous dysplasia. A pediatric disease reaches adulthood]. / Schimke-immunoossäre Dysplasie. Eine pädiatrische Erkrankung wird erwachsen.

BACKGROUND: Schimke immuno-osseous dysplasia (SIOD) is a rare autosomal recessive multisystemic disorder caused by mutations of the SMARCAL 1 gene (SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily a-like 1). CLINICAL FEATURES: Main clinical features are: disproportional growth deficiency due to spondyloepiphyseal dysplasia, nephrotic syndrome with focal and segmental glomerulosclerosis, and defective cellular immunity. Patients with severe SIOD have life-limiting complications like cerebral ischemia due to vaso-occlusive processes. Only a few patients reached adulthood. CASE REPORTS: The clinical course of four adult SIOD patients is presented. CONCLUSION: Even patients with severe SIOD can reach adulthood. Therefore, doctors working in the field of internal medicine and family doctors should be familiar with the clinical picture of SIOD.

Treatment of chronic respiratory failure in kyphoscoliosis: oxygen or ventilation?

Patients with kyposcoliosis and chronic respiratory insufficiency are treated either with home oxygen therapy or ventilation. Kyphoscoliotic patients demonstrate impaired ventilatory mechanics, consequently ventilation seems to be the treatment of choice. Yet, no randomised controlled trials (CRT) exist to prove it. Most investigators find it difficult to ethically justify a CRT. Therefore, the current authors performed the following retrospective study: survival and pulmonary function were analysed in all consecutive kyphoscoliotic patients who started long-term oxygen therapy (LTO group; n=15, aged 62+/-11 yrs (mean+/-SD)) or LTO plus nocturnal nasal intermittent positive pressure ventilation (nNIPPV group; n=18, aged 61+/-7 yrs) in the Dept of Pulmonology (University Hospital Gasthuisberg, Leuven) between 1990-2002. Prior to treatment partial pressure of oxygen (PO2) was lower, partial pressure of carbon dioxide (PCO2) tended to be higher and vital capacity (VC) tended to be lower in the nNIPPV group than in the LTO group (PO2 5.9+/-1 versus 6.7+/-0.9 kPa (44+/-8 versus 50+/-7 mmHg), PCO2 8+/-1 versus 7.3+/-0.9 kPa (60+/-8 versus 55+/-7 mmHg), VC 32+/-12 versus 40+/-16% predicted, or 645+/-244 versus 970+/-387 mL). In the nNIPPV group the 1-yr survival was higher (100% versus 66%). nNIPPV patients demonstrated an improvement in PO2 (breathing air) +54%, PCO2 (breathing air) -21%, VC +47% and maximal static inspiratory mouth pressure +33%; these improvements were absent in the LTO group. In conclusion, nocturnal nasal intermittent positive pressure ventilation, plus long-term oxygen therapy results in more favourable survival and changes in blood gases and respiratory function than long-term oxygen therapy alone.

Life expectancy in pediatric patients with cerebral palsy and neuromuscular scoliosis who underwent spinal fusion.

The aim of this study was to document the rate of survival among 288 severely affected pediatric patients (154 females, 134 males) with spasticity and neuromuscular scoliosis who underwent spinal fusion (mean age at surgery 13 years 11 months, SD 3 years 4 months), and to identify exposure variables that could significantly predict survival times. Kaplan-Meier survival analysis was performed demonstrating a mean predicted survival of 11 years 2 months after spinal surgery for this group of globally involved children with cerebral palsy (CP). Cox's proportional hazards model was used to evaluate predictive efficacy of exposure variables, such as sex, age at surgery, level of ambulation, cognitive ability, degree of coronal and sagittal plane spinal deformity, intraoperative blood loss, surgical time, days in hospital, and days in the intensive care unit. Number of days in intensive care unit after surgery and the presence of severe preoperative thoracic hyperkyphosis were the only factors affecting survival rates. This demonstrated statistically significant predictability for decreased life expectancy after spinal fusion in children with CP.

Vertebral fractures and mortality in older women: a prospective study. Study of Osteoporotic Fractures Research Group.

BACKGROUND: Osteoporotic fractures, including clinically detected vertebral fractures, are associated with increased mortality. However, only one third of vertebral fractures are diagnosed. It is unknown whether vertebral fractures, whether clinically apparent or not, are associated with greater mortality. OBJECTIVES: To test the hypothesis that women with prevalent vertebral fractures have greater mortality than those without fractures and to describe causes of death associated with vertebral fractures. DESIGN: Prospective cohort study with mean follow-up of 8.3 years. SETTING: Four clinical centers in the United States. PARTICIPANTS: A total of 9575 women aged 65 years or older and enrolled in the Study of Osteoporotic Fractures. MEASUREMENTS: Vertebral fractures by radiographic morphometry; calcaneal bone mineral density; demographic, medical history, and lifestyle variables; blood pressure; and anthropometric measures. In a subset of 606 participants, thoracic curvature was measured during a second clinic visit. MAIN OUTCOME MEASURES: Hazard ratios for mortality and cause-specific mortality. RESULTS: At baseline, 1915 women (20.0%) were diagnosed as having vertebral fractures. Compared with women who did not have a vertebral fracture, women with 1 or more fractures had a 1.23-fold greater age-adjusted mortality rate (95% confidence interval, 1.10-1.37). Mortality rose with greater numbers of vertebral fractures, from 19 per 1000 woman-years in women with no fractures to 44 per 1000 woman-years in those with 5 or more fractures (P for trend, <.001). In particular, vertebral fractures were related to the risk of subsequent cancer (hazard ratio, 1.4;95% confidence interval, 1.1-1.7) and pulmonary death (hazard ratio, 2.1;95% confidence interval, 1.4-3.0). In the subset of women who underwent thoracic curvature measurements, severe kyphosis was also related to pulmonary deaths (hazard ratio, 2.6;95% confidence interval, 1.3-5.1). CONCLUSION: Women with radiographic evidence of vertebral fractures have an increased mortality rate, particularly from pulmonary disease and cancer.

The effects of five years of nocturnal cuirass-assisted ventilation in chest wall disease.

We investigated the long-term effectiveness of cuirass-assisted ventilation, and examined whether mortality and morbidity could have been predicted at the time of admittance. Twenty five patients were commenced on nocturnal cuirass-assisted ventilation between 1983 and 1985, 10 with scoliosis or kyphosis, 8 with a thoracoplasty and 7 with neuromuscular disease. Mean pretreatment vital capacity was 30% of predicted, and arterial carbon dioxide tension (Paco2) was 8.2 kPa (62 mmHg). Fifteen patients were alive 5 yrs later. Two had discontinued assisted ventilation, both dying soon afterwards, and three had been changed to intermittent positive pressure ventilation. Survival could not have been predicted from age, severity of disease, lung volumes or arterial blood gases at presentation. Paco2 in the survivors had risen from a mean of 6.1 kPa (46 mmHg) after one year to 6.8 kPa (52 mmHg) after 5 yrs (p < 0.05), but remained significantly less than at presentation. There were no significant change in arterial oxygen tension (Pao2), lung volumes, respiratory muscle strength, haemoglobin, right heart failure, exercise tolerance, mental function and symptom scores after 5 yrs, compared to after 1 yr. The median amount of time spent in hospital declined from 15 days per patient in the first year after initial discharge with cuirass-assisted ventilation, to between 3-5.5 days per patient in subsequent years. We conclude that nocturnal cuirass-assisted ventilation has a role in long-term management of patients with neuromuscular and skeletal chest wall disorders. A randomized comparison with nasal intermittent positive pressure ventilation is now indicated.

Respiratory prognosis in chest wall diseases.

In this short review focusing essentially on thoracic kyphoscoliosis, respiratory and cardiovascular abnormalities observed in different forms of this rib cage disorder are briefly described. Chest wall and pulmonary mechanics, respiratory muscles, gas exchange, pulmonary vasculature and regulation of ventilation can be markedly affected by kyphoscoliosis and a substantial growth of knowledge of these functional alterations has been allowed recently by new technological approaches. The pathophysiological process which leads to cor pulmonale and respiratory failure in some patients with kyphoscoliosis is analysed. The prognostic aspects of surgical and non-surgical management on pulmonary function are addressed.

Survival of patients with severe thoracic spine deformities receiving domiciliary oxygen therapy.

Scoliosis can lead to respiratory failure and premature death. Alveolar hypoventilation is a dominant cause and artificial ventilation at home (AVH) is probably the treatment of choice. It has been suggested that long-term domiciliary oxygen therapy (LTO) is of little value because of the worsening of hypercapnia. We analyzed survival and predictors of death among 80 patients with scoliosis and other severe thoracic spine deformities receiving LTO for chronic hypoxia. The survival rate was higher in patients under the age of 65 (p = 0.01) and in patients without concomitant pulmonary or airways disease. Likewise, the survival rate was higher in patients with a PaCO2 of greater than 7.4 kPa than in patients with a lesser degree of hypoventilation and hypercapnia (p less than 0.05). The risk of developing life-threatening hypercapnia during well-controlled LTO appeared to be small. In younger patients without complicating disease, long-term survival was achieved with LTO, but with time, an increasing proportion of the patients changed to AVH, with or without LTO.