Scheuermann's Disease.

Scheuermann's disease is a rigid progressive kyphosis occurring in late childhood to adolescence. It is the most common cause of sagittal imbalance and angular progressive kyphosis in adolescents associated with back pain. The exact etiology of the disease is unclear, but it is characterized by defective growth of the end plate that may result from excessive mechanical stress on a weakened end plate during spinal growth. Several other theories have been proposed, and it is thought to be a multifactorial disease occurring as a result of the interplay of multiple factors. The radiographic features consist of anterior vertebral body wedging, irregular end plates, Schmorl's nodes, and intervertebral disk degeneration. The natural history and evolution of this disease is also unknown. Conservative management with physiotherapy, rehabilitation, and bracing is the first line of treatment. Mechanical bracing helps prevent further progression of the kyphotic deformity. Surgery is mostly indicated in patients with failure of conservative management, with neurologic compromise, and for cosmetic reasons.

Selecting the Vertebra above Sagittal Stable Vertebra as the Distal Fusion Level in Scheuermann's Kyphosis: A Prospective Study with a Minimum of 2-Year Follow-Up.

OBJECTIVE: The proper selection of the lower instrumented vertebra (LIV) remains controversial in the surgical treatment of Scheuermann's disease and there is a paucity of studies investigating the clinical outcomes of fusion surgery when selecting the vertebra one level proximal to the sagittal stable vertebra (SSV-1) as LIV. The purpose of this study is to investigate whether SSV-1 could be a valid LIV for Scheuermann kyphosis (SK) patients with different curve patterns. METHODS: This was a prospective study on consecutive SK patients treated with posterior surgery between January 2018 and September 2020, in which the distal fusion level ended at SSV-1. The LIV was selected at SSV-1 only in patients with Risser >2 and with LIV translation less than 40 mm. All of the patients had a minimum of 2-year follow-up. Patients were further grouped based on the sagittal curve pattern as thoracic kyphosis (TK, n = 23) and thoracolumbar kyphosis (TLK, n = 13). Radiographic parameters including global kyphosis (GK), lumbar lordosis (LL), sagittal vertical axis (SVA), LIV translation, pelvic incidence (PI), pelvic tilt (PT), and sacral slope (SS) were measured preoperatively, postoperatively, and at the latest follow-up. The intraoperative and postoperative complications were recorded. The Scoliosis Research Society (SRS)-22 scores were performed to evaluate clinical outcomes. RESULTS: A total of 36 patients were recruited in this study, with 23 in the TK group and 13 in the TLK group. In TK group, the GK was significantly decreased from 80.8° ± 10.1° to 45.4° ± 7.7° after surgery, and was maintained at 45.3° ± 8.6° at the final follow-up. While in the TLK group, GK was significantly decreased from 70.7° ± 9.2° to 39.1° ± 5.4° after surgery (p < 0.001) and to 39.3° ± 4.5° at the final follow-up. Meanwhile, despite presenting with different sagittal alignment, significant improvement was observed in LL, SVA, and LIV translation for both TK and TLK groups (p < 0.05). Self-reported scores of pain and self-image in TK group and scores of self-image and function in TLK group showed significant improvement at the final follow-up (all p < 0.05). Distal junctional kyphosis (DJK) was observed in two patients (8.7%) in TK group, and one patient (7.7%) in TLK group. No revision surgery was performed. CONCLUSION: Selecting SSV-1 as LIV can achieve satisfactory radiographic and clinical outcomes for SK patients with different curve patterns without increasing the risk of DJK. This selection strategy could be a favorable option for SK patients with Risser sign >2 and LIV translation less than 40 mm.

In old men Scheuermann's disease is not associated with neck or back pain: a Swedish cohort study.

BACKGROUND AND PURPOSE: Scheuermann's disease is characterized by kyphosis and frequently mild back pain. As the level of kyphosis may progress over time, also the level of pain may increase. We evaluated the prevalence of Scheuermann's disease, and their pain, in Swedish elderly men. PATIENTS AND METHODS: The Osteoporotic Fractures in Men (MrOS) Study Sweden (n = 3,014) is a population-based prospective observational study of community-living men aged 69-81 years. At baseline, participants answered a questionnaire including history of neck/back pain during the preceding year and characteristics of any pain (severity, sciatica, and neurological deficits). Lateral thoracic/lumbar spine radiographs were taken of 1,453 men. We included the 1,417 men with readable radiographs. Scheuermann's disease was defined as 3 or more consecutive vertebrae with > 5° wedging with no other explanation for the deformity. RESULTS: 92 of the 1,417 men (6.5%, 95% confidence interval 5.3-7.9) had Scheuermann's disease. 31% of men with and 31% without Scheuermann's disease reported neck pain (P = 0.90) and 51% with and 55% without the disease reported back pain (P = 0.4). Among men with Scheuermann's disease and back pain, none reported severe pain, 57% moderate, and 43% mild, compared with 7%, 50%, and 44% in those without Scheuermann's disease (P = 0.2). In those with Scheuermann's disease 63% reported no sciatica, 15% sciatica without neurological deficits, and 22% sciatica with neurological deficits, compared with 56%, 16%, and 28% in those without the disease (P = 0.6). CONCLUSION: The prevalence of Scheuermann's disease in elderly Swedish men is between 5.3% and 7.9%. The condition seems at this age not to be associated with neck or back pain.

Issues With Big Data: Variability in Reported Demographics and Complications Associated With Posterior Spinal Fusion in Pediatric Patients.

BACKGROUND: Clinical and administrative registries provide large volumes of data that can be used for clinical research. However, there are several limitations relating to the quality, consistency, and generalizability of big data. In this study, we aim to compare reported demographics and certain outcomes in patients undergoing posterior spinal fusion (PSF) for adolescent idiopathic scoliosis (AIS), neuromuscular scoliosis (NS), and Scheuermann kyphosis (SK) between 3 commonly utilized databases in pediatric orthopaedic research. METHODS: We used International Classification of Diseases, Ninth Revision (ICD-9), International Classification of Diseases, 10th Revision (ICD-10), and Current Procedural Terminology (CPT) codes to identify patients in the National Surgical Quality Improvement Program (NSQIP), Healthcare Cost and Utilization Project (HCUP), and Pediatric Health Information System (PHIS) between the ages of 10 to 18 that underwent PSF for AIS, SK, and NS from 2012 to 2015. We compared various demographic factors, such as sex, race/ethnicity, age, and rates of postsurgical infection and 30-day readmissions. Data was analyzed with descriptive and univariate statistics. RESULTS: We identified 9891 patients that underwent PSF in NSQIP, 10,771 patients in PHIS, and 4335 patients in HCUP over the study period. There were significant differences in patient demographics, readmission rates, and infection rates between all patients that underwent PSF across the databases (P<0.01), as well as specifically in patients with AIS (P<0.01). HCUP had the highest proportion of Hispanic patients that underwent PSF (13.5%), as well as patients who had AIS (13.3%) or NS (17.9%). The PHIS database had the highest proportion of patients undergoing PSF for SK. Among patients with NS, there were significant differences in race across the databases (P<0.01), but no significant differences in sex, ethnicity, or readmission (P>0.05). In addition, there were significant differences in race (P=0.04) and readmission (P=0.01) across databases for patients with SK, but no differences in sex or ethnicity (P>0.05). NSQIP reported the highest rate of 30-day readmissions for patients undergoing PSF (17.9%) compared with other databases (HCUP 4.1%, PHIS 12.1%). CONCLUSIONS: There are significant differences in patient demographics, sample sizes, and rates of complications for pediatric patients undergoing PSF across 3 commonly utilized US administrative databases. Given the variability in reported outcomes and demographics, generalizability is difficult to extrapolate from these large data sources. In addition, certain databases should be selected to appropriately power studies focusing on particular patient populations or outcomes.

Complications Following Surgery for Adult Scheuermann's Kyphosis: A 2-Year Follow-Up in 22 Patients.

AIM: To determine the rate of postoperative complications following surgery for Scheuermann?s kyphosis (SK) and ascertain whether restoration to an ideal Roussouly spine type reduced the incidence of postoperative proximal junctional kyphosis (PJK). MATERIAL AND METHODS: We retrospectively screened all patients undergoing SK surgery at our institution (2010?2017) and excluded patients with less than two years of follow-up. Postoperative complications were identified as early or late and minor or major. Successful restoration of Roussouly spine type was assessed and patients were classified as ?restored? or ?non-restored.? Associations between ideal Roussouly restoration and postoperative PJK were evaluated using logistic regression analysis. RESULTS: The study included 22 patients with a median age of 23 (IQR, 20.0?43.8) years. Postoperative complications developed in 17 (77%) of these cases. All 17 patients developed minor complications; seven (32%) patients also exhibited major complications. PJK was diagnosed in 55% of the patients with an 18% overall two-year revision rate. Forty-four percent of the patients in the restored group developed PJK compared to 83% in the non-restored group (p=0.162). Multivariable logistic regression analysis revealed a trend towards an increased incidence of PJK in the non-restored group, albeit without statistical significance (OR, 9.4; 95% CI, 0.7?122.5, p=0.087). CONCLUSION: Our study revealed that 77% of patients undergoing surgery for SK developed at least one complication with a two-year revision rate of 18%. PJK was detected less frequently in patients who were restored to their ideal Roussouly spine type, although this finding did not achieve statistical significance.

Combined surgical correction of pectus carinatum and juvenile kyphosis.

Pectus carinatum may rarely be associated with kyphosis. However, the correlation between both conditions is not well reported. Therefore, there are no reports for combined correction of both deformities in the same patient. Moreover, studies estimating the kyphosis prevalence in patients with pectus carinatum are lacking. To our knowledge, this is the first paper to present such a case. We report an 18-year-old boy with both pectus carinatum and kyphosis that were surgically corrected in a combined procedure. The indication of surgery is cosmetic, and the postoperative recovery included pneumothorax but was otherwise uneventful and satisfactory.

Selection of the optimal distal fusion level for Scheuermann kyphosis with different curve patterns: when can we stop above the sagittal stable vertebra?

PURPOSE: To investigate the optimal lowest instrumented vertebra (LIV) in the treatment of Scheuermann kyphosis (SK) with different curve patterns. METHODS: Fifty-two SK patients who underwent posterior surgery between January 2010 and December 2017 with a minimum follow-up of 2 years were retrospectively reviewed. Patients were divided into two groups based on the curve pattern: the Scheuermann thoracic kyphosis (STK group) or Scheuermann thoracolumbar kyphosis (STLK group). Based on the relationship between the sagittal stable vertebra (SSV) and LIV, both groups were further divided into the SSV group and SSV-1 group. Radiographic parameters, distal junctional kyphosis (DJK) incidence and SRS-22 questionnaire scores were evaluated. RESULTS: In STK and STLK groups, there were no significant differences in most pre- and postoperative radiographic assessments between SSV and SSV-1 subgroups. DJK incidence showed no significant differences between groups during follow-up (P > 0.05). LIV-PSVL was significantly more negative in the SSV-1 group than that in the SSV group (P < 0.001). Within the SSV-1 group, patients with DJK showed a more negative LIV-PSVL (P = 0.039). Moderate correlation was observed between preoperative LIV-PSVL and DJK with a Spearman coefficient of - 0.474 (P = 0.035). Receiver operative characteristic curve analysis showed that the threshold value of preoperative LIV-PSVL to predict DJK was - 37.35 mm (area under the curve 0.882). CONCLUSION: Shorter fusion stopping at SSV-1 achieved comparable clinical outcomes and did not increase the risk of DJK for both STK and STLK patients. For patients whose preoperative LIV-PSVL < - 37.35 mm, extending fusion to SSV is an acceptable solution to prevent DJK.

Surgical Procedures Used for Correction of Scheuermann's Kyphosis: A Meta-Analysis.

OBJECTIVES: Scheuermann's kyphosis can cause severe back pain and cosmetic disorders to patients. Previous studies on surgical procedure selection for correction of Scheuermann's kyphosis have drawn controversial conclusions. Here, a meta-analysis was performed to figure out a better way between anterior-posterior (AP) combined procedures and posterior-only (PO) procedures. METHODS: We searched PubMed database and Ovid database, as well as Cochrane Library (between January 2009 and December 2020, around recent ten years), for studies reporting Scheuermann's kyphosis correction in an anterior way or a posterior way. Random effects meta-analysis regarding correction degrees and incidence of proximal junctional kyphosis (PJK) was performed. RESULTS: Finally, 13 unique studies including 586 patients (AP: 300; PO: 286) were identified and included for this meta-analysis. Overall, 6 AP cohorts and 10 PO cohorts were pooled regarding the correction degrees of kyphosis in the analysis, respectively. Pooled correction degrees in AP cohorts were 33.31 (95% CI: 27.48-39.15; I 2 = 86%, P < 0.001) and in PO cohorts were 31.16 (95% CI: 26.97-35.35; I 2 = 81.1%, P < 0.001). Comparison of correction between AP and PO cohorts did not indicate any significant difference. Likewise, postoperative PJK incidence showed no difference. Back pain can be caused by both AP and PO procedures, but which causes less pain remains to be conclusive. The PO approach showed less blood loss and shorter surgical duration as compared to the AP approach. CONCLUSIONS: In summary, this meta-analysis shows similar treatment effects between AP and PO procedures in correcting Scheuermann's kyphosis, suggesting the advantage of PO procedures due to less blood loss and surgical duration. However, the postoperative complications PJK and distal junctional kyphosis (DJK) cannot be well concluded due to the limitation of existing data.

Long-term follow-up of untreated Scheuermann's kyphosis.

STUDY DESIGN: Long-term cross-sectional study. OBJECTIVES: To investigate the long-term effects of untreated Scheuermann's kyphosis on quality of life, and its relationship to radiographic parameters of spinal deformity. Previous studies reported reduced self-image, increased pain and impaired physical status. Little is known of the long-term impact of sagittal plane deformity in untreated SK. METHODS: One hundred and thirteen consecutive untreated patients with SK were identified from a national service database prior to 2000, when surgery was not offered at this unit. 81 of these patients were available for evaluation; 66 (81%) consented to questionnaire and clinical evaluation, and 47 (58%) consented to additional radiological evaluation. Health-related quality of life (HRQoL) was compared to normative population values. Mean age was 45.1 years (31-65), and mean follow-up was 27 years (16-36). 57 patients had thoracic kyphosis and 9 had thoracolumbar deformity. RESULTS: SRS-22 and SF-36 scores were lower, and ODI was greater in patients with untreated SK compared to normative population values. Kyphosis progressed from mean 66° at skeletal maturity to 78° (p < 0.001) after mean follow-up of 27 years. Long-term progression of untreated SK was 0.45°/year (n = 47). Multilinear regression showed good correlation between increasing SVA and worse ODI scores (r = 0.59; p = 0.001). Increasing SVA also correlated with worse function, pain and mental health scores reported by SRS-22, and with worse physical function and bodily pain scores reported by SF-36. Increasing CL correlated with worse SF-36 physical function scores. Increasing cSVA and increasing TK correlated with worse SRS-22 self-image scores. CONCLUSION: SRS-22 and SF-36 scores were lower, and ODI was greater in patients with untreated SK compared to normative data. Long-term progression of untreated SK was 0.45°/year (n = 47). Increasing SVA correlated with worse SF-36 physical function, SRS-22 function, SRS-22 pain and higher ODI scores. Total kyphosis (TK) and cSVA were independent predictors of low SRS self-image. LEVEL OF EVIDENCE: III.

Relationship between Scheuermann disease and symptomatic thoracic spinal stenosis: A retrospective study.

OBJECTIVE: This study aimed to investigate the possible relationship between Scheuermann disease (SD) and the pathophysiological factors of thoracic spinal stenosis (TSS), including ossification of the ligamentum flavum (OLF), ossification of the posterior longitudinal ligament (OPLL), and thoracic disc herniation (TDH) in patients with symptomatic TSS. METHODS: Demographic and radiological data from 66 consecutive patients diagnosed with symptomatic TSS from 2013 to 2018 were retrospectively collected and divided into 3 groups depending on the underlying pathomechanism of TSS: TDH group (18 patients; 6 women; mean age ± standard deviation [Sd] = 59.89 ± 11.34), OPLL group (12 patients; 8 women; mean age ± Sd = 56.08 ± 14.74), and OLF group (36 patients; 20 women; mean age ± Sd = 58.69 ± 9.77). A total of 41 age-matched healthy individuals (19 women; mean age ± Sd = 54.88 ± 13.63) were designated as the control group. In each group, both typical and atypical SD criteria were radiologically examined. The demographic data and presence of SD between the control group and 3 subgroups of TSS pathomechanisms were evaluated. RESULTS: SD characteristics were identified in 83.33% (15/18) of patients in the TDH group, 44.44% (16/36) in the OLF group, 25% (3/12) in the OPLL group, and 17.07% (7/41) of the control individuals. When analyzed by the chi-squared test and logistic regression analysis, the presence of SD was significantly associated with TDH (P < 0.01) and OLF (P < 0.05) but not OPLL (P > 0.05). Patients with TDH and OLF showed peak involvement of T10/11, and patients with OPLL did not. Furthermore, we determined that age, sex, body-mass index, and smoking status were not the risk factors for TDH, OPLL, and OLF (P > 0.05). SD was found to be a risk factor for TDH (P < 0.01) and OLF (P < 0.05) but not for OPLL (P > 0.05). CONCLUSION: Evidence from this study indicated that SD might be a risk factor for OLF and TDH but not for OPLL.

Treatment approaches for Scheuermann kyphosis: a systematic review of historic and current management.

OBJECTIVE: Scheuermann kyphosis (SK) is an idiopathic kyphosis characterized by anterior wedging of ≥ 5° at 3 contiguous vertebrae managed with either nonoperative or operative treatment. Nonoperative treatment typically employs bracing, while operative treatment is performed with either a combined anterior-posterior fusion or posterior-only approach. Current evidence for these approaches has largely been derived from retrospective case series or focused reviews. Consequently, no consensus exists regarding optimal management strategies for patients afflicted with this condition. In this study, the authors systematically review the literature on SK with respect to indications for treatment, complications of treatment, differences in correction and loss of correction, and changes in treatment over time. METHODS: Using PubMed, Embase, CINAHL, Web of Science, and the Cochrane Library, all full-text publications on the operative and nonoperative treatment for SK in the peer-reviewed English-language literature between 1950 and 2017 were screened. Inclusion criteria involved fully published, peer-reviewed, retrospective or prospective studies of the primary medical literature. Studies were excluded if they did not provide clinical outcomes and statistics specific to SK, described fewer than 2 patients, or discussed results in nonhuman models. Variables extracted included treatment indications and methodology, maximum pretreatment kyphosis, immediate posttreatment kyphosis, kyphosis at last follow-up, year of treatment, and complications of treatment. RESULTS: Of 659 unique studies, 45 met our inclusion criteria, covering 1829 unique patients. Indications for intervention were pain, deformity, failure of nonoperative treatment, and neural impairment. Among operatively treated patients, the most common complications were hardware failure and proximal or distal junctional kyphosis. Combined anterior-posterior procedures were additionally associated with neural, pulmonary, and cardiovascular complications. Posterior-only approaches offered superior correction compared to combined anterior-posterior fusion; both groups provided greater correction than bracing. Loss of correction was similar across operative approaches, and all were superior to bracing. Cross-sectional analysis suggested that surgeons have shifted from anterior-posterior to posterior-only approaches over the past two decades. CONCLUSIONS: The data indicate that for patients with SK, surgery affords superior correction and maintenance of correction relative to bracing. Posterior-only fusion may provide greater correction and similar loss of correction compared to anterior-posterior approaches along with a smaller complication profile. This posterior-only approach has concomitantly gained popularity over the combined anterior-posterior approach in recent years.

What Are the Indications for Spinal Fusion Surgery in Scheuermann Kyphosis?

BACKGROUND: Surgical indications for Scheuermann kyphosis are variable. We sought to evaluate the characteristics of patients undergoing operative versus nonoperative treatment of Scheuermann kyphosis to better understand current practices and the factors which contribute to the decision for surgical management. METHODS: Multicenter prospective cohort study. We evaluated consecutive patients presenting with Scheuermann kyphosis. Patients underwent either surgical or nonoperative management according to surgeon and patient discretion. Preoperative patient-reported outcome measures (Scoliosis Research Society and Spinal Appearance Questionnaire scores), demographics, and radiographic characteristics were assessed. RESULTS: Overall, 150 patients with Scheuermann kyphosis were enrolled, with 77 choosing nonoperative treatment and 73 treated operatively. Compared with the nonoperative cohort, patients treated operatively were older (16.3±2.0 vs. 15.1±2.2, P=0.0004), and had higher body mass index (26.3±7.2 vs. 22.7±6.5, P=0.003), had greater T2-T12 kyphosis (71±14 degrees vs. 61±12 degrees, P<0.001), increased pelvic incidence (46 vs. 41 degrees, P=0.03) and pelvic tilt (10 vs. 3 degrees, P=0.03). There was no detected difference in maximal sagittal Cobb angle in the operative versus nonoperative patients (73±11 vs. 70±12 degrees, P=0.11). Functionally, the operative patients had worse Scoliosis Research Society pain scores (3.7±0.9 vs. 4.1±0.7, P=0.0027) and appearance scores (2.9±0.7 vs. 3.4±0.8, P <0.0001). CONCLUSIONS: Patients undergoing surgical management of Scheuermann disease were more likely to have large body mass index and worse pain scores. Other factors beyond radiographic measurement likely contribute to the decision for surgical management of Scheuermann kyphosis. LEVEL OF EVIDENCE: Level II.

Spinal deformity and malocclusion association is not supported by high-quality studies: results from a systematic review of the literature.

BACKGROUND: Several reports in the literature have suggested a causative association between oral occlusion and spinal deformity such as scoliosis and Scheuermann's disease or kyphosis. Based on these findings, a growing number of adolescents with spinal deformity receive orthodontic treatment, supposing a beneficial effect on the spine. OBJECTIVE: The aim of this study was to verify the association between spinal deformity and malocclusion in the orthopedic population and potential effect of orthodontic treatment on the spinal deformity. METHOD: The databases: MEDLINE, CINAHL, EMBASE, Cochrane Register, OTseeker and ScienceDirect were searched up to August 2017 for studies reporting on associations between spinal and occlusal conditions. Case series, cohort, case-control studies and randomized clinical trials were considered for analysis. Two reviewers independently selected studies, conducted quality assessment and extracted results. Methodological quality was assessed using MINORS score. RESULTS: Nine publications reporting on 1424 patients were included. Studies were two case series, five case-control studies, one cohort study and one randomized clinical trial. The methodological quality was poor in 8/9 studies. CONCLUSION: Evidence from three low-quality studies suggests an increased prevalence of occlusal dysfunction in patients with known spinal deformity, but the conclusions have a high risk of bias. No evidence of beneficial effects of orthodontic treatment on spinal deformity was found. These slides can be retrieved under Electronic Supplementary Material.

Long term prognosis of Scheuermann's disease: The association with fragility fracture - The MINOS cohort.

The aim was to assess the association of Scheuermann's disease (SCD) with fracture risk (vertebral, peripheral) and bone mineral density (BMD) in older men. SCD was assessed on the baseline lateral spine radiographs using the Berlin criteria in 766 men aged 50-85. We evaluated the association of SCD and its diagnostic criteria with incident fracture (vertebral over 7.5 years, peripheral over 10 years) and BMD (baseline). SCD prevalence was 25.2%. SCD and its criteria showed inconsistent associations with BMD at different skeletal sites. Eighty-four men had incident fractures. After adjustment for age, weight, spine BMD, prevalent vertebral fractures, prior falls and score of disc space narrowing due to osteoarthritis (DSN-OA), SCD was not associated with vertebral fracture risk. Vertebral endplate irregularities (EI), one of its diagnostic criteria, were associated with higher vertebral fracture risk (OR = 3.26, 95% CI: 1.34-7.94, p < 0.01). Vertebral fracture risk was higher in men with EI and low spine BMD vs. men without these characteristics (OR = 12.84, 95% CI: 3.12-52.83, p < 0.005). EI was associated with higher vertebral fracture risk in men without severe DSN-OA and without prevalent vertebral fractures. Peripheral fracture risk was lower in men with SCD (HR = 0.39, 95% CI: 0.18-0.83, p < 0.02) and EI. Peripheral fracture risk was higher in men without SCD who had low femoral neck BMD vs. men with SCD and normal BMD (HR = 4.68, 95% CI: 1.09-20.03, p < 0.05). In conclusion, EI were associated with high vertebral fracture risk. SCD and EI were associated with lower peripheral fracture risk. The associations of SCD and its criteria with BMD were inconsistent.

Single-stage deformity correction in children with neurogenic kyphoscoliosis: nuances and a proposal for risk stratification.

OBJECTIVE Neurogenic kyphoscoliosis in pediatric patients has varied causes and diverse management options. The most common management strategy is to stage the orthopedic and neurosurgical aspects of the correction at an interval of 3-6 months from each other. The aim of this study was to report the authors' experience with correction of deformity in children with associated neurological abnormalities requiring intervention as a single-stage surgical treatment. METHODS Of 591 pediatric patients with scoliosis treated at the Amrita Institute of Medical Sciences and Research Centre between January 2001 and January 2011, the authors identified all patients with neurogenic kyphoscoliosis who underwent deformity correction and a neurosurgical procedure in the same surgical session when they were younger than 15 years. Data regarding the demographic details, preoperative Cobb angle/kyphotic angle, surgical details, postoperative complications, cost of the procedure, and long-term neurological outcome were collected for each case and analyzed. Based on the results of this analysis, the authors propose a 2-category risk stratification system for the timing of deformity correction depending on the primary neurogenic etiology. RESULTS Ten cases involving patients with neurogenic kyphoscoliosis requiring both deformity correction and neurosurgical procedure met the criteria and were included in the analysis. All 10 patients were younger than 15 years; their mean age was 10.8 years. The average Cobb angle was 59.5°. Five patients underwent foramen magnum decompression, 5 patients underwent spinal cord detethering, and 1 patient had intraspinal intradural tumor excision. The mean estimated blood loss was 1177 ml (range 700-1550 ml), and the mean operative time was 4.4 hours (range 3-5.5 hours). There were no intraoperative complications. None of the patients had any new postoperative neurological deficits. The mean postoperative Cobb angle of the main thoracic curve was 12.6°. CONCLUSIONS Simultaneous deformity correction along with a definitive neurosurgical procedure in patients with neurogenic kyphoscoliosis can be performed without added morbidity and avoids both the added costs and the risk of complications associated with a second surgery. The risk stratification system proposed in this paper may serve as a guideline for case selection and help to optimize outcome.

Familial lumbar Scheuermann disease with idiopathic scoliosis in China: First case report.

RATIONALE: Given that Scheuermann disease rarely occurs in the lumbar region and that the co-occurrence of Scheuermann disease and idiopathic scoliosis (IS) has not been reported-the etiology of Scheuermann disease and IS is not clear. In this case report, we present familaiar lumbar Scheuermann disease with IS, in a Chinese proband, who was successfully treated with surgery. PATIENT CONCERNS: A 16-year-old boy presented at the Second XiangYa Hospital of Central South University with a chief complaint of kyphotic deformity in the lower back for 4 years and obvious lower back pain. In addition, he complained of limited lumbar activity. And The proband's family history was obtained by routine inquiring. In this Chinese family with 17 members over 3 generations. The 3 patients (proband, proband's sister and father) shared the characteristics of vertebral wedging from L1 to L3 and a kyphosis Cobb angle of 37°, 70°, or 73°, respectively. The main deformity of the proband's mother was at T7-L1 with a Cobb angle of 102° in the coronal plane at T7-L1, thoracic kyphosis of 73°, and lumbar lordosis of 62°. DIAGNOSES: Scheuermann's disease. INTERVENTIONS: Clinical history, physical examination, laboratory tests, and radiographs of those in the pedigree were recorded, and the related literature was reviewed. The proband accepted osteotomy and orthopedic surgery for treatment. OUTCOMES: After 3 months of treatment, postoperative lateral radiographs showed a significantly improved sagittal vertical axis (SVA). The other patients were continued to be seen in follow-up visits. LESSONS: This series of lumbar Scheuermann patients with IS in a pedigree support the genetic contribution to Scheuermann disease. Therefore, this study provides some insight into the genetic etiology of Scheuermann disease with IS.

Progression of untreated mild thoracic Scheuermann's kyphosis - Radiographic and functional assessment after mean follow-up of 46 years.

BACKGROUND: There is no data available on the radiographic development of the Scheuermann's deformity. Our purpose was to investigate radiographic deformity progression and the relation between kyphosis progression and clinical outcome in patients with untreated Scheuermann's kyphosis. METHODS: Thoracic kyphosis (Th4-Th12) was measured from standing lateral radiographs in 19 patients at baseline and after mean 46-year follow-up. Mean age at baseline was 19.2 and at follow-up 64.7 years. At follow-up, height, weight, hand grip strength, and hamstring tightness were measured, and sit-to-stand and walking tests were performed. Additionally general health and quality of life questionnaires were administered. RESULTS: The mean thoracic kyphosis increased from 46° (range 25°-78°) at baseline to 60° (34°-82°) (p < 0.001) at follow-up. Mean of the vertebrae wedge increased from 8.8° to 9.9° (p = 0.046). There was no correlation between extent of kyphosis progression and function at follow-up. CONCLUSIONS: Among patients with Scheuermann's disease the degree of radiographic deformity progressed slightly during long-term follow-up. Progression did not predict symptoms.

Spinal epidural lipomatosis - an easily ignored secondary intraspinal disorder in spinal kyphotic deformities.

BACKGROUND: A previous study reported a high prevalence of spinal epidural lipomatosis (SEL) in patients with Scheuermann kyphosis (SK) and suggested that it may play a role in the pathogenesis of this disease. According to our observation, however, SEL occurs in other spinal kyphotic deformities as well. The aim of this study was to test the hypothesis that SEL commonly occurs in patients with different types of kyphotic deformities as a secondary intraspinal disorder. METHODS: MR images of 16 patients with congenital kyphosis (CK), 40 patients with SK, 13 patients with tuberculotic kyphosis (TK), and 69 age- and sex-matched controls were retrospectively evaluated. The body mass index (BMI), kyphosis Cobb angle, and sagittal diameters of spinal epidural fat (EF) and the dural sac (DS) in the apical region (EFA, DSA) and non-kyphotic region (EFN, DSN) were measured. The EF ratios at the apical vertebral level (EFRA) and in the non-kyphotic region (EFRN) were calculated as EF / (EF + DS). RESULTS: EFA and EFRA were significantly higher among patients with CK, SK, and TK than among controls (P < 0.05). Seven CK patients (43.8%), 8 SK patients (20.0%), and 11 TK patients (84.6%) fulfilled the diagnostic criteria for SEL, while only 6.3, 2.5, and 0% of patients in the control groups did (P = 0.019, 0.014, and < 0.001, respectively). Spearman's correlation analysis showed statistically significant correlations between the kyphosis Cobb angle and the amount of EF in all three patient groups. CONCLUSIONS: SEL is a common secondary intraspinal disorder in different types of kyphotic deformities, and surgeons should pay increased attention to this intraspinal anomaly because excessive EF may compress the spinal cord and cause neurological deficits.

MRI Screening in Operative Scheuermann Kyphosis: Is it Necessary?

STUDY DESIGN: Patients with preoperative spine magnetic resonance imaging (MRI) studies from a prospective multicenter study of operative adolescent Scheuermann kyphosis (SK). OBJECTIVES: To investigate the usefulness of MRI screening in operative planning for SK surgeries. SUMMARY OF BACKGROUND DATA: Neural axis abnormalities in operative SK have not been previously studied with MRI screening, despite its use. METHODS: One orthopedic surgeon and two radiologists evaluated all images retrospectively. Radiographs were evaluated for kyphosis apex and magnitude. MRIs were evaluated for spinal cord abnormalities, epidural lipomatosis, location and number of vertebral wedging, Schmorl nodes and posterior disc herniations, frequency of spondylolysis, etc. The relationship of these pathologies to the kyphosis apex was explored. This group was compared to a surgical SK group without preoperative MRIs. RESULTS: Eighty-six patients with MRIs, mean age 16.3 years, 64% male, and a mean preoperative kyphosis of 75.9° were evaluated. There were 17 spinal cord abnormalities. Low-lying conus was found in 2 patients, and syrinx in 15 (no Chiari malformations). Epidural lipomatosis was found in 49 patients, average of 5.7 levels. Anterior vertebral wedging occurred in all (mean 4.7 levels). Posterior disc herniations averaged 5.2 levels/patient and 1.8 levels caudad to the apex. Spondylolysis was reported in 8.1%. Four cases (4.7%) had the operative plan changed as a result of the preoperative MRI: two due to neural compression, one due to disc herniation and one due to a spinal cord draped over the apex. Thirty-one patients did not receive an MRI; there were no significant differences between the two groups. The rate of postoperative neurologic change was 3.5% in the MRI group and 3.2% in the no-MRI group. CONCLUSIONS: Based on 4.7% of cases requiring a change in the operative plan as a result of preoperative MRI, the authors recommend considering performing screening MRI in operative SK patients.