Safety of Traditional Growing Rods in Patients with Early-Onset Congenital Scoliosis Associated with Type-I Split Cord Malformation.

BACKGROUND: Literature regarding the application of traditional growing rod (TGR) instrumentation in patients with early-onset congenital scoliosis with type-I split cord malformation is scarce. The purpose of the present study was to assess the safety and effectiveness of TGR surgery and repeated lengthening procedures in patients with congenital scoliosis with type-I split cord malformation not treated with prophylactic osseous spur excision. METHODS: Thirteen patients with early-onset congenital scoliosis associated with type-I split cord malformation and a stable neurologic status between March 2009 and July 2020 were recruited. All patients underwent primary TGR surgery and subsequent repeated lengthening procedures without osseous spur excision by the same surgical team. Clinical information and radiographic data from the preoperative, postoperative, and latest follow-up periods were collected. RESULTS: The mean preoperative Cobb angle of the major coronal curve was 74.62° ± 25.59°, the mean early postoperative angle was 40.23° ± 17.89°, and the mean latest follow-up angle was 40.62° ± 16.60°. The scoliotic deformity correction percentage was 46.81% ± 12.26% after the initial operation and 45.08% ± 15.53% at the latest follow-up. Compared with the preoperative values, significant improvements were observed in the coronal and sagittal balance early postoperatively and at the latest follow-up (p < 0.05 for all). The average annual amounts of spinal height gained were 15.73 ± 5.95 mm at T1-S1, 8.94 ± 3.94 mm at T1-T12, and 12.02 ± 6.70 mm between the instrumented segments. The total height gained at T1-S1 and T1-T12 was 72.18 ± 28.74 mm and 37.62 ± 12.53 mm, respectively. No intraoperative neurophysiological monitoring events were observed, and no case of neurological deficit was observed postoperatively or during follow-up. CONCLUSIONS: Patients without neurologic deficit and having a stable neurologic exam who have early-onset congenital scoliosis associated with type-I split cord malformation can safely and effectively undergo TGR surgery, followed by repeated lengthening procedures, without prophylactic osseous spur excision. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Profound hypoxemia and hypotension during posterior spinal fusion in a spinal muscular atrophy child with severe scoliosis: a case report.

BACKGROUND: Anesthesia for spinal muscular atrophy (SMA) patients undergoing spinal deformity surgery is challenging. We report an unusual case of an SMA girl who developed severe intraoperative hypoxemia and hypotension during posterior spinal fusion related with surgical positioning. CASE PRESENTATION: A 13-yr-old girl diagnosed with SMA type 2, severe kyphoscoliosis and thoracic deformity was scheduled for elective posterior spinal fusion. She developed severe hypoxemia and profound hypotension intraoperatively in the prone position with surgical table tilted 45° to the right. Though transesophageal echocardiography (TEE) could not be performed due to limited mouth opening, her preoperative computed tomography revealed a severely distorted thoracic cavity with much reduced volume of the right side. A reasonable explanation was when the surgeons performed surgical procedure with the tilted surgical table, the pressure was directly put on the shortest diameter of the significantly deformed thoracic cavity, causing severe compression of the pulmonary artery, resulting in both hypoxemia and hypotension. The patient stabilized when the surgical table was tilted back and successfully went through the surgery in the leveled prone position. CONCLUSIONS: Spinal fusion surgery is beneficial for SMA patients in preventing scoliosis progression and improving ventilation. However, severe scoliosis and thoracic deformities put them at risk of both hemodynamic and respiratory instability during surgical positioning. When advanced monitoring like TEE is not practical intraoperatively, preoperative imaging may help with differential diagnosis, and guide the surgical positioning to minimize mechanical compression of the thoracic cavity, thereby helping the patient complete the surgery safely.

A Novel Classification of Juvenile and Adolescent Idiopathic Scoliosis for Conservative Treatment.

BACKGROUND: The operative classification of scoliosis is well-developed but inadequate for guiding conservative treatment. The current conservative classification for juvenile and adolescent idiopathic scoliosis (JAIS) exhibits noticeable deficiencies. This study aimed to establish the Peking Union Medical College Hospital (PUMCH) classification and assess its clinical value in the conservative treatment of JAIS. METHODS: This study consisted of 2 parts. First, it involved a retrospective analysis of patients treated for JAIS in the Department of Rehabilitation Medicine, the ∗∗∗ Union Medical College Hospital, between January 2013 and June 2020. Second, it involved an ambispective cohort study that enrolled patients with JAIS in the above hospital between July and December 2020. RESULTS: A total of 989 patients with JAIS were enrolled, with 899 patients for establishing the PUMCH classification and 90 patients with JAIS for validating the PUMCH classification. The classification demonstrated an average reliability of 88.22% with a kappa coefficient of 0.862. After 1 week, the remeasured results presented a mean reproducibility of 92.78% and a kappa coefficient of 0.908. After 1-year follow-up, the Cobb angle decreased significantly from 16.61 ± 2.88° to 12.16°± 9.97° (P = 0.002) in 51 patients with PUMCH-scoliosis-specific exercise (SSE) treatment, while the Cobb angle increased significantly from 15.74 ± 2.75° to 17.64 ± 5.60° (P = 0.014) in 39 patients without PUMCH-SSE treatment. CONCLUSIONS: The PUMCH-SSE classification demonstrates good inter-observer reliability and intra-observer reproducibility. In addition, the classification may be used to guide the conservative treatment of JAIS in clinical settings.

Comparative analysis of paraspinal muscle imbalance between idiopathic scoliosis and congenital scoliosis from the transcriptome aspect.

Background: Previous studies have analyzed paraspinal muscle imbalance in idiopathic scoliosis (IS) with methods including imaging, histology and electromyography. However, whether paraspinal muscle imbalance is the cause or the consequence of spinal deformities in IS remains unclear. Comparison of paraspinal muscle imbalance between IS and congenital scoliosis (CS) may shed some light on the causality of paraspinal muscle imbalance and IS. This study aimed to elucidate the generality and individuality of paraspinal muscle imbalance between IS and CS from gene expression. Methods: Five pairs of surgical-treated IS and CS patients were matched. Bilateral paraspinal muscles at the apex were collected for transcriptome sequencing. Differentially expressed genes (DEGs) between the convexity and concavity in both IS and CS were identified. Comparison of DEGs between IS and CS was conducted to discriminate IS-specific DEGs from DEGs shared by both IS and CS. Bioinformatics analysis was performed. The top 10 hub genes in the protein-protein interaction (PPI) network of IS-specific DEGs were validated by quantitative PCR (qPCR) in 10 pairs of IS and CS patients. Results: A total of 370 DEGs were identified in IS, whereas 380 DEGs were identified in CS. Comparison of DEGs between IS and CS identified 59 DEGs shared by IS and CS, along with 311 DEGs specific for IS. These IS-specific DEGs were enriched in response to external stimulus and signaling receptor binding in GO terms and calcium signaling pathway in KEGG pathways. The top 10 hub genes in the PPI network of IS-specific DEGs include BDKRB1, PRH1-TAS2R14, CNR2, NPY4R, HTR1E, CXCL3, ICAM1, ALB, ADIPOQ, and GCGR. Among these hub genes, the asymmetrical expression of PRH1-TAS2R14 and ADIPOQ in IS but not CS were validated by qPCR. Conclusions: Transcriptomic differences in bilateral paraspinal muscles between the convexity and concavity in IS share few similarities with those in CS.

Morphological analysis of isolated hemivertebra: radiographic manifestations related to the severity of congenital scoliosis.

PURPOSE: The natural history of congenital scoliosis (CS) caused by hemivertebra varies greatly. This study aimed to explore the association between the morphology of hemivertebra and the severity of CS, since the diagnosis of the hemivertebra. METHODS: Patients with isolated (single fully segmented) hemivertebra were enrolled. The degree and progression of deformity were compared by three morphological parameters of hemivertebra, comprising whether the width of hemivertebra extends across the central vertical line of lower adjacent vertebra (midline); the lateral height ratio (LHR, lateral height of hemivertebra× 2/(lateral height of HV-1 plus HV + 1) with the cut-point being 0.9; and the sagittal position of hemivertebra that was divided into the lateral and posterolateral group. RESULTS: In total, 156 patients (mean age 9.7 ± 6.2 years, 81 males) were enrolled. The number of thoracic, thoracolumbar (T12/13-L1), and lumbar hemivertebrae were 63, 41, and 52, respectively. Hemivertebrae across the midline had larger scoliosis and kyphosis (58.3 ± 20.6° vs. 42.8 ± 15.0°, P <  0.001; 45.1 ± 32.5° vs. 29.5 ± 25.7°, P = 0.013, respectively). Hemivertebrae with LHR ≥0.9 was associated with larger scoliosis (55.7 ± 20.6° vs. 41.4 ± 13.3°, P <  0.001). Larger scoliosis and kyphosis were observed in posterolateral hemivertebrae (54.4 ± 21.0° vs. 44.4 ± 15.6°, P = 0.026; 51.4 ± 31.5° vs. 20.6 ± 17.1°, P <  0.001, respectively). Co-occurrence of more than one of the three positive parameters above indicated higher annual progression (5.0 ± 2.2° vs. 3.3 ± 1.3°, P <  0.001). CONCLUSION: Three positive parameters, width across the midline, LHR ≥0.9, and posterolateral position were associated with a more severe deformity in patients with isolated hemivertebra. Hemivertebrae with more than one positive parameter may cause progressive deformity, and thus need prompt surgery. LEVEL OF EVIDENCE: Prognostic, level IV.

Effects of ferric derisomaltose on postoperative anaemia in adult spinal deformity surgery: a study protocol for a randomised controlled trial.

INTRODUCTION: Postoperative anaemia is prevalent in adult spinal deformity (ASD) surgery in association with unfavourable outcomes. Ferric derisomaltose, a novel iron supplement, offers a promising solution in rapidly treating postoperative anaemia. However, the clinical evidence of its effect on patients receiving spinal surgery remains inadequate. This randomised controlled trial aims to evaluate the safety and efficacy of ferric derisomaltose on postoperative anaemia in ASD patients. METHODS AND ANALYSIS: This single-centre, phase 4, randomised controlled trial will be conducted at Department of Orthopaedics at Peking Union Medical College Hospital and aims to recruit adult patients who received ASD surgery with postoperative anaemia. Eligible participants will be randomly assigned to receive ferric derisomaltose infusion or oral ferrous succinate. The primary outcome is the change in haemoglobin concentrations from postoperative days 1-14. Secondary outcomes include changes in iron parameters, reticulocyte parameters, postoperative complications, allogeneic red blood cell infusion rates, length of hospital stay, functional assessment and quality-of-life evaluation. ETHICS AND DISSEMINATION: This study has been approved by the Research Ethics Committee of Peking Union Medical College Hospital and registered at ClinicalTrials.gov. Informed consent will be obtained from all participants prior to enrolment and the study will be conducted in accordance with the principles of the Declaration of Helsinki. The results of this study are expected to be disseminated through peer-reviewed journals and academic conferences. TRIAL REGISTRATION NUMBER: NCT05714007.

Dual Growing Rods and the Apical Control Technique for Treating Congenital Early-Onset Scoliosis: Lessons Learned.

BACKGROUND: Congenital early-onset scoliosis (CEOS) is characterized by a spectrum of vertebral anomalies, including formation failures and segmentation failures at the apex segment, which makes CEOS different from other etiologies of early-onset scoliosis. To date, studies on patients who have graduated from CEOS treatment using traditional dual growing rods (TDGR) have been scarce, and the preliminary results of TDGR with or without the apical control technique (ACT) have varied. We therefore compared the final outcomes of patients with CEOS who graduated from TDGR with or without the ACT. METHODS: A retrospective study of patients with CEOS who had graduated from TDGR treatment performed from 2007 to 2020 was conducted. Graduation included final fusion or observation after reaching skeletal maturity. Patients were divided into the ACT-TDGR group (apical vertebrectomy and/or hemivertebrectomy with short fusion and TDGR) and the TDGR-only group. Demographic characteristics, radiographic data, patient-reported clinical outcomes, pulmonary function, and complications were analyzed. RESULTS: A total of 41 patients with CEOS were enrolled: 13 in the ACT-TDGR group and 28 in the TDGR-only group. The lengthening intervals were longer in the ACT-TDGR group (mean [and standard deviation], 1.26 ± 0.66 years) than in the TDGR-only group (0.80 ± 0.27 years). The preoperative main curve was larger in the ACT-TDGR group (80.53° ± 19.50°) than in the TDGR-only group (64.11° ± 17.50°). The residual curve was comparable between groups (26.31° ± 12.82° in the ACT-TDGR group compared with 27.76° ± 15.0° in the TDGR group) at the latest follow-up. The changes in apical vertebral rotation and thoracic rotation were significantly larger in the ACT-TDGR group. Patients had comparable T1-12 and T1-S1 heights, pulmonary function, and 22-item Scoliosis Research Society (SRS-22) scores at the latest follow-up. The mean number of mechanical-related complications per patient was lower in the ACT-TDGR group (0.77 ± 0.73) than in the TDGR-only group (1.54 ± 1.43). Seventeen patients underwent final fusion. CONCLUSIONS: In this small-scale study, we observed that both ACT-TDGR and TDGR-only could correct the deformity while allowing for spinal growth in patients with CEOS. ACT-TDGR yielded better correction in severe cases and did not have a deleterious effect on spinal height. A large number of cases will be needed to validate the clinical value of the ACT. LEVEL OF EVIDENCE: Therapeutic Level III . See Instructions for Authors for a complete description of levels of evidence.

Surgical treatment of atlantoaxial dysplasia and scoliosis in spondyloepiphyseal dysplasia congenita: A case report.

BACKGROUND: Spondyloepiphyseal dysplasia congenita (SEDC) is a rare autosomal dominant hereditary disease caused by COL2A1 mutations. SEDC primarily involves the skeletal system, with typical clinical manifestations, including short stature, hip dysplasia, and spinal deformity. Due to the low incidence of SEDC, there are only a few case reports regarding the surgical treatment of SEDC complicated with spinal deformities. CASE SUMMARY: We report a case of a 16-year-old male patient with SEDC. He presented with typical short stature, atlantoaxial dysplasia, scoliosis, and hip dysplasia. Cervical magnetic resonance imaging showed spinal canal stenosis at the atlas level and cervical spinal cord compression with myelopathy. The scoliosis was a right thoracic curve with a Cobb angle of 65°. He underwent atlantoaxial reduction, decompression, and internal fixation from C1-C2 to relieve cervical myelopathy. Three months after cervical surgery, posterior correction surgery for scoliosis was performed from T3 to L4. Scoliosis was corrected from 66° to 8° and remained stable at 2-year follow-up. CONCLUSION: This is the first case report of a patient with SEDC who successfully underwent surgery for atlantoaxial dysplasia and scoliosis. The study provides an important reference for the surgical treatment of SEDC complicated with spinal deformities.

Adolescent Idiopathic Scoliosis: A Cross-Sectional Study.

Objective: Adolescent idiopathic scoliosis (AIS) can lead to right heart dysfunction. Inferior vena cava (IVC) diameter is an essential parameter of the right-heart function, but the relationship between scoliosis and the IVC diameter has not been established. This study aimed to investigate the correlation between the IVC diameter and the severity of AIS. Methods: This retrospective study examined the data from patients admitted to Peking Union Medical College Hospital between January 2000 and June 2014 with a diagnosis of AIS. A preoperative spine full-length standing X-ray was collected. The IVC diameter was measured during the expiration phase as a routine examination. Results: Among the 339 included patients, 269 had thoracic curves, and 311 had at least a thoracic or thoracolumbar curve. Significant correlations between the diameter of IVC and the coronal Cobb angle of main thoracic curves (r=0.142, P = .015) and thoracolumbar curves (r=0.580, P = .015) were observed. Regarding the possible confounding factors, the IVC diameter was also significantly correlated to body weight (r=0.327, P < .001) and body height (r=0.134, P = .014). In the partial correlation analysis correcting for body weight and height, the IVC inner diameter was correlated to the Cobb angle of the thoracic curve (r=0.172, P = .003) and the larger curve of either the thoracic or thoracolumbar curve (r=0.190, P = .001). Discussion: The result of this study implies that even in patients with mild scoliosis, there might already be underlying right heart function changes. Heart function should be taken into more consideration for AIS paitents. For patients with higher IVC diameters, a more throught cardiology assessment should be performed. Conclusion: The IVC diameter is positively correlated with the Cobb angle of thoracic curves or the larger one of thoracic or thoracolumbar curves in patients with AIS.

Unilateral interlaminar fenestration on the convex side provides a reliable access for intrathecal administration of nusinersen in spinal muscular atrophy: a retrospective study.

BACKGROUND: As the first gene therapy for spinal muscular atrophy (SMA), nusinersen is supposed to be administrated via intrathecal injection regularly for a lifetime. However, for SMA patients with severe spinal deformities, bony fusion following posterior spinal instrumentation sets great obstacles for the application of nusinersen. Therefore, efforts have been devoted to the exploration of appropriate approach for nusinersen administration. This study aims to evaluate the safety and reliability of unilateral interlaminar fenestration on the convex side during spinal fusion surgery for intrathecal nusinersen injection in SMA. RESULTS: SMA patients receiving posterior spinal fusion and interlaminar fenestration in Peking Union Medical College Hospital from January 2020 to October 2021 were retrospectively analyzed. 13 patients were included. Of the 13 patients, 10 were classified into SMA type II and 3 into SMA type III. Distal fusion to pelvis was undertaken in 11 patients; while L5 was selected as the lowest instrumented vertebra in the other 2 patients. All patients received interlaminar fenestration on the convex side only with an area of about 15 mm × 20 mm. Fenestration at L2-L3 level was performed in 6 patients; while L3-L4 level was selected for windowing in the remaining 7 patients. 9 of the 13 patients received lumbar puncture and intrathecal nusinersen administration during the 1-year follow-up, with an accumulative total of 50 times. All injections were performed successfully under ultrasound guidance, with no one transferred to radiographic assistance. No severe complications occurred after injection. CONCLUSIONS: In SMA with severe scoliosis planning to receive posterior spinal fusion, unilateral lumbar interlaminar fenestration on the convex side provides a feasible and reliable access for intrathecal nusinersen administration after surgery.

A large pedigree study confirmed the CGG repeat expansion of RILPL1 Is associated with oculopharyngodistal myopathy.

BACKGROUND: Oculopharyngodistal myopathy (OPDM) is an autosomal dominant adult-onset degenerative muscle disorder characterized by ptosis, ophthalmoplegia and weakness of the facial, pharyngeal and limb muscles. Trinucleotide repeat expansions in non-coding regions of LRP12, G1PC1, NOTCH2NLC and RILPL1 were reported to be the etiologies for OPDM. RESULTS: In this study, we performed long-read whole-genome sequencing in a large five-generation family of 156 individuals, including 21 patients diagnosed with typical OPDM. We identified CGG repeat expansions in 5'UTR of RILPL1 gene in all patients we tested while no CGG expansion in unaffected family members. Repeat-primed PCR and fluorescence amplicon length analysis PCR were further confirmed the segregation of CGG expansions in other family members and 1000 normal Chinese controls. Methylation analysis indicated that methylation levels of the RILPL1 gene were unaltered in OPDM patients, which was consistent with previous studies. Our findings provide evidence that RILPL1 is associated OPDM in this large pedigree. CONCLUSIONS: Our results identified RILPL1 is the associated the disease in this large pedigree.

Association between Adiposity and Bone Mineral Density in Adults: Insights from a National Survey Analysis.

Adiposity and bone mineral density (BMD) are closely associated. The aim of this research was to investigate the association between BMD and adiposity measures in adults, including gynoid percent fat (GPF), android percent fat (APF), total percent fat (TPF), visceral adipose tissue percent (VAT%), and total lean mass percent (TLM%). Participants (n = 11,615) aged 18 years and older were analyzed using data from the National Health and Nutrition Examination Survey (NHANES) spanning from 1999 to 2018. Associations between BMD and adiposity measures were investigated, and potential differences based on gender and age were explored. Significant negative associations were observed among TPF, APF, GPF, VAT%, and BMD in the fully adjusted models, while TLM% and BMD were positively associated. Stratifying by age and sex, TPF, GPF, and VAT% consistently demonstrated a negative correlation with BMD. In the young adult group, a TPF of 38.2% eliminated the negative correlation between BMD and TPF. Male BMD exhibited an inverted U-shaped relationship with APF, peaking at 35.6%, while a similar pattern was observed for the middle-aged group BMD and APF, with a peak at 31.7%. This large-sample research found a significant negative association between adiposity measures and BMD, providing valuable revelations regarding the intricate connection between adiposity and bone health.

Proteomic Comparison of Paraspinal Muscle Imbalance Between Idiopathic Scoliosis and Congenital Scoliosis.

OBJECTIVE: This study aims to compare the proteomic profiles of paraspinal muscle imbalance between idiopathic scoliosis (IS) and congenital scoliosis (CS). METHODS: Bilateral paraspinal muscles of 5 pairs of matched IS and CS patients were collected. Proteome patterns of paraspinal muscles were established. Differentially expressed proteins (DEPs) in paraspinal muscles between the convexity and the concavity were screened out. DEPs shared by both IS and CS and IS-specific DEPs were identified. Bioinformatic analyses of DEPs were performed. RESULTS: Among 105 DEPs identified in IS, 30 displayed predominant expression on the convexity, whereas other 75 exhibited predominant expression on the concavity. DEPs in IS were mainly enriched in calcium ion binding and DNA binding in gene ontology (GO) term and glycolysis/gluconeogenesis and purine metabolism in Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway. Among 48 DEPs identified in CS, 25 were predominantly expressed on the convexity and 23 on the concavity. DEPs in CS were mainly enriched in receptor activity and immune response in GO term and glycolysis/gluconeogenesis and cellular senescence in KEGG pathway. Comparison of DEPs between IS and CS identified only 8 proteins shared by both types of scoliosis. Among the 97 IS-specific DEPs, 28 were predominantly expressed on the convexity and 69 on the concavity. IS-specific genes were enriched in calcium ion binding and protein glycosylation in GO term and glycolysis/gluconeogenesis and hypertrophic cardiomyopathy in KEGG pathway. CONCLUSION: IS and CS exhibit proteomic imbalance in bilateral paraspinal muscles but share few similarities. Paraspinal muscle imbalance in IS might not be the consequence of spinal deformities.

How do orphan disease patients live during the pandemic of Omicron variant? A nationwide survey of spinal muscular atrophy patients in China.

OBJECTIVES: To investigate the challenges of patients with spinal muscular atrophy (SMA) during the Omicron variant COVID-19 pandemic. DESIGN: A cross-sectional survey was conducted in China from January 02, 2023, to January 12, 2023, using a questionnaire that covered three aspects: (1) Demographic information; (2) SMA-related information; and (3) COVID-19-related information. We recruited patients with SMA from 33 provinces. The prevalence, course, and clinical manifestations of COVID-19 were calculated. The relationships between independent and outcome variables were investigated. RESULTS: In total, 677 patients (male: 349; female: 328) were included in this study (average age = 11.40 years); 534 (78.88%) suffered from COVID-19. The most common symptoms were fever (95.51%), cough (57.87%), and sputum (49.44%). Of the infected patients, 91.57% recovered with at-home care, and 8.43% were hospitalized; 1.31% were admitted to the intensive care unit (ICU). A positive correlation was observed between the SMA severity and hospitalization rate. The ICU stay rate in patients with SMA type I was significantly higher than that in other SMA types. CONCLUSION: This is the first large sample survey to timely reveal the living situation of patients with SMA during the COVID-19 pandemic's Omicron variant. Patients with SMA type I should be paid more attention in terms of hospitalization and ICU stay.

Ten-year trends in surgical management of 1207 congenital scoliosis.

PURPOSE: To report and analyze development trends in the surgical treatment of congenital scoliosis (CS) in a large CS cohort over a 10-year period. METHODS: We retrospectively searched and extracted medical records of CS inpatients receiving posterior instrumented fusion surgery at our institute from January 2010 to December 2019. We analyzed information on demographics and surgical information, including the surgical approach, number of fused segments, use of osteotomy and titanium cage implantation, length of stay, intraoperative blood loss, and rates of complications and readmission. RESULTS: 1207 CS inpatients were included. In the past decade, the proportion of patients younger than 5 years increased from 15.5 to 26.9%. The average number of fused segments decreased from 9.24 to 7.48, and the proportion of patients treated with short-segment fusion increased from 13.4 to 30.3%. The proportion of patients treated with osteotomy and titanium cage implantation increased from 55.65% and 12.03% to 76.5% and 40.22%. The average length of stay and blood loss decreased from 16.5 days and 816.1 ml to 13.5 days and 501.7 ml. The complication and readmission rates also decreased during these ten years. CONCLUSION: During this ten-year period, the surgical treatment of CS at our institute showed trends toward a younger age at fusion, lower number of fused segments, higher rate of osteotomy and titanium cage implantation, reduced blood loss, shorter length of stay and lower rate complications and readmission. These results suggest performing osteotomy combined with titanium cage implantation at an earlier age can achieve fewer fused segments and complications.

Retrospective Analysis of Associated Anomalies in 636 Patients with Operatively Treated Congenital Scoliosis.

BACKGROUND: Congenital scoliosis is frequently associated with anomalies in multiple organ systems. However, the prevalence and distribution of associated anomalies remain unclear, and there is a large amount of variation in data among different studies. METHODS: Six hundred and thirty-six Chinese patients who had undergone scoliosis correction surgery at Peking Union Medical College Hospital from January 2012 to July 2019 were recruited, as a part of the Deciphering disorders Involving Scoliosis and COmorbidities (DISCO) study. The medical data for each subject were collected and analyzed. RESULTS: The mean age (and standard deviation) at the time of presentation for scoliosis was 6.4 ± 6.3 years, and the mean Cobb angle of the major curve was 60.8° ± 26.5°. Intraspinal abnormalities were found in 186 (30.3%) of 614 patients, with diastematomyelia being the most common anomaly (59.1%; 110 of 186). The prevalence of intraspinal abnormalities was remarkably higher in patients with failure of segmentation and mixed deformities than in patients with failure of formation (p < 0.001). Patients with intraspinal anomalies showed more severe deformities, including larger Cobb angles of the major curve (p < 0.001). We also demonstrated that cardiac anomalies were associated with remarkably worse pulmonary function, i.e., lower forced expiratory volume in the first second (FEV1), forced vital capacity (FVC), and peak expiratory flow (PEF). Additionally, we identified associations among different concomitant malformations. We found that patients with musculoskeletal anomalies of types other than intraspinal and maxillofacial were 9.2 times more likely to have additional maxillofacial anomalies. CONCLUSIONS: In our cohort, comorbidities associated with congenital scoliosis occurred at a rate of 55%. To our knowledge, our study is the first to show that patients with congenital scoliosis and cardiac anomalies have reduced pulmonary function, as demonstrated by lower FEV1, FVC, and PEF. Moreover, the potential associations among concomitant anomalies revealed the importance of a comprehensive preoperative evaluation scheme. LEVEL OF EVIDENCE: Diagnostic Level III. See Instructions for Authors for a complete description of levels of evidence.

Does Progesterone Affect Perioperative Blood Loss during Posterior Spinal Fusion Surgeries in Female Patients with Adolescent Idiopathic Scoliosis? A Retrospective Study.

OBJECTIVE: Menstruation is considered a contraindication for elective surgery for higher operative blood loss. Progesterone is often used to postpone menstruation to avoid surgery during menstruation. This study aimed to explore whether using progesterone to postpone menstruation affects perioperative blood loss and complications in female patients with adolescent idiopathic scoliosis (AIS) who underwent posterior spinal fusion (PSF) surgery. METHODS: A retrospective study was performed for female patients diagnosed with AIS who underwent PSF surgery between March 2013 and January 2021. Patients scheduled to undergo PSF surgery from 2 days before menstruation to 3 days after menstruation were treated with progesterone preoperatively. The patients were divided into two groups according to progesterone use (progesterone injection group; control group). Demographic and surgical data including intraoperative blood loss (IBL), normalized blood loss (NBL), total blood loss (TBL), transfusion rate, perioperative complications, postoperative drainage time, postoperative hospital stay, and preoperative coagulation function data were collected. RESULTS: A total of 206 patients were included in the study. Among them, the progesterone injection group included 41 patients, with an average age of 14.8 years. While the control group included 165 patients, with an average age of 14.9 years. The two groups were matched for age, height, weight, operation time, Risser sign, correction rate, mean curve Cobb angle, bending Cobb angle, number of internal fixations, and number of fused levels (all P > 0.05). Regarding coagulation function, no significant differences were found in thrombin time, activated partial thromboplastin time, fibrinogen, prothrombin time, and platelet count between the two groups (all P > 0.05). IBL, NBL, and TBL were higher in progesterone injection group; however, the difference was nonsignificant (all P > 0.05). Transfusion rate, perioperative complications, postoperative drainage time, and postoperative hospital stay were not statistically different between groups (all P > 0.05). CONCLUSION: Intramuscular injection of progesterone to avoid menstruation during PSF surgery did not affect perioperative blood loss and complications in AIS patients. It may be a safe method for AIS patients to avoid menstrual problems affecting the operation time and receive PSF surgery as scheduled.

Dual Growing Rods Combined With the Apical Convex Control Pedicle Screw Technique Versus Traditional Dual Growing Rods for the Surgical Treatment of Early-Onset Scoliosis: A Case-Matched 2-Year Study.

BACKGROUND: Combination of apical control convex pedicle screws (ACPS) with traditional dual growing rods (TDGRs) had better apex control ability for early-onset scoliosis (EOS); however, there is a paucity of studies on the ACPS technique. OBJECTIVE: To compare 3-dimensional deformity correction parameters and complications between the apical control technique (DGR + ACPS) and TDGR in the treatment of EOS. METHODS: A retrospective case-match analysis consisting of 12 cases of EOS treated with the DGR + ACPS technique (group A) from 2010 to 2020, and matched with TDGR case (group B) at a ratio of 1:1 by age, sex, curve type, major curve degree, and apical vertebral translation (AVT). Clinical assessment and radiological parameters were measured and compared. RESULTS: Demographic characteristics, preoperative main curve, and AVT were comparable between groups. The correction ability of the main curve, AVT, and apex vertebral rotation were better in group A at index surgery ( P < .05). The increase in T1-S1 and T1-T12 height was large in group A at index surgery ( P = .011, P = .074). The annual increase in spinal height was slower in group A, but without significant difference. The surgical time and estimated blood loss were comparable. Six complications occurred in group A, and 10 occurred in group B. CONCLUSION: In this preliminary study, ACPS seems to provide better correction of apex deformity, while attaining the comparable spinal height at 2-year follow-up. Larger cases and longer follow-up are needed to achieve reproducible and optimal results.

Evaluation of Adjacent Segment With Pre-Existing Degeneration Using the Cerebrospinal Fluid Occlusion Sign on MRI Before Posterior Lumbar Fusion: A Multi-Center Observational Cohort Study.

STUDY DESIGN: Prospective cohort study. OBJECTIVE: To evaluate whether pre-existing adjacent spinal canal stenosis (SCS) is associated with short-term outcomes after lumbar fusion surgery. METHODS: We included patients with lumbar spinal stenosis treated surgically between July 2015 and December 2017 at 4 centers. All patients had the same pathology, with L4-S1 as the culprit sections. Patients were divided into 2 groups based on the cerebrospinal fluid occlusion sign on MRI at the adjacent L3/4 level. Patients without SCS (grade 0) and with mild SCS (grade 1) were classified into the non-stenosis (NS) and mild stenosis (MS) groups, respectively. All patients underwent PLIF and completed at least 1-year follow-up. The incidence of adjacent segment degeneration (ASDeg) and clinical outcomes were compared between the 2 groups. RESULTS: A total of 308 patients (NS, 156; MS, 152) met the inclusion criteria. The incidence of ASDeg in the NS group (n = 40, 25.6%) was significantly lower than that in the MS group (n = 74, 48.7%; P < .001). The most frequent type of ASDeg in the 2 groups was the SCS-aggravated type. No significant difference was observed in adjacent segment disease incidence between the 2 groups (P = .243). The NS group had better outcomes according to the clinical function scores (P < .05). CONCLUSIONS: The cerebrospinal fluid occlusion sign on MRI is valuable for evaluating the adjacent segment with pre-existing degeneration. Patients with mild SCS in adjacent segments were more likely to have ASDeg, and the most frequent type of ASDeg was the SCS-aggravated type at early follow-up.