Posterior Spinal Fusion Surgery for Neuromuscular Disease Patients with Severe Scoliosis Whose Cobb Angle Was over 100 Degrees.

Background and objectives: Patients with neuromuscular diseases usually have progressive neuromuscular scoliosis (NMS), requiring invasive surgery. Some patients present with severe scoliosis at the time of consultation and are difficult to treat. Posterior spinal fusion (PSF) surgery combined with anterior release and pre- or intraoperative traction would be effective for severe spinal deformities but would be invasive. This study aimed to evaluate the outcomes of PSF-only surgery for patients with severe NMS with a Cobb angle > 100°. Materials and Methods: Thirty NMS patients (13 boys and 17 girls; mean age 13.8 years) who underwent PSF-only surgery for scoliosis with a Cobb angle > 100° were included. We reviewed the lower instrumented vertebra (LIV), duration of surgery, blood loss, perioperative complications, preoperative clinical findings, and radiographic findings, including Cobb angle and pelvic obliquity (PO) in the sitting position pre- and postoperatively. The correction rate and correction loss of the Cobb angle and PO were also calculated. Results: The mean duration of surgery was 338 min, intraoperative blood loss was 1440 mL, preoperative %VC was 34.1%, FEV1.0 (%) was 91.5%, and EF was 66.1%. There were eight cases of perioperative complications. The Cobb angle and PO correction rates were 48.5% and 42.0%, respectively. We divided the patients into two groups: the L5 group, in which the LIV was L5, and the pelvis group, in which the LIV was the pelvis. The duration of surgery and PO correction rate in the pelvis group were significantly higher than those in the L5 group. Conclusions: Patients with severe NMS demonstrated severe preoperative restrictive ventilatory impairments. PSF surgery without anterior release or any intra-/preoperative traction showed satisfactory outcomes, including acceptable scoliosis correction and improved clinical findings, even in patients with extremely severe NMS. Instrumentation and fusion to the pelvis for severe scoliosis in patients with NMS showed good PO correction and low correction loss of Cobb angle and PO, but a longer duration of surgery.

Intraoperative Blood Loss at Different Surgical-Procedure Stages during Posterior Spinal Fusion for Idiopathic Scoliosis.

Background and Objectives: Several predictive factors have been reportedly associated with intraoperative total blood loss (TBL) during posterior spinal fusion (PSF) for idiopathic scoliosis (IS). To reduce TBL, preoperative factors and interoperative factors are considered important. However, there are few reports that have evaluated bleeding patterns according to surgical stages. This study aimed to elucidate bleeding patterns at different surgical stages and determine the predictive factors for TBL during PSF surgery in patients with IS. Materials and Methods: Preoperative data, radiographic parameters, and intraoperative data of patients undergoing PSF for IS were retrospectively collected. We divided the patients into six stages: stage 1, exposure; stage 2, implant placement; stage 3, release; stage 4, correction; stage 5, bone grafting; and stage 6, closure; then we reviewed the blood loss and bleeding speed. Multiple-regression analysis was performed to generate a predictive formula for blood loss using preoperative and intraoperative factors, including blood loss at stage 1, as explanatory variables. Results: Forty-five patients (mean age: 17.6 years) were included. The mean operative time and TBL were 287.9 min and 756.5 mL, respectively. Blood loss was the highest at stage 3, followed by stage 4. Bleeding speed was the highest at stage 4, followed by stage 3. Bleeding speeds at stages 3 and 4 were significantly higher than those at stages 1 and 2. Preoperative Cobb angle, activated partial thromboplastin time (aPTT), number of fused vertebrae, and blood loss at stage 1 were significant contributing factors. Conclusions: Blood loss and bleeding speed during the release and correction stages were high. Specifically, bleeding speed significantly increased during and after the release procedure. The preoperative Cobb angle, aPTT, number of fixed vertebrae, and blood-loss volume during PSF were significantly associated with TBL. Our findings would be helpful for reducing TBL in patients undergoing PSF for IS.

A High Body Mass Index and the Vacuum Phenomenon Upregulate Pain-Related Molecules in Human Degenerated Intervertebral Discs.

Animal studies suggest that pain-related-molecule upregulation in degenerated intervertebral discs (IVDs) potentially leads to low back pain (LBP). We hypothesized that IVD mechanical stress and axial loading contribute to discogenic LBP's pathomechanism. This study aimed to elucidate the relationships among the clinical findings, radiographical findings, and pain-related-molecule expression in human degenerated IVDs. We harvested degenerated-IVD samples from 35 patients during spinal interbody fusion surgery. Pain-related molecules including tumor necrosis factor alpha (TNF-alpha), interleukin (IL)-6, calcitonin gene-related peptide (CGRP), microsomal prostaglandin E synthase-1 (mPGES1), and nerve growth factor (NGF) were determined. We also recorded preoperative clinical findings including body mass index (BMI), Oswestry Disability Index (ODI), and radiographical findings including the vacuum phenomenon (VP) and spinal instability. Furthermore, we compared pain-related-molecule expression between the VP (-) and (+) groups. BMI was significantly correlated with the ODI, CGRP, and mPGES-1 levels. In the VP (+) group, mPGES-1 levels were significantly higher than in the VP (-) group. Additionally, CGRP and mPGES-1 were significantly correlated. Axial loading and mechanical stress correlated with CGRP and mPGES-1 expression and not with inflammatory cytokine or NGF expression. Therefore, axial loading and mechanical stress upregulate CGRP and mPGES-1 in human degenerated IVDs, potentially leading to chronic discogenic LBP.

Prevalence and location of endplate fracture and subsidence after oblique lumbar interbody fusion for adult spinal deformity.

BACKGROUND: Recently, Oblique lumbar interbody fusion (OLIF) is commonly indicated to correct the sagittal and coronal alignment in adult spinal deformity (ASD). Endplate fracture during surgery is a major complication of OLIF, but the detailed location of fracture in vertebral endplate in ASD has not yet been determined. We sought to determine the incidence and location of endplate fracture and subsidence of the OLIF cage in ASD surgery, and its association with fusion status and alignment. METHODS: We analyzed 75 levels in 27 patients were analyzed using multiplanar CT to detect the endplate fracture immediately after surgery and subsidence at 1 year postoperatively. The prevalence was compared between anterior and posterior, approach and non-approach sides, and concave and convex side. Their association with fusion status, local and global alignment, and complication was also investigated. RESULTS: Endplate fracture was observed in 64 levels (85.3%) in all 27 patients, and the incidence was significantly higher in the posterior area compared with the anterior area (85.3 vs. 68.0%, p=0.02) of affected vertebra in the sagittal plane. In the coronal plane, there was no significant difference in incidence between left (approach) and right (non-approach) sides (77.3 and 81.3%, respectively), or concave and convex sides (69.4 and 79.6%) of wedged vertebra. By contrast, cage subsidence at 1 year postoperatively was noted in 14/75 levels (18.7%), but was not associated with endplate fracture. Fusion status, local and global alignment, and complications were not associated with endplate fracture or subsidence. CONCLUSION: Endplate fracture during OLIF procedure in ASD cases is barely avoidable, possibly induced by the corrective maneuver with ideal rod counter and cantilever force, but is less associated with subsequent cage subsidence, fusion status, and sustainment of corrected alignment in long fusion surgery performed even for elderly patients.

Incidence of Unintentional Intraoperative Hypothermia in Pediatric Scoliosis Surgery and Associated Preoperative Risk Factors.

INTRODUCTION: Intraoperative hypothermia is associated with perioperative complications such as blood loss and wound infection. Thus, perioperative heat retention methods to prevent perioperative hypothermia such as providing a warmed blanket and active patients' warming are important. Although major surgery and pediatric patient age are noted as risk factors, only a few studies focus on hypothermia as an intraoperative complication in pediatric scoliosis surgery. The aim of this study is to investigate the incidence of intraoperative hypothermia in pediatric scoliosis surgery and the associated preoperative risk factors. METHODS: We retrospectively reviewed the records of pediatric patients who underwent posterior spinal fusion at a single institution between 2015 and 2019. We recorded the background data, perioperative data, lowest recorded core temperature, and perioperative complications. Patients were divided into those whose temperature decreased below 36°C (Group H) and those who maintained a temperature of 36°C or greater (Group N) during surgery. We compared the two groups and performed multivariate analysis to identify preoperative risk factors for intraoperative hypothermia. RESULTS: A total of 103 patients underwent posterior spinal fusion; 56 for adolescent idiopathic scoliosis and 47 for neuromuscular scoliosis. Hypothermia was observed in 40 patients (38.8%). Group H had more non-adolescent idiopathic scoliosis (AIS) patients, lower mean body mass index, greater mean blood loss, greater number of fused vertebrae, larger preoperative Cobb angle, and lower initial core body temperature (immediately after induction of anesthesia). On multivariate analysis, a diagnosis of neuromuscular scoliosis, a lower body mass index, and a lower initial core body temperature were identified as independent risk factors for intraoperative hypothermia. CONCLUSIONS: The incidence of hypothermia in pediatric posterior scoliosis surgery is 38.8%. Diagnosis of non-AIS, lower body mass index, and lower core body temperature at the time of anesthesia induction are preoperative risk factors for intraoperative hypothermia.

Body Composition in Japanese Girls with Adolescent Idiopathic Scoliosis.

INTRODUCTION: There are few reports on body composition, particularly muscle mass, in patients with adolescent idiopathic scoliosis (AIS). The purpose of this study was to measure body composition including muscle mass and estimated bone mass of patients with AIS using bioelectrical impedance analysis (BIA) and to clarify the relationship between the degree of scoliosis and body composition. METHODS: The subjects were 210 girls (mean age 14.0 years, range 10-18 years) whose body composition was evaluated using BIA (Tanita MC-780). Body mass index (BMI), percent body fat (%BF), lean muscle mass index (LMI: muscle mass/height^2), and estimated bone mass index (eBoneMI: estimated bone mass/height^2) were determined by age and compared with those of previous reports. We divided 111 subjects whose bone maturation was complete into two groups for comparison of body composition metrics: those with Cobb angle <40° (moderate scoliosis group) and those with Cobb angle ≥40° (severe scoliosis group). The relationships between Cobb angle and each body composition parameter were evaluated. RESULTS: Age-adjusted BMI, %BF, and LMI tended to be low at all ages compared with means for the healthy Japanese population as previously reported. BMI, LMI, and eBoneMI were significantly lower in the severe scoliosis group compared with those in the moderate scoliosis group (p<0.05). In addition, all BMI, LMI, and eBoneMI were weakly correlated with Cobb angle (r= -0.20, -0.26, and -0.24). CONCLUSIONS: On the basis of the results of this study, patients with AIS are thinner, with lower BMI, %BF, and LMI compared with healthy girls of the same age. Furthermore, factors such as lower BMI, lower muscle mass, and lower estimated bone mass were correlated with progressive scoliosis.

Posterior Spinal Correction and Fusion Surgery in Patients with Spinal Muscular Atrophy-Associated Scoliosis for Whom Treatment with Nusinersen Was Planned.

INTRODUCTION: Spinal muscular atrophy (SMA) is defined as a neuromuscular disorder induced by progressive weakness of the skeletal muscle and is usually accompanied by progressive spinal deformity including scoliosis. The newly developed Nusinersen, which is the first approved drug worldwide for SMA, requires accurate intrathecal injection, which is sometimes difficult in patients with severe spinal deformity. TECHNICAL NOTE: For an accurate intrathecal approach in patients who have spinal fusion surgery to treat neuromuscular scoliosis, we have combined an L3 laminectomy with spinal correction and fusion surgery. Here, we review four cases of SMA in patients who underwent the additional L3 laminectomy during surgery to treat spinal scoliosis. A successful intrathecal approach was made using fluoroscopic guidance in all four patients, who were then administered with Nusinersen. CONCLUSIONS: Our findings show that additional lumbar laminectomy during surgery for spinal scoliosis has effectively allowed for intrathecal injection of Nusinersen.

Chylous retroperitoneum following 720 degree anteroposterior-combined corrective surgery for adult spinal deformity with split vertebral fracture subluxation: a case report.

STUDY DESIGN: Clinical case report. PURPOSE: To report the rare case with post-operative chylous retroperitoneum after corrective surgery for adult spinal deformity. METHODS: We present a case of a 73-year-old woman with Parkinson's disease. She sustained a severe split fracture subluxation of the L3 vertebra with AO Spine Thoracolumbar classification type CN2M2, resulting in severe kyphoscoliosis in global alignment. She underwent a two-stage 720-degree anteroposterior-combined corrective surgery with anterior vertebral column resection of L3 and posterior fusion from T4 to the pelvis. On post-operative day 1, milky fluid in the drainage tube was noted, which was diagnosed as post-operative chylous retroperitoneum. RESULTS: Oral intake was discontinued immediately and peripheral parenteral nutrition was started. A low-fat, high-protein diet was started on post-operative day 4, and drainage was removed on day 6. A low-fat diet was continued until 3 months post-operatively, with dietary counselling by a nutritionist. The chylous retroperitoneum resolved without recurrence at the final follow-up evaluation at 3 years. CONCLUSION: Surgeons should recognize this rare complication, which might be induced by direct damage to the lymphatic flow during an operative maneuver anterior to the lumbar vertebral body and indirect damage due to shearing force during correction of a subluxated vertebra, especially in cases with a severe deformity.

Role of CD14-positive cells in inflammatory cytokine and pain-related molecule expression in human degenerated intervertebral discs.

Multiple human and animal studies suggest that the upregulation of inflammatory cytokines and other pain-related molecules in degenerated or injured intervertebral discs (IVDs) may cause discogenic low back pain (LBP). We previously reported that macrophages in injured IVD in mice produced inflammatory cytokines, but not other pain-related molecules. CD14 is a monocyte marker expressed mainly by macrophages. The aim of the current study was to evaluate the role of CD14-positive cells in inflammatory cytokine and pain-related molecule expression in human degenerated IVD. IVD samples were harvested from 14 patients, including 10 with lumbar spinal stenosis, four with adult spinal deformity, and one with lumbar disc herniation during spinal interbody fusion surgery. Harvested IVD-derived mononuclear cells were obtained and CD14-positive (+) and CD14-negative (-) cells were separated using CD14 antibody and streptavidin-labeled magnetic beads. Inflammatory cytokines messenger RNA (mRNA) in the CD14(+) and CD14(-) cells, including tumor necrosis factor ɑ (TNFA), in, terleukin-1ß (IL1B) and IL6, were determined using quantitative polymerase chain reaction (qPCR) and their expression levels were compared. To evaluate factors controlling the regulation of pain-related molecules mRNA expression, cultured CD14(-) and CD14(+) cells from IVDs were stimulated with recombinant human TNF-ɑ and IL-1ß and levels of pain-related molecules, including calcitonin gene-related peptide (CGRP) and nerve growth factor (NGF) were determined using qPCR. Levels of TNFA, IL1B, IL6, and NGF in CD14(+) cells were significantly increased compared with those in CD14(-) cells (TNFA, p = 0.006; IL1B, p = .017; IL6, p = .010; NGF, p = .027). Following TNFA stimulation, NGF levels were significantly increased in CD14(-) and CD14(+) cells (CD14(-), p = .003; CD14(+), p < .001) and CGRP was significantly increased in CD14(-) IVD cells (p = .040). Following IL1B stimulation, NGF levels were significantly increased in CD14(-) cells (p = .004). CD14(+) cells had higher TNFA, IL1B, IL6, and NGF expressions than CD14(-) cells in human degenerated IVDs. Additionally, TNFA stimulation promoted the upregulation of NGF and CGRP in CD14(-) cells. These findings suggested that CD14(+) cells directly and indirectly contributed to inflammatory cytokine and pain-related molecule expression in human degenerated IVD. CD14(+) cells might be important in the pathological mechanism of chronic discogenic LBP in humans.

Risk factors associated with prolonged intensive care unit stay after posterior spinal fusion for Duchenne muscular dystrophy.

BACKGROUND: The perioperative complication rate for spinal fusion in Duchenne muscular dystrophy (DMD) remains high and sometimes prolonged perioperative intensive care is needed. We investigated preoperative and intraoperative risk factors associated with prolonged intensive care unit (ICU) stay after posterior spinal fusion. METHODS: We reviewed the records of 49 consecutive DMD patients who underwent posterior spinal fusion. Instrumentation was performed from T4 to L5 (46 cases) or to the ilium (3 cases). We recorded the preoperative Cobb angle and perioperative clinical data from patient records. Patients were divided into two groups (ICU stay 0 or 1 day, 2 days or longer). Chi-square and t tests were used for univariate analysis. Factors with p < 0.05 in the univariate analysis were entered into a multilevel logistic regression analysis. RESULTS: The average age of patients at surgery was 14.2 years (range 11-20 years). Their preoperative Cobb angle was 79.2° (range 40°-154°). Average operative time was 325.3 min (range 225-507 min). The average estimated blood loss (EBL) was 1673.0 ml (range 500-3785 ml). Eight patients stayed in the ICU for 2 days or longer for postoperative monitoring and treatment. Univariate analysis found statistical difference between the two groups in preoperative Cobb angle, Thoracolumbar kyphosis, %VC, operation time, and EBL during surgery. After multiple logistic regression analysis, Cobb angle and EBL was identified as an independent factor. The patients with a larger Cobb angle and greater EBL had a higher rate of prolonged ICU stay in interquartile range-based comparison. CONCLUSIONS: The present study suggests the preoperative Cobb angle and intraoperative EBL could be predictors for postoperative course in posterior spinal fusion for DMD patients.

Cervical Hyperextension Treated by Posterior Spinal Correction and Fusion in A Patient with Ullrich Congenital Muscular Dystrophy: A Case Report.

CASE: An 18-year-old man with Ullrich congenital muscular dystrophy (UCMD) noted difficulty of looking forward and discomfort swallowing and breathing because of his hyperextended neck. We treated his cervical deformity with posterior spinal correction and fusion alone. He underwent a tracheotomy because of lung function deterioration 2 years after cervical surgery. The tracheotomy was performed safely because the anterior cervical spine anatomy was normalized and soft tissues around trachea were preserved by the posterior cervical correction. CONCLUSION: Cervical hyperextension can be a problem in patients with UCMD. Posterior spinal correction and fusion may be a preferable solution.

Prediction tool for high risk of surgical site infection in spinal surgery.

OBJECTIVE: The incidence of surgical site infection (SSI) is higher in spinal surgeries than in general orthopedic operations. In this study, we aimed to develop a scoring system with reduced health care costs for detecting spinal surgery patients at high risk for SSI. DESIGN: Retrospective cohort study. PATIENTS: In total, 824 patients who underwent spinal surgery at 2 university hospitals from September 2005 to May 2011. METHODS: We reviewed the medical records of 824 patients, and we examined 19 risk factors to identify high-risk patients. After narrowing down the variables by univariate analysis, multiple logistic analysis was performed for factors with P values <.2, using SSI as a dependent variable. Only factors that showed P values <.05 were included in the final models, and each factor was scored based on the ß coefficient values obtained. The clinical prediction rules were thereby prepared. RESULTS: "Emergency operation," "blood loss >400 mL," "presence of diabetes," "presence of skin disease," and "total serum albumin value <3.2 g/dL" were detected by multivariable modeling and were incorporated into the risk scores. Applying these 5 independent predictive factors, we were able to predict the infection incidence after spinal surgery. CONCLUSIONS: Our present study could aid physicians in making decisions regarding prevention strategies in patients undergoing spinal surgery. Stratification of risks employing this scoring system will facilitate the identification of patients most likely to benefit from complex, time-consuming and expensive infection prevention strategies, thereby possibly reducing healthcare costs.

Regression of Retro-odontoid Pseudotumor Using External Orthosis without Atlantoaxial Fusion: A Case Report.

CASE: We treated an 87-year-old woman with noninflammatory retro-odontoid pseudotumor induced by atlantoaxial instability (AAI) using only a Philadelphia collar. Because of perioperative risk factors, fusion surgery was canceled; nevertheless, the neurological symptoms improved gradually. After 11 months of nonoperative treatment, the follow-up magnetic resonance imaging demonstrated that the pseudotumor's size was obviously diminished. Orthosis was applied for 2 years, and after its removal, the pseudotumor's size remained the same at the final 7-year follow-up. CONCLUSIONS: External orthosis is one treatment option for pseudotumor with AAI, especially in patients with significant comorbidities for whom surgical procedures are relatively contraindicated.

Radiographic and Clinical Outcomes From the Use of S2 Alar Screws in Surgery for Adult Spinal Deformity.

STUDY DESIGN: Retrospective. OBJECTIVES: To evaluate the efficacy of S2 alar screws in surgery for correction of adult spinal deformity (ASD). METHODS: We retrospectively reviewed the cases of 23 patients (mean follow-up: 18.5 months, minimum 12 months) who underwent corrective surgery for ASD using S2 alar screws as anchors for instrumentation of lower vertebrae. The background of the patients and their spinopelvic parameters (pelvic incidence [PI], pelvic tilt [PT], lumbar lordosis [LL], thoracic kyphosis [TK], sagittal vertical axis [SVA], and PI-LL) were evaluated. RESULTS: LL was improved from 9.7 ± 20.5° and SVA from 141.0 ± 64.0 mm before surgery to 39.0 ± 9.6° and 51.7 ± 40.8 mm immediately after surgery, respectively, and 38.2 ± 12.7° and 70.5 ± 59.2 mm at final follow-up. In 13 patients without sufficient correction (postoperative PI-LL ≥10°), bone mineral density and postoperative LL were significantly less, and PI, PI-LL, and PT were significantly greater than in patients with postoperative PI-LL <10°, suggesting that these are risk factors for undercorrection. In 5 patients, SVA increased more than 40 mm during follow-up. Postoperative LL was significantly less (31.4° vs 41.0°) and postoperative PI-LL was significantly greater (21.6° vs 9.3°) in these patients, suggesting a PI-LL mismatch induces postoperative progression of global malalignment. CONCLUSIONS: Use of S2 alar screws as anchors for instrumentation in ASD surgery should be restricted. Their use might be an option for patients with low PI, and without severe osteoporosis, in whom efficient surgical correction can be obtained.

Segmental Pedicle Screw Instrumentation and Fusion Only to L5 in the Surgical Treatment of Flaccid Neuromuscular Scoliosis.

STUDY DESIGN: A retrospective cohort study was performed. OBJECTIVE: The purpose of this study was to determine the efficacy and safety of stopping segmental pedicle screw instrumentation constructs at L5 in the treatment of neuromuscular scoliosis. SUMMARY OF BACKGROUND DATA: Duchenne muscular dystrophy and spinal muscular atrophy are flaccid neuromuscular disorders in which gradual deterioration is the hallmark and have a lot of characteristics in common despite differences in etiology. Instrumentation and fusion to the sacrum/pelvis has been a mainstay in the surgical treatment of flaccid neuromuscular scoliosis and recommended to correct pelvic obliquity. However, the caudal extent of instrumentation and fusion in the surgical treatment of flaccid neuromuscular scoliosis has remained a matter of considerable debate and there have been few studies on the use of segmental pedicle screw instrumentation for flaccid neuromuscular scoliosis. METHOD: From 2005 to 2007, a total of 27 consecutive patients with neuromuscular disorders (20 Duchenne muscular dystrophy and 7 spinal muscular atrophy), aged 11 to 17 years, underwent segmental pedicle screw instrumentation and fusion only to L5. Assessment was performed clinically and with radiologic measurements. Minimum 2-year follow-up was required for inclusion in this study. RESULTS: Twenty patients were enrolled in this study. No patient was lost to follow-up. All patients had L5 tilt of less than 15° and a coronal curve with apex L2 or higher preoperatively. Preoperative coronal curve averaged 70° (range: 51°-88°), with a postoperative mean of 15° (range: 5°-25°) and 17° (range: 6°-27°) at the last follow-up. The pelvic obliquity improved from 15° (range: 9°-25°) preoperatively to 5° (range: 3°-8°) postoperatively and 6° (range: 3°-8°) at the last follow-up. The L5 tilt improved from 9° (range: 2°-14°) preoperatively to 2° (range: 0°-4°) postoperatively and 2° (range: 0°-5°) at the last follow-up. Physiologic sagittal plane alignment was recreated after surgery and maintained long-term. There was no significant loss of correction of coronal curve and pelvic obliquity. There was no major complication. CONCLUSION: Segmental pedicle screw instrumentation and fusion to L5 was safe and effective in patients with flaccid neuromuscular scoliosis with apex L2 or higher and minimal L5 tilt of less than 15°. Segmental pedicle screw instrumentation ending at L5 offered the ability to correct spinal deformity and pelvic obliquity initially, intermediate and even long-term, with no major complications. This method in appropriate patients can be a viable alternative to instrumentation and fusion to the sacrum/pelvis in the surgical treatment of flaccid neuromuscular scoliosis. LEVEL OF EVIDENCE: N/A.

Perioperative Complications in Posterior Spinal Fusion Surgery for Neuromuscular Scoliosis.

INTRODUCTION: Patients with neuromuscular disorders sometimes show progressive spinal scoliosis. The surgery for neuromuscular scoliosis (NMS) has high rates of complications. In this study, we elucidated the perioperative complications in patients with NMS. METHODS: We included 83 patients with NMS (58 boys and 25 girls; 61 with muscular dystrophy, 18 with spinal muscular atrophy, and 4 others) who had undergone posterior fusion surgery for scoliosis. We evaluated the perioperative complications (within 3 months), age at time of surgery, operative time, blood loss, preoperative %VC and FEV1.0 (%) for pulmonary function, and preoperative ejection fraction (EF) for cardiac function. RESULTS: There were 5 (6%) major complications, including pneumonia and a cardiovascular complication requiring intensive care unit (ICU) care, and 15 (18%) minor complications including viral enteritis and a urinary tract infection. Overall, there were 20 (24%) complications. Three of the 5 major complications were pulmonary. The mean age at the time of surgery was 13.7 y, operative time was 304 min, and blood loss was 1530 ml. The mean preoperative %VC was 41%, FEV1.0 was 91%, and EF was 60%. When we separated the patients into a group with major complications (n = 5) and a group without major complications (n = 78), the preoperative %VC in the group with major complications (23%) was significantly lower than that in the group without (42%) (p < 0.05). However, operative time, blood loss, preoperative FEV1.0 (%) and EF between the two groups were not significantly different (p > 0.05). CONCLUSIONS: Compared with the previous findings of the perioperative complication rate (45%-74%) for NMS, the complication rate was remarkably low in this case series. Because of advances in medical skills, including anesthesia and surgical instruments, surgery for NMS appears to be safe. However, patients with NMS with complications demonstrated severe restrictive ventilatory impairment preoperatively. Therefore, we should be vigilant for perioperative pulmonary complications especially in patients with NMS and preoperative severe restrictive ventilatory impairment.

Safety and Efficacy of Treatment for Scolios Is Secondary to Spinal Muscular Atrophy Fused to Lumbar 5 Level.

INTRODUCTION: Patients with spinal muscular atrophy (SMA) usually have progressive scoliosis. Although fusion of the sacrum or pelvis has been recommended for correcting pelvic obliquity (PO), the procedure is invasive. This study determined as to whether performing instrumentation to the fifth lumbar vertebra (L5) is safe and effective for scoliosis in patients with SMA. METHODS: Twelve patients with SMA underwent posterior spinal fusion and stopping instrumentation at the L5 level. We evaluated age at surgery, the duration of surgery, blood loss, complications, preoperative and postoperative Cobb angles, and PO. RESULTS: The mean age at surgery was 11.4 years; the mean duration of surgery was 319 minutes, and the mean blood loss was 1170 mL. The Cobb angle improved from 97.3° to 39.1° at 1 month postoperatively (correction rate, 60.9%) and to 42.3° at the final follow-up. PO was corrected from 27.8° to 13.1° at 1 month postoperatively (correction rate, 51.7%) and to 19.8° at the final follow-up. No complications were reported. All patients showed improvement in low back pain, with reduced difficulty while sitting. However, >10% correction loss of PO was observed in 6 patients with high preoperative PO. CONCLUSIONS: The correction rate of scoliosis in SMA patients with posterior spinal fusion and instrumentation to the L5 level was acceptable, and no complications occurred. Scoliosis associated with SMA was more rigid and severer than scoliosis associated with Duchenne muscular dystrophy. Correction rates of the Cobb angle and PO in SMA patients with instrumentation to L5 were similar to those in SMA patients with instrumentation to the sacrum or pelvis. Correction loss of PO was greater in patients with high preoperative PO than in those with low preoperative PO. Instrumentation and fusion to L5 for scoliosis in patients with SMA seems safe and effective, except in cases of high preoperative PO.

Spontaneous Spinal Epidural Hematoma in an Infant with Developmental Disabilities.

INTRODUCTION: Spontaneous spinal epidural hematomas (SSEHs) are rare in childhood, especially in infants. CASE REPORT: We present the case of a 17-month-old-boy with trisomy 21 and a large SSEH. He was hospitalized for acute onset paraplegia after 6 days of irritability. Nine days after symptom onset, magnetic resonance imaging (MRI) of the spine revealed an extensive epidural hematoma between C7 and T5 causing severe spinal cord compression. After a coagulation disorder was ruled out (12 days after onset), he underwent emergency hemilaminectomy with evacuation of the hematoma. His neurologic impairment gradually improved, and 4 months after surgery he was back to his neurologic baseline. At 18 months after surgery, he was walking independently, although he had some developmental disabilities due to trisomy 21. CONCLUSIONS: Only 20 cases of SSEH in infancy have been previously reported, and this is the first report of SSEH in an infant with developmental disabilities. Because of the non-specific symptoms and difficulty obtaining MRIs in infants, particularly in those with developmental disabilities, the diagnosis and treatment of SSEH may be delayed. However, early diagnosis with MRI and early evacuation of SSEH in patients with severe neurological impairments is important for good outcomes. Attention must be paid to postoperative spinal deformity in infants.

The Relationship between Preoperative Echocardiographic Evaluation and Spinal Deformity in Patients with Neuromuscular Scoliosis.

INTRODUCTION: Echocardiography is an important component of perioperative cardiac risk stratification in patients with neuromuscular scoliosis (NMS). However, there are little data regarding the relationship between preoperative echocardiographic findings and spinal deformity. We retrospectively reviewed preoperative echocardiographic data to investigate the relationship between echocardiographic evaluation and spinal deformity in NMS. METHODS: We reviewed 73 NMS patients (mean age: 13.3 years, male 66%) who underwent spinal correction surgery between 2008 and 2016. Echocardiographic data including ejection fraction (EF), valvar disease, and inferior vena cava diameter were collected from the preoperative exam. Demographic and radiographic data were also collected. RESULTS: Preoperative diagnoses included Duchenne muscular dystrophy, Fukuyama congenital muscular dystrophy, other dystrophy, spinal muscular atrophy, and congenital myopathies. Mean Body Mass Index (BMI) was 15.6 kg/m2. Mean major Cobb angle before surgery was 86.6 ± 28.2°. Because of technical difficulty, complete echocardiographic data could only be collected and evaluated in 49.3% of patients. Neither right nor left sided cardiac evaluation could be completed in 20.5%. Patients in whom complete echocardiographic data could not be collected had significantly more extensive thoracic scoliosis with a more rigid curve and hypokyphosis, and were of lower weight and BMI than patients in whom complete data could be collected. Ten cases (13.7%) were diagnosed as having minor heart-related complications immediately after surgery, and they had higher right atrial pressures preoperatively. CONCLUSIONS: Echocardiography can be technically difficult in NMS patients with extensive spinal deformities. We found that perioperative cardiac function could only be evaluated by echocardiogram in about half of NMS patients undergoing spinal correction surgery. The absence of an adequate preoperative cardiac evaluation could render these patients more susceptible to perioperative heart-related complications. Echocardiography may not be sufficient to evaluate cardiac conditions in children with extensive NMS.

Perioperative Evaluation of Respiratory Muscle Strength after Scoliosis Correction in Patients with Duchenne Muscular Dystrophy.

STUDY DESIGN: Retrospective cohort study. PURPOSE: To investigate the effect of spinal correction on respiratory muscle strength in patients with Duchenne muscular dystrophy (DMD). OVERVIEW OF LITERATURE: Several studies have reported that scoliosis correction in patients with DMD does not improve pulmonary function. In these studies, pulmonary function was evaluated using the traditional spirometric values of percent vital capacity (%VC) and percent forced vital capacity (%FVC). However, traditional spirometry may not be suitable for patients with DMD because the results can be influenced by patient fatigue or level of understanding. Therefore, we evaluated respiratory function focusing on respiratory muscle strength using maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and sniff nasal inspiratory pressure (SNIP), in addition to %VC and %FVC. METHODS: We retrospectively reviewed 16 patients with DMD who underwent spinal correction surgery between 2006 and 2011 at Kitasato University Hospital. All patients were males, and the mean age was 13.5 years. Respiratory muscle strength was evaluated using MIP, MEP, and SNIP. Measurements were obtained preoperatively and at 1 and 6 months postoperatively, and %VC and %FVC were obtained preoperatively and within 6 months postoperatively. RESULTS: The mean preoperative and postoperative %VC values were 54.0% and 51.7%, whereas the mean %FVC values were 53.9% and 53.2%, respectively. The mean MIP, MEP, and SNIP values obtained preoperatively and at 1 and 6 months postoperatively were as follows: MIP, 40.5, 42.7 and 47.2 cm H2O; MEP, 26.0, 28.0, and 29.0 cm H2O; and SNIP, 33.4, 33.0, and 33.0 cm H2O; respectively. The mean MIP and MEP values significantly improved postoperatively. There were no significant differences in SNIP, %VC, or %FVC preand postoperatively. CONCLUSIONS: By focusing on respiratory muscle strength, our results suggest that scoliosis correction in patients with DMD might have a favorable effect on respiratory function.