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

Study DesignRetrospective cohort study.PurposeTo investigate the effect of spinal correction on respiratory muscle strength in patients with Duchenne muscular dystrophy (DMD).Overview of LiteratureSeveral 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.MethodsWe 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.ResultsThe 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.ConclusionsBy focusing on respiratory muscle strength, our results suggest that scoliosis correction in patients with DMD might have a favorable effect on respiratory function

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

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

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

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). 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

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

Surgical treatment for suicidal jumper's fracture (unstable sacral fracture) with thoracolumbar burst fracture: a report of three cases

Introduction: Suicidal jumper's fracture (unstable sacral fracture) is characterized not only by multiple fractures including thoracolumbar fractures, but also major chest and abdominal injuries. Early stabilization of these fractures and early ambulation are required for the treatment and management of chest and abdominal injuries. We present 3 cases of suicidal jumper's fracture with thoracolumbar burst fracture, treated with minimally invasive posterior fixation surgery, which is a combination of percutaneous pedicle screws (PPS) and the mini-open Galveston technique. Case reports: Case 1. A 50-year-old woman was injured by a fall from the 5th floor of a building as the result of a suicide attempt. Computed tomography revealed an H-shaped unstable sacral fracture and thoracolumbar fractures with major chest and abdominal injuries. For early stabilization of spinopelvic instability and early ambulation, we treated the patient with PPS and the mini-open Galveston technique. Her early postoperative emergence from bedrest contributed to the improvement of her general condition. One year after surgery at the final follow-up, she was able to walk with a T-cane without any motor, bladder, or bowel dysfunction (BBD) and achieved almost complete healing of the fractures. Cases 2 and 3. A 25-year-old woman (Case 2) and a 43-year-old woman were injured in falls. They had multiple injuries including unstable sacral fractures, and thoracolumbar fractures with major chest and abdominal injuries. We treated these patients with PPS and the mini-open Galveston technique. One year after surgery, they were able to walk with a T-cane and achieved almost complete healing of thoracolumbar fractures, but delayed healing of an unstable sacral fracture in Case 2, and remaining BBD in Case 3. Conclusion: PPS and the mini-open Galveston technique is a good approach to fixation because they are minimally invasive and provide moderately rigid fixation, especially in patients with multiple trauma whose general condition is poor

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

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 Characteristics of Spinal Sagittal Malalignment in Patients with Osteoporosis

Spinal sagittal malalignment due to vertebral fractures (VFs) induces low back pain (LBP) in patients with osteoporosis. This study aimed to elucidate spinal sagittal malalignment prevalence based on VF number and patient characteristics in individuals with osteoporosis and spinal sagittal malalignment. Spinal sagittal alignment, and VF number were measured in 259 patients with osteoporosis. Spinal sagittal malalignment was defined according to the SRS-Schwab classification of adult spinal deformity. Spinal sagittal malalignment prevalence was evaluated based on VF number. In patients without VFs, bone mineral density, bone turnover markers, LBP scores and health-related quality of life (HRQoL) scores of normal and sagittal malalignment groups were compared. In 205 of the 259 (79.2%) patients, spinal sagittal malalignment was detected. Sagittal malalignment prevalence in patients with 0, 1, or ≥2 VFs was 72.1%, 86.0%, and 86.3%, respectively. All LBP scores and some subscale of HRQoL scores in patients without VFs were significantly worse for the sagittal malalignment group than the normal alignment group (p < 0.05). The majority of patients with osteoporosis had spinal sagittal malalignment, including ≥70% of patients without VFs. Patients with spinal sagittal malalignment reported worse LBP and HRQoL. These findings suggest that spinal sagittal malalignment is a risk factor for LBP and poor HRQoL in patients with osteoporosis