Comparison between Cases of Total Hip Arthroplasty Followed by Colonna Capsular Arthroplasty and Lorenz Cast Reduction in Patients with Developmental Dysplasia of the Hip.

Most patients with developmental dysplasia of the hip (DDH) now receive closed-reduction treatment within 6 months after birth. The long-term outcomes of patients with late-detection DDH have remained unclear. We reviewed the clinical records of 18 patients who underwent Colonna capsular arthroplasty (n=8) or closed reduction (n=10) for developmental dysplasia of the hip as infants or young children and underwent total hip arthroplasty approximately in midlife. Both the Colonna capsular arthroplasty and closed reduction groups achieved good clinical results after total hip arthroplasty. However, the operating time was longer and the improvements of hip range of motion and clinical score were significantly worse in the Colonna capsular arthroplasty group than in the closed reduction group.

Long-Term Results of Posterior Vertebral Column Resection for Severe Thoracolumbar Kyphosis with Achondroplastic Patients: A Case Series.

Background and Objectives: Thoracolumbar kyphosis is one of the most frequent skeletal manifestations in patients with achondroplasia. Few papers have been published on the surgical treatment of this condition, especially in skeletally mature patients. With this study, we presented a retrospective case series of long-term surgical results for achondroplastic patients with severe thoracolumbar kyphosis. This study was conducted to evaluate the outcome of surgical treatment for thoracolumbar kyphosis in patients associated with achondroplasia presenting with paraparesis. Materials and Methods: Three patients with achondroplasia who developed neurologic deficits due to severe thoracolumbar kyphosis and underwent surgical treatment were evaluated (mean age 22.3 years; mean follow-up 9.3 years). All patients were treated with posterior vertebral column resection (p-VCR) of hypoplastic apical vertebrae with a cage and segmental instrumentation. Neurologic outcomes (JOA scores), correction of kyphosis, and operative complications were assessed. Results: All patients had back pain, neurological deficits, and urinary disturbance before surgery. The average preoperative JOA score was 8.3/11 points, which was improved to 10.7/11 points at the final follow-up (mean recovery rate 83%). All patients obtained neurologic improvement after surgery. The mean preoperative kyphotic angle was 117° (range 103°-126°). The postoperative angles averaged 37° (range 14°-57°), resulting in a mean correction rate of 67%. All patients had postoperative complications such as rod breakage and/or surgical site infection. Conclusions: The long-term results of p-VCR were acceptable for treating thoracolumbar kyphosis in patients with achondroplasia. To perform this p-VCR safely, spinal navigation and neuromonitoring are inevitable when resecting non anatomical fused vertebrae and ensuring correct pedicle screw insertion. However, surgical complications such as rod breakage and surgical site infection may occur at a high rate, making informed consent very important when surgery is indicated.

Prevalence of and Risk Factors for the Progression of Upper Cervical Lesions in Patients with Rheumatoid Arthritis.

We investigated the prevalence of and risk factors for the progression of upper cervical lesions (UCLs) in patients with rheumatoid arthritis (RA). A retrospective analysis of 49 patients with RA (4 males, 45 females) was conducted. The UCLs included atlanto-axial subluxation and vertical subluxation. We investigated the clinical factors including the Disease Activity Score 28 based on C-reactive protein (DAS28-CRP) and the modified Health Assessment Questionnaire-Disability Index as well as radiographic changes between the baseline (at May 2010 to April 2013) and final follow-up. Forty patients (81.6%) were classified as the non-progressive group, and the other 9 patients (18.4%) comprised the progressive group. The progressive group's final CRP values, baseline or final MMP-3 levels, DAS28-CRP, and rate of pre-existing lesions at baseline were all significantly higher than those of the non-progressive group (p=0.017, p=0.043, p=0.002, p=0.008, p<0.001, and p=0.008 respectively). A multivariate logistic regression analysis demonstrated that DAS28-CRP at baseline was a risk factor for radiographic progression (p=0.018, odds ratio: 2.54, 95% confidence interval: 1.17-5.51). Our findings indicate that higher disease activity might influence the progression of UCLs in patients with RA.

Two-Year Survival After First-Ever Stroke in a General Population of 1.4 Million Japanese　- Shiga Stroke Registry.

BACKGROUND: Stroke is one of the leading causes of disability and mortality in Japan. The aim of the present analysis was to determine the non-acute survival rate after first-ever stroke using data from a large-scale population-based stroke registry in Japan. Methods and Results: Shiga Stroke Registry is an ongoing population-based registry of stroke, which covers approximately 1.4 million residents of Shiga Prefecture in central Japan. A total of 2,176 first-ever stroke patients, who were registered in 2011, were followed up until December 2013. The 2-year cumulative survival rates were estimated using Kaplan-Meier method according to index stroke subtype. Cox proportional hazards models were used to assess predictors of all-cause death. During a 2-year follow-up period, 663 patients (30.5%) died. The 2-year cumulative survival rate after first-ever stroke was 69.5%. There was heterogeneity in 2-year cumulative survival according to stroke subtype: lacunar infarction, 87.2%; large artery infarction, 76.1%; cardioembolic infarction, 55.4%; intracerebral hemorrhage, 65.9%; and subarachnoid hemorrhage, 56.7%. Older age, male sex, medical history, higher Japan coma scale score on admission, and stroke subtype were associated with risk of all-cause death in ≤2 years. CONCLUSIONS: In the present population-based stroke registry with a real-world setting in Japan, 2-year cumulative mortality after first-ever stroke is still high (>30%), particularly for cardioembolic infarction, subarachnoid hemorrhage and intracerebral hemorrhage.

Venous Thromboembolism in Patients with Acute Thoracolumbar Spinal Cord Injury.

Venous thromboembolism (VTE) is a major complication in patients with acute spinal cord injury. There are few reports of VTE with acute thoracolumbar spinal cord injury (TLSCI). We assessed the incidence of VTE with acute TLSCI using color Doppler ultrasonography. We retrospectively assessed 75 patients with acute TLSCI (T1 to L1). All patients were surgically treated. VTE of the lower extremity and pelvis was assessed using color Doppler ultrasound regardless of whether symptoms were present. This retrospective study included patients who were assessed between 6 and 10 days (mean 8.1 days) after injury. VTE was detected in 27 of the 75 patients (35.7%) with or without paralysis. Of the 13 patients who had complete motor paralysis, 8 (62%) had VTE; of the 31 patients with incomplete motor paralysis, 10 (32%) had VTE, and of the 31 patients without motor paralysis, 9 (29%) had VTE. Among the patients with TLSCI, those with VTE had a significantly higher mean age than those without. The incidence of VTE in TLSCI patients is not related to the severity of paralysis in a Japanese population. The incidence appears to be related primarily to age.

The JAGUAR Score Predicts 1-Month Disability/Death in Ischemic Stroke Patient Ineligible for Recanalization Therapy.

BACKGROUND AND PURPOSE: Most available scoring system to predict outcome after acute ischemic stroke (AIS) were established in Western countries. We aimed to develop a simple prediction score of 1-month severe disability/death after onset in AIS patients ineligible for recanalization therapy based on readily and widely obtainable on-admission clinical, laboratory and radiological examinations in Asian developing countries. METHODS: Using the Shiga Stroke Registry, a large population-based registry in Japan, multivariable logistic regression analysis was conducted in 1617 AIS patients ineligible for recanalization therapy to yield ß-coefficients of significant predictors of 1-month modified Rankin Scale score of 5-6, which were then multiplied by a specific constant and rounded to nearest integer to develop 0-10 points system. Model discrimination and calibration were evaluated in the original and bootstrapped population. RESULTS: Japan Coma Scale score (J), age (A), random glucose (G), untimely onset-to-arrival time (U), atrial fibrillation (A), and preadmission dependency status according to the modified Rankin Scale score (R), were recognized as independent predictors of outcome. Each of their ß-coefficients was multiplied by 1.3 creating the JAGUAR score. Its area under the curve (95% confidence interval) was .901 (.880- .922) and .901 (.900- .901) in the original and bootstrapped population, respectively. It was found to have good calibration in both study population (P = .27). CONCLUSIONS: The JAGUAR score can be an important prediction tool of severe disability/death in AIS patients ineligible for recanalization therapy that can be applied on admission with no complicated calculation and multimodal neuroimaging necessary, thus suitable for Asian developing countries.

Incidence, Management and Short-Term Outcome of Stroke in a General Population of 1.4 Million Japanese　- Shiga Stroke Registry.

BACKGROUND: This study determined the current status of the incidence, management, and prognosis of stroke in Japan using a population-based stroke registry.Methods and Results:Shiga Stroke Registry is an ongoing population-based registry that covers approximately 1.4 million residents of Shiga Prefecture. Cases of acute stroke were identified using standard definitions through surveillance of both all acute-care hospitals with neurology/neurosurgery facilities and death certificates in 2011. A total of 2,956 stroke cases and 2,176 first-ever stroke cases were identified. The age- and sex-adjusted incidence rate for first-ever stroke using the 2013 European Standard Population as standard was per 100,000 person-years: 91.3 for ischemic stroke, 36.4 for intracerebral hemorrhage, and 13.7 for subarachnoid hemorrhage. It was estimated that approximately 220,000 new strokes occurred in 2011 in Japan. Among the 2,956 cases, most stroke patients underwent neuroimaging, 268 received surgical or endovascular treatment, and 2,158 had rehabilitation therapy; 78 patients received intravenous thrombolysis. A total of 1,846 stroke patients had died or were dependent at hospital discharge, and 390 died within 28 days of onset. CONCLUSIONS: Incidence rates of stroke by subtypes were clarified and the total number of new strokes in Japan was estimated. More than half of stroke patients die or become dependent after a stroke. This study re-emphasized the importance of public health measures in reducing the burden of stroke in Japan.

Accuracy of pedicle screw insertion in posterior scoliosis surgery: a comparison between intraoperative navigation and preoperative navigation techniques.

PURPOSE: This study was conducted to compare the efficacy and accuracy of intraoperative navigation (O-arm or Arcadis navigation) and preoperative CT-based navigation in adolescent idiopathic scoliosis (AIS) surgery. METHODS: Sixty-seven patients with scoliosis were grouped according to the method of navigation used in their fixation surgeries. A total of 492 pedicle screws were implanted in 27 patients using intraoperative navigation, and 626 screws were implanted in 40 patients using preoperative navigation. We analyzed the postoperative CT images for pedicle violations using the Gertzbein classification. RESULTS: There was no statistical difference in the accuracy of pedicle screw placement between two groups. However, in the apical region (the apex ± 2 vertebrae), the accuracy of safe pedicle screw placement (grades 0, 1) was significantly higher in the intraoperative navigation group than in the preoperative navigation group (94.8 vs 89.2%, respectively; P = 0.035). Intraoperative navigation significantly diminished medial perforation compared to preoperative navigation (P = 0.027), and the number of screws per vertebra that could be placed in the apical region was significantly higher in intraoperative navigation group (P < 0.001). In addition, the time required for the registration procedure and insertion of one pedicle screw was 11.3 ± 2.1 min in the preoperative group, but significantly decreased to 5.1 ± 1.1 min in the intraoperative group (P < 0.001). CONCLUSIONS: Both preoperative CT-based and intraoperative navigation systems provide sufficient accuracy and safety in pedicle screw insertion for AIS surgery. Intraoperative navigation systems facilitate pedicle screw insertion in the apical region and reduce registration time during AIS surgery which improves the efficacy and accuracy of pedicle screw insertion.

Cervical Spine Osteoradionecrosis.

Osteoradionecrosis (ORN), a well-known complication of radiotherapy in the mandibular bone, is very rare in the cervical spine. The authors report the result of a 3-year follow-up of a 63-year-old female patient with ORN of the cervical spine. The patient had a history of laryngeal carcinoma and was treated with chemotherapy and radiation therapy with a total of 120 Gy. Eight years later, she developed acute, severe neck pain due to cervical spine necrosis. The authors performed vascularized fibular bone graft and posterior pedicle screw fixation to reconstruct her cervical spine. The patient was successfully treated with surgery, and cervical alignment was preserved. She had neither neurological deficits nor severe neck pain at her final follow-up 3 years later. Delaying treatment of ORN may be life threatening, so the early diagnosis of this condition is important for patients who receive radiotherapy. Otolaryngologists and spine surgeons should understand this potential complication to speed diagnosis and treatment as early as possible.

Scoliosis in Patients with Severe Cerebral Palsy: Three Different Courses in Adolescents.

Patients with cerebral palsy (CP) frequently present with scoliosis; however, the pattern of curve progression is difficult to predict. We aimed to clarify the natural course of the progression of scoliosis and to identify scoliosis predictors. This was a retrospective, single-center, observational study. Total of 92 CP patients from Asahikawasou Ryouiku Iryou Center in Okayama, Japan were retrospectively analyzed. Cobb angle, presence of hip dislocation and pelvic obliquity, and Gross Motor Function Classification System (GMFCS) were investigated. Severe CP was defined as GMFCS level IV or V. The mean observation period was 10.7 years. Thirtyfour severe CP patients presented with scoliosis and were divided into 3 groups based on their clinical courses: severe, moderate and mild. The mean Cobb angles at the final follow-up were 129°, 53°, and 13° in the severe, moderate, and mild groups, respectively. The average progressions from 18 to 25 years were 2.7°/year, 0.7°/year, and 0.1°/year in the severe, moderate, and mild curve groups, respectively. We observed the natural course of scoliosis and identified 3 courses based on the Cobb angle at 15 and 18 years of age. This method of classification may help clinicians predict the patients' disease progression.

Short-term outcomes of patients being treated for chronic intractable pain at a liaison clinic and exacerbating factors of prolonged pain after treatment.

BACKGROUND: Although a multidisciplinary approach is often recommended to treat intractable pain, this approach does not completely prevent uncontrolled pain in some patients. The aim of this retrospective study was to investigate the exacerbating factors of prolonged, intractable pain among patients being treated at a pain liaison clinic. METHODS: The participants of this study were 94 outpatients (32 men, 62 women) with chronic intractable pain who visited our hospital between April 2013 and February 2015. Demographic and clinical information was obtained from all patients at baseline. Experts in various fields, including anesthesia, orthopedic surgery, psychiatry, physical therapy, and nursing, were involved in the treatment procedures. All patients were assessed before and after a 6-month treatment period using the following measures: the Numeric Rating Scale (NRS); the Pain Catastrophizing Scale (PCS); the Hospital Anxiety and Depression Scale (HADS); the Pain Disability Assessment Scale (PDAS); and the Oswestry Disability Index (ODI). All participants were then divided into two groups based on their self-reported pain after treatment: a pain relief group (n = 70) and a prolonged pain group (n = 24). The exacerbating factors of prolonged pain after treatment in the pain liaison outpatient clinic were analyzed using univariate and multiple logistic regression analysis. RESULTS: A significant improvement in NRS scores was observed after the 6-month follow-up period. After treatment, 24 (25.5%) of the 94 patients reported having prolonged pain. Significant improvements were seen in the PCS, PDAS, and ODI scores in the pain relief group, and in the HADS depression scores in the prolonged pain group. On univariate and multiple regression analysis, HADS depression scores were identified as a factor related to prolonged pain after treatment. CONCLUSIONS: The results of the present study suggest that severe depression at the initial visit to the liaison outpatient clinic was an exacerbating factor for prolonged pain after treatment.

Denosumab and alendronate treatment in patients with back pain due to fresh osteoporotic vertebral fractures.

INTRODUCTION: Denosumab specifically inhibits the receptor activator for nuclear factor-kappa B ligand (RANKL), and prevents osteoporotic fractures. Several reports have analyzed the effects of denosumab and alendronate alone on bone mineral density (BMD) or reduction of fracture risk. The objective of this study was to analyze the effects of antiresorptive osteoporosis pharmacotherapy on pain relief in patients with fresh vertebral fracture. METHODS: This retrospective, single-center study included 80 patients (10 males, 70 females) with fresh osteoporotic vertebral fractures treated using denosumab at a dose of 60 mg subcutaneously every 6 months (40 patients) or alendronate at a dose of 35 mg orally every week (40 patients) for 6 months in our hospital. The mean age of subjects was 77 years (range, 55-92 years). The primary outcome was duration of back pain. Secondary outcomes included changes in BMD, serum type 1 collagen cross-linked N-telopeptide (NTX), and serum N-terminal propeptide of type 1 collagen (P1NP) from baseline to 6 months. Pain catastrophizing due to back pain was assessed using the Pain Catastrophizing Scale (PCS). The incidences of further vertebral fracture and adverse events were also assessed. RESULTS: Pain relief was obtained at a mean of 3.3 weeks with denosumab and 5.4 weeks with alendronate. Pain relief was achieved significantly earlier with denosumab than with alendronate. At 6 months, change in BMD was higher with denosumab (6.1%) than with alendronate (0.8%). No significant differences in changes in NTX and P1NP were observed between groups. Scores for PCS were significantly lower for denosumab than for alendronate. The incidence of further vertebral fractures was 5% with denosumab and 10% with alendronate. Adverse event rates were similar between groups. CONCLUSIONS: Denosumab enabled earlier pain relief than alendronate and avoided catastrophizing in patients with osteoporotic vertebral fractures after 6 months of treatment.

Transuncovertebral joint screw placement: technical note.

PURPOSE: Although a C2 pedicle screw and a C1-2 transarticular screw are the most rigid anchors, these screws cannot be used in cases with bilateral high-riding vertebral arteries. The authors describe their recent experience using a novel method of C2-3 transuncovertebral joint screw placement for occipitocervical fixation. METHODS: A 67-year-old patient suffered myelopathy due to instability at C1-2. The patient had bilateral high-riding vertebral arteries that precluded the use of a C2 pedicle screw or a C1-2 transarticular screw. RESULTS: A C2-3 transuncovertebral joint screw was applied bilaterally under 3D navigation guidance. The patient's postoperative course was uneventful, and his neurological status improved after the surgery. Bony fusion was achieved after the surgery. CONCLUSIONS: This is the first report to describe the technique of transuncovertebral joint screw. Using a C2-3 transuncovertebral joint screw, a long screw could be used, and it provided an anchor at C3 and C2 from a posterior approach.

Carbon-ion radiotherapy of spinal osteosarcoma with long-term follow.

PURPOSE: Primary spinal osteosarcoma is quite rare, and the 5-year survival rate is very low. Because of its rarity, successful treatment experience with spinal osteosarcoma is limited. The purpose of this study is to report the effect of therapy of primary osteosarcoma of spine by carbon-ion radiotherapy (CIRT) and long-term follow. METHODS: A 70-year-old with primary spinal osteosarcoma who received CIRT underwent combined anterior artificial vertebral body replacement and posterior lumbar fusion (L1-L5) 3 years later. RESULTS: According to the surgical resection of tumoral lesion, pathological results showed that the intertrabecular space previously filled with tumor cells on the initial biopsy sample now contained necrotic tissue without tumor cells. This means that primary osteosarcoma of the spine was completely eliminated and achieved local control with CIRT, with a 7-year follow-up after the initial treatment. CONCLUSIONS: Carbon ion beam treatment is an effective local treatment for patients with spinal osteosarcoma for whom surgical resection is not a feasible option, especially for elderly patients. However, more patients need to be evaluated over a longer term to assess the curative effect of CIRT.

Laminar closure rates in patients with cervical myelopathies treated with either open-door laminoplasty with reattachment of spinous processes and extensor musculature or Hirabayashi open-door laminoplasty: a case-control study.

PURPOSE: The presence of preoperative kyphosis has been identified as a risk factor for laminar closure (LC). We have been performing modified open-door laminoplasty with reattachment of the spinous processes and extensor musculature for patients with reduced cervical lordosis because this procedure can prevent progression of cervical kyphosis. This procedure may also prevent LC. The purpose of this study was to evaluate the rate of LC after the modified open-door laminoplasty. METHODS: A total of 104 patients who underwent open-door laminoplasty were retrospectively reviewed. Of these patients, 18 underwent modified open-door laminoplasty (group M), whereas the other 86 underwent Hirabayashi open-door laminoplasty (group H). Group H was divided into two classes according to the preoperative cervical lordosis angle (group H1 ≤ 10° vs. group H2 ≥ 11°). All 18 patients in group M had a preoperative lordosis angle ≤10°. The outcomes were assessed using the rate of LC, the cross-sectional area (CSA) of the erector muscle on magnetic resonance imaging, and the Japanese Orthopaedic Association score (JOA score). RESULTS: The rate of laminar closure was significantly lower in group M (39 %) than in group H1 (76 %) (P < 0.01). The CSAs tended to be larger in the muscle preservation side of group M than in the other groups. There was no significant difference in the JOA score among the three groups. CONCLUSIONS: Although patients with decreased cervical lordosis have a higher risk of LC, the results suggested that modified open-door laminoplasty could reduce the rate of LC compared with Hirabayashi open-door laminoplasty.

Neurological Recovery after Posterior Spinal Surgery in Patients with Metastatic Epidural Spinal Cord Compression.

Metastatic epidural spinal cord compression (MESCC) is a common complication in patients with a malignant tumor, but it is difficult to decide the proper time to perform the necessary surgery. Here we analyzed the prognostic factors for postoperative walking ability. We retrospectively reviewed the cases of 112 MESCC patients treated surgically at our institute and divided them into ambulatory (n＝ 88) and non-ambulatory (n＝24) groups based on their American Spinal Injury Association (ASIA) Impairment Scale grades at the final follow-up. We also classified the patients preoperatively using the revised Tokuhashi score. We assessed the correlation between preoperative or intraoperative factors and postoperative walking ability in both groups. Of the 10 patients classified preoperatively as grade A or B, 2 (20% ) were ambulatory at the final follow-up. Of the 102 patients classified preoperatively as grade C, D or E, 86 (84% ) were ambulatory at the final follow-up (p＜0.001). There were no significant differences between the groups in the average total Tokuhashi score. Our analysis revealed that the severity of paralysis significantly affects neurological recovery in patients with MESCC. Patients with MESCC should receive surgery before the preoperative ASIA Impairment Scale grade falls below grade C.

Biomechanical Comparison of Posterior Fixation Using Spinal Instrumentation and Conventional Posterior Plate Fixation in Unstable Vertical Sacral Fracture.

Vertical sacral fracture is one of the most difficult fractures to treat. Posterior fixation using spinal dual rods is a novel method for treating this fracture, but its biomechanical strength has not yet been reported. The aim of this study was to evaluate the biomechanical strength produced by posterior fixation using spinal instrumentation. Sacral fractures were created in eight pelvic bone models and classifi ed into a posterior plate fixation group [P group, n＝4] and a spinal instrumentation group [R group, n＝4]. The biomechanical strength was tested by pushing down on the S1 vertebra from the top. The mean maximum loads were 1,057.4 N and 1,489.4 N in the P and R groups, respectively (p＝ 0.014). The loads applied to the construct at displacements of 5mm and 7.5mm from the start of the universal testing machine loading were also significantly higher in the R group. The mean stiffness levels in the P and R groups were 88.3N/mm and 119.6N/mm, respectively (p＝0.014). Posterior fixation using spinal instrumentation is biomechanically stronger than conventional posterior plate fixation. This procedure may be the optimal method for treating unstable sacral fracture fixation.

Scoliosis correction surgery for patients with McCune-Albright syndrome using pedicle screws: a report of two cases with different characteristics and a review of the literature.

PURPOSE: Scoliosis can occur secondary to McCune-Albright syndrome (MAS); it can be progressive and sometimes requires surgical treatment. It is still unclear if pedicle screw (PS) fixation in these patients with poor bone quality can be considered an effective treatment for scoliosis. The purpose of this study is to report two MAS patients with spinal fibrous dysplasia (FD) who underwent scoliosis surgeries with the PS system. METHODS: Case 1: a 12-year-old girl. Standing posteroanterior radiographs revealed a 58° right curve from T7 to L2. Computed tomography (CT) showed small areas of FD throughout the spine. A posterior spinal arthrodesis from T4 to L3 using PS fixation was performed with a CT-based navigation system. Case 2: a 26-year-old woman. Radiographs in the standing position revealed a right 87° curve from T8 to L2 and a 55° kyphosis from T8 to T12. CT images showed multiple areas of severe spinal FD causing angular deformity and collapse of vertebral bodies. The patient underwent posterior spinal arthrodesis from T8 to her pelvis using a CT-based navigation system for PS fixation. RESULTS: Superior scoliosis corrections were obtained using PS instrumentation, attaining complete bony union in both cases without major complications. However, Case 2 had some technical difficulties in treating due to the multiple large FD lesions. CONCLUSIONS: PS fixation can be considered an effective treatment for correcting scoliosis and maintaining the correction at follow-up in MAS patients with poor bone quality. However, great care must be taken when performing correction and follow-up.

A rare course of scoliosis associated with Chiari malformation and syringomyelia.

Spinal deformity is an important clinical manifestation of Chiari I malformation (CM-I) and syringomyelia. Here we report the result of an 8-year follow-up of a 13-year-old girl with severe scoliosis associated with Chiari malformation and a large syringomyelia. The patient presented at our hospital at the age of 13 with a 68° scoliosis. Magnetic resonance imaging showed Chiari malformation and a large syringomyelia. Neurosurgical treatment involved foramen magnum decompression and partial C1 laminectomy, but the scoliosis still progressed. We present the first case report of a rare course of scoliosis in a patient with CM-I and a large syringomyelia.