Intraspinal Abnormalities Research Articles

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

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. 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. 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. 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. Diagnostic Level III. See Instructions for Authors for a complete description of levels of evidence.

Intra-Operative Neurophysiological Monitoring in Patients with Intraspinal Abnormalities Undergoing Posterior Spinal Fusion.

ObjectiveTo analyze the intraoperative neurophysiological monitoring (IONM) data of patients with intraspinal abnormalities undergoing posterior spinal fusion and to determine how intraspinal abnormalities influence IONM results.MethodsPatients with severe kyphoscoliosis and intraspinal abnormalities who underwent posterior spinal correction and fusion between September 2015 and January 2019 were retrospectively reviewed. Candidate intraspinal abnormalities included Chiari malformation, syringomyelia, split cord malformation, and tethered cord syndrome. Total intravenous anesthesia was administered, and no muscle relaxant or inhalation anesthesia was used for maintenance. IONM data, including somatosensory evoked potentials (SSEP) and motor evoked potentials (MEP), were recorded. The P37 and N50 latencies and amplitude were recorded for SSEP, whereas only the amplitude was recorded for MEP. The possible high‐risk factors for abnormal IONM results were analyzed.ResultsThe current study included 87 patients (40 men, 47 women) with an average age of 20.2 ± 10.4 years. The etiologies were neuromuscular in 45 patients, idiopathic in four, and congenital in 38. A total of 136 intraspinal abnormalities were detected, including Chiari malformation in 33 patients, syringomyelia in 55, split‐cord malformation in 25, and tethered cord syndrome in 23. Forty patients had one intraspinal abnormality, whereas 47 patients had two or three intraspinal abnormalities. The monitorabilities were 87.4% and 97.7% for the SSEP and MEP, respectively. SSEP alerts were reported in five patients and MEP alerts in four patients, and new neurological deficits were observed in three patients postoperatively. The sensitivity and specificity were 100% and 97.3% for SSEP, and 100% and 98.8% for MEP, respectively. A significant difference in MEP amplitude between the concave and convex sides was observed, while significantly higher SSEP latency was observed on the concave side in patients with preoperative neurological deficits. There were 52 (59.8%) patients with abnormal IONM data. Preoperative neurological deficits (χ2 = 7.715, p = 0.005) and more than one intraspinal abnormality (χ2 = 9.186, p = 0.004) indicated a higher risk of abnormal IONM data.ConclusionsIONM can be effectively used in patients with intraspinal abnormalities who undergo posterior spinal fusion. Patients with preoperative neurological deficits and more than one intraspinal abnormality have a higher risk of abnormal IONM monitoring.

Neurosurgical management of patients with neurocutaneous melanosis: a systematic review.

Neurocutaneous melanocytosis (NCM), also referred to as neurocutaneous melanosis, is a rare neurocutaneous disorder characterized by excess melanocytic proliferation in the skin, leptomeninges, and cranial parenchyma. NCM most often presents in pediatric patients within the first 2 years of life and is associated with high mortality due to proliferation of melanocytes in the brain. Prognosis is poor, as patients typically die within 3 years of symptom onset. Due to the rarity of NCM, there are no specific guidelines for management. The aims of this systematic review were to investigate approaches toward diagnosis and examine modern neurosurgical management of NCM. A systematic review was performed using the PubMed database between April and December 2021 to identify relevant articles using PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. Search criteria were created and checked independently among the authors. Inclusion criteria specified unique studies and case reports of NCM patients in which relevant neurosurgical management was considered and/or applied. Exclusion criteria included studies that did not report associated neurological diagnoses and neuroimaging findings, clinical reports without novel observations, and those unavailable in the English language. All articles that met the study inclusion criteria were included and analyzed. A total of 26 extracted articles met inclusion criteria and were used for quantitative analysis, yielding a cumulative of 74 patients with NCM. These included 21 case reports, 1 case series, 2 retrospective cohort studies, 1 prospective cohort study, and 1 review. The mean patient age was 16.66 years (range 0.25-67 years), and most were male (76%). Seizures were the most frequently reported symptom (55%, 41/74 cases). Neurological diagnoses associated with NCM included epilepsy (45%, 33/74 cases), hydrocephalus (24%, 18/74 cases), Dandy-Walker malformation (24%, 18/74 cases), and primary CNS melanocytic tumors (23%, 17/74 cases). The most common surgical technique was CSF shunting (43%, 24/56 operations), with tethered cord release (4%, 2/56 operations) being the least frequently performed. Current management of NCM includes CSF shunting to reduce intracranial pressure, surgery, chemotherapy, radiotherapy, immunotherapy, and palliative care. Neurosurgical intervention can aid in the diagnosis of NCM through tissue biopsy and resection of lesions with surgical decompression. Further evidence is required to establish the clinical outcomes of this rare entity and to describe the diverse spectrum of intracranial and intraspinal abnormalities present.

Imaging and Clinical Features of Neurocutaneous Melanosis in the Pediatric Population.

Neurocutaneous Melanosis (NCM) is a rare nonfamilial phakomatosis characterized by the presence of congenital melanocytic nevi and abnormal melanocyte infiltration of the leptomeninges. This paper shows the importance of early diagnosis and the most important imaging features of the disease on CT and MR scans. PubMed database was searched from January 1972 to September 2020. Papers including imaging findings of NCM, clinical, follow-up, and treatment features were collected, selecting only 89 studies. NCM is a term used for the first time by van Bogaert in 1948. It refers to a condition caused by an error during morphogenesis and migration leading to leptomeningeal melanocytic accumulation. Although histological findings are the gold standard for diagnosis confirmation, neuroimaging and clinical features strongly support the suspect of NCM. Localization and extension of the lesions are predictive of neurological manifestations related to increased intracranial pressure, mass lesions, or spinal cord compression. CT demonstrates sites of increased density in the anterior temporal lobe, mainly the amygdala, thalami, cerebellum, and frontal lobes base. However, MRI is the best imaging method to diagnose central nervous system lesions, often appearing as T1-short signal areas of the cerebral parenchyma, indicative of central nervous system melanosis. MRI can also reveal associated intracranial and intraspinal abnormalities. Early imaging, when available, is helpful if NCM suspect is raised and may be of guidance in comparing later studies. NCM requires a multidisciplinary approach since it is a multisystem disease with a genetic component.

New Association between Idiopathic Scoliosis and Luckenschadel Skull (Lacunar Skull)

Objective: Luckenschadel skull is a skull that is radiologically characterized by lacunae in the cranial vault. To date, although the association between neurological abnormalities and scoliosis is well recognized, no relationship between idiopathic scoliosis and a lacunar skull has been defined. We explored the incidence and time courses of lacunar skulls in patients with idiopathic scoliosis. Materials and Methods: Spinal X-rays of 3,170 children aged 6–16 years with idiopathic scoliosis evaluated from October 2010 to August 2020 were examined for the presence of an irregular inner calvarial table indicative of a Luckenschadel skull. A total of 1,760 (55.5%) of the 3,170 images included the skull. We also explored the frequency of intraspinal abnormalities in children with lacunar skull images who underwent spinal magnetic resonance imaging. Results: The study population consisted of 1,760 children, 1,203 girls (68.4%) and 557 (31.6%) boys. A total of 268 (15.2%) clearly evidenced lacunar skulls in their radiographs; 186 (69.4%) girls (11.3 ± 4.3 years) and 82 (30.6%) boys (12.6 ± 3.3 years). Two of the 56 patients (3.6%) who underwent spinal MRI had intraspinal abnormalities (isolated Chiari malformation-I). No additional neurological problems were detected in children with lacunar skulls. Conclusion: We conclude that the lacunar skull is very common in children with idiopathic scoliosis who lack any other neurological pathology. The lacunar skull does not disappear even in adolescence. Although previous publications have stated that lacunar skull disappears over time in radiographic images, we observed that it became more noticeable over time in children with scoliosis.

Intraspinal anomalies: presentation, assessment, treatment and association with the development of scoliosis

The most common congenital intraspinal abnormalities presenting to medical attention is either a Chiari malformation with or without associated syringomyelia or caudal anomalies, such as tethered cord and fatty filum terminale. These intraspinal defects are usually diagnosed at birth or in early childhood, but they may also be identified in adult life due to newly presented neurological symptoms. Availability and use of appropriate imaging modalities, such as MRI and regular fetal screening has reduced the incidence of delayed diagnosis of spinal dysraphism and other accompanying congenital systemic anomalies. In view of the risk for neurological complications, prophylactic surgery is often recommended in spinal dysraphism; hence knowledge of various intraspinal abnormalities, their clinical presentation and natural history will allow early diagnosis and treatment in order to prevent neurological deterioration and irreversible morbidity. This review summarizes the embryological aspects and clinical manifestations of intraspinal abnormalities and provides a rational approach for evaluation and management in this group of patients.

Incidence of intraspinal abnormalities in congenital scoliosis: a systematic review and meta-analysis

ObjectiveThis is the first systematic review and meta-analysis on the overall incidence of intraspinal abnormalities in patients with congenital scoliosis (CS) and potential influencing factors.MethodsWe searched three large electronic databases (PubMed, EMBASE, and Cochrane Library) for potentially relevant studies. The quality of the included studies was assessed independently by two authors using the Methodological Index for Non-Randomized Studies (MINORS) criteria. Data on the number of CS patients, number of CS patients with intraspinal abnormalities, sex of the patients, and CS types were extracted from the included studies. R software was used to pool and analyze all the extracted data.ResultsThis meta-analysis included 10 articles, and 671 of 1863 CS patients undergoing magnetic resonance imaging (MRI) examinations were identified to have intraspinal abnormalities. The overall incidence of intraspinal abnormalities in the patients with CS was 37% (95% CI, 29–45%). Diastematomyelia was the most common intraspinal abnormality and was detected in 45.60% of the patients with intraspinal abnormalities (306/671). The remaining intraspinal abnormalities included syringomyelia (273/671, 40.69%), tethered cord (190/671, 28.32%), low conus (58/671, 8.64%), intraspinal mass (39/671, 5.81%), Chiari malformation (32/671, 4.77%), fatty filum (27/671, 4.02%), spina bifida (occulta excluded) (17/671, 2.53%), tumor (17/671, 2.53%), cyst (12/671, 1.79%), syringomyelus (4/671, 0.60%), dural ectasia (1/671, 0.15%), and undiagnosed cord MRI hyperintensity (1/671, 0.15%). The patient’s sex and CS type were not factors that affected the incidence of intraspinal abnormalities in CS patients (all P > 0.05).ConclusionsThis meta-analysis revealed that the overall incidence of intraspinal abnormalities detected by MRI in CS patients was 37%. Diastematomyelia was the most common intraspinal abnormality. The patient’s sex and CS type were not factors that affected the incidence of intraspinal abnormalities in CS patients.

Effect of simultaneous surgical treatment in scoliosis associated with intraspinal abnormalities: A retrospective study.

To evaluate the outcomes of single-stage surgical treatment for spinal deformity and coexisting intraspinal pathologies, 12 patients who underwent single-stage surgical treatment for spinal deformity and co-existing intraspinal abnormalities between October 2016 and January 2017 were enrolled in the present study. Treatment for intraspinal abnormalities, posterior correction, osteotomy and internal fixation were performed simultaneously. The clinical and radiological outcomes, surgical details, complications and postoperative outcomes were evaluated. The mean fusion length was 11.0±2.8. Both scoliosis Cobb angle (pre-surgery 65.9±13.4 vs. post-surgery 21.7±9.4) and kyphosis (pre-surgery 71.1±19.5 vs. post-surgery 31.4±10.4) were significantly improved post-surgery. Tethered cords were released and epidermoid cysts, gangliogliomas, meningiomas and lipomas were resected. Muscle strength in all patients was improved. The muscular tone of 8 patients was improved. No severe complications occurred postoperatively. None of the patients experienced deterioration in their neurological status nor loss of correction during the 12-24 months' follow-up. The simultaneous surgical treatment for spinal deformity and intraspinal pathology seems to be a safe and effective approach. Neurological deficits were improved postoperatively. Osteotomy produces satisfactory correction results.

The safety and efficacy of one-stage posterior surgery in the treatment of presumed adolescent idiopathic scoliosis associated with intraspinal abnormalities a minimum 3-year follow-up comparative study.

Adolescent idiopathic scoliosis (AIS) is a common type of idiopathic scoliosis. Previous studies reported that the incidence of intraspinal abnormalities among the presumed idiopathic scoliosis was 13-43%. Intraspinal abnormalities were also considered increasing the risks of progressing of scoliosis and neurological complications following scoliosis corrective surgery. The surgical strategy of presumed adolescent idiopathic scoliosis (PAIS) associated with intraspinal abnormalities remains controversial. The purpose of this study was to investigate whether one-stage posterior surgery safe and effective for the PAIS patients associated with intraspinal abnormalities. One hundred and thirteen consecutive patients who underwent one-stage posterior correction surgery were included. Thirty PAIS patients with intraspinal abnormalities without preoperative neurological symptoms were matched with eighty-three AIS patients for sex, age, blood loss, operating time, number of levels and location of instrumentation and curve magnitude. Radiographic and clinical parameters of the patients were evaluated before surgery, within 1 week after surgery, and more than 3 years at the last follow-up for complications and changes in main curve correction, global coronal balance, thoracic kyphosis, sagittal vertical axis, and ODI scores. On average, the duration of follow-up was 51.5months in the PAIS group compared to 52.5months in the AIS group. The preoperative mean major coronal curve was 79.6° (ranged 56.2°-106.7°) and improved to 22.4° (ranged 6.4°-58.1°) at the last follow-up for a 71.9% of correction in the AIS group. The preoperative mean major coronal curve was 80.4° (ranged 63.4°-108.1°) and improved to 23.2° (ranged 4.8°-66.2°) at the last follow-up for a 71.1% of correction in PAIS group. The preoperative ODI score was 32.4 (10-42) in the PAIS group and improved to 11.4 (4-22) at last follow-up, 33.4 (12-42) in the AIS group and improved to 11.5 (5-22) at last follow-up. The global coronal balance, TK and SVA were all significantly improved after surgery and maintained to the last follow-up in the two groups. The neurological complications were observed in 3.3% of PAIS patients and 3.6% of AIS patients. No statistical difference in the parameters between the two groups was observed at the last follow-up. One-stage posterior corrective surgery is safe and effective in PAIS patients associated with intraspinal abnormalities without preoperative neurological symptoms. Surgical guidelines of AIS are appropriate for the treatment of PAIS patients associated with intraspinal abnormalities.

MRI utilization and rates of abnormal pretreatment MRI findings in early-onset scoliosis: review of a global cohort.

Retrospective review OBJECTIVES: To report the frequency of pretreatment magnetic resonance imaging (MRI) utilization and rates and types of intra-spinal abnormalities identified on MRI in patients with early-onset scoliosis (EOS). MRI can help identify spinal cord abnormalities in patients with EOS. We reviewed data from patients enrolled from 1993-2018 in an international EOS registry. Patients with incomplete/unverifiable data and those with spinal deformities secondary to infection or tumor were excluded, leaving 1343 patients for analysis. Demographic characteristics, pretreatment major curve magnitude, treatment type, and MRI findings were analyzed. Patients were categorized by EOS type (congenital, idiopathic, neuromuscular, syndromic), pretreatment MRI utilization, and presence of intra-spinal abnormality on MRI. Univariate testing and multivariate logistic regression were performed to identify demographic, radiographic, and clinical predictors of MRI utilization and abnormal MRI findings. MRI was used in 836 patients (62%). Pretreatment MRI utilization rates ranged from 42% in neuromuscular EOS to 74% in congenital EOS. Prevalence of abnormal MRI findings was 24% overall, ranging from 13% in patients with idiopathic EOS to 39% in neuromuscular EOS. Compared with white/Caucasian patients, Asian/Asian-American patients had higher odds of MRI utilization and abnormal MRI findings. Treatment type, pretreatment major curve magnitude, age at MRI, and age at treatment were not associated with abnormal MRI findings. Overall, 249 abnormalities were identified in 197 patients. The most common findings were syrinx and tethered cord. Syrinx with Chiari malformation was the most frequent combination of abnormal findings. In the two-thirds of patients who underwent MRI before EOS treatment, findings were abnormal in 24%. EOS type and race/ethnicity were associated with both MRI utilization and abnormal findings. The most frequent abnormalities were syrinx and tethered cord, and the type of abnormalities appeared to differ by EOS type. Prognostic, Level III.

Determining the optimal timing of screening spinal cord ultrasonography to detect filum terminale lipoma in infants.

PurposeThe purpose of this study was to identify the optimal timing for screening spinal cord ultrasonography (US) to detect filum terminale lipoma in infants.MethodsWe retrospectively reviewed infants (<12 months old) who underwent repeated spinal cord US between April 2011 and January 2019. We excluded infants if they only had one US examination, or if they had lesions other than filum terminale lipoma. Infants with filum terminale lipoma on magnetic resonance imaging were included in the lipoma group and the others in the control group. A linear mixed model was used to assess differences in the growth pattern of filum terminale thickness by age and group. The cutoff thickness on US and its diagnostic performance were assessed according to age.ResultsAmong 442 infants with 901 US examinations, 46 were included in the lipoma group and 58 in the control group. Sixty-seven infants had unmeasurable filum terminale thickness on initial US, including 55 neonates (82.1%) before 1 month of age. The lipoma group had significantly greater filum terminale thickness than the control group (P<0.001). Thickness increased with age in the lipoma group (P=0.027). The sensitivity of US was 87.5% and the area under the receiver operating characteristic curve was 0.949 (95% confidence interval, 0.849 to 0.991) with a cutoff value of 1.1 mm in 4- to 6-month-old infants.ConclusionScreening spinal cord US could effectively diagnose filum terminale lipoma in 4- to 6-month-old infants with a cutoff thickness of 1.1 mm. Spinal cord US can be used to screen young infants with intraspinal abnormalities.

Frequency and characteristics of congenital intraspinal abnormalities in a cohort of 128 patients with congenital scoliosis.

Objectives:The aims of this study were to determine the incidence and main characteristics of associated intraspinal anomalies in patients with congenital scoliosis (CS) and to analyze the different factors that influence the curve progression.Design:This was a retrospective comparative study.Methods:This was a retrospective study of 128 patients with CS.Main Outcome Measurements:The incidence of the patients with intraspinal anomalies and their demographic, clinical, and radiological values was described.Results:Intraspinal anomalies were present in 13.3% of the patients. Among them, the most frequent anomaly was syringomyelia. The most frequent curve was the thoracic curve. The main deformity based on McMaster classification was formation failure. The curve progression during follow-up did not show significant differences between vertebral anomalies, syringomyelia, presence of thoracic anomalies, and gender (P > 0.05).Conclusions:Our study showed a lower percentage of spinal anomalies compared to other series. As other studies, the progression of the scoliosis curve in patients with spinal anomalies seems primarily to be determined by the type of vertebral malformation.Level of Evidence:Level II.

One stage surgical treatment for scoliosis associated with intraspinal abnormalities

Objective To evaluate the effectiveness and safety of one stage surgical treatment for scoliosis and coexisting intraspinal abnormalities. Methods The data of 6 patients who underwent one stage surgical treatment for scoliosis and coexisting intraspinal abnormalities from October 2016 to January 2017 were retrospectively analyzed. Treatment for intraspinal abnormalities, posterior correction, osteotomy and internal fixation were performed simultaneously. The clinical and radiologic presentations, operative details, complications and postoperative outcomes were evaluated. Results The success rate was 100%. The operating time was (470.83 ± 136.20) min and intraoperative bleeding amount was 1350 (625, 2150) ml. Total fusion segments were 11.00 ± 2.76. Both Cobb angle of scoliosis [postoperation (19.60 ± 5.94)° vs. preoperation (59.40 ± 14.31)°, P = 0.007] and kyphosis [postoperation (25.80 ± 10.87)° vs. preoperation (62.40 ± 21.04)°, P = 0.005] were improved after operation. Tethered cords were released and epidermoid cyst, ganglioglioma and lipoma were excised. Syringomyelia was left untreated. No neurological functional defect or worsening was found. Muscle strength of all patients was improved. Muscular tone of 4 patients and difficulty in urination of 5 patients were also improved. The mean hospital stay was (8.83 ± 3.31) d. No severe complications, such as infection, cerebrospinal fluid (CSF) leakage, failed internal fixation, fractured pedicle screws or rods occurred after operation. None of the patients died, or experienced deterioration of neurological function, delayed infection, pseudoarthrosis, or loss correction during the (7.50 ± 1.22) months follow - up. Conclusions The one stage surgical treatment for scoliosis and intraspinal abnormalities seems to be a safe and effective approach. Neurological functional defect can be improved after operation. Osteotomy can improve correction result. DOI: 10.3969/j.issn.1672-6731.2017.09.011

Intraspinal anomalies in early-onset idiopathic scoliosis.

In the United Kingdom, lower incidences of intraspinal abnormalities in patients with early onset idiopathic scoliosis have been observed than in studies in other countries. We aimed to determine the rates of these abnormalities in United Kingdom patients diagnosed with idiopathic scoliosis before the age of 11 years. This retrospective study of patients attending an urban scoliosis clinic identified 71 patients satisfying a criteria of: clinical diagnosis of idiopathic scoliosis; age of onset ten years and 11 months or less; MRI screening for intraspinal abnormalities. United Kingdom census data combined with patient referral data was used to calculate incidence. Mean age at diagnosis was six years with 39 right-sided and 32 left-sided curves. Four patients (5.6%) were found to have intraspinal abnormalities on MRI. These consisted of: two combined Arnold-Chiari type 1 malformations with syrinx; one syrinx with a low lying conus; and one isolated syrinx. Overall annual incidence of early onset idiopathic scoliosis was one out of 182 000 (0.0006%). This study reports the lowest rates to date of intraspinal anomalies in patients with early onset idiopathic scoliosis, adding to knowledge regarding current incidences of these abnormalities as well as any geographical variation in the nature of the disease. Cite this article: Bone Joint J 2017;99-B:829-33.

Congenital Scoliosis

ABSTRACT Congenital scoliosis is a scoliosis that occurs as a result of bony abnormalities that arise between 4 to 16 weeks of gestation. There are genetic and environmental factors involved in etiology. The majority of congenital vertebral anomalies are in the thoracic spine (64%). The patients often have associated skeletal anomalies and can also be part of a syndrome. Skeletal, cardiac, genitourinary intraspinal abnormalities are most commonly found in association with congenital scoliosis. The natural history seems to be related to the morphology of the bony the site in the spine, and the patient's age. Congenital scoliosis usually progresses and often requires intervention. The aim of intervention is to achieve a flexible and balanced spine with normal truncal height and space available for lung ratio. Early intervention for scoliosis seems to be instrumental in trying to achieve these goals. How to cite this article Jasani V. Congenital Scoliosis. J Postgrad Med Edu Res 2017;51(2):95-102.

Two-staged Correction of Severe Congenital Scoliosis Associated With Intraspinal Abnormalities.

A retrospective study. To analyze the efficacy and safety of perioperative halo-gravity traction as an adjunct to posterior vertebral column resection (PVCR) in the treatment of patients with severe congenital scoliosis and coexisting asymptomatic intraspinal pathologies (diastematomyelia and/or tethered cord). Few reports to our knowledge review the use of perioperative halo-gravity traction and PVCR in this patient population. A total of 17 patients with a minimum 2-year follow-up who underwent PVCR using perioperative halo-gravity traction were analyzed. Patients were analyzed by age at date of examination (range, 11-23 y; mean, 14.4 y), sex (7 male, 10 female), major coronal curve magnitude (range, 108-149 degrees; average, 125 degrees), major sagittal curve magnitude (range, 72-118 degrees; average, 91 degrees). Complications related to halo traction and PVCR were reviewed. Radiographic outcomes demonstrated Cobb angle of major curve had an average correction of 28% after halo traction and it measured 53 degrees (range, 42-84 degrees) at the last follow-up, for a 58% correction. Maximal kyphosis improved to 58 degrees (range, 43-76 degrees) at ultimate follow-up. There were no permanent neurological deficits in this series. The study results suggested that surgical treatment for intraspinal abnormality may be unnecessary in asymptomatic patients with severe congenital scoliosis who are undergoing scoliosis corrective surgery. PVCR combined with perioperative traction is a safe and effective alternative for such patients.

The prevalence of intraspinal anomalies in infantile and juvenile patients with “presumed idiopathic” scoliosis: a MRI-based analysis of 504 patients

BackgroundThough several studies have reported the incidence of intraspinal neural axis abnormalities in infantile and juvenile “presumed idiopathic” scoliosis, there has been a varying prevalence ranging from 11.1 to 26.0 % based on a limited sample size. Therefore, such inconclusive findings have resulted in some questions on the MRI-associated role in the management of these patients. We aimed to investigate the prevalence and distribution of intraspinal anomalies in the infantile and juvenile patients with “presumed idiopathic” scoliosis and to explore the radiographic and clinical indicators with large sample size.MethodsA total of 504 infantile and juvenile patients diagnosed with “presumed idiopathic” scoliosis were examined for potentially-existing neural axis abnormalities by MRI. Patients were grouped into two cohorts according to the presence of neural axis abnormalities. Radiographic parameters including curve magnitude, curve pattern, location of apex, degree of thoracic kyphosis, and span of curve were recorded and compared between the two groups. The prevalence of the neural abnormalities between the infantile-age group and juvenile-age group was also compared. The student t test was used to evaluate the differences of continuous variables and the chi-square test was used to evaluate the difference of categorical variables. Fisher exact test was applied to detect the difference of the rate of intraspinal anomalies between the “infantile idiopathic scoliosis” and “juvenile idiopathic scoliosis” group.ResultsInvolving the spinal cord, 94 patients (18.7 %) were found to have a neural abnormality: Arnold-Chiari malformation alone in 43 patients, Arnold-Chiari malformation combined with syringomyelia in 18 patients, isolated syringomyelia in 13 patients, diastematomyelia in six patients, tethered cord combined with diastematomyelia in six patients, tethered cord alone in four patients, and other uncommon intraspinal abnormalities in the remaining four patients. Totally Arnold-Chiari malformation with or without syringomyelia accounted for 64.8 % (61/94) among all these abnormalities. Male gender, left thoracic curve and right lumbar curve were found to be significantly associated with the presence of neural axis abnormalities on MRI.ConclusionsThe incidence of neural axis abnormalities in the presumed IIS and JIS was 18.7 %. Thus a routine MRI evaluation appears warranted for those “presumed idiopathic” scoliosis patients if aged less than 10 years, being male or having left thoracic or right lumbar curve.

Imaging features of split cord malformation associated with scoliosis and its correlation with neurologic symptoms

Objective To analyze the imaging features of congenital spinal deformity (CSD) associated with split cord malformation (SCM) and other intraspinal abnormalities, and to investigate the relationship to neurological symptoms. Methods 105 cases CSD with SCM were retrospectively studied. Analysis the imaging features of SCM (including type of SCM, location of SCM, location and apical vertebrae, symmetry of divided cord) and other intraspinal abnormalities. To investigate the relationship of the factors and neurological symptoms using Chi-square test of one factor and multiple factors logistic regression analysis. Results 28 cases (26.7%) were formation failure, 33 cases (31.4%) were segmentation failure, and 44 cases (41.9%) were combination of 2 disorders. 41 cases had neurological symptoms, 64 cases were asymptomatic. The distribution of SCM combined with spinal deformities: thoracic (11 cases), thoracolumbar (18 cases) and lumbar (20 cases) in type I SCM, thoracic (31 cases), thoracolumbar (20 cases) and lumbar (5 cases) in type II, none was in cervical. The location of SCM upper than apical vertebrae 29 cases, on apical vertebrae 25 cases, lower than apical vertebrae 51 cases. Spinal cord was splitted symmetric 27 cases and asymmetric 78 cases. 66 cases combined with other intraspinal abnormalities, lower conus 42 cases, syringomyelia 38 cases, meningocele 10 cases and sakrale zyste 5 cases. Associated with intraspinal abnormalities, the rate of neural symptoms was different. According to Chisquare test of one factor and multiple factors logistic regression analysis, lumbar SCM, spinal cord asymmetric and lower conus were related with neurological symptoms. Conclusion The predilection spinal deformity of type I is combination, type II SCM is segmentation failure. When SCM patients associated with other intraspinal abnormalities, the incidence of neurologic symptoms is increased. The lumbar SCM, hemicords asymmetry and lower lying conus have significant relationship with neurologic symptoms. Key words: Scoliosis; Neural tube defects; Syringomyelia; Spinal cord diseases

Vertebral and Intraspinal Anomalies in Indian Population with Congenital Scoliosis: A Study of 119 Consecutive Patients.

Study DesignRetrospective case study by clinical and radiological data analysis.PurposeTo analyze different types of vertebral anomalies and the incidence of associated intraspinal anomalies in the Indian population.Overview of LiteratureThis is the largest study of congenital scoliosis and associated intraspinal anomalies in Indian population. Incidence of intraspinal anomaly in this series is 47% which is higher than previous literature. Hemivertebra was the most common anomaly as seen in previous studies.MethodsA total of 119 patients with congenital scoliosis who underwent surgery between December 2006 and December 2012 were studied. Data was reviewed with medical records, plain radiographs, and magnetic resonance imaging (MRI) scans.ResultsThoracolumbar curve was most common, seen in 43.6% of patients. In addition to scoliotic deformity, kyphosis was seen in 26% of patients. Failure of formation, the most common vertebral anomaly, was seen in 51.2% of patients, failure of segmentation was seen in 19.3% of patients, and there were 29.4% patients having both formation and segmentation anomalies. Hemivertebra was the most common vertebral anomaly seen in 66.3% of patients and for whom 63.2% were in thoracic spine. Intraspinal anomalies were associated with 47% of patients with congenital scoliosis. Tethered cord was the most common intraspinal abnormality and was found in 48.2% patients with intraspinal anomalies. The patients with failure of segmentation and mixed deformities were found to have a significantly higher incidence of intraspinal anomalies (65% and 57%, respectively) than those with failure of formation (34%). Out of 31 patients with kyphotic deformity 29% had intraspinal anomalies, and amongst them tethered cord was the most common anomaly seen in 66% patients. Out of 12 patients with neurocutaneous markers, 83% patients had intraspinal anomaly.ConclusionsIntraspinal anomalies were seen in 47% of patients with congenital scoliosis in the Indian population. Tethered cord was the most common intraspinal anomaly, seen in 48% cases of congenital scoliosis. Hemivertebra was seen in 66% cases and was the most common vertebral defect. Hence MRI imaging of whole spine should be done in all cases of congenital scoliosis before any surgical intervention. Special attention should be given to physical examination and visualization of any neurocutaneous markers, which are associated with a higher incidence of intraspinal anomalies; absence of a neurocutaneous marker, however, does not rule out intraspinal anomaly.

Intraspinal lesions associated with sacrococcygeal dimples.

OBJECT.: Sacrococcygeal dimples in the gluteal fold, also known as coccygeal pits, are observed in 2%-4% of newborns. Sacrococcygeal dimples are not generally considered to be associated with a significant risk of intraspinal anomalies and therefore are not thought to require further radiographic evaluation. Accordingly, the precise incidence and nature of intraspinal lesions that may be associated with sacrococcygeal dimples is unclear. This study was conducted to determine the incidence of intraspinal lesions in patients with intergluteal dimples. In this study, the authors used MRI to evaluate 103 children who were seen at the Niigata University Medical and Dental Hospital between 2006 and 2011 because of skin abnormalities in the lumbosacral region. Of these children, 14 were excluded as having a subcutaneous fatty mass, and 5 were excluded because the dimples were above the gluteal fold or did not end at the coccyx. The remaining 84 patients were classified according to whether the bottom of the dimple was visible (shallow) or not (deep). The authors also retrospectively examined other skin abnormalities and coexisting anomalies. The mean age at the time of MRI evaluation was 11.7 months. Magnetic resonance imaging led to the identification of fibrolipoma of the terminal filum (FTF) in 14 cases (16.7%); 6 of these patients also had a low conus. Classified by depth, there were 58 cases with shallow and 26 with deep dimples. Fibrolipoma of the terminal filum was found in significantly more patients with deep dimples (9 [34.6%]) than in those with shallow dimples (5 [8.6%]). The frequency of other congenital anomalies was significantly higher in patients with FTF-associated dimples (6 [42.9%] of 14) than in those with dimples that were not associated with FTF (9 [12.9%] of 70). Fibrolipoma of the terminal filum was identified by MRI in 16.7% of patients with sacrococcygeal dimples. The risk of FTF increased when the dimples were deeply excavated or were accompanied by congenital anomalies. Magnetic resonance imaging should be performed to identify intraspinal lesions when there are high risk factors for intraspinal abnormalities, or when an ultrasound screening suggests intraspinal abnormalities.