Radiological factors affecting functional outcome after the implantation of BDYN™ dynamic stabilization system for low-grade lumbar degenerative spondylolisthesis.

BACKGROUND: The objectives of this study are to identify radiological factors associated with good functional outcomes after the implantation of BDYN™ dynamic stabilization system in the setting of painful low-grade degenerative lumbar spondylolisthesis (DLS). MATERIAL AND METHODS: In this monocentric, retrospective study, we followed 50 patients, a 5-years period, with chronic lower back pain, radiculopathy and/or neurogenic claudication evolving for at least one year that failed conservative treatment. All patients presented low-grade DLS and underwent lumbar dynamic stabilization. Radiological and clinical outcomes were assessed preoperatively and 24months after surgery. Functional evaluation was based on the Oswestry Disability Index (ODI), the Numerical Rating Scale (NRS), and the Walking Distance (WD). Radiological analysis was based on lumbar X-rays and MRI parameters. Patients were divided into two groups according to the reduction in the postoperative ODI score (more or less than 15 points), and statistical analysis was performed between both groups to find predictive radiological factors for a satisfying functional outcome. RESULTS: Clinically, 80% (40 patients) had a satisfying functional result, and 20% (10 patients) were considered having a poor outcome according to the ODI score. Radiologically, the loss in segmental lordosis was statistically associated with bad functional outcomes (18° for ODI decrease>15 versus 11° for ODI decrease<15). There is also a tendency showing that a higher Pfirmann disc signal grade (grade IV) and a severe canal stenosis according to Schizas classification (grade C & D) are predictive of a poor clinical result, but that must be confirmed in future studies. CONCLUSIONS: BDYN™ appears safe and well-tolerated. This new device should be effective for the treatment of patients with low-grade DLS. It provides significant improvement in terms of daily life activity and pain. Moreover, we have been able to deduce that a kyphotic disc is associated with a bad functional outcome after BDYN™ device implantation. It may represent a contraindication for the implantation of such DS device. Moreover, it seems that it is better to implant BDYN™ in DLS with mild or moderate disc degeneration and canal stenosis.

Do Age-Related Variations Of Sagittal Alignment Rely On SpinoPelvic Organization? An Observational Study Of 1540 Subjects.

STUDY DESIGN: Descriptive radiographic analysis of a prospective multi-center database. OBJECTIVE: This study aims to provide normative values of spinopelvic parameters and their correlations according to age and pelvic incidence (PI) of subjects without spinal deformity. METHODS: After Institutional Review Board (IRB) approval, 1540 full spine radiographs were analyzed. Subjects were divided into 3 groups of PI: low PI < 45°, intermediate PI 45-60°, high PI > 60°, and then stratified by age (20-34, 35-49, 50-64, > 65 Y.O). Pelvic and spinal parameters were measured. Statistical analysis between parameters was performed using Bayesian inference and correlation. RESULTS: Mean age was 53.5 years (845 females, 695 males, range 20-93 years).In low PI group, lumbar lordosis (LL) decrease was mainly observed in the 2 younger age groups.In medium and high PI groups, loss of lordosis was linear during aging and occurred mainly on the distal arch of lordosis. Moderate PI group had a stable lordosis apex and thoracolumbar inflection point. High PI group had a stable thoracolumbar inflection point and a more distal lordosis apex in elderly subjects.For all subjects, kyphosis and pelvic tilt (PT) increased with age.There was a constant chain of correlation between PI and age groups. Proximal lumbar lordosis (PLL) was correlated with kyphosis and sagittal vertical axis (SVA C7), while the distal lumbar lordosis (DLL) was correlated with PI and PT. CONCLUSION: This study provides a detailed repository of sagittal spinopelvic parameters normative values with detailed analysis of segmental kyphosis and lordosis distribution according to gender, age, and PI.

Can Lumbopelvic Parameters Be Used to Predict Thoracic Kyphosis at all Ages? A National Cross-Sectional Study.

STUDY DESIGN: National cross-sectional study. BACKGROUND: Thoracic kyphosis (TK) is related to sagittal parameters as pelvic tilt (PT), lumbar lordosis (LL) and pelvic incidence (PI). The equation TK =2 (PT+LL-PI) was validated for adolescents.Objective: to investigate if this equation correctly predicts TK regardless of age. METHODS: Sagittal alignment parameters were assessed on full spine radiographs of 2599 individuals without spine pathology (1488 females, 1111 males). Calculated TK (CTK) = 2 (PT+LL-PI) and measured TK (MTK) were compared by calculating the gap and using a linear regression between both parameters. Subgroup analyses were performed for gender, age, TK groups (≤20°, 21°-40°, 41°-60°, >60°), and PI groups (<45°, 45°-60°, >60°). RESULTS: Average values in the total population were: MTK 45.0°, CTK 36.9°. Average TK gap was 8.1°, 5.2° in females (intercept 11.7, slope .61) and 11.9° in males (intercept 7.1, slope .58). The mean gap was 3.6° for 15-34 years, 5.7° under 15 years and it increased progressively after 35 years with a maximum of 19.9° over 80 years. The gap also increased with the amount of MTK: -3.5° for TK<20° up to 17.3° for TK >60°. Differences in gaps were minor between PI groups. The intercept was smallest and slopes >.6 for PI <45° and TK ≤20°. CONCLUSION: The formula TK=2 (PT+LL-PI) yielded moderate accuracy for adolescents and young adults, but did not fit for over 35 years and under 15. The amount and variance in TK increased in elderly subjects, which made the formula less accurate.

Comparison of Spinopelvic Configuration and Roussouly Alignment Types Between Pediatric and Adult Populations.

STUDY DESIGN: Retrospective cross-sectional study. OBJECTIVE: The aim was to describe spinopelvic alignment types by pelvic incidence (PI) and age to compare the Roussouly classification between pediatric and adult populations. SUMMARY OF BACKGROUND DATA: The Roussouly classification was validated for adults. Alignment types may vary during growth. MATERIALS AND METHODS: Radiographs of 1706 non pathologic individuals (5-49 yr) were analyzed. Individuals ≤19 years were stratified by chronological age and skeletal maturity (triradiate cartilage, Risser), and compared with adults. Global and spinopelvic alignment parameters were assessed. Roussouly Types 1, 2, 3, 3A (anteverted pelvis), and 4 were determined. The distribution of parameters was analyzed by Bayesian inference. The relationship between PI and age by Roussouly type was modeled by linear regression. RESULTS: The Sagittal Vertical Axis C7 decreased during growth and was significantly smaller in adults (20-34 yr) (Pr>0.99). Thoracic kyphosis and lumbar lordosis increased during growth and were larger in adults (Pr<0.025). Lordosis increased mainly in the cranial arch (Pr<0.025). PI and pelvic tilt increased during growth and were larger in adults (Pr<0.025). In children and adolescents, PI<45° represented the largest proportion, significantly larger compared with adults (Pr>0.99). Proportions of Roussouly Types 1 and 2 were similar throughout ages. Types 3 and 4 were rarer during the prepubertal period (Pr<0.025). The proportion of Type 3A was significantly higher in children and adolescents (Pr>0.99). Linear regression showed that Type 4 had the largest PI increase with age, with significantly higher curve slope compared with other types (Pr>0.9999). Types 3, 3A and 2 had similar slopes and lowest PI increase with age. CONCLUSION: Global and spinopelvic alignment changed during childhood and adolescence, leading to different kyphosis and lordosis distribution compared with adults. Growth-related PI increase influenced Roussouly types with typical predominance of Type 3A in the pediatric population and larger PI increase in Type 4. LEVEL OF EVIDENCE: Level III.

CT-Guided Aspiration of a Hemorrhagic Tarlov Cyst for the Treatment of a Post-Partum Sciatica: A Case Report and a Review of the Literature.

Background: Tarlov or perineural cysts are dilations of nerve roots resulting from a pathologically increased cerebrospinal fluid pressure. Although it is very common in the general population, most of these cysts remain asymptomatic. In some cases, they can evolve and exert pressure on neural elements, independently from their initial size. Case report: In this paper, we describe the case of a 33-year-old female known to have asymptomatic multiple and large radicular and pelvic Tarlov cysts. One cyst located in the right pelvic space progressed acutely after delivery, inducing a painful sciatica without neurological deficit. The intracystic bleeding can be a direct consequence of the delivery, leading to an acute and mechanical local compression of the right S1 root. A CT-guided puncture and aspiration allowed a complete recovery. This case report was completed by a review of the literature of these rare intracystic Tarlov bleedings. Conclusions: Intracystic hemorrhage is a rare complication of Tarlov cysts. Delivery-induced cyst bleeding was not described before. Patients known to have large and multiple Tarlov cysts should be monitored in post-partum, as their presence is considered a risk factor. Percutaneous cyst aspiration seems to be an effective and safe treatment to relieve symptoms.

Fatigue in Multiple Sclerosis: A Review of the Exploratory and Therapeutic Potential of Non-Invasive Brain Stimulation.

Fatigue is the most commonly reported symptom in patients with multiple sclerosis (MS). It is a worrisome, frequent, and debilitating manifestation that could occur at any time during the course of MS and in all its subtypes. It could engender professional, familial, and socioeconomic consequences and could severely compromise the patients' quality of life. Clinically, the symptom exhibits motor, cognitive, and psychosocial facets. It is also important to differentiate between perceived or subjective self-reported fatigue and fatigability which is an objective measure of decrement in the performance of cognitive or motor tasks. The pathophysiology of MS fatigue is complex, and its management remains a challenge, despite the existing body of literature on this matter. Hence, unraveling its neural mechanisms and developing treatment options that target the latter might constitute a promising field to explore. A PubMed/Medline/Scopus search was conducted to perform this review which aims (a) to reappraise the available electrophysiological studies that explored fatigue in patients with MS with a particular focus on corticospinal excitability measures obtained using transcranial magnetic stimulation and (b) to assess the potential utility of employing neuromodulation (i.e., non-invasive brain stimulation techniques) in this context. A special focus will be put on the role of transcranial direct current stimulation and transcranial magnetic stimulation. We have provided some suggestions that will help overcome the current limitations in upcoming research.

Variation of Global Sagittal Alignment Parameters According to Gender, Pelvic Incidence, and Age.

STUDY DESIGN: Retrospective cross-sectional study. OBJECTIVE: The aim was to describe existing global sagittal alignment parameters across ages and to analyze differences according to gender and pelvic incidence (PI). SUMMARY OF BACKGROUND DATA: Variability with age has been reported. It remains unclear how gender and spinopelvic morphology could additionally influence global alignment parameters. MATERIALS AND METHODS: Radiographs of 2599 individuals (5-93 y) were analyzed. Translation parameters were: Sagittal Vertical Axis (SVA)-C7, SVA-C2, SVA-Center Acoustic Meatus (CAM), C7/Sacrofemoral Distance (SFD) ratio. Inclination parameters were: C7-Vertical Tilt (VT), T1-VT and T9-VT, Odontoid-Hip Axis (OD-HA), OD-CAM. Pelvic compensation parameters were: T1-Pelvic Angle (TPA), Global Tilt (GT), Spino-Sacral Angle (SSA). Global sagittal alignment (GSA) was considered among formulae. The distribution of parameters was analyzed using a Bayesian inference. Correlations with spinopelvic parameters were investigated. RESULTS: SVA-C7, SVA-C2, SVA-CAM were larger in males and high PI, and increased significantly after 50 years (Pr>0.9999). C7/SFD decreased during growth and was larger in low PI (Pr=0.951). There was no correlation with spinopelvic parameters. Age-related variations of inclination parameters were nonsignificant. T1-VT and T9-VT increased with PI and were significantly larger in high PI (Pr>0.95). C7-VT was significantly larger in low PI (Pr>0.9999). OD-HA and OD-CAM were constant and increased after 80 years. TPA and GT increased with PI (Pr>0.9999) and age after 35 years (Pr>0.9999). SSA decreased nonsignificantly after 50 years. TPA correlated with PI (ρ=0.6130) and pelvic tilt (PT) (ρ=0.8375). GT correlated with PI (ρ=0.5961) and PT (ρ=0.8996). SSA correlated with sacral slope (ρ=0.9026). GSA was larger in high PI (Pr>0.9999) and increased after 35 years (Pr>0.9999). GSA correlated with PT (ρ=0.7732). CONCLUSION: Translation parameters increase with age, more prominently in males and high PI. Variations of inclination parameters are smaller. Pelvic compensation parameters and GSA increase with age and are closely related to PT and spinopelvic morphology. LEVEL OF EVIDENCE: Level III.

Ten Years After SINS: Role of Surgery and Radiotherapy in the Management of Patients With Vertebral Metastases.

The objective of the different types of treatments for a spinal metastasis is to provide the best oncological and functional result with the least aggressive side effects. Initially created in 2010 to help clinicians in the management of vertebral metastases, the Spine Instability Neoplastic Score (SINS) has quickly found its place in the decision making and the treatment of patients with metastatic spinal disease. Here we conduct a review of the literature describing the different changes that occurred with the SINS score in the last ten years. After a brief presentation of the spinal metastases' distribution, with or without spinal cord compression, we present the utility of SINS in the radiological diagnosis and extension of the disease, in addition to its limits, especially for scores ranging between 7 and 12. We take this opportunity to expose the latest advances in surgery and radiotherapy concerning spinal metastases, as well as in palliative care and pain control. We also discuss the reliability of SINS amongst radiologists, radiation oncologists, spine surgeons and spine surgery trainees. Finally, we will present the new SINS-derived predictive scores, biomarkers and artificial intelligence algorithms that allow a multidisciplinary approach for the management of spinal metastases.

Variation of cervical sagittal alignment parameters according to gender, pelvic incidence and age.

PURPOSE: The aim was to describe radiographic cervical sagittal alignment variations according to age, gender and pelvic incidence (PI) and to investigate relationships with thoracic alignment. METHODS: A total of 2599 individuals (5-93 years) without spinal deformity were studied. Cranial cervical parameters were: McGregor slope, occipita-C2 angle, McGregor-C2 lordosis and C1-C2 lordosis. Caudal cervical parameters were: C2-C7, cranial arch and caudal arch lordosis and C7- and T1-slope. A Bayesian inference compared parameter distributions. Correlations with spinopelvic and global alignment parameters were investigated. RESULTS: Among cranial cervical parameters, variations of McGregor slope were non-significant. McGregor-C2 lordosis and C1-C2 lordosis were smaller in males and increased significantly during growth, whereas the occipito-C2 angle decreased (Pr > 0.95). The occipito-C2 angle was larger and McGregor-C2 lordosis was smaller in low PI (Pr > 0.95). Among caudal cervical parameters, C2-C7 lordosis and C7- and T1-slope were larger in males and increased after 50 years (Pr > 0.95). Lordosis changes were non-significant in the cranial arch, whereas values increased in the caudal arch after 35 years (Pr > 0.95). Caudal parameter differences were non-significant between PI groups. Strong correlations existed between C2-C7, caudal arch lordosis, C7-slope, T1-slope and thoracic kyphosis. The sagittal vertical axis C2 correlated with caudal arch lordosis and T1-slope (ρ > 0.5; Pr > 0.95). CONCLUSION: Cervical alignment parameters vary according to age, gender and PI. In the cranial cervical spine, changes occur mainly during growth. In the caudal cervical spine, lordosis increases in the caudal arch, which is related to thoracic kyphosis increase with age. The caudal cervical arch acts as a compensatory segment by progressive extension, allowing horizontal gaze.

Spinal meningiomas, from biology to management - A literature review.

Meningiomas arise from arachnoidal cap cells of the meninges, constituting the most common type of central nervous system tumors, and are considered benign tumors in most cases. Their incidence increases with age, and they mainly affect females, constituting 25-46% of primary spinal tumors. Spinal meningiomas could be detected incidentally or be unraveled by various neurological symptoms (e.g., back pain, sphincter dysfunction, sensorimotor deficits). The gold standard diagnostic modality for spinal meningiomas is Magnetic resonance imaging (MRI) which permits their classification into four categories based on their radiological appearance. According to the World Health Organization (WHO) classification, the majority of spinal meningiomas are grade 1. Nevertheless, they can be of higher grade (grades 2 and 3) with atypical or malignant histology and a more aggressive course. To date, surgery is the best treatment where the big majority of meningiomas can be cured. Advances in surgical techniques (ultrasonic dissection, microsurgery, intraoperative monitoring) increase the complete resection rate. Operated patients have a satisfactory prognosis, even in those with poor preoperative neurological status. Adjuvant therapy has a growing role in treating spinal meningiomas, mainly in the case of subtotal resection and tumor recurrence. The current paper reviews the fundamental epidemiological and clinical aspects of spinal meningiomas, their histological and genetic characteristics, and their management, including the various surgical novelties and techniques.

Case Report: A Rare Case of Fourth Ventricle to Spinal Subarachnoid Space Shunt Migration: Surgical Pearl and Literature Review.

Background: In the event of syringomyelia communicating with the fourth ventricle, a fourth ventricle to cervical subarachnoid space shunting could be proposed. Case Report: In this review article, we describe the case of a 40-year-old woman who had a previously implanted fourth ventricle to spinal subarachnoid space shunt for the treatment of syringomyelia in the context of Chiari syndrome. The catheter migrated intradurally to the lumbosacral space, but in the absence of neurological repercussions, we decided to leave it in place. Conclusions: To the best of our knowledge, this is the first case described in the literature review of a catheter migration in the subarachnoid space from occipitocervical to lumbosacral level.

Real-time Brain Tumor imaging with endogenous fluorophores: a diagnosis proof-of-concept study on fresh human samples.

The primary line of therapy for high-grade brain tumor is surgical resection, however, identifying tumor margins in vivo remains a major challenge. Despite the progress in computer-assisted imaging techniques, biopsy analysis remains the standard diagnostic tool when it comes to delineating tumor margins. Our group aims to answer this challenge by exploiting optical imaging of endogenous fluorescence in order to provide a reliable and reproducible diagnosis close to neuropathology. In this study, we first establish the ability of two-photon microscopy (TPM) to discriminate normal brain tissue from glioblastomas and brain metastasis using the endogenous fluorescence response of fresh human brain sample. Two-photon fluorescence images were compared to gold standard neuropathology. "Blind" diagnosis realized by a neuropathologist on a group of TPM images show a good sensitivity, 100%, and specificity, 50% to discriminate non tumoral brain tissue versus glioblastoma or brain metastasis. Quantitative analysis on spectral and fluorescence lifetime measurements resulted in building a scoring system to discriminate brain tissue samples.

Extent of resection and Carmustine wafer implantation safely improve survival in patients with a newly diagnosed glioblastoma: a single center experience of the current practice.

For newly diagnosed glioblastomas treated with resection in association with the standard combined chemoradiotherapy, the impact of Carmustine wafer implantation remains debated regarding postoperative infections, quality of life, and feasibility of adjuvant oncological treatments. To assess together safety, tolerance and efficacy of Carmustine wafer implantation and of extent of resection for glioblastoma patients in real-life experience. Observational retrospective monocentric study including 340 consecutive adult patients with a newly diagnosed supratentorial glioblastoma who underwent surgical resection with (n = 123) or without (n = 217) Carmustine wafer implantation as first-line oncological treatment. Carmustine wafer implantation and extent of resection did not significantly increase postoperative complications, including postoperative infections (p = 0.269, and p = 0.446, respectively). Carmustine wafer implantation and extent of resection did not significantly increase adverse events during adjuvant oncological therapies (p = 0.968, and p = 0.571, respectively). Carmustine wafer implantation did not significantly alter the early postoperative Karnofsky performance status (p = 0.402) or the Karnofsky performance status after oncological treatment (p = 0.636) but a subtotal or total surgical resection significantly improved those scores (p < 0.001, and p < 0.001, respectively). Carmustine wafer implantation, subtotal and total resection, and standard combined chemoradiotherapy were independently associated with longer event-free survival (adjusted Hazard Ratio (aHR), 0.74 [95% CI 0.55-0.99], p = 0.043; aHR, 0.70 [95% CI 0.54-0.91], p = 0.009; aHR, 0.40 [95% CI 0.29-0.55], p < 0.001, respectively) and with longer overall survival (aHR, 0.69 [95% CI 0.49-0.96], p = 0.029; aHR, 0.52 [95% CI 0.38-0.70], p < 0.001; aHR, 0.58 [95% CI 0.42-0.81], p = 0.002, respectively). Carmustine wafer implantation in combination with maximal resection, followed by standard combined chemoradiotherapy is safe, efficient, and well-tolerated in newly diagnosed supratentorial glioblastomas in adults.

Multimodal optical analysis discriminates freshly extracted human sample of gliomas, metastases and meningiomas from their appropriate controls.

Delineating tumor margins as accurately as possible is of primordial importance in surgical oncology: extent of resection is associated with survival but respect of healthy surrounding tissue is necessary for preserved quality of life. The real-time analysis of the endogeneous fluorescence signal of brain tissues is a promising tool for defining margins of brain tumors. The present study aims to demonstrate the feasibility of multimodal optical analysis to discriminate fresh samples of gliomas, metastases and meningiomas from their appropriate controls. Tumor samples were studied on an optical fibered endoscope using spectral and fluorescence lifetime analysis and then on a multimodal set-up for acquiring spectral, one and two-photon fluorescence images, second harmonic generation signals and two-photon fluorescence lifetime datasets. The obtained data allowed us to differentiate healthy samples from tumor samples. These results confirmed the possible clinical relevance of this real-time multimodal optical analysis. This technique can be easily applied to neurosurgical procedures for a better delineation of surgical margins.

Multimodal optical analysis of meningioma and comparison with histopathology.

Meningioma is the most frequent primary central nervous system tumor. The risk of recurrence and the prognosis are correlated with the extent of the resection that ideally encompasses the infiltrated dura mater and, if required, the infiltrated bone. No device can deliver real-time intraoperative histopathological information on the tumor environment to help the neurosurgeon to achieve a gross total removal. This study assessed the abilities of nonlinear microscopy to provide relevant and real-time data to help resection of meningiomas. Nine human meningioma samples (four World Health Organization Grade I, five Grade II) were analyzed using different optical modalities: spectral analysis and imaging, lifetime measurements, fluorescence lifetime imaging microscopy, fluorescence emitted under one- and two-photon excitation and the second-harmonic generation signal imaging using a multimodal setup. Nonlinear microscopy produced images close to histopathology as a gold standard. The second-harmonic generation signal delineated the collagen background and two-photon fluorescence underlined cell cytoplasm. The matching between fluorescence images and Hematoxylin and Eosin staining was possible in all cases. Grade I meningioma emitted less autofluorescence than Grade II meningioma and Grade II meningioma exhibited a distinct lifetime value. Autofluorescence was correlated with the proliferation rates and seemed to explain the observed differences between Grade I and II meningiomas. This preliminary multimodal study focused on human meningioma samples confirms the potential of tissue autofluorescence analysis and nonlinear microscopy in helping intraoperatively neurosurgeons to reach the actual boundaries of the tumor infiltration. Correspondence between H&E staining (top pictures) and the two-photon fluorescence imaging (bottom pictures).

Spontaneous Regression of a Third Ventricle Colloid Cyst.

BACKGROUND: Colloid cysts represent 0.5%-1% of intracranial tumors and most commonly occur in the third ventricle near the Monro foramen. Although benign, if the lesion obstructs the foramen abruptly, sudden death may ensue. Evolution of these cysts is poorly understood. Spontaneous regression has been reported in only 2 other cases. Management of such cysts depends on whether the cyst continues to grow, its location, and clinical presentation. Incidental asymptomatic colloid cysts are typically followed with neuroimaging surveillance. CASE DESCRIPTION: We present a case of an incidental third ventricle colloid cyst in a 46-year-old patient who was managed conservatively with neuroimaging surveillance. Thereafter, she started developing some working memory deficits and intermittent headaches, with the cyst volume increasing, leading to the decision to perform a resection. However, the cyst underwent spontaneous regression before the scheduled surgery date, 3 years after initial diagnosis. CONCLUSIONS: This case confirms that some colloid cysts could regress spontaneously. Disappearance of the cyst is not necessarily accompanied by clinical worsening, as was reported by other investigators who noted deteriorating neurologic deficits as a result of worsening hydrocephalus. Assuming the cysts rupture, some patients tolerate the contents of the cyst leaking into the ventricular system, whereas others may mount an inflammatory reaction, causing a disruption in cerebrospinal fluid flow. In addition, it is still unclear what factors increase the likelihood of cysts to suddenly rupture.

An Unusual Case of Constitutional Mismatch Repair Deficiency Syndrome With Anaplastic Ganglioglioma, Colonic Adenocarcinoma, Osteosarcoma, Acute Myeloid Leukemia, and Signs of Neurofibromatosis Type 1: Case Report.

BACKGROUND AND IMPORTANCE: Constitutional mismatch repair deficiency (CMMRD) syndrome is a disorder with recessive inheritance caused by biallelic mismatch repair gene mutations, in which mismatch repair defects are inherited from both parents. This syndrome is associated with multiple cancers occurring in childhood. The most common tumors observed with CMMRD include brain tumors, digestive tract tumors, and hematological malignancies. The aim of this study was to report new phenotypic expressions of CMMRD syndrome and add new insight to the existing knowledge about this disease. A review of the literature was conducted and recommendation for surveillance and follow-up in patients with CMMRD are proposed. CLINICAL PRESENTATION: We report for the first time in the literature, the case of a 22-year-old female patient who was diagnosed with CMMRD syndrome, with the development of 2 unusual tumors: an anaplastic ganglioglioma and an osteosarcoma. She presented initially with an anaplastic ganglioglioma and later developed several malignancies including colonic adenocarcinoma, osteosarcoma, and acute myeloid leukemia. The patient had an atypical course of her disease with development of the initial malignancy at an older age and a remarkably long survival period despite developing aggressive tumors. CONCLUSION: Many aspects of this disease are still unknown. We identified a case of CMMRD in a patient presenting with an anaplastic ganglioglioma, who underwent successful surgical resection, chemotherapy, and radiotherapy and has had one of the longest survival periods known with this disease. This case broadens the tumor spectrum observed with CMMRD syndrome with anaplastic ganglioglioma and osteosarcoma as new phenotypic expressions of this genetic defect.

Combining intraoperative carmustine wafers and Stupp regimen in multimodal first-line treatment of primary glioblastomas.

BACKGROUND: The study investigated if intraoperative use of carmustine wafers, particularly in combination with Stupp regimen, is a viable and safe first-line treatment option of glioblastomas. METHODS: Eighty-three consecutive adult patients (50 men; mean age 60 years) with newly diagnosed supratentorial primary glioblastomas that underwent surgical resection with intraoperative carmustine wafers implantation (n = 7.1 ± 1.7) were retrospectively studied. RESULTS: The median overall survival (OS) was 15.8 months with 56 patients dying over the course of the study. There was no significant association between the number of implanted carmustine wafers and complication rates (four surgical site infections, one death). The OS was significantly longer in Stupp regimen patients (19.5 months) as compared with patients with other postoperative treatments (13 months; p = 0.002). In addition patients with eight or more implanted carmustine wafers survived longer (24.5 months) than patients with seven or less implanted wafers (13 months; p = 0.021). Finally, regardless of the number of carmustine wafers, median OS was significantly longer in patients with a subtotal or total resection (21.5 months) than in patients with a partial resection (13 months; p = 0.011). CONCLUSIONS: The intraoperative use of carmustine wafers in combination with Stupp regimen is a viable first-line treatment option of glioblastomas. The prognostic value of this treatment association should be evaluated in a multicenter trial, ideally in a randomized and placebo-controlled one.

Surgical Management of Spinal Synovial Cysts: A Series of 23 Patients and Systematic Analysis of the Literature.

STUDY DESIGN: Retrospective cohort and review of the literature. OBJECTIVE: To compare surgical strategies for the management of spinal synovial cysts. SUMMARY OF BACKGROUND DATA: The recent multiplication of retrospective series of patients with spinal synovial cysts has led to a reappraisal of their incidence and clinical significance. Although surgery is considered the treatment of choice, there is still no agreement over which surgical technique should be used. METHODS: We retrospectively reviewed 23 consecutive patients undergoing surgery for a spinal synovial cyst in our department between 2004 and 2010. Surgical procedures were classified into the following 4 categories: cystectomy by an interlaminar approach, hemilaminectomy, laminectomy, or associated with instrumented spinal fusion. Clinical outcome, cyst recurrence, need for subsequent fusion, and perioperative complications were compared between these groups. RESULTS: Of the patients included in the present cohort, 11 underwent cyst excision by an interlaminar approach, 8 had a hemilaminectomy, 2 had a laminectomy, and 2 underwent instrumented fusion. "Excellent" or "good" clinical outcome on the Macnab modified scale were achieved for 16 patients (69.6%), and there were 2 perioperative complications, 2 cyst recurrences, and 1 secondary fusion. Of the 519 patients reported in the literature, overall clinical outcome was either "excellent" or "good" for 83% of all patients. However, unfavorable outcome was more likely in patients treated with decompression alone (80/396) than decompression with fusion (10/123) (20.2% vs. 8.1%; P=0.003) and cyst recurrence (13/396 vs. 0/123; P=0.028). In contrast, the rate of perioperative complications was significantly higher in the fusion group (23/123) compared with decompression alone (11/396) (18.7% vs. 2.8%; P<0.0001). CONCLUSIONS: In patients with spinal synovial cyst, spinal fusion seems to decrease the risk of unfavorable clinical outcome and cyst recurrence and associated with a considerably higher rate of perioperative complications.