Quantification of cervical spinal stenosis by automated 3D MRI segmentation of spinal cord and cerebrospinal fluid space.

DESIGN: Prospective diagnostic study. OBJECTIVES: Anatomical evaluation and graduation of the severity of spinal stenosis is essential in degenerative cervical spine disease. In clinical practice, this is subjectively categorized on cervical MRI lacking an objective and reliable classification. We implemented a fully-automated quantification of spinal canal compromise through 3D T2-weighted MRI segmentation. SETTING: Medical Center - University of Freiburg, Germany. METHODS: Evaluation of 202 participants receiving 3D T2-weighted MRI of the cervical spine. Segments C2/3 to C6/7 were analyzed for spinal cord and cerebrospinal fluid space volume through a fully-automated segmentation based on a trained deep convolutional neural network. Spinal canal narrowing was characterized by relative values, across sever segments as adapted Maximal Canal Compromise (aMCC), and within the index segment as adapted Spinal Cord Occupation Ratio (aSCOR). Additionally, all segments were subjectively categorized by three observers as "no", "relative" or "absolute" stenosis. Computed scores were applied on the subjective categorization. RESULTS: 798 (79.0%) segments were subjectively categorized as "no" stenosis, 85 (8.4%) as "relative" stenosis, and 127 (12.6%) as "absolute" stenosis. The calculated scores revealed significant differences between each category (p ≤ 0.001). Youden's Index analysis of ROC curves revealed optimal cut-offs to distinguish between "no" and "relative" stenosis for aMCC = 1.18 and aSCOR = 36.9%, and between "relative" and "absolute" stenosis for aMCC = 1.54 and aSCOR = 49.3%. CONCLUSION: The presented fully-automated segmentation algorithm provides high diagnostic accuracy and objective classification of cervical spinal stenosis. The calculated cut-offs can be used for convenient radiological quantification of the severity of spinal canal compromise in clinical routine.

Photon-Counting Computed Tomography (PC-CT) of the spine: impact on diagnostic confidence and radiation dose.

OBJECTIVES: Computed tomography (CT) is employed to evaluate surgical outcome after spinal interventions. Here, we investigate the potential of multispectral photon-counting computed tomography (PC-CT) on image quality, diagnostic confidence, and radiation dose compared to an energy-integrating CT (EID-CT). METHODS: In this prospective study, 32 patients underwent PC-CT of the spine. Data was reconstructed in two ways: (1) standard bone kernel with 65-keV (PC-CTstd) and (2) 130-keV monoenergetic images (PC-CT130 keV). Prior EID-CT was available for 17 patients; for the remaining 15, an age-, sex-, and body mass index-matched EID-CT cohort was identified. Image quality (5-point Likert scales on overall, sharpness, artifacts, noise, diagnostic confidence) of PC-CTstd and EID-CT was assessed by four radiologists independently. If metallic implants were present (n = 10), PC-CTstd and PC-CT130 keV images were again assessed by 5-point Likert scales by the same radiologists. Hounsfield units (HU) were measured within metallic artifact and compared between PC-CTstd and PC-CT130 keV. Finally, the radiation dose (CTDIvol) was evaluated. RESULTS: Sharpness was rated significantly higher (p = 0.009) and noise significantly lower (p < 0.001) in PC-CTstd vs. EID-CT. In the subset of patients with metallic implants, reading scores for PC-CT130 keV revealed superior ratings vs. PC-CTstd for image quality, artifacts, noise, and diagnostic confidence (all p < 0.001) accompanied by a significant increase of HU values within the artifact (p < 0.001). Radiation dose was significantly lower for PC-CT vs. EID-CT (mean CTDIvol: 8.83 vs. 15.7 mGy; p < 0.001). CONCLUSIONS: PC-CT of the spine with high-kiloelectronvolt reconstructions provides sharper images, higher diagnostic confidence, and lower radiation dose in patients with metallic implants. KEY POINTS: • Compared to energy-integrating CT, photon-counting CT of the spine had significantly higher sharpness and lower image noise while radiation dose was reduced by 45%. • In patients with metallic implants, virtual monochromatic photon-counting images at 130 keV were superior to standard reconstruction at 65 keV in terms of image quality, artifacts, noise, and diagnostic confidence.

Automated signal intensity analysis of the spinal cord for detection of degenerative cervical myelopathy - a matched-pair MRI study.

PURPOSE: Detection of T2 hyperintensities in suspected degenerative cervical myelopathy (DCM) is done subjectively in clinical practice. To gain objective quantification for dedicated treatment, signal intensity analysis of the spinal cord is purposeful. We investigated fully automated quantification of the T2 signal intensity (T2-SI) of the spinal cord using a high-resolution MRI segmentation. METHODS: Matched-pair analysis of prospective acquired cervical 3D T2-weighted sequences of 114 symptomatic patients and 88 healthy volunteers. Cervical spinal cord was segmented automatically through a trained convolutional neuronal network with subsequent T2-SI registration slice-by-slice. Received T2-SI curves were subdivided for each cervical level from C2 to C7. Additionally, all levels were subjectively classified concerning a present T2 hyperintensity. For T2-positive levels, corresponding T2-SI curves were compared to curves of age-matched volunteers at the identical level. RESULTS: Forty-nine patients showed subjective T2 hyperintensities at any level. The corresponding T2-SI curves showed higher signal variabilities reflected by standard deviation (18.51 vs. 7.47 a.u.; p < 0.001) and range (56.09 vs. 24.34 a.u.; p < 0.001) compared to matched controls. Percentage of the range from the mean absolute T2-SI per cervical level, introduced as "T2 myelopathy index" (T2-MI), was correspondingly significantly higher in T2-positive segments (23.99% vs. 10.85%; p < 0.001). ROC analysis indicated excellent differentiation for all three parameters (AUC 0.865-0.920). CONCLUSION: This fully automated T2-SI quantification of the spinal cord revealed significantly increased signal variability for DCM patients compared to healthy volunteers. This innovative procedure and the applied parameters showed sufficient diagnostic accuracy, potentially diagnosing radiological DCM more objective to optimize treatment recommendation. TRIAL REGISTRATION: DRKS00012962 (17.01.2018) and DRKS00017351 (28.05.2019).

Diffusion tensor imaging in unclear intramedullary tumor-suspected lesions allows separating tumors from inflammation.

DESIGN: Prospective diagnostic study. OBJECTIVES: Primary imaging-based diagnosis of spinal cord tumor-suspected lesions is often challenging. The identification of the definite entity is crucial for dedicated treatment and therefore reduction of morbidity. The aim of this trial was to investigate specific quantitative signal patterns to differentiate unclear intramedullary tumor-suspected lesions based on diffusion tensor imaging (DTI). SETTING: Medical Center - University of Freiburg, Germany. METHODS: Forty patients with an unclear tumor-suspected lesion of the spinal cord prospectively underwent DTI. Primary diagnosis was determined by histological or clinical work-up or remained indeterminate with follow-up. DTI metrics (FA/ADC) were evaluated at the central lesion area, lesion margin, edema, and normal spinal cord and compared between different diagnostic groups (ependymomas, other spinal cord tumors, inflammations). RESULTS: Mean DTI metrics for all spinal cord tumors (n = 18) showed significantly reduced FA and increased ADC values compared to inflammatory lesions (n = 8) at the lesion margin (p < 0.001, p = 0.001) and reduced FA at the central lesion area (p < 0.001). There were no significant differences comparing the neoplastic subgroups of ependymomas (n = 10) and other spinal cord tumors (n = 8), but remaining differences for both compared to the inflammation subgroup. We found significant higher ADC (p = 0.040) and a trend to decreased FA (p = 0.081) for ependymomas compared to inflammations at the edema. CONCLUSION: Even if distinct differentiation of ependymomas from other spinal cord neoplasms was not possible based on quantitative DTI metrics, FA and ADC were feasible to separate inflammatory lesions. This may avoid unnecessary surgery in patients with unclear intramedullary tumor-suspected lesions.

The challenge of measuring spinopelvic parameters: inter-rater reliability before and after minimally invasive lumbar spondylodesis.

BACKGROUND: The common manual measurement technique of spinal sagittal alignment on X-rays is susceptible to rater-dependent variability, which has not been adequately considered in previous publications. This study investigates the effect of those variations in the characterization of patients receiving lumbar spondylodesis. METHODS: General alignment parameters on pre- and postoperative X-rays were evaluated by four raters in 43 prospectively sampled patients undergoing monolevel spondylodesis. The Intra-class Correlation Coefficient (ICC) for each rater pair and all raters together was calculated for inter-rater reliability. For the operation-induced change of the sagittal alignment in every patient the Wilcoxon test was applied to compare for each rater separately. RESULTS: The ICCs were "good" (>0.75) to "excellent" (>0.9) for all raters together and for 45 of the 48 single rater pairs (93.75%). All revealed a significant increase of the addressed segmental lordosis and disc height and no significant change for spinopelvic parameters and sagittal vertical axis from pre- to postoperative. The lumbar lordosis showed a significant increase through the operation of +2.5° (p = 0.014) and +3.7° (p = 0.015) in two raters and no difference for the other ones (+2.1°, p = 0.171; -2.2°, p = 0.522). CONCLUSIONS: The pre- to postoperative change of lumbar lordosis revealed different significance levels for different raters, although the ICCs were formally good. Accordingly, the evaluation by only one rater would lead to different conclusions. Due to this susceptibility of alignment measurements to rater-dependent variability, the exact evaluation process should be described in every publication and the consistency of significant results be validated through multiple raters. TRIALS REGISTRATION: The trial was approved by the local ethics committee and listed at the national clinical trials register ( DRKS00004514 , date of registration: 08/11/2012).

Cranial facet joint injuries in percutaneous lumbar pedicle screw placement: a matched-pair analysis comparing intraoperative 3D navigation and conventional fluoroscopy.

PURPOSE: The violation of the cranial adjacent facet is a frequent complication in lumbar instrumentations and can induce local pain and adjacent segment disease. Minimally invasive screw implantation is often stated as risk factor in comparison with open approaches. Percutaneous pedicle screw placement (PPSP) can be performed using single X-ray images (fluoroscopy) or intraoperative 3D navigation. The study compares top-level screws in percutaneous lumbar instrumentations regarding facet violations and screw pedicle position using navigation or fluoroscopy. METHODS: Patients after lumbar PPSP were retrospectively separated according to the intraoperative technique: navigation (NAV) or fluoroscopy (FLUORO). Two blinded investigators graded the top-level screws regarding facet violations and pedicle breach in postoperative CT scans. Subsequent matched cohort analysis was performed for comparable groups. RESULTS: Evaluating 768 screws, we assessed 70 (9.1%) facet violations. Overall, 186 (24.2%) screws were implanted using navigation. There was no significant difference in the rate of facet violations between both imaging groups (NAV 19/186, 10.2%, FLUORO 51/582, 8.8%, p = 0.55). Totally, 728 (94.8%) of all screws showed a correct pedicle position. Most of the 40 unfavorable pedicle positions were placed by fluoroscopy (NAV 4/186, 2.2%, FLUORO 36/582, 6.6%, p = 0.03). The matched cohorts verified these results (facet violations: NAV 19/186, 10.2%, FLUORO 18/186, 9.7%, p = 0.55; pedicle penetrations: NAV 4/186, 2.2%, FLUORO 12/186, 6.9%, p = 0.04). CONCLUSIONS: Both intraoperative imaging techniques allow lumbar PPSP with low rates of cranial facet violations if the surgeon intends to preserve facet integrity. Navigation was superior concerning accurate pedicle screw position, but could not significantly prevent facet violations.

Radiation exposure of a mobile 3D C-arm with large flat-panel detector for intraoperative imaging and navigation - an experimental study using an anthropomorphic Alderson phantom.

BACKGROUND: Intraoperative 3-dimensional (3D) navigation is increasingly being used for pedicle screw placement. For this purpose, dedicated mobile 3D C-arms are capable of providing intraoperative fluoroscopy-based 3D image data sets. Modern 3D C-arms have a large field of view, which suggests a higher radiation exposure. In this experimental study we therefore investigate the radiation exposure of a new mobile 3D C-arm with large flat-panel detector to a previously reported device with regular flat-panel detector on an Alderson phantom. METHODS: We measured the radiation exposure of the Vision RFD 3D (large 30 × 30 cm detector) while creating 3D image sets as well as standard fluoroscopic images of the cervical and lumbar spine using an Alderson phantom. The dosemeter readings were then compared with the radiation exposure of the previous model Vision FD Vario 3D (smaller 20 × 20 cm detector), which had been examined identically in advance and published elsewhere. RESULTS: The larger 3D C-arm induced lower radiation exposures at all dosemeter sites in cervical 3D scans as well as at the sites of eye lenses and thyroid gland in lumbar 3D scans. At ​​male and especially female gonads in lumbar 3D scans, however, the larger 3D C-arm showed higher radiation exposures compared with the smaller 3D C-arm. In lumbar fluoroscopic images, the dosemeters near/in the radiation field measured a higher radiation exposure using the larger 3D C-arm. CONCLUSIONS: The larger 3D C-arm offers the possibility to reduce radiation exposures for specific applications despite its larger flat-panel detector with a larger field of view. However, due to the considerably higher radiation exposure of the larger 3D C-arm during lumbar 3D scans, the smaller 3D C-arm is to be recommended for short-distance instrumentations (mono- and bilevel) from a radiation protection point of view. The larger 3D C-arm with its enlarged 3D image set might be used for long instrumentations of the lumbar spine. From a radiation protection perspective, the use of the respective 3D C-arm should be based on the presented data and the respective application.

Hemangioblastoma and von Hippel-Lindau disease: genetic background, spectrum of disease, and neurosurgical treatment.

INTRODUCTION: Hemangioblastomas are rare, histologically benign, highly vascularized tumors of the brain, the spinal cord, and the retina, occurring sporadically or associated with the autosomal dominant inherited von Hippel-Lindau (VHL) disease. Children or adults with VHL disease have one of > 300 known germline mutations of the VHL gene located on chromosome 3. They are prone to develop hemangioblastomas, extremely rarely starting at age 6, rarely at age 12-18, and, typically and almost all, as adults. There is a plethora of VHL-associated tumors and cysts, mainly in the kidney, pancreas, adrenals, reproductive organs, and central nervous system. Due to a lack of causal treatment, alleviation of symptoms and prevention of permanent neurological deficits as well as malignant transformation are the main task. Paucity of data and the nonlinear course of tumor progression make management of pediatric VHL patients with hemangioblastomas challenging. METHODS: The Freiburg surveillance protocol was developed by combining data from the literature and our experience of examinations of > 300 VHL patients per year at our university VHL center. RESULTS: Key recommendations are to start screening of patients at risk by funduscopy with dilated pupils for retinal tumors with admission to school and with MRI of the brain and spinal cord at age 14, then continue biannually until age 18, with emergency MRI in case of neurological symptoms. Indication for surgery remains personalized and should be approved by an experienced VHL board, but we regard neurological symptoms, rapid tumor growth, or critically large tumor/cyst sizes as the key indications to remove hemangioblastomas. Since repeated surgery on hemangioblastomas in VHL patients is not rare, modern neurosurgical techniques should encompass microsurgery, neuronavigation, intraoperative neuromonitoring, fluorescein dye-based intraoperative angiography, intraoperative ultrasound, and minimally invasive approaches, preceded in selected cases by endovascular embolization. Highly specialized neurosurgeons are able to achieve a very low risk of permanent morbidity for the removal of hemangioblastomas from the cerebellum and spinal cord. Small retinal tumors of the peripheral retina can be treated by laser coagulation, larger tumors by cryocoagulation or brachytherapy. CONCLUSION: We consider management at experienced VHL centers mandatory and careful surveillance and monitoring of asymptomatic lesions are required to prevent unnecessary operations and minimize morbidity.

Polyglobulia in patients with hemangioblastomas is related to tumor size but not to serum erythropoietin.

BACKGROUND: Hemangioblastomas are associated with elevated hemoglobin (Hb) levels (polyglobulia), which is associated with a higher risk for cerebral stroke, cardiac infarction and pulmonary embolism. The pathomechanism of polyglobulia remains unclear and different theories have been postulated. Among those are elevated serum erythropoietin (EPO) levels caused by secretion of the tumor or associated tumor cyst. METHODS: To elucidate the pathomechanism, we systematically investigated the relation between polyglobulia, serum EPO level, size of the solid tumor and associated cyst in hemangioblastomas. We prospectively evaluated hemoglobin and EPO levels in a series of 33 consecutive patients operated on hemangioblastomas in our center. We measured the size of the solid tumor and associated cyst in magnetic resonance imaging. Statistical evaluations were performed using the Fisher's exact test and student's t-test. RESULTS: As a result five patients had elevated hemoglobin levels. Only one of these had an elevated serum EPO level. Of 26 patients with normal hemoglobin levels, 4 patients had elevated EPO levels.Patients with low or normal hemoglobin levels (84%) had an average tumor size of 0.8 cm3, which differed significantly from patients with elevated hemoglobin levels (16%), who had an average solid tumor size of 8.0 cm3 (p < 0.05). We did not observe a significant correlation between EPO levels or polyglobulia and associated cysts. CONCLUSIONS: We therefore conclude that in contrast to previous case reports and interpretations, our data show no correlation between polyglobulia and EPO levels or associated cysts in patients with hemangioblastomas. In fact, it is the size of the solid tumor that correlates with polyglobulia.The study was retrospectively registered in the German Clinical Trial Registry on 10 July 2014; Trial registration: DRKS00006310.

Giant central thoracic disc herniations: surgical outcome in 17 consecutive patients treated by mini-thoracotomy.

PURPOSE: Safe treatment of giant central thoracic disc herniations (cTDHs) remains a surgical challenge due to frequent calcifications, intradural extension and, importantly, the rare exposure of spine surgeons to these lesions. We report our 10-year experience in the management of giant cTDH by mini-thoracotomy and offer a detailed description of the technique. METHODS: 17 patients harboring 17 giant cTDH operated on via a mini-thoracotomy at the authors' institution between 2004 and 2014 were reviewed. All patients presented with myelopathy of varying magnitude. Mean patient age was 47 years. The mean follow-up period was 5.5 years. Median canal compromise of the cTDH was 66 %. cTDH were densely calcified in 7 (41 %), partially calcified in 6 (35 %) and soft in 4 (24 %) patients. Intradural extension of cTDH was noted in six patients (35 %). Benzels' modified myelopathy score of the Japanese Orthopedic Association was adjusted for the evaluation of thoracic myelopathy (mJOA) to assess functional outcomes. RESULTS: Successful removal of the offending cTDH was achieved in all patients. The overall mJOA Score improved from 7.9/13 to 11.1/13. Two patients with giant and densely calcified cTDH experienced a transient post-operative neurological decline. There was a statistically significant correlation between size of cTDH and intradural extension. CONCLUSION: Patients with myelopathy due to giant cTDH can be safely treated by the mini-thoracotomy approach. Postoperative neurological worsening and severe complications or incisional pain are rare. In contrast to complex posterior or thoracoscopic approaches, the mini-thoracotomy is technically straightforward and thus easy to learn for experienced spine surgeons.

Accidental Durotomy in Minimally Invasive Transforaminal Lumbar Interbody Fusion: Frequency, Risk Factors, and Management.

PURPOSE: To assess the frequency, risk factors, and management of accidental durotomy in minimally invasive transforaminal lumbar interbody fusion (MIS TLIF). METHODS: This single-center study retrospectively investigates 372 patients who underwent MIS TLIF and were mobilized within 24 hours after surgery. The frequency of accidental durotomies, intraoperative closure technique, body mass index, and history of previous surgery was recorded. RESULTS: We identified 32 accidental durotomies in 514 MIS TLIF levels (6.2%). Analysis showed a statistically significant relation of accidental durotomies to overweight patients (body mass index ≥ 25 kg/m(2); P = 0.0493). Patient age older than 65 years tended to be a positive predictor for accidental durotomies (P = 0.0657). Mobilizing patients on the first postoperative day, we observed no durotomy-associated complications. CONCLUSIONS: The frequency of accidental durotomies in MIS TLIF is low, with overweight being a risk factor for accidental durotomies. The minimally invasive approach seems to minimize durotomy-associated complications (CSF leakage, pseudomeningocele) because of the limited dead space in the soft tissue. Patients with accidental durotomy can usually be mobilized within 24 hours after MIS TLIF without increased risk. The minimally invasive TLIF technique might thus be beneficial in the prevention of postoperative immobilization-associated complications such as venous thromboembolism. This trial is registered with DRKS00006135.

Minimally Invasive Technique for PMMA Augmentation of Fenestrated Screws.

PURPOSE: To describe the minimally invasive technique for cement augmentation of cannulated and fenestrated screws using an injection cannula as well as to report its safety and efficacy. METHODS: A total of 157 cannulated and fenestrated pedicle screws had been cement-augmented during minimally invasive posterior screw-rod spondylodesis in 35 patients from January to December 2012. Retrospective evaluation of cement extravasation and screw loosening was carried out in postoperative plain radiographs and thin-sliced triplanar computed tomography scans. RESULTS: Twenty-seven, largely prevertebral cement extravasations were detected in 157 screws (17.2%). None of the cement extravasations was causing a clinical sequela like a new neurological deficit. One screw loosening was noted (0.6%) after a mean follow-up of 12.8 months. We observed no cementation-associated complication like pulmonary embolism or hemodynamic insufficiency. CONCLUSIONS: The presented minimally invasive cement augmentation technique using an injection cannula facilitates convenient and safe cement delivery through polyaxial cannulated and fenestrated screws during minimally invasive screw-rod spondylodesis. Nevertheless, the optimal injection technique and design of fenestrated screws have yet to be identified. This trial is registered with German Clinical Trials DRKS00006726.

PEEK cages versus PMMA spacers in anterior cervical discectomy: comparison of fusion, subsidence, sagittal alignment, and clinical outcome with a minimum 1-year follow-up.

PURPOSE: To compare radiographic and clinical outcomes after anterior cervical discectomy in patients with cervical degenerative disc disease using PEEK cages or PMMA spacers with a minimum 1-year follow-up. METHODS: Anterior cervical discectomy was performed in 107 patients in one or two levels using empty PEEK cages (51 levels), Sulcem PMMA spacers (49 levels) or Palacos PMMA spacers (41 levels) between January, 2005 and February, 2009. Bony fusion, subsidence, and sagittal alignment were retrospectively assessed in CT scans and radiographs at follow-up. Clinical outcome was measured using the VAS, NDI, and SF-36. RESULTS: Bony fusion was assessed in 65% (PEEK cage), 57% (Sulcem), and 46% (Palacos) after a mean follow-up of 2.5 years. Mean subsidence was 2.3-2.6 mm without significant differences between the groups. The most pronounced loss of lordosis was found in PEEK cages (-4.1°). VAS was 3.1 (PEEK cage), 3.6 (Sulcem), and 2.7 (Palacos) without significant differences. Functional outcome in the PEEK cage and Palacos group was superior to the Sulcem group. CONCLUSIONS: The substitute groups showed differing fusion rates. Clinical outcome, however, appears to be generally not correlated with fusion status or subsidence. We could not specify a superior disc substitute for anterior cervical discectomy. This trial is registered with DRKS00003591.

Associations of collagen type I α2 polymorphisms with the presence of intracranial aneurysms in patients from Germany.

OBJECTIVE: Subarachnoid hemorrhage from ruptured intracranial aneurysms is associated with a severe prognosis. Preventive treatment of unruptured intracranial aneurysms is possible and recommended. However, the identification of risk patients by genetic analyses is not possible because of lack of candidate genes. Collagen type I α2 (COL1A2) has been associated with the presence of aneurysms in patients from Japan, China, and Korea. In this study, we investigate whether COL1A2 is a possible aneurysm candidate gene in the German population. METHODS: Patients admitted with intracranial aneurysms to our department and collaborating departments were enrolled. Three single-nucleotide polymorphisms (SNPs) of the COL1A2 gene, namely rs42524 in exon 28, rs1800238 in exon 32, and rs2621215 in intron 46 were investigated using restriction enzymes and sequencing. HapMap data were used for comparison of allelic frequencies with the normal population by χ2 test to identify significant associations between genotypes and the presence of aneurysms. RESULTS: Two hundred sixty-nine patients were enrolled into the study. There was a significant correlation with the presence of aneurysms for the GC allele of the SNP rs42524 in exon 28 (P = .02). The other polymorphisms did not show significant correlations. CONCLUSIONS: The COL1A2 gene is associated with intracranial aneurysms in a subset of the German population. However, it is not responsible for the majority of aneurysms, and further candidate genes need to be identified to develop sensitive genetic screening for patients at risk.

Optic nerve and chiasm hemangioblastomas in von Hippel-Lindau disease: report of 12 cases and review of the literature.

Introduction: Optic nerve and chiasm hemangioblastomas are rare tumors, occurring sporadically or in the context of von Hippel-Lindau (VHL) disease. They have only been portrayed in isolated case reports and small cohorts. Their natural history and therapeutic strategies are only scarcely described. To better characterize these rare tumors, we retrospectively analyzed an optic nerve and chiasm hemangioblastoma series of 12 VHL patients. By combining our own experience to a review of all known cases in literature, we intended to create treatment recommendations for optic nerve and chiasm hemangioblastomas in VHL patients. Methods: We reviewed two electronic databases in the hospitals of our senior authors, searching for VHL patients with optic nerve or chiasm hemangioblastomas. Clinical data were summarized. Tumor size and growth rate were measured on contrast enhanced MRI. Comparable data were collected by literature review of all available cases in VHL patients (Pubmed, Trip, Google and Google Scholar). Results: Of 269 VHL patients, 12 had optic nerve or chiasm hemangioblastomas. In 10 of 12 patients, tumors were diagnosed upon annual ophthalmoscopic/MRI screening. Of 8 patients who were asymptomatic at diagnosis, 7 showed absent or very slow annual progression, without developing significant vision impairment. One patient developed moderate vision impairment. Two symptomatic patients suffered from rapid tumor growth and progressive vision impairment. Both underwent late-stage surgery, resulting in incomplete resection and progressive vision impairment. One patient presented with acute vision field loss. A watchful-waiting approach was adopted because the hemangioblastoma was ineligible for vision-sparing surgery. One patient developed progressive vision impairment after watchful waiting. In the literature we found 45 patient cases with 48 hemangioblastomas. Discussion: When optic nerve and chiasm hemangioblastomas are diagnosed, we suggest annual MRI follow-up as long as patients do not develop vision impairment. If tumors grow fast, threaten the contralateral eye, or if patients develop progressive vision deficiency; surgical resection must be considered because neurological impairment is irreversible, and resection of large tumors carries a higher risk of further visual decline.

Impact of Spinal CSF Leaks on Quality of Life and Mental Health and Long-Term Reversal by Surgical Closure.

Background and Objectives: Spontaneous intracranial hypotension (SIH) caused by a spinal CSF leak is a multisymptom syndrome, which can dramatically affect physical and mental health. However, systematic data on health-related quality of life (HRQoL) and mental health are scarce. We hypothesized that surgical treatment leads to significant and sustained improvements in HRQoL and mental health in patients with SIH. Methods: In this single-center cohort study, we prospectively collected HRQoL and mental health data in patients undergoing surgical closure of a spinal CSF leak from September 2020 to November 2022. EuroQoL (EQ-5D-5L), including the health state index (EQ-Index) and the visual analog scale (EQ-VAS), measured HRQoL. The 21-item version of the Depression Anxiety Stress Scales (DASS-21) measured symptoms of mental health. Follow-ups were performed 3 and 6 months postoperatively. Primary outcome was the change in EQ-Index, EQ-VAS, and DASS-21 subscales. Secondary outcome was the impact of baseline depression symptoms on HRQoL outcomes following surgery. Results: Seventy-four patients were included. EQ-VAS improved from 40 (interquartile range [IQR] 30-60) preoperatively to 70 (IQR 55-85) at 3 months and to 72 (IQR 60-88) at 6 months postoperatively (p < 0.001, respectively). EQ-Index increased from 0.683 (IQR 0.374-0.799) to 0.877 (0.740-0.943) at 3 months and to 0.907 (0.780-0.956) at 6 months postoperatively (p < 0.001, respectively). Depression, anxiety, and stress significantly improved after surgery. Preoperative depressive symptoms did not affect the HRQoL outcome. Discussion: The severe impact of a spinal CSF leak on HRQoL and mental health significantly improved after closure of the leak. Higher levels of depressive symptoms do not predict worse outcomes and should not discourage invasive treatment. Further systematic evaluation of outcomes, with special regard to quality of life, is needed, as it allows a comparison of symptom burden between SIH and more familiar diseases as well as a comparison of different treatment modalities in future studies.

Sequence variations in the von Hippel-Lindau tumor suppressor gene in patients with intracranial aneurysms.

BACKGROUND: The rupture of intracranial aneurysms leads to subarachnoid hemorrhage, which is often associated with poor outcome. Preventive treatment of unruptured intracranial aneurysms is possible and recommended. However, the lack of candidate genes precludes identifying patients at risk by genetic analyses. We observed intracranial aneurysms in 2 patients with von Hippel-Lindau (VHL) disease and the known disease-causing mutation c.292T > C (p.Tyr98His) in the VHL tumor suppressor gene. This study investigates whether the VHL gene is a possible candidate gene for aneurysm formation. METHODS: Patients with intracranial aneurysms admitted to our department between 2006 and 2009 were enrolled. The peripheral leukocyte DNA of 200 patients was investigated for sequence variations in the VHL gene using denaturing high performance liquid chromatography. Peripheral leukocyte DNA of 100 randomly sampled probands was investigated as a control group. The allelic frequencies of sequence variations between both groups were compared using the Fisher exact test. RESULTS: Fourteen of 200 patients with intracranial aneurysms had sequence variations at 6 different loci in the VHL gene. In contrast, no sequence variations were identified in 100 probands in the control group (P = 0.0062). However, none of the single-sequence variations had a statistically significant difference in the allelic frequencies compared to the control group. CONCLUSIONS: There is accumulating evidence for a genetic basis of aneurysm development. Our investigations lead to the conclusion that the VHL gene is potentially involved in the formation of intracranial aneurysms in a subset of patients. Additional candidate genes need to be identified in order to develop sensitive genetic screening for at-risk patients.

Free-Hand MIS TLIF without 3D Navigation-How to Achieve Low Radiation Exposure for Both Surgeon and Patient.

BACKGROUND: Transforaminal lumbar interbody fusion (TLIF) is one of the most frequently performed spinal fusion techniques, and this minimally invasive (MIS) approach has advantages over the traditional open approach. A drawback is the higher radiation exposure for the surgeon when conventional fluoroscopy (2D-fluoroscopy) is used. While computer-assisted navigation (CAN) reduce the surgeon's radiation exposure, the patient's exposure is higher. When we investigated 2D-fluoroscopically guided and 3D-navigated MIS TLIF in a randomized controlled trial, we detected low radiation doses for both the surgeon and the patient in the 2D-fluoroscopy group. Therefore, we extended the dataset, and herein, we report the radiation-sparing surgical technique of 2D-fluoroscopy-guided MIS TLIF. METHODS: Monosegmental and bisegmental MIS TLIF was performed on 24 patients in adherence to advanced radiation protection principles and a radiation-sparing surgical protocol. Dedicated dosemeters recorded patient and surgeon radiation exposure. For safety assessment, pedicle screw accuracy was graded according to the Gertzbein-Robbins classification. RESULTS: In total, 99 of 102 (97.1%) pedicle screws were correctly positioned (Gertzbein grade A/B). No breach caused neurological symptoms or necessitated revision surgery. The effective radiation dose to the surgeon was 41 ± 12 µSv per segment. Fluoroscopy time was 64 ± 34 s and 75 ± 43 radiographic images per segment were performed. Patient radiation doses at the neck, chest, and umbilical area were 65 ± 40, 123 ± 116, and 823 ± 862 µSv per segment, respectively. CONCLUSIONS: Using a dedicated radiation-sparing free-hand technique, 2D-fluoroscopy-guided MIS TLIF is successfully achievable with low radiation exposure to both the surgeon and the patient. With this technique, the maximum annual radiation exposure to the surgeon will not be exceeded, even with workday use.

Minimally invasive surgery for spinal cerebrospinal fluid leaks in spontaneous intracranial hypotension.

OBJECTIVE: Spinal CSF leaks cause spontaneous intracranial hypotension (SIH). Surgical closure of spinal CSF leaks is the treatment of choice for persisting leaks. Surgical approaches vary, and there are no studies in which minimally invasive techniques were used. In this study, the authors aimed to detail the safety and feasibility of minimally invasive microsurgical sealing of spinal CSF leaks using nonexpandable tubular retractors. METHODS: Consecutive patients with SIH and a confirmed spinal CSF leak treated at a single institution between April 2019 and December 2020 were included in the study. Surgery was performed via a dorsal 2.5-cm skin incision using nonexpandable tubular retractors and a tailored interlaminar fenestration and, if needed, a transdural approach. The primary outcome was successful sealing of the dura, and the secondary outcome was the occurrence of complications. RESULTS: Fifty-eight patients, 65.5% of whom were female (median age 46 years [IQR 36-55 years]), with 38 ventral leaks, 17 lateral leaks, and 2 CSF venous fistulas were included. In 56 (96.6%) patients, the leak could be closed, and in 2 (3.4%) patients the leak was missed because of misinterpretation of the imaging studies. One of these patients underwent successful reoperation, and the other patient decided to undergo surgery at another institution. Two other patients had to undergo reoperation because of insufficient closure and a persisting leak. The rate of permanent neurological deficit was 1.7%, the revision rate for a persisting or recurring leak was 3.4%, and the overall revision rate was 10.3%. The rate of successful sealing during the primary closure attempt was 96.6% and 3.4% patients needed a secondary attempt. Clinical short-term outcome at discharge was unchanged in 14 patients and improved in 25 patients, and 19 patients had signs of rebound intracranial hypertension. CONCLUSIONS: Minimally invasive surgery with tubular retractors and a tailored interlaminar fenestration and, if needed, a transdural approach is safe and effective for the treatment of spinal CSF leaks. The authors suggest performing a minimally invasive closure of spinal CSF leaks in specialized centers.

The Impact of Implementing a Radiation-Sparing Protocol for Percutaneous Kyphoplasty-A Prospective Dosemetric Study.

STUDY DESIGN: Prospective cohort study. OBJECTIVES: The purpose of this prospective study was to evaluate a protocol for radiation-sparing kyphoplasty by assessing dosemetrically recorded radiation exposures to both patient and surgeon. METHODS: This prospective clinical study examines the radiation exposure to patient and surgeon during single-level kyphoplasty in 32 thoracolumbar osteoporotic vertebral body fractures (12 OF 2, 9 OF 3, 11 OF 4 types) using a radiation aware surgical protocol between May 2017 and November 2019. The radiation exposure was measured at different locations using film, eye lens and ring dosemeters. Dose values are reported under consideration of lower detection limits of each dosemeter type. RESULTS: A high proportion of dosemeter readings was below the lower detection limits, especially for the surgeon (>90%). Radiation exposure to the surgeon was highest at the unprotected thyroid gland (0.053 ± 0.047 mSv), however only slightly above the lower detection limit of dosemeters (0.044 mSv). Radiation exposure to the patient was highest at the chest (0.349 ± 0.414 mSv) and the gonad (0.186 ± 0.262 mSv). Fluoroscopy time, dose area product and number of fluoroscopic images were 46.0 ± 17.9 sec, 124 ± 109 cGy×cm2, and 35 ± 13 per kyphoplasty, respectively. Back pain significantly improved from 6.8 ± 1.6 to 2.5 ± 1.7 on the numeric rating scale on the first postoperative day (P < 0.0001). CONCLUSIONS: The implementation of a strict intraoperative radiation protection protocol allows for safely performed kyphoplasty with ultra-low radiation exposure for the patient and surgeon without exceeding the annual occupational dose limits. TRIAL REGISTRATION: The study was registered in the German Clinical Trials Register (DRKS00011908, registration date 16/05/2017).