Oral extrusion of a vertebral body replacement device after chordoma tumor growth and radiation: case report and review.

BACKGROUND: Screw migration following anterior cervical discectomy and fusion is a very rare complication and it is often related to device failure. Even more exceptional is the extrusion of an intervertebral graft. CASE PRESENTATION: We report the second case of migration and extrusion through the oral cavity of a cervical vertebral body replacement device (expandable cylinder) in a patient that had undergone cervical corpectomy due to a vertebral chordoma. CONCLUSION: The antecedent of radiation therapy as well as progressive tumor re-growth may have favored the development of this complication. A literature review is added.

Intrathecal administration of autologous mesenchymal stromal cells for spinal cord injury: Safety and efficacy of the 100/3 guideline.

BACKGROUND AIMS: Cell therapy with autologous mesenchymal stromal cells (MSCs) in patients with spinal cord injury (SCI) is beginning, and the search for its better clinical application is an urgent need. METHODS: We present a phase 2 clinical trial in patients with chronic SCI who received three intrathecal administrations of 100 x 106 MSCs and were followed for 10 months from the first administration. Efficacy analysis was performed on nine patients, and safety analysis was performed on 11 patients. Clinical scales, urodynamic, neurophysiological and neuroimaging studies were performed previous to treatment and at the end of the follow-up. RESULTS: The treatment was well-tolerated, without any adverse event related to MSC administration. Patients showed variable clinical improvement in sensitivity, motor power, spasms, spasticity, neuropathic pain, sexual function or sphincter dysfunction, regardless of the level or degree of injury, age or time elapsed from the SCI. In the course of follow-up three patients, initially classified as ASIA A, B and C, changed to ASIA B, C and D, respectively. In urodynamic studies, at the end of follow-up, 66.6% of the patients showed decrease in postmicturition residue and improvement in bladder compliance. At this time, neurophysiological studies showed that 55.5% of patients improved in somatosensory or motor-evoked potentials, and that 44.4% of patients improved in voluntary muscle contraction together with infralesional active muscle reinnervation. CONCLUSIONS: The present guideline for cell therapy is safe and shows efficacy in patients with SCI, mainly in recovery of sphincter dysfunction, neuropathic pain and sensitivity.

Cell therapy with autologous mesenchymal stromal cells in post-traumatic syringomyelia.

BACKGROUND AIMS: Recently, clinical studies show that cell therapy with mesenchymal stromal cells (MSCs) improves the sequelae chronically established in paraplegic patients, being necessary to know which of them can obtain better benefit. METHODS: We present here a phase 2 clinical trial that includes six paraplegic patients with post-traumatic syringomyelia who received 300 million MSCs inside the syrinx and who were followed up for 6 months. Clinical scales, urodynamic, neurophysiological, magnetic resonance (MR) and studies of ano-rectal manometry were performed to assess possible improvements. RESULTS: In all the cases, MR at the end of the study showed a clear reduction of the syrinx, and, at this time, signs of improvement in the urodynamic studies were found. Moreover, four patients improved in ano-rectal manometry. Four patients improved in neurophysiological studies, with signs of improvement in evoked potentials in three patients. In the American Spinal Injury Association (ASIA) assessment, only two patients improved in sensitivity, but clinical improvement in neurogenic bowel dysfunction was observed in four patients and three patients described improvement in bladder dysfunction. Spasms reduced in two of the five patients who had them previous to cell therapy, and spasticity was improved in the other two patients. Three patients had neuropathic pain before treatment, and it was reduced or disappeared completely during the study. Only two adverse events ocurred, without relation to the cell therapy. CONCLUSIONS: Cell therapy can be considered as a new alternative to the treatment of post-traumatic syringomyelia, achieving reduction of syrinx and clinical improvements in individual patients.

[Erdheim-Chester disease, an incredible simulator. Cases reports and review of literature]. / Enfermedad de Erdheim-Chester, una gran imitadora. Presentación de casos y revisión de la literatura.

Erdheim-Chester disease is a non-Langerhans histiocytosis. Until 2014 at least 550 cases have been reported. According to European Rare Disease Organization and National Organization for Rare Disorders it is a rare disease. The most common symptom is bone pain in the lower extremities and it usually appears between the 5th and 7th decades of life. The diagnostic is based on immunohistochemical results: S100(+/-), CD68(+), and CD1a(-), the latter 2 are mandatory. The best treatment nowadays is alpha-interferon or pegylated alpha-2. The overall survival is 96% at one year and 68% at 5 years. Central nervous system involvement is associated with a worse outcome. Two cases are presentedwith central nervous system lesions in the absence of lesions in other organs on their onset. Very few cases have been reported with this kind of presentation. We also noted that these patients had recurrences or new lesions at 8 months. A follow-up is proposed with brain MRI and thoraco-abdominal PET every 3-4 months.

Normal perfusion pressure breakthrough phenomenon: experimental models.

One of the most life-threatening complications after the obliteration of intracranial arteriovenous malformations is the development of oedema and/or multifocal haemorrhage. Two main theories have been postulated so far in order to explain this situation. On one hand, "normal perfusion pressure breakthrough phenomenon" is based on the loss of cerebral vessel autoregulation due to the chronic vasodilation of perinidal microcirculation. On the other hand, the "occlusive hyperaemia" deals with thrombotic and venous obstruction phenomena that may also generate such manifestations. The aim of this study is to resume the main concepts of the "normal perfusion pressure breakthrough phenomenon" theory as well as the related animal models described up to date, their advantages and disadvantages, and the main conclusions obtained as a result of the experimental research.

Sinking bone flap due to overdrainage of a ventriculoperitoneal shunt. A case report and review of the literature.

The widespread use of decompressive craniectomy and subsequent cranioplasty has led to a better understanding of its complications. However, cases of a sunken bone flap have hardly ever been described. We present the eighth case reported up to date and perform a review of the literature of this sporadic complication. A 40-year-old Caucasian male suffered a traumatic brain injury that required a decompressive craniectomy. One month after initial trauma autologous cranioplasty was performed. A ventriculoperitoneal shunt was also placed. Neurological status progressively improved but his therapist noted cognitive status decline 8 months later. Follow-up computed tomography showed a progressive sinking bone flap. The patient underwent bone flap removal and a custom-made calcium phosphate-based implant was inserted, leading to symptoms resolution. Bone resorption has been described as the main cause of sinking bone flap following cranioplasty. This entity may manifest with symptoms of overdrainage in patients with cerebrospinal fluid shunt devices.

Tunneled antibiotic-impregnated vs. bolt-connected, non-coated external ventricular drainage: a comparison of complications.

Background: External ventricular drainage (EVD) is a common emergency neurosurgical procedure, but it is not free of adverse events. The aim of this study is to compare the complication rate of two frequently used EVD types, namely, tunneled antibiotic-impregnated catheters (Bactiseal©) and bolt-connected non-coated devices (Camino©). Methods: All EVDs placed between 1 March 2015 and 31 December 2017 were registered. Procedures performed with any catheter different from Bactiseal© or Camino© EVD with incomplete follow-up and those EVDs placed due to infectious disease were excluded. Demographic and clinical variables, as well as the overall complication rate (infection, hemorrhage, obstruction, malposition of the catheter, and involuntary pull-out of the device) and the need for replacement of the EVD, were collected. Results: A total of 77 EVDs were finally considered for analysis (40 Bactiseal® and 37 Camino®). There was a statistically significant difference in diagnosis and also in the location of the procedure, as more bolt-connected EVD was placed outside the operating room (97.3 vs. 23.5%, p < 0.001) due to emergent pathologies such as vascular diseases and spontaneous hemorrhages. In the univariate analysis, a statistically significantly higher rate of catheter involuntary pull-out (29.7 vs. 7.5%, p = 0.012) and the need for EVD replacement (32.4 vs. 12.5%, p = 0.035) was found in the Camino cohort. However, those differences could not be confirmed with multivariable analysis, which showed no association between the type of catheter and any of the studied complications. Ventriculostomy duration was identified as a risk factor for infection (OR 1.09, 95% CI 1.02-1.18). Conclusion: No significant differences were observed regarding infection, hemorrhage, obstruction, malposition, involuntary catheter pull-out, and the need for EVD replacement when comparing non-impregnated bolt-connected EVDs (Camino®) with tunneled antibiotic-impregnated catheters (Bactiseal®). The duration of EVD was associated with an increased risk of infection.

[Past, present and future of the neurosurgery]. / Pasado, presente y futuro de la neurocirugía.

In this work a both historical and current journey for the main pathologies regarding the Neurosurgery is achieved, that is to say, the head injuries, the brain tumors, the cerebrovascular diseases, the pathology concerning the cerebrospinal fluid and malformations, the functional neurosurgery, the degenerative spine disease, the spine injuries, the spine tumors and eventually, the pathology of the Peripheral and Autonomic Nervous System. Also, references to what can be the Neurosurgery in a not very distant future are taken into account. Finally, some notes about investigation in the Neurosurgery field are added.

[Schwannoma of the brachial plexus resembling a breast adenocarcinoma metastasis]. / Neurinoma del plexo braquial simulando metastasis de adenocarcinoma de mama.

Schwanomas originating from the brachial plexus, although rare, may be mistaken for another type of tumour. A 40 year-old woman, who had been treated years earlier for a breast adenocarcinoma, showed in the 5-year follow-up magnetic resonance examination a localized lesion in the right brachial plexus. The presumptive radiological diagnosis was a metastasis from the primary adenocarcinoma. Following surgical access via the right axilla, a well-circumscribed mass in the brachial plexus was detected. Under intraoperative electrophysiological guidance, the lesion was observed to depend on the ulnar nerve and its complete resection was possible without compromising nerve function. Histological findings indicated a schwannoma thus ruling out the presence of metastasis. The postoperative development was uneventful and six years after surgery, the patient is to date tumour-free. To the best of our knowledge, this is the first report of a brachial plexus schwannoma arising from the ulnar branch. Intraoperative electrophysiological monitoring is essential for a good surgical outcome.

[Outlining the limits of salvageability in severe head injury]. / Perfilando los límites de la supervivencia en el tratamiento craneoencefálico grave.

Severe head injury is one of the most important health, social, and economic problems in industrialized countries. The percentages of mortality and unfavourable outcomes are very similar to those reported in the last quarter of century. In order to make predictions, different "prognostic formulas or models" have been carried out. These models will be constructed on the one hand, by a group of "prognostic indicators or factors" and on the other hand, by different "prognostic scales" that are useful for measuring the final outcome of these patients. Several "statistical techniques or methods" are necessary to develop these ones.

Retrospective Evaluation of Thoracolumbar Injury Classification System and Thoracolumbar AO Spine Injury Scores for the Decision Treatment of Thoracolumbar Traumatic Fractures in 458 Consecutive Patients.

BACKGROUND: Thoracolumbar Injury Classification System (TLICS) score and Thoracolumbar AO Spine Injury Score (TLAOSIS) are the scores preferred to classify and treat thoracolumbar fractures. Our study evaluates the reliability of both as guidelines for treatment. METHODS: Single-center and retrospective case series of 458 patients. Clinical variables, radiology, and treatment were analyzed. We classified fractures according to the AO Spine Thoracolumbar System and retrospectively applied both scales in 2 groups (surgical and conservative). A concordance analysis and statistical measures comparing both were performed. RESULTS: The patients were divided as follows: 257 patients (56.1%) in the conservative group and 201 patients (43.9%) in the surgical group. The concordance analysis between both scales was 89.7% (95% confidence interval, 86.5%-92.3%), and the Cohen kappa coefficient was 0.68 (95% confidence interval, 59%-76%). TLAOSIS had a higher tendency to classify patients in the gray zone (10.3% vs. 2.8%, P < 0.001), whereas TLICS had a more conservative nature (85.2% vs. 78.4%, P = 0.01). In the surgical group, the matching decision ratio was 29.9% for TLICS and 42.8% for TLAOSIS, but differences were found in TLICS being more conservative (70.1% vs. 57.2%, P = 0.01). In the conservative group, the matching decision ratio was 98.1% for both scales, being the main difference in the gray zone for TLAOSIS. CONCLUSIONS: Both scales have a good concordance in general, with TLICS being more conservative overall. They had rather low coincidence when predicting surgery. Because TLAOSIS placed more patients in the gray zone, we think it might be slightly better for giving surgeons more license to decide a surgical approach on certain controversial types of fractures.

[New therapeutical approach of the complex cerebral vascular malformations]. / Nuevo enfoque terapéutico de las malformaciones vasculares cerebrales complejas.

The final therapeutical target of the cerebral vascular malformations has to be its complete resection or its entire vascular exclusion. To date, the cerebral vascular malformations named complex (grades III, IV and V of the Spetzler & Martin classification) did not have a well-defined treatment. Nowadays, thanks to a multidisciplinary approach by means of several sessions of embolization with Onyx and later surgical treatment, these lesions can be "eradicated" and therefore, "cured" in a definitive way and without any delay. Following this strategy, our experience on this matter in the "Hospital Clínico San Carlos" of Madrid is presented.

Neuroprotective effect of indomethacin in normal perfusion pressure breakthrough phenomenon.

Loss of cerebral autoregulation in normal perfusion pressure breakthrough (NPPB) phenomenon has been reported in other Central Nervous System diseases such as neonatal intraventricular haemorrhage. Several studies have demonstrated that low-dose indomethacin prevents this latter condition. A previous rat model was used to resemble NPPB phenomenon. Study animals were distributed in 4 groups that received 3 doses of indomethacin at different concentrations prior to fistula occlusion 60 days after its creation. Control animals received saline solution. Intracranial pressure (ICP) increased in all groups following fistula creation, whereas mean arterial pressure (MAP) and cerebral perfusion pressure (CPP) decreased as a manifestation of cerebral hypoperfusion and intracranial hypertension. The administration of indomethacin was associated with raised MAP and CPP, as well as decreased ICP. Sodium fluorescein extravasation was slight in study animals when comparing with control ones. Histological analysis evidenced diffuse ischaemic changes with signs of neuronal apoptosis in all brain layers in control animals. These findings were only focal and slight in study animals. The results suggest the usefulness of indomethacin to revert, at least partially, the haemodynamic effects of NPPB phenomenon in this experimental model, as well as to reduce BBB disruption and histological ischemia observed in absence of indomethacin.

Experimental rat model of chronic cerebral hypoperfusion-reperfusion mimicking normal perfusion pressure breakthrough phenomenon.

BACKGROUND: Normal perfusion pressure breakthrough (NPPB) phenomenon is a major life-threatening complication that restricts the treatment of complex intracranial arteriovenous malformations. The aim of the study it to develop a rat model mimicking NPPB phenomenon that enables the evaluation of any therapy to prevent such complication. METHODS: Twenty Wistar male rats were randomly assigned to either a study or a control group. Study animals underwent an end-to-side left external jugular vein-common carotid artery anastomosis and ligation of bilateral external carotid arteries. Control animals only underwent ligation of bilateral external carotid arteries. All animals were sacrificed sixty days after the procedure. Hemodynamic parameters [mean arterial pressure (MAP), intracranial pressure (ICP) and cerebral perfusion pressure (CPP)], blood-brain barrier (BBB) permeability (measured by fluorescein staining) and histological features were then compared between both groups. RESULTS: A significant decrease in MAP and CPP was confirmed in the study group. An increase in ICP was also observed. A significant decrease in MAP and CPP was also present in the study group when comparing preoperative values with those recorded on days 0 (postoperative), 7 and 60. Fluorescein staining findings were consistent with signs of BBB disruption in study animals. Histological analysis demonstrated an increased number of pyknotic neurons in the ipsilateral hemisphere of rat brains included in the study group. CONCLUSION: These results confirm that this model mimics a vascular steal state with chronic cerebral hypoperfusion comparable to patients with AVMs behavior and disruption of the BBB after fistula closure comparable to NPPB phenomenon disorders.

Variability of Clinical and Angiographic Results Based on the Treatment Preference (Endovascular or Surgical) of Centers Participating in the Subarachnoid Hemorrhage Database of the Working Group of the Spanish Society of Neurosurgery.

OBJECTIVES: Since the introduction of endovascular treatment for cerebral aneurysms, hospitals in which subarachnoid hemorrhage is treated show different availability and/or preferences towards both treatment modalities. The main aim is to evaluate the clinical and angiographic results according to the hospital's treatment preferences applied. METHODS: This study was conducted based on use of the subarachnoid hemorrhage database of the Vascular Pathology Group of the Spanish Neurosurgery Society. Centers were classified into 3 subtypes according to an index in the relationship between endovascular and surgical treatment as: endovascular preference, high endovascular preference, and elevated surgical preference. The clinical results and angiographic results were evaluated among the 3 treatment strategies. RESULTS: From November 2004 to December 2017, 4282 subarachnoid hemorrhage patients were selected for the study: 630 (14.7%) patients from centers with surgical preference, 2766 (64.6%) from centers with endovascular preference, and 886 (20.7%) from centers with high endovascular preference. The surgical preference group obtained the best angiographic results associated with a greater complete exclusion (odds ratio: 1.359; 95% confidence interval: 1.025-1.801; P = 0.033). The surgical preference subgroup obtained the best outcome at discharge (65.45%), followed by the high endovascular preference group (61.5%) and the endovascular preference group (57.8%) (odds ratio: 1.359; 95% confidence interval: 1.025-1.801; P = 0.033). CONCLUSIONS: In Spain, there is significant variability in aneurysm exclusion treatment in aneurysmal subarachnoid hemorrhage. Surgical centers offer better results for both surgical and endovascular patients. A multidisciplinary approach and the maintenance of an elevated quality of surgical competence could be responsible for these results.

Cell Therapy as a New Approach to the Treatment of Posttraumatic Syringomyelia.

BACKGROUND: Cell transplantation with autologous bone marrow-derived mesenchymal stromal cells (MSCs) seems to be a therapeutic promise for patients with established spinal cord injury, achieving improvement in their quality of life, but there is no experience with the application of this type of cell therapy in patients suffering posttraumatic syringomyelia. OBJECTIVE: To study the possible utility of cell therapy with autologous MSCs in posttraumatic syringomyelia. METHODS: A 40-year-old man with complete paraplegia since 1991 as a consequence of a Th4 vertebral fracture showed a great posttraumatic syringomyelia that extended up to C2 vertebral level, without signs of recent worsening. Autologous MSCs (150 × 106) were injected into the syrinx, without drainage or aspiration. RESULTS: One year after cell therapy, syrinx was reduced without collapse of cervical spinal cord. During the course of follow-up, clear clinical improvement was observed, mainly in sphincter dysfunction. CONCLUSIONS: Injection of MSCs in the syrinx of posttraumatic syringomyelia is safe and is associated with clinical and neuroimaging improvement. The possibility of cell therapy as a new approach to posttraumatic syringomyelia, or even for idiopathic syringomyelia, is an open door that requires further study.

Genetic alterations of IDH1 and Vegf in brain tumors.

BACKGROUND: This study evaluates the presence of R132H mutation in isocitrate dehydrogenase (IDH1) gene and the vascular endothelial growth factor (VEGF) +936 C/T polymorphism in brain tumors. The impact of these genetic alterations on overall survival (OS) and progression free survival (PFS) was evaluated. METHODS: A cohort of 80 patients surgically treated at Hospital Clínico San Carlos, Madrid, between March 2004 and November 2012, was analyzed. Tumors were distributed in 73 primary brain tumors (gliomas, meningiomas, hemangiopericytomas and hemangioblastomas) and seven secondary tumors evolved from a low grade glioma, thus providing a mixed sample. RESULTS: IDH1R132H gene mutation was found in 12 patients (15%) and appears more frequently in secondary tumors (5 (71.4%) whereas in 7 (9.7%) primary tumors (p < .001)). The mutation is related to WHO grade II in primary tumors and a supratentorial location in secondary tumors. The OS analysis for IDH1 showed a tendency towards a better prognosis of the tumors containing the mutation (p = .059).The IDH1R132H mutation confers a better PFS (p = .025) on primary tumors. The T allele of VEFG +936 C/T polymorphism was found in 16 patients (20%). No relation was found between this polymorphism and primary or secondary tumor, neither with OS or PFS. CONCLUSIONS: IDH1R132H gene mutation is exclusive in supratentorial tumors and more frequent in secondary ones, with a greater survival trend and better PFS in patients who carry it. The T allele of VEGF +936 C/T polymorphism is more common in primary tumors, although there is no statistical relation with survival.

Multiple spinal arteriovenous fistulas: A case-based review.

The occurrence of multiple spinal dural arteriovenous fistulas (AVFs) is rare. The majority of cases reported are synchronous and the lesions are mainly found at different spinal levels. Metachronous AVFs have been defined as lesions that manifest in a temporal sequence after treatment of a first AVF. In this report, we present two distinct cases of multiple spinal AVFs. Also, we review the main features of the cases previously reported, with emphasis on the proposed theories for the origin of multiple AVFs. In patients with failure to improve after treatment of a spinal DAVF, a whole-spine angiographic examination is mandatory, not only to ascertain the complete closure of the treated fistula, but also to look for a possible second lesion at a different spinal level.

Validation of a prognostic score for early mortality in severe head injury cases.

OBJECT: Traumatic brain injury (TBI) represents a large health and economic burden. Because of the inability of previous randomized controlled trials (RCTs) on TBI to demonstrate the expected benefit of reducing unfavorable outcomes, the IMPACT (International Mission on Prognosis and Analysis of Clinical Trials in TBI) and CRASH (Corticosteroid Randomisation After Significant Head Injury) studies provided new methods for performing prognostic studies of TBI. This study aimed to develop and externally validate a prognostic model for early death (within 48 hours). The secondary aim was to identify patients who were more likely to succumb to an early death to limit their inclusion in RCTs and to improve the efficiency of RCTs. METHODS: The derivation cohort was recruited at 1 center, Hospital 12 de Octubre, Madrid (1990-2003, 925 patients). The validation cohort was recruited in 2004-2006 from 7 study centers (374 patients). The eligible patients had suffered closed severe TBIs. The study outcome was early death (within 48 hours post-TBI). The predictors were selected using logistic regression modeling with bootstrapping techniques, and a penalized reduction was used. A risk score was developed based on the regression coefficients of the variables included in the final model. RESULTS: In the validation set, the final model showed a predictive ability of 50% (Nagelkerke R(2)), with an area under the receiver operating characteristic curve of 89% and an acceptable calibration (goodness-of-fit test, p = 0.32). The final model included 7 variables, and it was used to develop a risk score with a range from 0 to 20 points. Age provided 0, 1, 2, or 3 points depending on the age group; motor score provided 0 points, 2 (untestable), or 3 (no response); pupillary reactivity, 0, 2 (1 pupil reacted), or 6 (no pupil reacted); shock, 0 (no) or 2 (yes); subarachnoid hemorrhage, 0 or 1 (severe deposit); cisternal status, 0 or 3 (compressed/absent); and epidural hematoma, 0 (yes) or 2 (no). Based on the risk of early death estimated with the model, 4 risk of early death groups were established: low risk, sum score 0-3 (< 1% predicted mortality); moderate risk, sum score 4-8 (predicted mortality between 1% and 10%); high risk, sum score 9-12 (probability of early death between 10% and 50%); and very high risk, sum score 13-20 (early mortality probability > 50%). This score could be used for selecting patients for clinical studies. For example, if patients with very high risk scores were excluded from our study sample, the patients included (eligibility score < 13) would represent 80% of the original sample and only 23% of the patients who died early. CONCLUSIONS: The combination of Glasgow Coma Scale score, CT scanning results, and secondary insult data into a prognostic score improved the prediction of early death and the classification of TBI patients.