Treatments and outcomes of untreated cerebral cavernous malformations in China: study protocol of a nationwide multicentre prospective cohort study

IntroductionThe treatment decision and long-term outcomes of previously untreated cerebral cavernous malformation (U-CCM) are still controversial. Therefore, we are conducting a nationwide multicentre prospective registry study in China to determine the natural history and effect of surgical treatment on long-term outcomes in Chinese people with U-CCM.Methods and analysisThis study was started on 1 January 2018 and is currently ongoing. It is a cohort follow-up study across a 5-year period. Patients will be followed up for at least 3 years after inception. Patients with U-CCM will be enrolled from 24 Grade III, level A hospitals distributed all over China. The cohort size is estimated to be 1200 patients. Patients are registered in surgically treated group and conservatively treated group. Clinical characteristics, radiology information and laboratory data are prospectively collected using an electronic case report form through an electronic data capture system. The primary outcome of this study is poor clinical outcome at the last follow-up (modified Rankin Scale score >2 lasting at least 1 year). The secondary outcome includes symptomatic haemorrhage, drug refractory epilepsy, focal neurological deficits, morbidity and all-cause mortality during follow-up. Univariate and multivariate regression analysis will be performed to determine the risk factors for poor outcomes in all patients, and to estimate the effect of surgery. Life tables, Kaplan-Meier estimates, log-rank test and proportional hazards Cox regression will be used to analyse the follow-up data of conservatively treated patients to determine the natural history of U-CCM. Initial presentation and location of U-CCM are prespecified subgroup factors.Ethics and disseminationThe study protocol and informed consent form have been reviewed and approved by the Research Ethical Committee of First Affiliated Hospital of Fujian Medical University (FAHFMU-2018-003).Written informed consent will be obtained from each adult participant or from the guardian of each paediatric participant. The final results will be published in peer-reviewed journals.Trial registration numberNCT03467295.

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Rajaie Cardiomyopathy and Myocarditis Registry: Protocol for an Observational Study

ACTA MEDICA IRANICA ◽

10.18502/acta.v59i5.6659 ◽

2021 ◽

Author(s):

Majid Maleki ◽

Freidoun Noohi ◽

Parham Sadeghipour ◽

Mohammad Mehdi Peighambari ◽

Ahmad Amin ◽

...

Keyword(s):

Natural History ◽

Clinical Signs ◽

Response To Treatment ◽

Cardiac Resynchronization ◽

Therapeutic Approaches ◽

Long Term Outcomes ◽

Prediction Of Mortality ◽

Number Of Patients

Most of the information on the natural history and management of cardiomyopathies and myocarditis in Iran has been obtained from cohort studies in a small number of patients. The prevalence of patients with cardiomyopathies referred to Rajaei Cardiovascular medical and research centers from all over the country is remarkable. Rajaie Cardiomyopathy and myocarditis Registry (RCMR) study is an observational registry of patients with four subtype of cardiomyopathy include: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM) as well as myocarditis designed to determine clinical characteristics, natural history, current therapeutic approaches, response to treatment and long-term outcomes of patients with cardiomyopathy and myocarditis. Prediction of mortality and response to different treatments in these patients using artificial intelligence is another aim of this Registry. COVID 19 Myocarditis and its sequence as cardiomyopathy seem a new challenge in forthcoming years. At the baseline visit, past medical history, clinical signs/symptoms, risk factors, physical examination and family history of cardiomyopathy, current standards for diagnostic workup and clinical follow-up, and relevant electrocardiogram echocardiography, cardiac magnetic resonance, Holter monitoring, or biomarker analyses will be checked. The outcome and results of various therapeutic approaches currently employed for patients, including implantable cardioverter defibrillator, cardiac resynchronization therapy, septal myomectomy, ablation, cardiac transplantation, and medications, will be assessed. Long-term outcomes, including the benefits and complications of therapeutic interventions, will be collected. A follow-up visit will be scheduled after 12 months for all patients, and survival status, hospitalizations, co-morbidities, medications will be assessed.

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Contemporary radiosurgery of cerebral cavernous malformations: Part 2. Treatment outcome for hemispheric lesions

Journal of Neurosurgery ◽

10.3171/2018.2.jns171267 ◽

2019 ◽

Vol 130 (6) ◽

pp. 1826-1834 ◽

Cited By ~ 2

Author(s):

Gábor Nagy ◽

Stuart S. Stokes ◽

Loránd G. Erőss ◽

Debapriya Bhattacharyya ◽

John Yianni ◽

...

Keyword(s):

Natural History ◽

Neurological Deficits ◽

Time Interval ◽

Lesion Volume ◽

Cavernous Malformations ◽

Antiepileptic Medication ◽

Deep Lesion ◽

Prescription Isodose

OBJECTIVEThe role of radiosurgery (RS) in treating superficial cavernous malformations (CMs) is insufficiently studied in part because of the disappointing results of early experimental attempts as compared to the mostly safe and effective microsurgery. Nonetheless, because of lesion- or treatment-specific factors, a therapeutic alternative may be required. In this study, the authors aimed to assess the safety of RS in treating superficial CMs and to analyze its long-term effect on hemorrhage rates and epilepsy control.METHODSThe authors conducted a retrospective analysis of 96 patients with 109 CMs located in the cerebral or cerebellar hemispheres and treated with RS between 1995 and 2014. A median of 15 Gy (range 10–25 Gy) was given to the 50% prescription isodose level, lesion volume was 604 mm3 (4–8300 mm3), and the prescription isodose volume was 638.5 mm3 (4–9500 mm3). Outcomes were compared to those of 206 deep-seated lesions reported on in another study. Ninety-five patients had available follow-up, which was a median of 7 years (1–21 years). Median patient age was 42 years (0.5–77) at presentation and 45 (3–80) at treatment. Seventy-one CMs presented with symptomatic hemorrhage, and 52 caused seizures.RESULTSIn the nonhemorrhagic group (37 lesions), one bleed occurred during the follow-up period, for an annual bleed rate of 0.4% per lesion. The lifetime annual bleed rate of CMs having a single hemorrhage prior to treatment was 2.5%. The rebleed rate in the single-bleed group decreased from 1.8% within the first 2 years after RS to 0.7% thereafter. The pretreatment rebleed rate for lesions having multiple bleeds prior to RS was 14.15%, which fell to 3.85% for the first 2 years after RS and declined to 1.3% thereafter. Multivariate analysis showed younger age, deep lesion location, and multiple pretreatment hemorrhages as significant predictors of posttreatment hemorrhage.Pretreatment hemorrhages led to permanent deficits in 41.4% of the cases with a single bleed and in 46.1% of cases with multiple bleeds. Only mild (modified Rankin Scale score 1) and a low rate of permanent neurological deficits were caused either by posttreatment hemorrhages (4.3%) or by radiation (2%).The rate of improvement in epilepsy was 84.9% after RS in patients with at least one seizure prior to treatment, not depending on the presence of hemorrhage or the time interval between presentation and treatment. Favorable outcome occurred in 81% of patients whose seizures were not controlled with antiepileptic medication prior to RS.CONCLUSIONSRadiosurgery for superficial CMs is safe and appears to be effective, offering a real treatment alternative to surgery for selected patients. Given their relatively benign natural history, superficial CMs require further study to verify the long-term benefit of RS over the lesions’ natural history.

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Laser-assisted in situ keratomileusis long term outcomes in late adolescence

European Journal of Ophthalmology ◽

10.1177/1120672120969039 ◽

2020 ◽

pp. 112067212096903

Author(s):

Jorge L Alió del Barrio ◽

Mario Canto-Cerdán ◽

María Bo ◽

Nuria Subirana ◽

Jorge L Alió

Keyword(s):

Refractive Error ◽

Spherical Aberration ◽

Late Adolescence ◽

Secondary Outcome ◽

Secondary Outcome Measure ◽

Late Adolescent ◽

Long Term Outcomes ◽

Time Of Surgery

Purpose: Evaluate the long term outcomes of myopic-LASIK in a late adolescent population (age ⩾17 and <20 at the time of surgery). Methods: Monocentric retrospective case series study. Eyes with at least 3 years of follow-up time were included. Primary outcome measures were long term efficacy, safety and stability of the refractive error. Secondary outcome measure was the evaluation of the relation between the postoperative spherical aberration and the long term stability of the refractive error. Results: Forty-seven eyes of 25 patients were included. Mean follow-up was 9.23 ± 3.16 years. Mean age at the time of surgery was 18.74 ± 0.44 years. With time, postoperative UDVA showed a mild but significant deterioration of 1–2 Snellen lines ( p = 0.012), in connection with a mild but significant myopization of the SE (mean increase of −0.43 D; p < 0.001), sphere (mean increase of −0.29 D; p = 0.004) and cylinder (mean increase of −0.16 D; p = 0.013). CDVA remained stable over time ( p > 0.05). Efficacy index decreased from 1.01 to 0.87 in the long term (77% UDVA ⩾ 20/32). Safety remained at 1.06. 66% and 74% of eyes presented a SE within ±0.50 D and ±1.00 D respectively. SE changed over 0.50D in 33% of eyes. No correlation could be detected between the SE and the postoperative spherical aberration. No cases of corneal ectasia were detected. Conclusions: Myopic-LASIK in late adolescence is safe and effective, but a mild myopic progression occurs. Despite presence of refractive stability is preferable, if necessary, myopic LASIK provides relatively good outcomes in the long term in this young population.

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Surgical approaches and long-term outcomes of intramedullary spinal cord cavernous malformations: a single-center consecutive series of 219 patients

Journal of Neurosurgery Spine ◽

10.3171/2018.12.spine181263 ◽

2019 ◽

Vol 31 (1) ◽

pp. 123-132 ◽

Cited By ~ 5

Author(s):

Jian Ren ◽

Tao Hong ◽

Chuan He ◽

Xiaoyu Li ◽

Yongjie Ma ◽

...

Keyword(s):

Spinal Cord ◽

Surgical Approaches ◽

Consecutive Series ◽

Single Center ◽

Long Term Outcomes ◽

Intramedullary Spinal Cord ◽

Cavernous Malformations ◽

Surgical Strategies

OBJECTIVEOptimal surgical strategies for intramedullary spinal cord cavernous malformations (ISCCMs) are not optimized and remain problematic. In this study the authors identify rational surgical strategies for ISCCMs and predictors of outcomes after resection.METHODSA single-center study was performed with 219 consecutive surgically treated patients who presented from 2002 to 2017 and were analyzed retrospectively. The American Spinal Injury Association (ASIA) Impairment Scale was used to evaluate neurological functions. Patient characteristics, surgical approaches, and immediate and long-term postoperative outcomes were identified.RESULTSThe average ISCCM size was 10.5 mm. The spinal level affected was cervical in 24.8% of patients, thoracic in 73.4%, and lumbar in 1.8%. The locations of the lesions in the horizontal plane were 30.4% ventral, 41.6% dorsal, and 28.0% central. Of the 214 patients included in the cohort for operative evaluation, 62.6% had superficially located lesions, while 37.4% were embedded. Gross-total resection was achieved in 98.1% of patients. The immediate postoperative neurological condition worsened in 10.3% of the patients. Multivariate logistic regression identified mild preoperative function (p = 0.014, odds ratio [OR] 4.5, 95% confidence interval [CI] 1.4–14.8) and thoracolumbar-level lesions (p = 0.01, OR 15.7, 95% CI 1.9–130.2) as independent predictors of worsening. The mean follow-up duration in 187 patients was 45.9 months. Of these patients, 63.1% were stable, 33.2% improved, and 3.7% worsened. Favorable outcomes were observed in 86.1% of patients during long-term follow-up and were significantly associated with preoperative mild neurological and disability status (p = 0.000) and cervically located lesions (p = 0.009). The depths of the lesions were associated with worse long-term outcomes (p = 0.001), and performing myelotomy directly through a yellowish abnormal surface in moderate-depth lesions was an independent predictor of worsening (p = 0.023, OR 35.3, 95% CI 1.6–756.3).CONCLUSIONSResection performed with an individualized surgical approach remains the primary therapeutic option in ISCCMs. Performing surgery in patients with mild symptoms at the thoracolumbar level and embedded located lesions requires more discretion.

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The effect of the hypertensive phase on the long-term outcomes of Ahmed glaucoma valve (AGV) implantation

10.21203/rs.3.rs-939083/v1 ◽

2021 ◽

Author(s):

Naveed Nilforushan ◽

Kiana Hassanpour ◽

Fatemeh Vafaei ◽

Maryam Yadgari

Keyword(s):

Neovascular Glaucoma ◽

Secondary Outcome ◽

Ahmed Glaucoma Valve ◽

Long Term Outcomes ◽

Long Term Effect ◽

Long Term Follow Up ◽

Surgical Failure ◽

Hypertensive Phase

Abstract BackgroundTo investigate the long-term effect of hypertensive phase(HP) on the clinical outcomes of Ahmed Glaucoma Valve (AGV) implantation.MethodThe records of patients with different etiologies of glaucoma who underwent AGV implantation were retrospectively reviewed. HP was defined as the IOP> 21 mm Hg during the first three months after surgery. The main outcome measure was cumulative success defined as 5 < IOP< 21 mmHg and 20 % reduction from the baseline with or without IOP lowering medications. The secondary outcome measures were intraocular pressure (IOP) and the number of glaucoma medications. ResultsA total of 120 patients (28 patients of HP, 92 patients without HP) with an average age (±SD) of 48.9 ± 19.6 years and a mean follow-up of 4.5 ± 1.4 years were enrolled. The mean duration of survival was 5.3 ± 0.5 years in HP which was significantly shorter than 6.4 ± 0.2 years in non-HP. (log Rank= 4.2, P= 0.04) Mean IOP and number of IOP lowering agents were higher in postoperative visits at 1,2, 3, and 4 years in HP patients compared with non-HP. (All Ps < 0.01) Higher Baseline IOP was significantly associated with higher rates of surgical failure. Neovascular glaucoma was the only factor associated with the occurrence of HP in a logistic regression model. (Odds ratio: 3.14 CI: 1.2-8.1). Conclusion In the long-term follow up the duration of survival was significantly longer in the non-HP group. In the non-HP group, the success rate was significantly higher than the HP group

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Contemporary radiosurgery of cerebral cavernous malformations: Part 1. Treatment outcome for critically located hemorrhagic lesions

Journal of Neurosurgery ◽

10.3171/2017.5.jns17776 ◽

2019 ◽

Vol 130 (6) ◽

pp. 1817-1825 ◽

Cited By ~ 4

Author(s):

Gábor Nagy ◽

Wendy Burkitt ◽

Stuart S. Stokes ◽

Debapriya Bhattacharyya ◽

John Yianni ◽

...

Keyword(s):

Natural History ◽

Current Knowledge ◽

Neurological Deficits ◽

Temporary Increase ◽

Long Term Effects ◽

Cavernous Malformations ◽

Intention To Treat ◽

Study Results

OBJECTIVELong-term benefits of radiosurgery (RS) applying modern protocols to treat cavernous malformations (CMs) remain unclear as critics may consider the decrease in the rebleed rate generally observed 2 years after RS as a reflection of the lesion’s natural history. The authors adopted an early intention-to-treat attitude since rehemorrhage from deep-seated CMs ultimately leads to stepwise neurological deterioration. The safety of this early policy was previously demonstrated. Here, the authors revisit their current practice in a larger population with a longer follow-up time to assess the long-term effects of RS in the context of current knowledge on the natural history of CMs.METHODSThe authors conducted a retrospective analysis of 210 patients with 210 hemorrhagic CMs located in the brainstem, thalamus, or basal ganglia and treated with Gamma Knife RS between 1995 and 2014. Two hundred six patients had available follow-up, which was a median of 5.5 years (range 1–20 years). The median age was 37 years (0.5–77 years) at presentation and 43 (2–78) at treatment. One hundred twenty-seven CMs had bled once and 83 had had multiple hemorrhages prior to treatment.RESULTSThe lifetime annual bleed rate of CMs having a single hemorrhage prior to treatment was 2.4% per lesion. The hemorrhage rate stabilized at 1.1% after a temporary increase of 4.3% within the first 2 years after RS. The annual pretreatment hemorrhage rate was 2.8% for the lesions having multiple bleeds prior to RS with a pretreatment rebleed rate of 20.7% and with a modest gradual decrease within the first 5 years and remaining stable at 11.55% thereafter. The rebleed rate fell to 7.9% for the first 2 years after RS and declined further to 1.3% thereafter, which was significantly lower than the long-term pretreatment rebleed risk. The rate of hemorrhage-free survival remained 86.4% and 75.1% (1 patient each) at 20 years after RS in the single- and multiple-bleed groups, respectively.Pretreatment hemorrhages resulted in permanent deficits in 48.8% of the cases with a single bleed and in 77.1% of the cases with multiple bleeds. Both the rate and severity of deficits were significantly lower in the first group. Only mild and a low rate of permanent neurological deficits were caused either by posttreatment hemorrhages (7.4%) or by radiation (7.2%). The rate of persistent morbidity in the single-bleed group remained significantly lower at the end of the study than pretreatment morbidity in the multiple-bleed group (OR 2.9, 95% CI 1.6–5.3). Lesion-specific mortality was < 1%.CONCLUSIONSThe hemorrhage rate of CMs after RS remained low after the first 2 years during the longer follow-up period. The benefit of early treatment appears to be confirmed by the study results as repeated hemorrhages carry the risk of significantly higher cumulative morbidity than the morbidity associated with RS.

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Periprocedural risk and long-term outcome of intracranial angioplasty based on a single-centre experience

VASA ◽

10.1024/0301-1526/a000287 ◽

2013 ◽

Vol 42 (4) ◽

pp. 264-274

Author(s):

Dagmar Krajíčková ◽

Antonín Krajina ◽

Miroslav Lojík ◽

Martina Mulačová ◽

Martin Vališ

Keyword(s):

Death Rate ◽

Long Term Outcomes ◽

Single Centre ◽

Intracranial Atherosclerotic Stenosis ◽

Atherosclerotic Stenosis ◽

Angioplasty And Stenting ◽

Aggressive Medical Management ◽

Stroke Death

Background: Intracranial atherosclerotic stenosis is a major cause of stroke and yet there are currently no proven effective treatments for it. The SAMMPRIS trial, comparing aggressive medical management alone with aggressive medical management combined with intracranial angioplasty and stenting, was prematurely halted when an unexpectedly high rate of periprocedural events was found in the endovascular arm. The goal of our study is to report the immediate and long-term outcomes of patients with ≥ 70 % symptomatic intracranial atherosclerotic stenosis treated with balloon angioplasty and stent placement in a single centre. Patients and methods: This is a retrospective review of 37 consecutive patients with 42 procedures of ballon angioplasty and stenting for intracranial atherosclerotic stenosis (≥ 70 % stenosis) treated between 1999 and 2012. Technical success (residual stenosis ≤ 50 %), periprocedural success (no vascular complications within 72 hours), and long-term outcomes are reported. Results: Technical and periprocedural success was achieved in 90.5 % of patients. The within 72 hours periprocedural stroke/death rate was 7.1 % (4.8 % intracranial haemorrhage), and the 30-day stroke/death rate was 9.5 %. Thirty patients (81 %) had clinical follow-up at ≥ 6 months. During follow-up, 5 patients developed 6 ischemic events; 5 of them (17 %) were ipsilateral. The restenosis rate was 27 %, and the retreatment rate was 12 %. Conclusions: Our outcomes of the balloon angioplasty/stent placement for intracranial atherosclerotic stenosis are better than those in the SAMMPRIS study and compare favourably with those in large registries and observational studies.

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