Materiały źródłowe

• #1 Positive and negative predictors for good outcome after decompressive surgery for Chiari malformation type 1 as scored on the Chicago Chiari Outcome Scale

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6718292/

  Posterior fossa decompression (PFD) is commonly applied as treatment for Chiari malformation type 1 (CM1), an entity which is associated with a variety of presenting symptoms but little data correlating symptoms to surgical outcome. […] Our results identify specific presenting factors that correlated with a better or worse outcome after CM1 decompression. […] Sensory deficits and peripheral neuropathy correlated with a lower score on the CCOS. Younger age at the time of surgery and, strikingly, presence of syringomyelia both correlated with a higher CCOS score. […] Patients presenting with peripheral neuropathy (dysesthesia, parasthesia, or hyperesthesia) were 2.91 times more likely to experience a poor outcome of surgery. […] Patients presenting with peripheral neuropathy and Valsalva headache were 2.85 times more likely to experience a poor outcome of surgery.

• #2 Machine learning for enhanced prognostication: predicting 30-day outcomes following posterior fossa decompression surgery for Chiari malformation type I in a pediatric cohort in: Journal of Neurosurgery: Pediatrics Volume 34 Issue 1 (2024) Journals

  https://thejns.org/pediatrics/view/journals/j-neurosurg-pediatr/34/1/article-p57.xml

  Using a large-scale nationwide dataset, machine learning models for the prediction of both 30-day readmissions and reoperations were developed and achieved high accuracy. This highlights the utility of machine learning in risk stratification and surgical decision-making for pediatric CM-I. […] The 30-day readmission and reoperation rates were 7.5% and 3.4%, respectively. […] Related hospital 30-day readmissions occurred in 7.5% (n = 532) of the patients. […] Overall, the 30-day reoperation rate was 3.4% (n = 239), with a median time to reoperation of 14 days (IQR 9, 20 days). […] In the multivariable logistic regression model, several variables were found to be significant and independent predictors of 30-day readmission. […] The best performing algorithms had AUCs of 0.960 and 0.990 for 30-day readmission and reoperation, respectively.

• #3 Positive and negative predictors for good outcome after decompressive surgery for Chiari malformation type 1 as scored on the Chicago Chiari Outcome Scale

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6718292/

  Patients presenting with peripheral neuropathy and neck pain were 2.49 times more likely to experience a poor outcome of surgery. […] Patients presenting with peripheral neuropathy and syncope were 4.64 times more likely to experience a poor outcome of surgery. […] There were three factors found to be positive prognostic indicators, i.e. they correlated with a favorable outcome of surgery. Patients who were less than 18 years old at the time of surgery were 2.72 times more likely to have a good outcome than those who were 18 years or older at the time of surgery. […] The most striking finding in our study is that patients presenting with syringomyelia were significantly more likely to achieve a better outcome score. […] In our hands, younger patients and patients presenting with syringomyelia are more likely to experience a good outcome of surgery, and patients presenting with peripheral neuropathy or loss of sensitivity to pinprick are frequently less likely to experience a good outcome from surgery.

• #4 Syringomyelia Associated with Chiari 1 Malformation in Adults: Positive Outcome Predictors after Posterior Fossa Decompression with Duraplasty

  https://www.mdpi.com/2077-0383/12/8/3019

  Presurgery CSF flow at the CCJ is proposed as a radiological marker with the ability to predict a positive outcome after PFDD in adults with syringomyelia and CM1. […] A young age at surgery (<47 years) was a significant good outcome predictor; according to these results, as also proposed by Aghakani et al., surgery should be proposed as soon as possible in adults affected by syringomyelia with CM1. [...] The presence of CSF flow at the CCJ before surgery predicted (86% specificity) a positive outcome of PFDD surgery.

• #5

  https://journals.lww.com/neur/fulltext/2022/70030/development_and_validation_of_a_multivariable.13.aspx

  Surgical outcome predictive models for Chiari malformations (CM) which are applicable to all age groups and simple enough to use on outpatient basis are lacking. […] The aim of this study was to develop and validate a preoperative index for predicting long-term outcomes in Chiari 1 (CM1) and Chiari 0 (CM0) malformations. […] Outcome was negatively associated with presence of motor, sensory or cranial nerve symptoms, poor functional status, basilar invagination, and tonsillar descent. It was positively associated with shorter duration of presenting symptom (9 months) and syrinx diameter 6 mm. […] COPI predicted CCOS with 91.1% accuracy (10-fold cross-validation). It had excellent discrimination for improved outcome (c = 0.968 in development and 0.976 in validation cohort), at threshold index of -1.

• #6

  https://link.springer.com/article/10.1007/s00701-024-06332-3

  Posterior fossa decompression for Chiari malformation type I (Chiari 1) is effective and associated with a low risk of complication. However, up to 20% of patients may experience continued deficits or recurring symptoms after surgical intervention. […] For pediatric patients, there are no established tools to predict outcomes, and the risk factors for unfavorable postoperative outcomes are poorly understood. […] On multivariable proportional odds logistic regression, motor deficits (OR: 0.09; CI95%: [0.010.62]; p=0.015), and surgical complications (OR: 0.16; CI95%: [0.410.66]; p=0.011) were significant predictors of worse outcomes. The presence of syringomyelia was identified as a predictor of better outcomes (OR: 4.42 CI95% [1.0219.35]; p=0.048). A persistent hydrocephalus during the early postoperative period after posterior fossa decompression was a strong predictor of worse long-term CCOS (OR: 0.026; CI95%: [0.0020.328]; p=0.005).

• #7

  https://journals.lww.com/neur/fulltext/2022/70030/development_and_validation_of_a_multivariable.13.aspx

  COPI is simple tool that can be administered in outpatient setting. It can facilitate evidence-based preoperative counseling of patients, to help them develop reasonable expectations regarding surgical outcomes. […] Pre-operative counselling is a key factor in post-operative satisfaction level of patients. Chiari outcome predictive index is a simple tool which can facilitate preoperative counselling of patients, to help them develop reasonable expectations regarding surgical outcomes. […] Various clinical and radiological factors have been analyzed to predict post-surgical outcome in CM. […] COPI reliably predicts outcome based on CCOS and hence predicts patient’s self-perceived improvement in quality of life as well as postoperative objective improvement in sign and symptoms. […] We found that shorter duration of presenting symptoms (9 months), small syrinx (6 mm) and good initial functional status to favor improved outcome, and presence of motor, sensory, LCN symptoms, poor functional status, tonsillar descent, and BI to negatively affect the outcome.

• #8 Syringomyelia Associated with Chiari 1 Malformation in Adults: Positive Outcome Predictors after Posterior Fossa Decompression with Duraplasty

  https://www.mdpi.com/2077-0383/12/8/3019

  Syringomyelia (Syr) in patients with Chiari 1 malformation (CM1) may be attributable to abnormal dynamics of cerebrospinal fluid (CSF) in the upper cervical segment; fourth ventricle enlargement has been reported in association with a worse clinical and radiological presentation, independently of the posterior fossa volume. […] As a primary endpoint, we aimed to correlate improvement in the fourth ventricle area with positive clinical outcomes. […] Surgery was associated with positive clinical and radiological outcomes in more than 90% of cases. […] The presurgical presence of CSF flow at the CCJ was able to predict a good outcome (AUC = 0.68, 95% CI 0.50–0.87 and LH+ = 2.1, IC 95% 1.16–3.07) and was also significantly associated with post-surgical pain relief (rho = 0.61 and p = 0.0144).

• #9

  https://link.springer.com/article/10.1007/s00701-024-06332-3

  Results from this study indicate that the existence of motor deficits and syringomyelia prior to surgery, and surgical complications and persistent hydrocephalus despite posterior fossa decompression, were useful predictors of long-term outcome. […] The presence of motor deficits and occurrence of perioperative complications was associated with worse CCOS, while the presence of syringomyelia was associated with improved CCOS at the last follow-up. […] A hydrocephalus not responding to decompression surgery during the early postoperative period (median: 5 months) was a strong predictor of worse long-term CCOS, as compared to patients with no, or those with postoperatively improved hydrocephalus (OR: 0.026; CI95%: [0.0020.328]; p=0.005). […] The occurrence of peri- and postoperative complications were found to be a significant predictor of negative outcomes.

• #10 A points-based algorithm for prognosticating clinical outcome of Chiari malformation Type I with syringomyelia: results from a predictive model analysis of 82 surgically managed adult patients in: Journal of Neurosurgery: Spine Volume 28 Issue 1 (2017) Jo

  https://thejns.org/spine/view/journals/j-neurosurg-spine/28/1/article-p23.xml

  The presence of gait imbalance and motor deficits independently predict worse clinical and radiological outcomes, respectively, after decompressive surgery for CMI with altered hindbrain CSF flow. […] The proposed points-based algorithm has good predictive value for postoperative multifactorial outcome in these patients.

• #11

  https://link.springer.com/article/10.1007/s00701-024-06332-3

  The current study revealed that the early hydrocephalus status, at a median of 5 months postoperatively, was a significant predictor of outcomes. In fact, patients with new onset postoperative hydrocephalus or those with worsening hydrocephalus were more likely to present worse CCOS scores at last follow-up. […] Implementation of strategies for the prevention of perioperative complications and appropriate management of hydrocephalus is essential to achieve good outcomes following posterior fossa decompression in Chiari 1 patients.

• #12 Surgical Outcomes in Chiari 1 and Chiari 1.5 Malformation Treated by Posterior Fossa Reconstruction: A Comprehensive Analysis of 110 Pediatric Cases and Literature Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11242314/

  The mean age of our child cohort was 9.9 4.7 years, with 54 girls (49%) and 56 boys (51%). Sixty-six children had CM-1 (60%) while forty-four had CM-1.5 (40%). Following surgery, there was no neurological worsening or death among the children. Most children (70%) had an uneventful recovery and were discharged home on average one week after surgery. However, in 33 children (30%), we recorded at least one postoperative adverse event. Aseptic meningitis syndrome was the most frequent adverse event (n = 25, 22.7%). The final surgical outcome was evaluated one year after PFR by using both clinical and neuroradiological results. The one-year surgical outcome was excellent in 101 children (91.9%), good in 5 (4.5%), and bad in 4 (3.6%). […] PFR significantly enlarges the volume of the posterior fossa and recreates a CSF environment that generates buoyancy of the cerebellum, with a high percentage of excellent and good clinical results evaluated one year post-surgery. […] The final surgical outcome was excellent in 101 children (91.9%), good in 5 (4.5%), and bad in 4 (3.6%). In the four children with bad one-year outcomes, the reasons were no changes in Syr in three children and the development of a new Syr in one.

• #13

  https://link.springer.com/article/10.1007/s00701-024-05999-y

  The purpose of our study was to examine the long-term outcomes of operated Chiari malformation type 1 (CM1) patients and evaluate whether different duraplasty techniques affected outcome after surgery in Kuopio University Hospital catchment area. […] The mean clinical follow-up time was 26.5 months (SD 33.5 months). […] After surgery, preoperative symptoms alleviated in 84.9% (79/93) and the postoperative syringomyelia regression rate was 89.2% (33/37) of all patients. […] No significant correlation between postoperative outcome and extent of dural decompression, or type of duraplasty performed was found. […] This is the largest reported series of surgically treated CM1 patients in Finland. […] Duraplasty technique had no significant difference in complication rate or long-term outcomes.

• #14

  https://link.springer.com/article/10.1007/s00701-024-05999-y

  In our cohort, which is, the largest reported series of surgically treated CM1 patients in Finland, most of the patients underwent PFDD and no statistically significant factors between the variable extent of dural decompression, or type of duraplasty performed were identified to affect any postoperative outcomes, i.e., complication rate either. […] In this study, the most common complication after CM1 surgery was aseptic meningitis in 25.8%, which is in line with previous literature. […] We found an 89.2% decrease of syrinx size after surgery, and the duraplasty technique did not significantly affect the outcome. […] Considering the relatively high rate of postoperative complications with or without syringomyelia, the correct indication to surgery and a patient-tailored choice of surgical techniques are crucial in treatment.

• #15

  https://journals.lww.com/neur/fulltext/2022/70030/development_and_validation_of_a_multivariable.13.aspx

  The classes of COPI probably defines the severity of disease, with class A being least severe and class C being most severe. […] COPI is a simple tool that can be used in the outpatient clinic to predict clinical outcome in CM patients with good accuracy and discriminative power. It can facilitate evidence based preoperative counselling of patients and help them develop reasonable expectations regarding surgical outcomes.

• #16 Chiari Malformation: What It Is, Symptoms, Types & Treatment

  https://my.clevelandclinic.org/health/diseases/6008-chiari-malformation

  A Chiari malformation can be very serious in some cases, but not all. In most cases, babies are born with a Chiari malformation. However, symptoms arent always present, and a diagnosis wont happen until late childhood or adulthood when a healthcare provider orders an imaging test for another unrelated reason. […] Although there isnt a cure for a Chiari malformation, treatment like surgery can help manage your symptoms to help you feel better and prevent life-threatening complications. […] Your outlook varies depending on the severity and type of Chiari malformation. Its important to work closely with your healthcare provider and care team, who can monitor your symptoms and determine the right treatment plan for you. […] If you dont have symptoms or you have mild symptoms after a Chiari malformation diagnosis, youll likely have a normal life expectancy. Severe symptoms and certain types of Chiari malformation can be fatal. Talk to your healthcare provider about what to expect after a diagnosis.

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