Treatment Of Chiari Malformation Type I Research Articles

Craniocervical Stabilization After Failed Chiari Decompression: A Case Series of a Population with High Prevalence of Ehlers-Danlos Syndrome

In the treatment of Chiari malformation type I (CM-I), posterior fossa decompression is achieved via suboccipital craniectomy (SOC); however, some patients continue to experience symptoms after treatment, which may be due to craniocervical instability (CCI). The purposes of this study were to analyze data from patients who required an occipitocervical fusion (OCF) for the management of CCI after having previously undergone SOC for CM-I to determine if OCF is a safe and effective option and to determine any identifiable risk factors for CCI in these patients. A retrospective review was done on all patients who underwent an OCF performed by the senior author between November 2013 and June 2020 after having previously undergone SOC for CM-I. Demographic, radiographic, perioperative, and outcome data were collected and clivoaxial angles (CXAs) were measured pre- and postoperatively. Fifteen patients were identified who developed symptomatic CCI after previously undergoing a suboccipital craniectomy for the treatment of CM-I. All 15 patients were treated by OCF with good outcome. Of these, 12 patients had a known diagnosis of Ehlers-Danlos syndrome (EDS). Overall, the CXAs of these patients were found to be corrected to a more anatomical alignment. Symptomatic CCI should be recognized as a delayed postoperative complication in the surgical treatment of CM-I, with an underlying connective tissue hypermobility disorder such as EDS serving as a potential risk factor its development. CCI can be managed with OCF as a safe and effective treatment option for this patient population.

Posterior calvarial distraction for complex craniosynostosis and cerebellar tonsillar herniation.

Children with syndromic, multisuture, and lambdoid craniosynostosis undergoing calvarial surgery often have Chiari malformation type I (CM-I) (or cerebellar tonsillar herniation). The optimal management of this patient group, including the surgical techniques and timing of surgery, remains uncertain. Posterior calvarial distraction (PCD) is an effective method to increase the supratentorial cranial volume and improve raised intracranial pressure in children with complex craniosynostosis. This study investigated the efficacy of PCD in posterior fossa (PF) volume expansion and treatment of CM-I and associated syringomyelia (syrinx) in this group of children. This retrospective study included patients who were surgically treated between 2006 and 2015. Over 10 years, 16 patients with multisuture synostosis, lambdoid synostosis, or craniosynostosis associated with a confirmed genetic syndrome, and a concurrent CM-I, were included. The mean age at the time of surgery was 5.1 years (range 8 months-18 years). Fourteen patients had pansynostosis and 2 had lambdoid synostosis. Eight had a confirmed syndromic diagnosis (Crouzon in 8, Apert in 4, Pfeiffer in 1, and Saethre-Chotzen in 1). Ten patients had raised intracranial pressure; 4 had syringomyelia. The average clinical follow-up was 50 months (range 9-116 months). Clinically, 9 patients improved, 7 remained stable, and none deteriorated. The average distraction distance was 23 mm (range 16-28 mm). The PF anterior-posterior (AP) distance/width ratio increased from 0.73 to 0.80 mm (p = 0.0004). Although an osteotomy extending inferior to the torcula (compared with superior) was associated with a larger absolute PF AP distance increase (13 vs 6 mm, p = 0.028), such a difference was not demonstrable when the PF AP distance/width ratio was calculated. Overall, the mean tonsillar herniation improved from 9.3 to 6.0 mm (p = 0.011). Syrinx dimensions also improved in the AP (from 7.9 to 3.1 mm) and superior-inferior (from 203 to 136 mm) dimensions. No patients required further foramen magnum decompression for CM. Of the 16 patients, 2 had subsequent frontoorbital advancement and remodeling, of which 1 was for volume expansion and 1 was for cosmetic purposes. Two patients required CSF shunt insertion after PCD. Following PCD, PF volume increased as well as supratentorial volume. This morphometric change was observed in osteotomies both inferior and superior to the torcula. The PF volume increase resulted in improvement of cerebellar tonsillar herniation and syrinx. PCD is an efficacious first-line, single-stage treatment for concurrent pansynostosis and lambdoid craniosynostosis, CM-I, and syrinx.

Minimally invasive posterior fossa decompression with duraplasty in Chiari malformation type I with and without syringomyelia

Background:Posterior fossa decompression (PFD), with and without duraplasty, represents a valid treatment in Chiari malformation Type I (CM-I) with and without syringomyelia. Despite a large amount of series reported in literature, several controversies exist regarding the optimal surgical approach yet. In this study, we report our experience in the treatment of CM-I, with and without syringomyelia, highlighting how the application of some technical refinements could lead to a good outcome and a lesser rate of complications.Methods:Twenty-six patients with CM-I, with and without syringomyelia, underwent PFD through a 3 cm × 3 cm craniectomy with the removal of the most median third of the posterior arch of C1 and duraplasty. Signs and symptoms included sensory deficits, motor deficits, neck pain, paresthesias, headache, dizziness, lower cranial nerve deficits, and urinary incontinence. Postoperative magnetic resonance (MR) was performed in all patients.Results:Signs and symptoms improved in 76.9% of cases. Postoperative MR revealed a repositioning of cerebellar tonsils and the restoration of cerebrospinal fluid circulation. In our experience, the rate of complication was 23% (fistula, worsening of symptoms, and respiratory impairment).Conclusion:PFD through a 3 cm × 3 cm craniectomy and the removal of the most median third of posterior arch of C1 with duraplasty represents a feasible and valid surgical alternative to treat patients with CM-I, with and without syringomyelia, achieving a good outcome and a low rate of complications.

Posterior fossa decompression for Chiari malformation type I: clinical and radiological presentation, outcome and complications in a retrospective series of 105 procedures.

Determining clinical and radiological characteristics, complication rates and outcome for patients undergoing posterior fossa decompression (PFD) and duraplasty for Chiari malformation type I (CM-I). Retrospective, single-university hospital study of all PFDs for CM-I between January 1995 and December 2016. PFD was performed in 105 patients with CM-I (n = 105), of whom 62 suffered from associated syringomyelia and 37 were pediatric cases. There was a female preponderance in the syringomyelia, non-syringomyelia, pediatric and adult groups (62.9%, 65.1%, 54.1% and 69.1%, respectively). Mean age at diagnosis was higher in the syringomyelia group (32.3 versus 26.9 years, p = 0.06) and in both groups delay for diagnosis was long (33.4 and 47.1 months, p = 0.50). Headache during Valsalva maneuver and on exertion was significantly more present in the non-syringomyelia group (27.9% versus 16.1%, p < 0.0001 and 39.5% versus 11.3%, p = 0.0009); cranial nerve dysfunction and cerebellar signs did not differ significantly (p = 0.07 and p = 0.53). Spinal cord syndrome was significantly more present in the syringomyelia group (72.6% versus 25.6%, p < 0.0001). Scoliosis was present in five patients, of whom four had syringomyelia. Duraplasty was performed in 101 surgeries. For 100 patients, PFD was the primary treatment of CM-I; 2 patients had previously undergone syrinx drainage and 3 ventriculoperitoneal shunting for hydrocephalus. Only one patient presented with hydrocephalus. Duraplasty (n = 101) was complicated by CSF leak in 4.0% (n = 4), symptomatic pseudomeningocele in 4.0% (n = 4), aseptic meningitis in 2.0% (n = 2) and hydrocephalus in 1.0% (n = 1). Osseous decompression without duraplasty was performed in only four highly selected patients, not allowing a comparative analysis with duraplasty. Post-operative symptom improvement is reported for 67.3% of all patients and stabilization of symptoms in 23.9%. Symptom improvement was significantly more frequent in the non-syringomyelia group (p = 0.03). Outcome seemed similarly good in the pediatric and the adult groups. PFD performed with duraplasty is a safe procedure. The majority of patients do report symptom stabilization or improvement. Non-syringomyelia patients reported significantly more frequent improvement of clinical symptoms.

Comparison between decompressed and non-decompressed Chiari Malformation type I patients: A neuropsychological study

BackgroundPrevious studies have suggested an association of Chiari Malformation type I (CM-I) and cognitive deficits. CM-I is a neurological disorder characterized by a descent of cerebellar tonsils into the foramen magnum, resulting in overcrowding of the upper cervical spine region. Posterior fossa decompression (PFD) is the surgical treatment of choice, however, the literature on the consequences for patients is mainly reduced to the assessment of physical symptoms. MethodsData from a neuropsychological assessment of 76 patients with CM-I, both with PFD (n = 37) and without PFD (n = 39) surgery, and 76 healthy controls, matched by gender, age and years of education are reported. ResultsCM-I patients show a generally lower cognitive performance in executive function, verbal fluency, spatial cognition, language (naming), verbal memory, processing speed, emotional facial recognition and theory of mind, compared to control group. The results are maintained even after statistically controlling for the influence of perceived physical pain and the presence of anxious-depressive symptomatology. Data also illustrate a similar cognitive profile between both groups with CM-I. ConclusionThese findings provide evidence of a deficient cognitive profile associated with CM-I, regardless of the PFD surgery. According to these results, both physical and cognitive consequences must be considered in the treatment of CM-I.

Autologous cervical fascia duraplasty in 123 children and adults with Chiari malformation type I: surgical technique and complications.

OBJECT Techniques for combined extradural and intradural decompression with expansile duraplasty for Chiari malformation type I (CM-I) have been well described, with various allogenic and autologous materials used for duraplasty. However, the approach and surgical technique used for duraplasty in our treatment of CM-I and developed by the senior author in the 1990s has not been described. METHODS A prospective database was initiated in March 2003 to denote the use of cervical fascia for duraplasty and incorporate an ongoing detailed record of complications during the surgical treatment of children and adults with CM-I with and without syringomyelia. A total of 389 surgeries for CM-I were performed on 379 patients from March 2003 to June 2016. A total of 123 posterior procedures were performed on 123 patients in which both a posterior fossa extradural and intradural decompression with duraplasty (extra-intradural) was performed. In this paper the authors describe the surgical technique for harvesting and using cervical fascia for duraplasty in the surgical treatment of CM-I and analyze and discuss complications from a prospective database spanning 2003-2016. RESULTS The authors found that cervical fascia can be harvested in patients of all ages (2-61 years old) without difficulty, and it provides a good substitute for dura in creating an expansile duraplasty in patients with CM-I. Cervical fascia is an elastic-like material with a consistency that allows for a strong watertight closure. Harvesting the cervical fascia graft does not require any further extension of the incision superiorly or inferiorly to obtain the graft. Complications were uncommon in this study of 123 children and adults. The risk of any type of complication (aseptic meningitis, CSF leak, pseudomeningocele, infection, development of hydrocephalus, and need for ventriculoperitoneal shunt) for the 78 patients in the pediatric age group was 0%. The risk of complication in the adult group was 6.7% (1 patient with aseptic meningitis and 2 patients with CSF leak). CONCLUSIONS Autologous cervical fascia is easy to obtain in patients of all ages and provides an effective material for duraplasty in the treatment of CM-I. Complications from the combination of both an extradural and intradural decompression with autologous cervical fascia duraplasty are uncommon.

Time to Resolution of Symptoms After Suboccipital Decompression with Duraplasty in Children with Chiari Malformation Type I

Duraplasty is a technique successfully used to treat Chiari malformation type I (CM-I). This study describes the timely manner of clinical outcomes and the postoperative course after craniectomy and duraplasty for the treatment of symptomatic CM-I in children. A retrospective chart review was performed in 105 consecutive children who underwent surgical decompression of symptomatic CM-I with dural opening by a single surgeon between 1999 and2015. In 16 of 28 children (57%) with typical Valsalva-related/tussive and mixed headaches, the symptoms resolved before discharge; by 6 months, all children were headache-free. Two of 28 children (7%) had recurrent headaches 9 months after surgery. Among the 78 children with syrinx, syrinx resolved or decreased in 68 (87%), recurred in 8 (10%), and was stable in 2 children (3%). Syrinx was resolved or decreased by 3 months in 51 children (65%) and by 6 months in 62 children (79%). Complications included aseptic meningitis requiring reoperation in 3 children (3%) and infection in one child (1%). Twelve children underwent reoperation, none within the first 30 days. No child had a major morbidity or mortality. In carefully selected children with CM-I, a high success rate can be achieved with suboccipital decompression and duraplasty. Valsalva-related/tussive headaches resolved by the time of discharge from the hospital in the majority of children, and syrinx resolved or decreased in two-thirds of the children by 3 months. By 6 months, headaches were resolved in all cases, and syrinx was resolved or decreased in 79% of cases.

The addition of duraplasty to posterior fossa decompression in the surgical treatment of pediatric Chiari malformation Type I: a systematic review and meta-analysis of surgical and performance outcomes.

OBJECTIVE Surgery is the definitive treatment of Chiari malformation Type I (CM-I). It involves posterior fossa decompression, which can be performed along with C-1 laminectomy, reconstructive duraplasty, or tonsil shrinkage. The aim of this study was to provide an updated systematic review and meta-analysis of the latest available evidence regarding posterior fossa decompression only (PFDO) versus posterior fossa decompression with duraplasty (PFDD) in the treatment of CM-I in children. METHODS A literature search was performed in compliance with the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines for article identification, screening, eligibility, and inclusion. Relevant articles were identified from 6 electronic databases from their inception to April 2016. These articles were screened against established criteria for inclusion into this study. RESULTS From 12 relevant studies identified, 1492 pediatric patients treated via PFDD were compared with 1963 pediatric patients treated by PFDO for CM-I. PFDD was associated with greater overall clinical improvement (p = 0.009), along with longer length of stay (p < 0.0001) and more postoperative complications (p = 0.0001) compared with PFDO. No difference was observed between PFDD and PFDO in terms of revision surgery incidence (p = 0.13), estimated blood loss (p = 0.14), syrinx improvement (p = 0.09), or scoliosis improvement (p = 0.95). CONCLUSIONS It appears that the addition of duraplasty to posterior decompression in the definitive treatment of CM-I in children may alter surgical and performance outcomes. In particular, parameters of overall clinical improvement, length of stay, and postoperative complication may differ between children undergoing PFDD and those undergoing PFDO. Current evidence in the literature is of low to very low quality that, as of yet, has not been able to completely control for inherent selection bias both in study design and surgeon preference. Future, large prospective registries and randomized controlled trials are warranted to validate the findings of this study.

Trends in surgical treatment of Chiari malformation Type I in the United States

OBJECTIVE The goal of this analysis was to define temporal and geographic trends in the surgical treatment of Chiari malformation Type I (CM-I) in a large, privately insured health care network. METHODS The authors examined de-identified insurance claims data from a large, privately insured health care network of over 58 million beneficiaries throughout the United States for the period between 2001 and 2014 for all patients undergoing surgical treatment of CM-I. Using a combination of International Classification of Diseases (ICD) diagnosis codes and Current Procedural Terminology (CPT) codes, the authors identified CM-I and associated diagnoses and procedures over a 14-year period, highlighting temporal and geographic trends in the performance of CM-I decompression (CMD) surgery as well as commonly associated procedures. RESULTS There were 2434 surgical procedures performed for CMD among the beneficiaries during the 14-year interval; 34% were performed in patients younger than 20 years of age. The rate of CMD increased 51% from the first half to the second half of the study period among younger patients (p < 0.001) and increased 28% among adult patients between 20 and 65 years of age (p < 0.001). A large sex difference was noted among adult patients; 78% of adult patients undergoing CMD were female compared with only 53% of the children. Pediatric patients undergoing CMD were more likely to be white with a higher household net worth. Regional variability was identified among rates of CMD as well. The average annual rate of surgery ranged from 0.8 surgeries per 100,000 insured person-years in the Pacific census division to 2.0 surgeries per 100,000 insured person-years in the East South Central census division. CONCLUSIONS Analysis of a large nationwide health care network showed recently increasing rates of CMD in children and adults over the past 14 years.

Surgical Outcome after Posterior Fossa Decompression with and without Duraplasty in Adult Chiari Malformation Type-I

Background Data: Adult Chiari malformation is a heterogeneous group of conditions, with the underlying commonality of disruption of normal CSF flow through the foramen magnum. Some cases are congenital, but others are acquired. The optimal surgical treatment of adult Chiari malformation type-I is unclear. Purpose: To evaluate the operative and postoperative results of extradural and intradural approaches in the treatment of Chiari malformation type-I.Study Design: A descriptive retrospective clinical case study. Patients and Methods: Twenty patients underwent surgery for adult Chiari malformation type-I. They were divided into two groups; posterior fossa decompression group and posterior fossa decompression with duroplasty group. Each group included 10 patients. They were operated between 2008and 2015. Participants were evaluated pre-operatively and post-operatively every three months. Operative time, hospital stay and complications were assessed. The clinical outcomes were compared between the two groups using The Chicago Chiari Outcome Scale. Results: No statistically significant differences were found between the decompression and duroplasty groups with regard to demographics, preoperative symptoms, radiographic characteristics, and clinical outcomes. However, the operative time, hospital stay and aseptic meningitis were higher in the duroplasty group.Conclusion: The clinical outcome of posterior fossa decompression is nearly identical to that of posterior fossa decompression with duroplasty in adult patients with Chiari type-I; however, the operative time, hospital stay and complication rate is higher in duroplasty group. (2016ESJ113)

A novel autologous duraplasty in situ technique for the treatment of Chiari malformation Type I.

OBJECTIVE The purpose of this study was to introduce a novel autologous duraplasty procedure for the treatment of Chiari malformation Type I (CM-I). METHODS The authors retrospectively reviewed data from patients who had been diagnosed with CM-I and had undergone suboccipital decompression and autologous duraplasty in situ or synthetic dural graft duraplasty; patients were treated in the authors' department between 2011 and 2014. All procedures were performed by the same surgeon. The 2 duraplasty methods were compared in terms of surgical factors and complications. The authors assessed the neurological outcome and MRI-documented syrinx size at the 6-month follow-up visit. RESULTS Twenty-seven patients were enrolled in this study, 13 in the duraplasty in situ group and 14 in the synthetic dural graft duraplasty group. The results showed no significant differences between the duraplasty in situ and synthetic dural graft duraplasty groups in overall operative time (4.9 hours vs 4.1 hours; p = 0.070), estimated blood loss (229 ml vs 254 ml; p = 0.159), and duration of hospital stay after the operation (13.5 days vs 12.8 days; p = 0.808). In the duraplasty in situ group, 1 case of meningitis occurred (7.7%). In the synthetic dural graft duraplasty group, the complications included 1 case of meningitis (7.1%) and 1 CSF leak (7.1%). The mean cost of hospitalization in the duraplasty in situ group (CNY 23,354) was significantly lower than that in the synthetic dural graft duraplasty group (CNY 29,385; p = 0.036). CONCLUSIONS Compared with synthetic dural graft duraplasty, autologous duraplasty in situ is a safe, effective, and cost-effective procedure for the treatment of CM-I. The long-term outcome of this procedure requires investigation.

Chiari malformation Type I in children younger than age 6 years: presentation and surgical outcome

The authors conducted a study to evaluate the unique presenting signs and symptoms of Chiari malformation Type I (CM-I) in children younger than 6 years of age and highlight the benefits of early surgical treatment in this patient population. The authors reviewed the medical records of patients who presented to the neurosurgery department before their 6th birthday and subsequently underwent surgery for CM-I. They identified 39 patients who had been evaluated between 1984 and 2007 and examined the medical records for presentation, surgical intervention, and outcome. Children aged 0-2 years commonly presented with oropharyngeal dysfunction (77.8%). Children aged 3-5 years more frequently presented with syringomyelia (85.7%), scoliosis (38.1%), and/or headache (57.1%). All patients underwent posterior fossa craniectomy. Additionally, in many patients cervical laminectomy and/or duraplasty was performed. A few patients required transoral decompression and occipitocervical fusion. In most cases, surgery led to resolution or dramatic improvement of initial symptoms. Early recognition and surgical treatment of CM-I in young children leads to good outcomes in the majority of patients. Additional therapies for oropharyngeal dysfunction, syringomyelia, and scoliosis can frequently be avoided.

Comparison of posterior fossa decompression with and without duraplasty for the surgical treatment of Chiari malformation Type I in pediatric patients: a meta-analysis

Surgery for Chiari malformation Type I (CM-I) is one of the most common neurosurgical procedures performed in children, although there is clearly no consensus among practitioners about which surgical method is preferred. The objective of this meta-analysis was to compare the outcome of posterior fossa decompression with duraplasty (PFDD) and posterior fossa decompression without duraplasty (PFD) for the treatment of CM-I in children. The authors searched Medline-Ovid, The Cochrane Library, and the conference proceedings of the American Association of Neurological Surgeons and the Congress of Neurological Surgeons (2000-2007) for studies meeting the following inclusion criteria: 1) surgical treatment of CM-I; 2) surgical techniques of PFD and PFDD being reported in a single cohort; and 3) patient age < 18 years. Five retrospective and 2 prospective cohort studies involving a total of 582 patients met the criteria for inclusion in the meta-analysis. Of the 582 patients, 316 were treated with PFDD and 266 were treated with PFD alone. Patient age ranged from 6 months to 18 years. Patients undergoing PFDD had a significantly lower reoperation rate (2.1 vs 12.6%, risk ratio [RR] 0.23, 95% confidence interval [CI] 0.08-0.69) and a higher rate of cerebrospinal fluid-related complications (18.5 vs 1.8%, RR 7.64, 95% CI 2.53-23.09) than those undergoing PFD. No significant differences in either clinical improvement (78.6 vs 64.6%, RR 1.23, 95% CI 0.95-1.59) or syringomyelia decrease (87.0 vs 56.3%, RR 1.43, 95% CI 0.91-2.25) were noted between PFDD and PFD. Posterior fossa decompression with duraplasty is associated with a lower risk of reoperation than PFD but a greater risk for cerebrospinal fluid-related complications. There was no significant difference between the 2 operative techniques with respect to clinical improvement or decrease in syringomyelia.