Natural history of Chiari malformation Type I following decision for conservative treatment

Strahle, Jennifer; Muraszko, Karin M.; Kapurch, Joseph; Bapuraj, Jayapalli Rajiv; Garton, Hugh; Maher, Cormac O.

doi:10.3171/2011.5.peds1122

Cited by 130 publications

(69 citation statements)

References 44 publications

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“…Because spontaneous syrinx improvement has been reported, it is difficult to draw any conclusions from individual case reports. 5,14,16,24,29,32 Despite the theoretical basis for such a relationship, we did not find any association between elevated BMIs and risk of syrinx in our own series. On the basis of these findings and our own data, we suggest that no association between BMI and syrinx formation in patients with CM-I can be proven with the existing data.…”

Section: Discussioncontrasting

confidence: 37%

Impact of body mass index on cerebellar tonsil position in healthy subjects and patients with Chiari malformation

Smith¹,

Strahle²,

Kazarian³

et al. 2015

JNS

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OBJECT It is unclear if there is a relationship between Chiari malformation Type I (CM-I) and body mass index (BMI). The aim of this study was to identify the relationship between BMI and cerebellar tonsil position in a random sample of people. METHODS Cerebellar tonsil position in 2400 subjects from a cohort of patients undergoing MRI was measured. Three hundred patients were randomly selected from each of 8 age groups (from 0 to 80 years). A subject was then excluded if he or she had a posterior fossa mass or previous posterior fossa decompression or if height and weight information within 1 year of MRI was not recorded in the electronic medical record. RESULTS There were 1310 subjects (54.6%) with BMI records from within 1 year of the measured scan. Of these subjects, 534 (40.8%) were male and 776 (59.2%) were female. The average BMI of the group was 26.4 kg/m2, and the average tonsil position was 0.87 mm above the level of the foramen magnum. There were 46 subjects (3.5%) with a tonsil position ≥ 5 mm below the level of the foramen magnum. In the group as a whole, there was no correlation (R2 = 0.004) between BMI and cerebellar tonsil position. CONCLUSIONS In this examination of 1310 subjects undergoing MRI for any reason, there was no relationship between BMI and the level of the cerebellar tonsils or the diagnosis of CM-I on imaging.

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Section: Discussioncontrasting

confidence: 37%

Impact of body mass index on cerebellar tonsil position in healthy subjects and patients with Chiari malformation

Smith¹,

Strahle²,

Kazarian³

et al. 2015

JNS

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“…The authors proposed that the presence of a syrinx was not always associated with symptomatic progression since only 6 of 20 patients with a syrinx had surgery during the follow-up interval, and 2 of these patients underwent surgery for reasons other than the syrinx. 31 Other investigators have added to the debate of surgery as necessary intervention in the setting of CM-I and syrinx. 8,24 Nishizawa and colleagues found over a 10-year follow-up interval that only 1 of 9 adult patients with incidental CM-I and syrinx required decompression.…”

Section: Discussionmentioning

confidence: 99%

“…The authors found that the majority of patients did not progress clinically or radiologically, and no new neurological deficits were observed during a mean follow-up period of 2.83 years. 6 Another analysis by Strahle et al in 2011 found that the majority of 147 pediatric patients with CM-I, who were also managed conservatively, tended to have a benign natural history over the course of clinical and MRI follow-up, with a mean duration of 4.6 and 3.8 years, respectively.…”

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confidence: 99%

Natural and surgical history of Chiari malformation Type I in the pediatric population

Pomeraniec

Ksendzovsky

Awad

et al. 2016

PED

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OBJECT The natural and surgical history of Chiari malformation Type I (CM-I) in pediatric patients is currently not well described. In this study the authors discuss the clinical and radiological presentation and outcomes in a large cohort of pediatric CM-I patients treated with either conservative or surgical management. METHODS The authors retrospectively reviewed 95 cases involving pediatric patients with CM-I who presented between 2004 and 2013. The patients ranged in age from 9 months to 18 years (mean 8 years) at presentation. The cohort was evenly split between the sexes. Twenty-five patients underwent posterior fossa decompression (PFD) with either dural splitting or duraplasty. Seventy patients were managed without surgery. Patients were followed radiologically (mean 44.8 months, range 1.2–196.6 months) and clinically (mean 66.3 months, range 1.2–106.5 months). RESULTS Seventy patients were treated conservatively and followed with serial outpatient neurological and radiological examinations, whereas 25 patients were treated with PFD. Of these 25 surgical patients, 11 were treated with duraplasty (complete dural opening) and 14 were treated with a dura-splitting technique (incomplete dural opening). Surgical intervention was associated with better clinical resolution of symptoms and radiological resolution of tonsillar ectopia and syringomyelia (p = 0.0392). Over the course of follow-up, 20 (41.7%) of 48 nonsurgical patients who were symptomatic at presentation experienced improvement in symptoms and 18 (75%) of 24 symptomatic surgical patients showed clinical improvement (p = 0.0117). There was no statistically significant difference in resolution of symptoms between duraplasty and dura-splitting techniques (p = 0.3572) or between patients who underwent tonsillectomy and tonsillopexy (p = 0.1667). Neither of the 2 patients in the conservative group with syrinx at presentation showed radiological evidence of resolution of the syrinx, whereas 14 (87.5%) of 16 patients treated with surgery showed improvement or complete resolution of syringomyelia (p = 0.0392). In the nonsurgical cohort, 3 patients (4.3%) developed new or increased syrinx. CONCLUSIONS The overwhelming majority of CM-I patients (92.9%) managed conservatively do not experience clinical or radiological progression, and a sizeable minority (41.7%) of those who present with symptoms improve. However, appropriately selected symptomatic patients (sleep apnea and dysphagia) and those presenting with syringomyelia should be considered surgical candidates because of the high rates of clinical (75%) and radiological improvement (87.5%).

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“…150,151,156 Symptoms and MRI findings are stable over time for most patients, with no symptoms or minimal symptoms that are followed without surgical treatment, although spontaneous improvement or worsening of both the CM as well as syringomyelia does occasionally occur. 150,151,156 Although most cases of clinically relevant syringomyelia are associated with CM, it may also be seen in patients with spinal cord tumor, tethered cord, or arachnoiditis. 157,158 In general, the management of syringomyelia should be directed at the primary disorder.…”

Section: Chiari and Syringomyeliamentioning

confidence: 99%

Incidental Findings on Brain and Spine Imaging in Children

Maher¹,

Piatt²,

Ragheb³

et al. 2015

Pediatrics

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In recent years, the utilization of diagnostic imaging of the brain and spine in children has increased dramatically, leading to a corresponding increase in the detection of incidental findings of the central nervous system. Patients with unexpected findings on imaging are often referred for subspecialty evaluation. Even with rational use of diagnostic imaging and subspecialty consultation, the diagnostic process will always generate unexpected findings that must be explained and managed. Familiarity with the most common findings that are discovered incidentally on diagnostic imaging of the brain and spine will assist the pediatrician in providing counseling to families and in making recommendations in conjunction with a neurosurgeon, when needed, regarding additional treatments and prognosis.

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Natural history of Chiari malformation Type I following decision for conservative treatment

Cited by 130 publications

References 44 publications

Impact of body mass index on cerebellar tonsil position in healthy subjects and patients with Chiari malformation

Impact of body mass index on cerebellar tonsil position in healthy subjects and patients with Chiari malformation

Natural and surgical history of Chiari malformation Type I in the pediatric population

Incidental Findings on Brain and Spine Imaging in Children

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