Defining the signs and symptoms of 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.

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

Journal of Neurosurgery Pediatrics ◽

10.3171/2010.3.peds09489 ◽

2010 ◽

Vol 5 (6) ◽

pp. 554-561 ◽

Cited By ~ 69

Author(s):

Gregory W. Albert ◽

Arnold H. Menezes ◽

Daniel R. Hansen ◽

Jeremy D. W. Greenlee ◽

Stuart L. Weinstein

Keyword(s):

Surgical Treatment ◽

Medical Records ◽

Chiari Malformation ◽

Early Recognition ◽

Signs And Symptoms ◽

Dramatic Improvement ◽

Type I ◽

Chiari Malformation Type I ◽

Initial Symptoms ◽

Oropharyngeal Dysfunction

Object 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. Methods 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. Results 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. Conclusions 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.

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Intraoperative ultrasonography used to determine the extent of surgery necessary during posterior fossa decompression in children with Chiari malformation Type I

Yearbook of Diagnostic Radiology ◽

10.1016/s0098-1672(08)70086-9 ◽

2007 ◽

Vol 2007 ◽

pp. 116-117

Author(s):

A.E. Oestreich

Keyword(s):

Posterior Fossa ◽

Chiari Malformation ◽

Intraoperative Ultrasonography ◽

Posterior Fossa Decompression ◽

Type I ◽

Chiari Malformation Type I ◽

Extent Of Surgery

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Management of Chiari Malformation Type I with Posterior Fossa Decompression without Dural Opening

Journal of Neurological Surgery Part A Central European Neurosurgery ◽

10.1055/s-0035-1566365 ◽

2015 ◽

Vol 76 (S 02) ◽

Author(s):

Walid Elhalaby

Keyword(s):

Posterior Fossa ◽

Chiari Malformation ◽

Posterior Fossa Decompression ◽

Type I ◽

Chiari Malformation Type I ◽

Dural Opening

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Prognostic significance of C1–C2 facet malalignment after surgical decompression in adult Chiari malformation type I: a pilot study based on the Chicago Chiari Outcome Scale

Journal of Neurosurgery Spine ◽

10.3171/2020.6.spine20544 ◽

2020 ◽

pp. 1-7

Author(s):

Michael Lumintang Loe ◽

Tito Vivas-Buitrago ◽

Ricardo A. Domingo ◽

Johan Heemskerk ◽

Shashwat Tripathi ◽

...

Keyword(s):

Pilot Study ◽

Chiari Malformation ◽

Prognostic Significance ◽

Foramen Magnum ◽

Bivariate Analysis ◽

Type I ◽

Foramen Magnum Decompression ◽

Swallowing Function ◽

Chiari Malformation Type I

OBJECTIVEThe authors assessed the prognostic significance of various clinical and radiographic characteristics, including C1–C2 facet malalignment, in terms of surgical outcomes after foramen magnum decompression of adult Chiari malformation type I.METHODSThe electronic medical records of 273 symptomatic patients with Chiari malformation type I who were treated with foramen magnum decompression, C1 laminectomy, and duraplasty at Mayo Clinic were retrospectively reviewed. Preoperative and postoperative Neurological Scoring System scores were compared using the Friedman test. Bivariate analysis was conducted to identify the preoperative variables that correlated with the patient Chicago Chiari Outcome Scale (CCOS) scores. Multiple linear regression analysis was subsequently performed using the variables with p < 0.05 on the bivariate analysis to check for independent associations with the outcome measures. Statistical software SPSS version 25.0 was used for the data analysis. Significance was defined as p < 0.05 for all analyses.RESULTSFifty-two adult patients with preoperative clinical and radiological data and a minimum follow-up of 12 months were included. Motor deficits, syrinx, and C1–C2 facet malalignment were found to have significant negative associations with the CCOS score at the 1- to 3-month follow-up (p < 0.05), while at the 9- to 12-month follow-up only swallowing function and C1–C2 facet malalignment were significantly associated with the CCOS score (p < 0.05). Multivariate analysis showed that syrinx presence and C1–C2 facet malalignment were independently associated with the CCOS score at the 1- to 3-month follow-up. Swallowing function and C1–C2 facet malalignment were found to be independently associated with the CCOS score at the 9- to 12-month follow-up.CONCLUSIONSThe observed results in this pilot study suggest a significant negative correlation between C1–C2 facet malalignment and clinical outcomes evaluated by the CCOS score at 1–3 months and 9–12 months postoperatively. Prospective studies are needed to further validate the prognostic value of C1–C2 facet malalignment and the potential role of atlantoaxial fixation as part of the treatment.

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