Treatment of Syringomyelia Associated with Chiari malformation (Type I), FMD was effective-2 Case Reports-

Chiari malformations (Types I–IV) are abnormalities of the posterior fossa that affect the cerebellum, brainstem, and the spinal cord with prevalence rates of 0.1%–0.5%. Case reports of familial aggregation of Chiari malformation, twin studies, cosegregation of Chiari malformation with known genetic conditions, and recent gene and genome-wide association studies provide strong evidence of the genetic underpinnings of familial Chiari malformation. The authors report on a series of 3 family pairs with Chiari malformation Type I: 2 mother-daughter pairs and 1 father-daughter pair. The specific genetic causes of familial Chiari malformation have yet to be fully elucidated. The authors review the literature and discuss several candidate genes. Recent advances in the understanding of the genetic influences and pathogenesis of familial Chiari malformation are expected to improve management of affected patients and monitoring of at-risk family members.

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Two Case Reports of Pediatric Chiari Malformation Type I

Practica Oto-Rhino-Laryngologica ◽

10.5631/jibirin.103.987 ◽

2010 ◽

Vol 103 (11) ◽

pp. 987-996

Author(s):

Nobuyuki Fujimoto

Keyword(s):

Chiari Malformation ◽

Case Reports ◽

Type I ◽

Chiari Malformation Type I

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Spontaneous resolution of Chiari malformation Type I in monozygotic twins

Journal of Neurosurgery Pediatrics ◽

10.3171/ped.2008.2.11.317 ◽

2008 ◽

Vol 2 (5) ◽

pp. 317-319 ◽

Cited By ~ 14

Author(s):

Jeffrey H. Miller ◽

David D. Limbrick ◽

Matthew Callen ◽

Matthew D. Smyth

Keyword(s):

Chiari Malformation ◽

Case Reports ◽

Monozygotic Twins ◽

Spontaneous Resolution ◽

Breath Holding ◽

Type I ◽

Chiari Malformation Type I ◽

Atypical Symptoms ◽

Nonsurgical Management

The spontaneous resolution of isolated tonsillar ectopia in Chiari malformation Type I (CM-I) is a known and reported entity in 2 previous single study case reports. However, it has not been previously described in monozygotic twins. Two children, ~ 1 year of age with CM-I and presumed episodes of pallid syncope or breath-holding spells presented for neurosurgical evaluation. Although Chiari decompression was considered, the authors decided to proceed with conservative management with close follow-up due to the uncertain nature of these episodes. Approximately 4 years later, both children's symptoms had resolved. Repeated MR imaging examinations also showed spontaneous resolution of the malformation in both girls. These cases emphasize that when patients with CM-I present with atypical symptoms, spontaneous resolution or improvement is possible, which may influence the decision to pursue a trial of nonsurgical management. The possible pathophysiological mechanisms and genetic influences of CM-I are also briefly discussed.

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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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