Chiari 1 Malformation in a Child with Febrile Seizures, Parasomnias, and Sleep Apnea Syndrome

Introduction. The type I is the most common Chiari malformation in children. In this condition, the lower part of the cerebellum, but not the brain stem, extends into the foramen magnum at the base of the skull leading to disturbances in cerebrospinal fluid circulation and to direct compression of nervous tissue. Case report. We describe a 4-year-old Caucasian female child with febrile seizures, headache, parasomnias, and a delay of speech. The child underwent a magnetic resonance imaging to investigate these neurological signs, disclosing a Chiari malformation type 1. The polysomnography showed a mild-moderate sleep-disordered breathing, increased number of central sleep apneas, and generalized spike waves at sleep onset. Conclusions. Seizures have been seldom described in CM1 patients. The main reasons for performing MRI in this case were frequent seizures, a delay of speech, and headache, leading to an unexpected diagnosis of CM1. Polysomnography detected a discrete SDB.

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The Association of Chiari Type I Malformation and Neurofibromatosis Type 1

Clinical Pediatrics ◽

10.1177/000992289303200316 ◽

1993 ◽

Vol 32 (3) ◽

pp. 189-190 ◽

Cited By ~ 9

Author(s):

Joseph Dooley ◽

Daniel Vaughan ◽

Michael Riding ◽

Peter Camfield

Keyword(s):

Neurofibromatosis Type 1 ◽

Chiari Malformation ◽

Neurofibromatosis Type ◽

Foramen Magnum ◽

Type I ◽

Chiari Type ◽

Chiari Type I Malformation ◽

Chiari Type I ◽

Chiari Malformations

The association of neurofibromatosis type 1 (NF1) with Chiari malformations of the cerebellum and brain stem has been reported on only two previous occasions.1,2 The pathogenesis of both conditions has remained unclear, although the Chiari type I malformation is most likely due to hypoplasia of the posterior fossa with subsequent extension of the cerebellum through the foramen magnum.3 NF1 is also associated with a variety of cerebral dysplasias.4 We present a patient with both of these dysplastic lesions whose Chiari malformation was asymptomatic.

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Spinal neuraxial anaesthesia for caesarean section in a parturient with type I Arnold Chiari malformation and syringomyelia

SAGE Open Medical Case Reports ◽

10.1177/2050313x18786114 ◽

2018 ◽

Vol 6 ◽

pp. 2050313X1878611

Author(s):

Miqi Mavis Teo

Keyword(s):

Caesarean Section ◽

Chiari Malformation ◽

Elective Caesarean Section ◽

Foramen Magnum ◽

Surgical Decompression ◽

Type I ◽

Arnold Chiari Malformation ◽

Neuraxial Anaesthesia ◽

Current Guidelines

Introduction: Type 1 Arnold Chiari malformation is associated with prolapse of the cerebellar tonsils into or below the level of the foramen magnum and is usually diagnosed in adults. There are no current guidelines for the management of patients with a residual type I Arnold Chiari malformation, planned for a caesarean section under spinal neuraxial anaesthesia. The paucity in the literature on this topic presents as a management dilemma. Case report: We report a case of a term parturient with type 1 Arnold Chiari malformation, following surgical decompression 4 years earlier, with a residual syringomyelia that underwent an elective caesarean section under spinal neuraxial anaesthesia. Conclusion: This case highlights that multidisciplinary management and early anaesthetic consult are of paramount importance in the outcome of the patient, and that spinal neuraxial anaesthesia can be considered as a safe anaesthetic option.

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Clinical outcome following duraplasty in type 1 Arnold Chiari malformation

International Surgery Journal ◽

10.18203/2349-2902.isj20190553 ◽

2019 ◽

Vol 6 (3) ◽

pp. 857

Author(s):

Guruprasad Bettaswamy ◽

Rajesh R. Raykar ◽

Rajesh Kumar Singh ◽

Mahendra M.

Keyword(s):

Clinical Outcome ◽

Neck Pain ◽

Clinical Improvement ◽

Chiari Malformation ◽

Foramen Magnum ◽

Type I ◽

Foramen Magnum Decompression ◽

Improvement Rate ◽

Female Ratio

Background: Few authors support the use of duraplasty and few authors have reported a higher rate of complications associated with the same. The objective of the present endeavor was to study clinical outcome following duraplasty in type 1 Arnold Chiari malformation.Methods: Retrospectively, 24 cases and prospectively 18 cases diagnosed and operated for Chiari malformation type I were included. Patients with Chiari type II, III, and IV were excluded. A questionnaire was used to assess the improvement in neck pain and disability due to it, head pain and disability due to it and improvement in general health before and one year after surgery. The results of the questionnaire of both groups were analyzed and compared.Results: The most common age group of presentation was 2nd decade (35.71%) followed by 3rd decade (26.19%). The male to female ratio was 1.2:1. The most common presenting complaint was sensory disturbances (66.66%) followed by neck pain in 14 patients (33.33%). The most common sign was limb weakness in 21 patients (50%). 24 patients were operated with foramen magnum decompression with duraplasty and 18 patients were operated without duraplasty. There were more complications in the duraplasty group. Patients showed an overall clinical improvement of 83.33% in the duraplasty group compared to a lower overall clinical improvement rate of 55.50% in the no duraplasty group. Specific symptoms like neck pain showed similar rate of improvement of (88.89%) in the duraplasty group compared to no duraplasty group (80%).Conclusions: Foramen magnum decompression with duraplasty is superior to foramen magnum decompression without duraplasty although slightly higher rate of complication is seen with duraplasty.

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Spinal neuraxial anaesthesia for Caesarean section in a parturient with Type I Arnold–Chiari malformation and syringomyelia

Proceedings of Singapore Healthcare ◽

10.1177/2010105818784060 ◽

2018 ◽

Vol 28 (2) ◽

pp. 135-137

Author(s):

Mavis Miqi Teo

Keyword(s):

Caesarean Section ◽

Chiari Malformation ◽

Elective Caesarean Section ◽

Foramen Magnum ◽

Surgical Decompression ◽

Type I ◽

Arnold Chiari Malformation ◽

Neuraxial Anaesthesia ◽

Current Guidelines

Type 1 Arnold–Chiari Malformation is associated with prolapse of the cerebellar tonsils into or below the level of the foramen magnum and is usually diagnosed in adults. There are no current guidelines for the management of patients with a residual Type I Arnold–Chiari Malformation, planned for a Caesarean section under spinal neuraxial anaesthesia. The paucity of literature on this topic presents as a management dilemma. We report a case of a term parturient with Type 1 Arnold–Chiari Malformation, following surgical decompression four years earlier, with a residual syringomyelia who underwent an elective Caesarean section under spinal neuraxial anaesthesia. This case highlights that multidisciplinary management and an early anaesthetic consult is of paramount importance in the outcome of the patient, and that spinal neuraxial anaesthesia can be considered as a safe anaesthetic option.

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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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Standard and cardiac-gated phase-contrast magnetic resonance imaging in the clinical course of patients with Chiari malformation Type I

Neurosurgical FOCUS ◽

10.3171/2011.7.focus11105 ◽

2011 ◽

Vol 31 (3) ◽

pp. E5 ◽

Cited By ~ 19

Author(s):

Uwe Max Mauer ◽

Andreas Gottschalk ◽

Carolin Mueller ◽

Linda Weselek ◽

Ulrich Kunz ◽

...

Keyword(s):

Mr Imaging ◽

Chiari Malformation ◽

Phase Contrast ◽

Foramen Magnum ◽

Central Canal ◽

Type I ◽

Csf Flow ◽

Chiari Malformation Type I ◽

Cerebrospinal Fluid Flow ◽

Contrast Magnetic Resonance

Object The causal treatment of Chiari malformation Type I (CM-I) consists of removing the obstruction of CSF flow at the level of the foramen magnum. Cerebrospinal fluid flow can be visualized using dynamic phase-contrast MR imaging. Because there is only a paucity of studies evaluating CSF dynamics in the region of the spinal canal on the basis of preoperative and postoperative measurements, the authors investigated the clinical usefulness of cardiacgated phase-contrast MR imaging in patients with CM-I. Methods Ninety patients with CM-I underwent preoperative MR imaging of CSF pulsation. Syringomyelia was present in 59 patients and absent in 31 patients. Phase-contrast MR imaging of the entire CNS was used to investigate 22 patients with CM-I before surgery and after a mean postoperative period of 12 months (median 12 months, range 3–33 months). In addition to the dynamic studies, absolute flow velocities, the extension of the syrinx, and tonsillar descent were also measured. Results The changes in pulsation were highly significant in the region of the (enlarged) cistern (p = 0.0005). Maximum and minimum velocities (the pulsation amplitude) increased considerably in the region where the syrinx was largest in diameter. The changes of pulsation in these patients were significant in the subarachnoid space in all spinal segments but not in the syrinx itself and in the central canal. Conclusions The demonstration of CSF flow pulsation can contribute to assessments of surgical outcomes. The results presented here, however, raise doubts about current theories on the pathogenesis of syringomyelia.

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A Case of Chiari Malformation Type I : Improvement of Syringomyelia and Scoliosis by Foramen Magnum Decompression Alone

Japanese Journal of Neurosurgery ◽

10.7887/jcns.8.727 ◽

1999 ◽

Vol 8 (11) ◽

pp. 727-731

Author(s):

Tetsuya Kubota ◽

Kenichi Nishiyama ◽

Akira Tamura ◽

Kouichi Kawasaki ◽

Hiroshi Masuda ◽

...

Keyword(s):

Chiari Malformation ◽

Foramen Magnum ◽

Type I ◽

Foramen Magnum Decompression ◽

Chiari Malformation Type I

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Trzyipółletni chłopiec z malformacją Chiariego typu I – opis przypadku

Nowa Pediatria ◽

10.25121/np.2017.21.3.87 ◽

2017 ◽

Vol 21 (3) ◽

Author(s):

Anna Worch ◽

Małgorzata Wielopolska

Keyword(s):

Chiari Malformation ◽

Foramen Magnum ◽

Common Type ◽

Type I ◽

Chiari Malformation Type I ◽

Chiari Type I Malformation ◽

Large Head ◽

Exacerbation Of Symptoms ◽

Chiari Type I ◽

Cerebellar Tonsils

Chiari malformation is the most common structural defect of the posterior fossa and cerebellum, consisting of displacements of hindbrain inclination through the foramen magnum, often associated with syringomyelia. In the most common type I of this disease, only the cerebellar tonsils are descended. Chiari type I malformation may remain asymptomatic in early childhood, however a number of unspecific symptoms, such as: neck pains and headaches, vertigos, balance and sight disorders, may strengthen with age. Because of unspecificity and various exacerbation of symptoms the disease is often misdiagnosed or undiagnosed. We describe a case of a 3.5-year old boy with whom his mother visited a pediatrician because of a chronic cough. During the examination mother of the patient informed that all infections start with vomiting and the patient is not able to stand up from horizontal position without turning first to the side and then to the abdomen. During the examination, a large head circumference was noticed (56 cm, > 97 percentile). MRI showed elongated cerebellar tonsils descended below the foramen magnum (left 14 mm, right 11 mm). Chiari malformation type I with associated syringomyelia was diagnosed. The patient was reffered to neurosurgery consultation.

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Traumatic Transient Herniation Concomitant with Tonsillar Hemorrhagic Contusion in a Child

Open Access Macedonian Journal of Medical Sciences ◽

10.3889/oamjms.2017.167 ◽

2017 ◽

Vol 5 (6) ◽

pp. 771-773 ◽

Cited By ~ 1

Author(s):

Ahmet Öğrenci ◽

Orkun Koban ◽

Murat Ekşi ◽

Onur Yaman ◽

Sedat Dalbayrak

Keyword(s):

Foramen Magnum ◽

Type I ◽

Tonsillar Ectopia ◽

Chiari Type ◽

Chiari Type I Malformation ◽

Pediatric Age Group ◽

Downward Displacement ◽

The Relationship

Downward displacement of cerebellar tonsils more than 5 mm below the foramen magnum is named as Chiari type I malformation and named benign tonsillar ectopia if herniation is less than 3 mm. It does not just depend on congenital causes. There are also some reasons for acquired Chiari Type 1 and benign tonsillar ectopia/herniation. Trauma is one of them. Trauma may increase tonsillar ectopia or may be the cause of new-onset Chiari type 1. The relationship between the tonsil contusion and its position is unclear. We present a case of pediatric age group with tonsillar herniation with a hemorrhagic contusion. Only 1 case has been presented so far in the literature. A case with unilateral tonsil contusion has not been presented to date. We will discuss the possible reasons for taking the place of the tonsils to the above level of the foramen magnum in the follow-up period, by looking at the literature.

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