scholarly journals Chiari Malformation-I with Syringomyelia: A Non-specific Presentation and Surgical Consideration

2016 ◽  
Vol 13 (2) ◽  
pp. 105-108
Author(s):  
Jemesh S Maharjan ◽  
Pranaya Shrestha ◽  
Avinash Chandra ◽  
Pravesh Rajbhandari ◽  
Samir Acharya ◽  
...  
Keyword(s):  
Case Report ◽  
Chiari Malformation ◽  
Foramen Magnum ◽  
Clinical Findings ◽  
Tonsillar Herniation ◽  
Diverse Range ◽  
Broad Perspective ◽  
Surgical Options ◽  
Chiari I ◽  
Selection Of

Chiari Malformation-I is a congenital disorder characterized by the anatomical defect of the base of skull with tonsillar herniation (≥5 mm) below the foramen magnum, which is detected on MRI. It has a diverse range of symptoms with non-specific presentation leading to the chances of misdiagnosis and untimely recognition of the disorder. Syringomyelia is the most common result of Chiari-I. The selection of surgical or non-surgical management depends upon the patient symptoms and the presence of absence of Syringomyelia. The objective of this case report is to give a broad perspective on Chiari Malformation-I from the symptoms and clinical findings obtained in a patient with Syringomyelia associated with Chiari Malformation-I and to discuss about the different surgical options as well as the psychological support required for the management of the condition.Nepal Journal of Neuroscience. Vol. 13, No. 2, 2016, Page: 105-108 

Factors predicting the occurrence of syringomyelia in patients with Chiari I malformations

2019 ◽  
Vol 7 (8) ◽  
pp. 2967
Author(s):  
Vijayan Peettakkandy ◽  
Bijukrishnan Rajagopalawarrier ◽  
Sreenath Kuniyil ◽  
Jithin Veliyath Thankaraj
Keyword(s):  
Foramen Magnum ◽  
P Value ◽  
Tonsillar Herniation ◽  
Duration Of Symptoms ◽  
Medical College ◽  
Chiari I ◽  
The Mean ◽  
Time Of Presentation ◽  
Common Association ◽  
Cerebellar Tonsils

Background: Chiari I malformations are defined as the downward herniation of cerebellar tonsils more than 5 mm through the foramen magnum. Syringomyelia is a common association of Chiari I malformation.Methods: This purpose of this study was to determine the various factors predicting the occurrence of syringomyelia in patients with Chiari I malformations. This retrospective study was conducted in Government medical college, Thrissur, Kerala, India. 27 patients admitted with diagnosis of symptomatic Chiari I malformations during the period of January 2015 to January 2019 were selected for the study.Results: The mean age was 22.8 years and syringomyelia was more common in patients older than 10 years (p value0.005). Females were 17(63%) and males were 10(37%) but there no difference in occurrence of syringomyelia among both sexes(p value is 0.16). Syringomyelia was present in 17(63%) cases. Association between various factors and syringomyelia measured by calculating P value which was 0.005 for age >10 years, 0.16 for sex, 0.093 for duration of symptoms >12 months, 0.05 for tonsillar descend >10mm and 0.097 for hypertension. The p value for the association of duration of symptoms >12months and extend of tonsillar descend is 0.001 Mean duration of symptoms (onset of first symptom to the time of presentation) was 16.3 months and syringomyelia was more common in patients with duration of symptoms more than 12 months but this difference was statistically not significant (p value 0.093). Mean tonsillar descend from the level of foramen magnum was 11.3 mms and syringomyelia was more common in patients with tonsillar herniation more than 10mm (p value 0.05). There is a statistically significant relationship between duration of symptoms more than 12 months and tonsillar descend more than 10mm(p value 0.001).There is no significant association between hypertension and occurrence of syringomyelia in patients with Chiari I malformation.Conclusions: Incidence of Chiari I Malformations is more among adults and it is slightly higher in females. Syringomyelia is a common association of Chiari I Malformations. The occurrence of syringomyelia in patients with Chiari I Malformations associated with increasing age of patients and extend of tonsillar herniation. There is no statistically significant association between syringomyelia and duration of symptoms or hypertension.

Spontaneous Resolution of Chiari I Malformation and Syringomyelia: Case Report and Review of the Literature

Neurosurgery ◽  
2001 ◽  
Vol 48 (3) ◽  
pp. 664-667 ◽  
Author(s):  
Jörg Klekamp ◽  
Giorgio Iaconetta ◽  
Madjid Samii
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cerebrospinal Fluid ◽  
Case Report ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Spontaneous Resolution ◽  
Magnetic Resonance Imaging Scan ◽  
Resonance Imaging ◽  
Chiari I ◽  
Resonance Imaging Scan

Abstract OBJECTIVE AND IMPORTANCE Indications for surgery and the surgical technique of foramen magnum decompression for patients with Chiari I malformation and syringomyelia are controversial issues. This case report supports the view that observation may be adequate for patients without progressive symptoms or with mild clinical symptoms. CLINICAL PRESENTATION A 37-year-old woman presented with a 3-month history of burning dysesthesias and hypesthesia in her right arm. A neurological examination revealed hypesthesia in the right trigeminal distribution. A magnetic resonance imaging scan revealed a Chiari I malformation with syringomyelia between C2 and T2. No hydrocephalus was observed. CLINICAL COURSE Because the patient's symptoms regressed spontaneously, surgery was not performed. Thirty-two months after her initial examination, the patient was asymptomatic. A second magnetic resonance imaging scan was obtained, which demonstrated complete spontaneous resolution of the Chiari I malformation and syringomyelia. CONCLUSION We attribute the regression of the patient's symptoms to spontaneous recanalization of cerebrospinal fluid pathways at the foramen magnum, which most likely was due to rupture of the arachnoid membranes that had obstructed cerebrospinal fluid flow.

Otoneurological manifestations in Chiari-I malformation

1993 ◽  
Vol 107 (5) ◽  
pp. 441-443 ◽  
Author(s):  
F. W. J. Albers ◽  
K. J. A. O. Ingels
Keyword(s):  
Hearing Loss ◽  
Case Report ◽  
Cranial Nerve ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Type I ◽  
Cranial Nerve Involvement ◽  
Presenting Symptoms ◽  
Chiari I ◽  
Cerebellar Tonsils

The type I Chiari malformation consists of a caudal displacement of the cerebellar tonsils through the foramen magnum into the cervical spinal canal. The most common presenting symptoms, such as pain, weakness and headache, are frequently preceded by otoneurological symptoms. Sensorineural hearing loss, vertigo, nystagmus, dysequilibrium, tinnitus and other cranial nerve involvement have been reported in Chiari-I malformation. A case report is presented and the clinical features of the disease are discussed with emphasis on the otoneurological aspects.

Hemifacial Spasm as a Feature of Chiari Malformation: Case Report

Neurosurgery ◽  
2010 ◽  
Vol 67 (6) ◽  
pp. E1826-E1830 ◽  
Author(s):  
Nitin Mukerji ◽  
Peter Newman ◽  
Fred P Nath
Keyword(s):  
Case Report ◽  
Hemifacial Spasm ◽  
Clinical Presentation ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Basilar Impression ◽  
Foramen Magnum Decompression ◽  
First Line ◽  
Chiari I ◽  
Possible Cause

Abstract BACKGROUND AND IMPORTANCE: Hemifacial spasm has rarely been described as one of the presenting features of Chiari I malformation. We present a case in which we found an association between the two in the absence of a basilar impression. CLINICAL PRESENTATION: A case of a 39-year-old man who presented with a disabling hemifacial spasm and was found to have Chiari I malformation as the possible cause is described. A foramen magnum decompression successfully relieved the hemifacial spasm. The patient remains symptom free at 20 months after the operation. CONCLUSION: Neurosurgeons may want to consider foramen magnum decompression as first-line surgical treatment for hemifacial spasm when it coexists with Chiari I malformation. Chiari I malformation should be considered one of the rare causes of hemifacial spasm.

Chiari malformation Type I and syrinx in children undergoing magnetic resonance imaging

2011 ◽  
Vol 8 (2) ◽  
pp. 205-213 ◽  
Author(s):  
Jennifer Strahle ◽  
Karin M. Muraszko ◽  
Joseph Kapurch ◽  
J. Rajiv Bapuraj ◽  
Hugh J. L. Garton ◽  
...  
Keyword(s):  
Cervical Spine ◽  
Mr Imaging ◽  
Chiari Malformation ◽  
Foramen Magnum ◽  
Type I ◽  
Csf Flow ◽  
Tonsillar Herniation ◽  
Chiari Malformation Type I ◽  
Age Related ◽  
Imaging Characteristics

Object Chiari malformation Type I (CM-I) with an associated spinal syrinx is a common pediatric diagnosis. A better understanding of the relative age-related prevalence and MR imaging characteristics of these associated conditions may lead to improved treatment decisions. Methods The authors performed a retrospective review of 14,116 consecutive individuals 18 years of age or younger who had undergone brain or cervical spine MR imaging at the University of Michigan between November 1997 and August 2008. In the patients with CM-I, demographic, clinical, and radiographic information was recorded. Results Five hundred nine children (3.6%) with CM-I were identified. Among these patients, 23% also had a spinal cord syrinx, and 86% of the syringes were found in the cervical spine. The MR imaging prevalence of CM-I with a syrinx was 1.2% in girls and 0.5% in boys (p < 0.0001). The severity of impaired CSF flow at the foramen magnum was associated with the amount of tonsillar herniation (p < 0.0001) and conformation of the tonsils (p < 0.0001). Patients with CM-I were treated surgically in 35% of cases; these patients exhibited more severe tonsillar herniation (p < 0.0001) and impaired CSF flow (p < 0.0001) as compared with those who did not undergo surgery. On imaging, 32% of all the patients with CM-I were considered symptomatic by the treating physician. Patients were more likely to be considered symptomatic if they were female, had a syrinx, displayed abnormal tonsillar pulsations, or had a greater amount of tonsillar herniation. Conclusions In this study the authors describe the age-related prevalence and MR imaging characteristics of CM-I and its association with a syrinx and other abnormalities in a large group of children who underwent MR imaging for any indication. Syringes are more common in older children, in girls, and in patients with a greater degree of tonsillar descent and CSF flow impairment.

scholarly journals Arachnoid cyst resulting in tonsillar herniation and syringomyelia in a patient with achondroplasia

2005 ◽  
Vol 19 (5) ◽  
pp. 1-7 ◽  
Author(s):  
Andrew M. Bauer ◽  
Diane M. Mueller ◽  
John J. Oró
Keyword(s):  
Mr Imaging ◽  
Arachnoid Cyst ◽  
Chiari Malformation ◽  
Pediatric Population ◽  
Foramen Magnum ◽  
Tonsillar Herniation ◽  
Residual Symptoms ◽  
Presenting Symptoms ◽  
Suboccipital Craniectomy ◽  
History Of

Achondroplasia has been associated with varying degrees of cervicomedullary and spinal compression, although usually in the pediatric population. Large arachnoid cysts have also been found to result in tonsillar herniation and syringomyelia. The authors present the case of a patient with achondroplasia who presented with symptoms of foramen magnum compression and syringomyelia, and who was subsequently found to have a large posterior fossa arachnoid cyst. This 38-year-old woman with achondroplasia presented with an 8-month history of headache and numbness of the hands and fingers. Admission magnetic resonance (MR) imaging of the head and spine revealed a large arachnoid cyst in the posterior cranial fossa, a 6-mm tonsillar herniation consistent with an acquired Chiari malformation, and a large cervicothoracic syrinx. The patient was treated using suboccipital craniectomy, C-1 laminectomy, fenestration of the arachnoid cyst, and decompression of the acquired Chiari malformation with duraplasty. Surgical decompression resulted in improvement of the presenting symptoms, adequate decompression of crowding at the foramen magnum, and resolution of the syrinx. Although there was only partial reduction in the retrocerebellar cisternal space on follow-up MR imaging, no residual symptoms were related to this.

Resolution of type I Chiari malformation and associated syringomyelia following intrathecal chemotherapy: case report

2020 ◽  
pp. 1-6
Author(s):  
Chia-En Wong ◽  
Yi-Shan Tsai ◽  
Jiann-Shiuh Chen ◽  
Yu-Ning Chen ◽  
Jung-Shun Lee
Keyword(s):  
Case Report ◽  
Chiari Malformation ◽  
Brain Mri ◽  
B Cell Lymphoma ◽  
Intrathecal Chemotherapy ◽  
Foramen Magnum ◽  
Type I ◽  
Cns Involvement ◽  
First Case

Type I Chiari malformation (CM-I) consists of downward herniation of the cerebellar tonsils below the foramen magnum and often requires surgical decompression if symptomatic. Spontaneous resolution of CM-I is rare. We present a case of resolved CM-I without surgery in a 6-year-old boy with B-cell lymphoma who was diagnosed with CM-I during lymphoma staging. Cerebrospinal fluid cytology and brain MRI revealed negative CNS involvement but showed CM-I with tonsillar ectopia 19 mm below the foramen magnum. The patient underwent induction chemotherapy including 5 doses of intrathecal chemotherapy. Follow-up MRI demonstrated marked regression of CM-I to less than 6 mm in 3 months, and complete resolution of CM-I was observed in 2 years. To the best of our knowledge, this is the first case of resolved CM-I and syringomyelia following chemotherapy. In this case report, the authors summarize all of the clinical characteristics, the radiological appearance, and the potential causes of resolution based on a review of the literature and propose the mechanisms through which intrathecal chemotherapy contributed to the CM-I and syringomyelia resolution in the present case.

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