scholarly journals Cerebellar fits in children with Chiari I malformation

2001 ◽  
Vol 11 (1) ◽  
pp. 1-4 ◽  
Author(s):  
Aditya Pandey ◽  
Shenandoah Robinson ◽  
Alan R. Cohen
Keyword(s):  
Correct Diagnosis ◽  
Epileptic Seizures ◽  
Holter Monitoring ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Respiratory Compromise ◽  
Drop Attacks ◽  
Cardiogenic Syncope ◽  
Chiari I ◽  
Cerebellar Tonsils

Object The authors describe a series of children with Chiari I malformation who presented with fulminating symptoms of “cerebellar fits” characterized by drop attacks with or without deterioration of consciousness, opisthotonic posturing, and varying degrees of respiratory compromise. Methods A retrospective analysis was undertaken of the medical records of 47 consecutive patients undergoing surgery for symptomatic Chiari I malformations at Rainbow Babies and Children's Hospital. Thirteen (28%) of the 47 patients presented with complaints consistent with cerebellar fits. Before the correct diagnosis was made, nine (69%) of the 13 children had previously undergone evaluation with electroencephalography and/or electrocardicography and Holter monitoring because of suspected cortical epilepsy or cardiogenic syncope. In each of the 13 children magnetic resonance imaging demonstrated pegged cerebellar tonsils herniated below the foramen magnum. A deep indentation or blanched discoloration of the cerebellar tonsils was noted in five (38%) of these 13 patients at the time of surgery. Of patients with symptomatic Chiari I malformations, the mean degree of tonsillar herniation was significantly less for those in whom cerebellar fits occurred than those in whom they were absent (8.8 mm and 13.9 mm, respectively; p = 0.007). In only one of the patients with cerebellar fits was a syrinx present, and this was a small focal lower thoracic collection. Spells resolved after surgery in all patients who presented with cerebellar fits. Conclusions Cerebellar fits may mimic other disorders such as cardiogenic syncope and epileptic seizures. The correct diagnosis may be delayed or the conditions may be misdiagnosed by those who fail to consider Chiari I malformation as a cause of drop attacks, abnormal extensor posturing, and apneic spells in children. The response to decompressive surgery in these patients is gratifying.

Chiari Malformation with Syringomyelia

2019 ◽  
pp. 57-64
Author(s):  
Yiping Li ◽  
Bermans J. Iskandar
Keyword(s):  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Neurological Deficits ◽  
Csf Leakage ◽  
Craniocervical Instability ◽  
Chiari I ◽  
Spine Mri ◽  
Operative Monitoring ◽  
Associated Abnormalities ◽  
Cerebellar Tonsils

Chiari I malformation (CIM) is defined by pathological herniation of the cerebellar tonsils below the foramen magnum. Operative intervention for CIM is generally undertaken to treat neurological deficits associated with tonsillar herniation or with associated syringomyelia. A complete brain and spine MRI is indicated to rule out associated abnormalities and to identify and the presence and extent of syringomyelia. The type of surgical decompression remains controversial but may include bone-only decompression, bony decompression followed by duraplasty, and bony decompression followed by duraplasty and tonsillar shrinkage. Post-operative monitoring for CSF leakage, inadequate decompression, subtle chronic craniocervical instability, and hydrocephalus is critical.

Elucidating the pathophysiology of syringomyelia

1999 ◽  
Vol 91 (4) ◽  
pp. 553-562 ◽  
Author(s):  
John D. Heiss ◽  
Nicholas Patronas ◽  
Hetty L. DeVroom ◽  
Thomas Shawker ◽  
Robert Ennis ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Subarachnoid Space ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Content Type ◽  
Cine Mr ◽  
Spinal Subarachnoid Space ◽  
Chiari I ◽  
Cerebellar Tonsils ◽  
Fine Print

Object. Syringomyelia causes progressive myelopathy. Most patients with syringomyelia have a Chiari I malformation of the cerebellar tonsils. Determination of the pathophysiological mechanisms underlying the progression of syringomyelia associated with the Chiari I malformation should improve strategies to halt progression of myelopathy.Methods. The authors prospectively studied 20 adult patients with both Chiari I malformation and symptomatic syringomyelia. Testing before surgery included the following: clinical examination; evaluation of anatomy by using T1-weighted magnetic resonance (MR) imaging; evaluation of the syrinx and cerebrospinal fluid (CSF) velocity and flow by using phase-contrast cine MR imaging; and evaluation of lumbar and cervical subarachnoid pressure at rest, during the Valsalva maneuver, during jugular compression, and following removal of CSF (CSF compliance measurement). During surgery, cardiac-gated ultrasonography and pressure measurements were obtained from the intracranial, cervical subarachnoid, and lumbar intrathecal spaces and syrinx. Six months after surgery, clinical examinations, MR imaging studies, and CSF pressure recordings were repeated. Clinical examinations and MR imaging studies were repeated annually. For comparison, 18 healthy volunteers underwent T1-weighted MR imaging, cine MR imaging, and cervical and lumbar subarachnoid pressure testing.Compared with healthy volunteers, before surgery, the patients had decreased anteroposterior diameters of the ventral and dorsal CSF spaces at the foramen magnum. In patients, CSF velocity at the foramen magnum was increased, but CSF flow was reduced. Transmission of intracranial pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was partially obstructed. Spinal CSF compliance was reduced, whereas cervical subarachnoid pressure and pulse pressure were increased. Syrinx fluid flowed inferiorly during systole and superiorly during diastole on cine MR imaging. At surgery, the cerebellar tonsils abruptly descended during systole and ascended during diastole, and the upper pole of the syrinx contracted in a manner synchronous with tonsillar descent and with the peak systolic cervical subarachnoid pressure wave. Following surgery, the diameter of the CSF passages at the foramen magnum increased compared with preoperative values, and the maximum flow rate of CSF across the foramen magnum during systole increased. Transmission of pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was normal and cervical subarachnoid mean pressure and pulse pressure decreased to normal. The maximum syrinx diameter decreased on MR imaging in all patients. Cine MR imaging documented reduced velocity and flow of the syrinx fluid. Clinical symptoms and signs improved or remained stable in all patients, and the tonsils resumed a normal shape.Conclusions. The progression of syringomyelia associated with Chiari I malformation is produced by the action of the cerebellar tonsils, which partially occlude the subarachnoid space at the foramen magnum and act as a piston on the partially enclosed spinal subarachnoid space. This creates enlarged cervical subarachnoid pressure waves that compress the spinal cord from without, not from within, and propagate syrinx fluid caudally with each heartbeat, which leads to syrinx progression. The disappearance of the abnormal shape and position of the tonsils after simple decompressive extraarachnoidal surgery suggests that the Chiari I malformation of the cerebellar tonsils is acquired, not congenital. Surgery limited to suboccipital craniectomy, C-1 laminectomy, and duraplasty eliminates this mechanism and eliminates syringomyelia and its progression without the risk of more invasive procedures.

scholarly journals Elucidating the pathophysiology of syringomyelia

1999 ◽  
Vol 7 (2) ◽  
pp. E1 ◽  
Author(s):  
John D. Heiss ◽  
Nicholas Patronas ◽  
Hetty L. DeVroom ◽  
Thomas Shawker ◽  
Robert Ennis ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Pulse Pressure ◽  
Subarachnoid Space ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Imaging Studies ◽  
Cine Mr ◽  
Spinal Subarachnoid Space ◽  
Chiari I ◽  
Cerebellar Tonsils

Object Syringomyelia causes progressive myelopathy. Most patients with syringomyelia have a Chiari I malformation of the cerebellar tonsils. Determination of the pathophysiological mechanisms underlying the progression of syringomyelia associated with the Chiari I malformation should improve strategies to halt progression of myelopathy. Methods The authors prospectively studied 20 adult patients with both Chiari I malformation and symptomatic syringomyelia. Testing before surgery included the following: clinical examination; evaluation of anatomy by using T1-weighted magnetic resonance (MR) imaging; evaluation of the syrinx and cerebrospinal fluid (CSF) velocity and flow by using phase-contrast cine MR imaging; and evaluation of lumbar and cervical subarachnoid pressure at rest, during the Valsalva maneuver, during jugular compression, and following removal of CSF (CSF compliance measurement). During surgery, cardiac-gated ultrasonography and pressure measurements were obtained from the intracranial, cervical subarachnoid, and lumbar intrathecal spaces and syrinx. Six months after surgery, clinical examinations, MR imaging studies, and CSF pressure recordings were repeated. Clinical examinations and MR imaging studies were repeated annually. For comparison, 18 healthy volunteers underwent T1-weighted MR imaging, cine MR imaging, and cervical and lumbar subarachnoid pressure testing. Compared with healthy volunteers, before surgery, the patients had decreased anteroposterior diameters of the ventral and dorsal CSF spaces at the foramen magnum. In patients, CSF velocity at the foramen magnum was increased, but CSF flow was reduced. Transmission of intracranial pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was partially obstructed. Spinal CSF compliance was reduced, whereas cervical subarachnoid pressure and pulse pressure were increased. Syrinx fluid flowed inferiorly during systole and superiorly during diastole on cine MR imaging. At surgery, the cerebellar tonsils abruptly descended during systole and ascended during diastole, and the upper pole of the syrinx contracted in a manner synchronous with tonsillar descent and with the peak systolic cervical subarachnoid pressure wave. Following surgery, the diameter of the CSF passages at the foramen magnum increased compared with preoperative values, and the maximum flow rate of CSF across the foramen magnum during systole increased. Transmission of pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was normal and cervical subarachnoid mean pressuree and pulse pressure decreased to normal. The maximum syrinx diameter decreased on MR imaging in all patients. Cine MR imaging documented reduced velocity and flow of the syrinx fluid. Clinical symptoms and signs improved or remained stable in all patients, and the tonsils resumed a normal shape. Conclusions The progression of syringomyelia associated with Chiari I malformation is produced by the action of the cerebellar tonsils, which partially occlude the subarachnoid space at the foramen magnum and act as a piston on the partially enclosed spinal subarachnoid space. This creates enlarged cervical subarachnoid pressure waves that compress the spinal cord from without, not from within, and propagate syrinx fluid caudally with each heartbeat, which leads to syrinx progression. The disappearance of the abnormal shape and position of the tonsils after simple decompressive extraarachnoidal surgery suggests that the Chiari I malformation of the cerebellar tonsils is acquired, not congenital. Surgery limited to suboccipital craniectomy, C-1 laminectomy, and duraplasty eliminates this mechanism and eliminates syringomyelia and its progression without the risk of more invasive procedures.

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.

scholarly journals Chiari I malformation with Klippel-Trenaunay syndrome: case report and review of the literature

2021 ◽  
Author(s):  
Isabel A. Snee ◽  
Catherine A. Mazzola ◽  
Tatiana Sikorskyj
Keyword(s):  
Facial Asymmetry ◽  
Clinical History ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Posterior Fossa Decompression ◽  
Leg Length ◽  
Upper And Lower Extremities ◽  
Chiari I ◽  
Klippel Trenaunay Syndrome ◽  
Cerebellar Tonsils

AbstractWe present a rare case of an 8-year-old male with Klippel-Trenaunay syndrome (KTS) and a Chiari I malformation (CIM). Magnetic resonance imaging (MRI) to investigate facial asymmetry and speech delay at age two revealed CIM with cerebellar tonsils 1.3 cm below the foramen magnum without syringomyelia. The patient underwent a craniectomy and posterior fossa decompression with C1 laminectomy. While gene sequencing determined the patient was negative for the PIK3CA gene mutation, the patient’s clinical history strongly suggests KTS. He has hemihypertrophy, leg length discrepancy, hemangiomas and pigmentary mosaicism along the upper and lower extremities, heart murmur, chronic low heart rate, recurrent hip pain, and mild scoliosis. Neurodevelopmental concerns include difficulty reading, attention deficit hyperactivity disorder (ADHD), anxiety, and difficulty running and going downstairs. His most recent MRI shows good decompression at the cervicomedullary junction, global cerebrospinal fluid (CSF) flow, and less peg-like cerebellar tonsils. Also noted were two intravertebral hemangiomas at T5 and T6. While the patient’s speech has improved, there is still difficulty with the expressive language. He still has mild delays, runs slowly, and does not alternate feet when climbing stairs. The patient is being followed by multiple specialists including neurology, hematology-oncology, genetics, orthopedic surgery, and developmental pediatrics.

scholarly journals The surgical procedure of syringomyelie with Chiari I in adults regarding the intrapial aspiration of cerebellar tonsils: does this procedure improve symptoms with less complication?

2019 ◽  
pp. 37-48
Author(s):  
Ait Bachir Mustapha ◽  
T. Benbouzid
Keyword(s):  
Clinical Signs ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Spinal Fluid ◽  
Motor Deficits ◽  
Common Denominator ◽  
Consecutive Series ◽  
Dural Graft ◽  
Chiari I ◽  
Cerebellar Tonsils

Introduction. Cranio-vertebral decompression remains the common denominator for the treatment of syringomyelia associated with Chiari I. On the other hand, the details of the procedure, remains controversial. The success of the surgery is to restore the circulation of cerebro-spinal fluid at the level of the foramen magnum. How is this circulation restored to the level of foramen is the question? We offer our attitude towards the treatment of syringomyelia with Chiari I. Material and method. Consecutive series of 32/121 patients benefiting from cranio-vertebral decompression associated with intrapial aspiration of cerebellar tonsils treated for syringomyelia with a Chiari I malformation in adults. Result. Motor deficits were present in 20 / 22 patients, representing 90% of the entire patient group. these motor deficits are improved in 16 out of 22 cases, and remained unchanged in 06cas. no motor aggravation occurred. in our study, bone decompression and intra pial aspiration of cerebellar tonsils (sub arachnoids manipulation) were found to be associated with favourable results on clinical signs and symptoms. However, sub arachnoids manipulation and intra pial aspiration of cerebellar tonsils showed a little more complication compared with bone decompression with dural plasty. Conclusion. The bone decompression with dural graft and intradural dissection of adhesions and reduction by intra pial aspiration or resection of the tonsils is indicated on the MRI aspect of cerebellar tonsils of considerable size totally obstructing the foramen Magnum, the intraoperative finding, through the arachnoid, of the absence of passage of the cerebro-spinal fluid because of the bulging of the cerebellar tonsils.

scholarly journals Re-evaluation of foramen magnum decompression with dura left open for Chiari I malformation

2017 ◽  
Vol 10 ◽  
pp. 150-154 ◽  
Author(s):  
Tetsuryu Mitsuyama ◽  
Yasuo Aihara ◽  
Takaomi Taira ◽  
Seiichiro Eguchi ◽  
Kentaro Chiba ◽  
...  
Keyword(s):  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Foramen Magnum Decompression ◽  
Chiari I

scholarly journals Posterior odontoid process angulation in pediatric Chiari I malformation: an MRI morphometric external validation study

2015 ◽  
Vol 16 (2) ◽  
pp. 138-145 ◽  
Author(s):  
Travis R. Ladner ◽  
Michael C. Dewan ◽  
Matthew A. Day ◽  
Chevis N. Shannon ◽  
Luke Tomycz ◽  
...  
Keyword(s):  
External Validation ◽  
Age Groups ◽  
Odontoid Process ◽  
Line Length ◽  
Craniocervical Junction ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Posterior Fossa Decompression ◽  
Tonsillar Ectopia ◽  
Chiari I

OBJECT Osseous anomalies of the craniocervical junction are hypothesized to precipitate the hindbrain herniation observed in Chiari I malformation (CM-I). Previous work by Tubbs et al. showed that posterior angulation of the odontoid process is more prevalent in children with CM-I than in healthy controls. The present study is an external validation of that report. The goals of our study were 3-fold: 1) to externally validate the results of Tubbs et al. in a different patient population; 2) to compare how morphometric parameters vary with age, sex, and symptomatology; and 3) to develop a correlative model for tonsillar ectopia in CM-I based on these measurements. METHODS The authors performed a retrospective review of 119 patients who underwent posterior fossa decompression with duraplasty at the Monroe Carell Jr. Children’s Hospital at Vanderbilt University; 78 of these patients had imaging available for review. Demographic and clinical variables were collected. A neuroradiologist retrospectively evaluated preoperative MRI examinations in these 78 patients and recorded the following measurements: McRae line length; obex displacement length; odontoid process parameters (height, angle of retroflexion, and angle of retroversion); perpendicular distance to the basion-C2 line (pB–C2 line); length of cerebellar tonsillar ectopia; caudal extent of the cerebellar tonsils; and presence, location, and size of syringomyelia. Odontoid retroflexion grade was classified as Grade 0, > 90°; Grade I,85°–89°; Grade II, 80°–84°; and Grade III, < 80°. Age groups were defined as 0–6 years, 7–12 years, and 13–17 years at the time of surgery. Univariate and multivariate linear regression analyses, Kruskal-Wallis 1-way ANOVA, and Fisher’s exact test were performed to assess the relationship between age, sex, and symptomatology with these craniometric variables. RESULTS The prevalence of posterior odontoid angulation was 81%, which is almost identical to that in the previous report (84%). With increasing age, the odontoid height (p < 0.001) and pB–C2 length (p < 0.001) increased, while the odontoid process became more posteriorly inclined (p = 0.010). The pB–C2 line was significantly longer in girls (p = 0.006). These measurements did not significantly correlate with symptomatology. Length of tonsillar ectopia in pediatric CM-I correlated with an enlarged foramen magnum (p = 0.023), increasing obex displacement (p = 0.020), and increasing odontoid retroflexion (p < 0.001). CONCLUSIONS Anomalous bony development of the craniocervical junction is a consistent feature of CM-I in children. The authors found that the population at their center was characterized by posterior angulation of the odontoid process in 81% of cases, similar to findings by Tubbs et al. (84%). The odontoid process appeared to lengthen and become more posteriorly inclined with age. Increased tonsillar ectopia was associated with more posterior odontoid angulation, a widened foramen magnum, and an inferiorly displaced obex.

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