Correlation of Functional Outcome and Natural History With Clinicoradiological Factors in Surgically Managed Pediatric Chiari I Malformation

Neurosurgery ◽  
2011 ◽  
Vol 68 (2) ◽  
pp. 319-328 ◽  
Author(s):  
Sunil V. Furtado ◽  
Sumit Thakar ◽  
Alangar S. Hegde
Keyword(s):  
Spinal Cord ◽  
Posterior Fossa ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Functional Improvement ◽  
Duration Of Symptoms ◽  
Mean Values ◽  
Significant Difference ◽  
Chiari I

Abstract BACKGROUND: Chiari I malformation occurs because of an underdeveloped posterior fossa with reduced volume that cannot accommodate the normally developed hindbrain. OBJECTIVE: To study the clinical presentation and surgical outcome of pediatric Chiari I malformation and to correlate outcome with demographic and clinical factors and radiological changes in the syrinx, spinal cord, and preoperative intracranial, posterior fossa, and foramen magnum dimensions. METHODS: This retrospective study spanning 9 years included 20 symptomatic patients who underwent foramen magnum decompression, shrinkage of tonsils, and duraplasty. Improvement at follow-up was assessed with a modified Asgari scoring system. Mean differences in the dimensions of the syrinx and cord, foramen magnum morphometry, and intracranial and posterior fossa for 2 groups (with or without improvement) were analyzed with the independent-sample Student t test. Correlation of outcome in relation to change in radiological factors and influence of variables such as age, type and duration of symptoms, and presence of syrinx were evaluated with the Pearson χ2 test. RESULTS: Sixty percent of patients showed functional improvement at follow-up. Of various demographic and radiological factors assessed, there was significant difference in mean values of change in cord diameter for the entire cohort (P = .05) and for the subgroup with preoperative syringes (P = .03). There was no correlation between change in any of these factors and functional improvement (χ2 range, 0-4.673; P > .05). CONCLUSION: More than half the pediatric patients with Chiari I malformation improve after surgery. The age at presentation, duration and type of symptoms, cranial and foramen magnum morphometry, and syrinx-related changes have no bearing on outcome at short-term follow-up. The spinal cord diameter differs significantly in patients with and without functional improvement.

Syrinx shunt to posterior fossa cisterns (syringocisternostomy) for bypassing obstructions of upper cervical theca

1992 ◽  
Vol 77 (6) ◽  
pp. 871-874 ◽  
Author(s):  
Thomas H. Milhorat ◽  
Walter D. Johnson ◽  
John I. Miller
Keyword(s):  
Posterior Fossa ◽  
Symptomatic Improvement ◽  
Foramen Magnum ◽  
Content Type ◽  
Results Of Treatment ◽  
Spinal Subarachnoid Space ◽  
Upper Cervical ◽  
Spinal Arachnoiditis ◽  
Chiari I

✓ Syrinx shunts to the spinal subarachnoid space are likely to fail if the cerebrospinal fluid pathways rostral to the syrinx are blocked. To bypass obstructions at or below the level of the foramen magnum, a technique was developed for shunting the syrinx to the posterior fossa cisterns, termed “syringocisternostomy.” Syrinxes were shunted to the cisterna magna in two patients with spinal arachnoiditis and to the cerebellopontine angle cistern in four patients with Chiari I malformations. There was symptomatic improvement and collapse of the syrinx in each case, with no complications or recurrences over a follow-up interval of 14 to 27 months (average 20.3 months). The surgical technique and results of treatment are described.

Advanced Chiari malformation Type I with marked spinal cord atrophy

2005 ◽  
Vol 3 (4) ◽  
pp. 324-327 ◽  
Author(s):  
Yoshiro Ito ◽  
Koji Tsuboi ◽  
Hiroyoshi Akutsu ◽  
Satoshi Ihara ◽  
Akira Matsumura
Keyword(s):  
Spinal Cord ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Type I ◽  
Foramen Magnum Decompression ◽  
Spinal Cord Atrophy ◽  
Chiari Malformation Type I ◽  
Content Type ◽  
Neurological Improvement ◽  
Chiari I

✓ The authors discuss the results obtained in patients who underwent foramen magnum decompression for longstanding advanced Chiari I malformation in which marked spinal cord atrophy was present. This 50-year-old woman presented with progressive quadriparesis and sensory disorders. Magnetic resonance imaging revealed the descent of cerebellar tonsils and medulla associated with remarkable C1—L2 spinal cord atrophy. After a C-1 laminectomy—based foramen magnum decompression, arachnoid dissection and duraplasty were undertaken. These procedures resulted in remarkable neurological improvement, even after 40 years of clinical progression. Spinal cord atrophy may be caused by chronic pressure of entrapped cerebrospinal fluid in the spinal canal.

scholarly journals Is Chiari I Malformation Associated With Fibromyalgia?

Neurosurgery ◽  
2011 ◽  
Vol 68 (2) ◽  
pp. 443-449 ◽  
Author(s):  
Nathaniel F. Watson ◽  
Dedra Buchwald ◽  
Jack Goldberg ◽  
Kenneth R. Maravilla ◽  
Carolyn Noonan ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Sleep Disturbances ◽  
Cervical Spinal Cord ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Upper Cervical Spine ◽  
Fisher Exact Test ◽  
Contrast Imaging ◽  
Control Subjects ◽  
Chiari I

Abstract BACKGROUND: The symptoms of Chiari I Malformation (CIM) and fibromyalgia (FM) overlap. Some FM patients have been surgically treated for presumed CIM-type pathology. OBJECTIVE: To determine whether CIM is more common among FM patients than pain- and fatigue-free controls. METHODS: One hundred seventy-six participants with FM and 67 pain- and fatigue-free control subjects underwent magnetic resonance imaging of the brain and upper cervical spine. Posterior fossa cerebrospinal fluid flow was assessed with cardiac gated cine phase-contrast imaging at the craniocervical region. CIM was defined as inferior extension of cerebellar tonsils ≥ 5 mm below the basion-opisthion line of the foramen magnum or tonsillar position 3 to 5 mm below the basion-opisthion line plus abnormalities of CSF flow, posterior fossa volume, or hindbrain or cervical spinal cord movement. Visual analog scales, questionnaires, and interviews were used to collect data on sleep quality, fatigue, pain, and headache. We used regression techniques to examine the association of outcome measures with disease status and the Fisher exact test to compare the CIM prevalence in the 2 groups. RESULTS: The FM group was older (mean age, 50 vs 40 years) and more likely to be white (89% vs 73%) and female (93% vs 54%; P < .01). Mean tonsillar position and the prevalence of CIM (2.8% vs 4.5%; P = .69) were similar in the FM and control groups. FM patients experienced more headaches, pain, fatigue, and sleep disturbances than control subjects (P < .01). CONCLUSION: Most patients with FM do not have CIM pathology. Future studies should focus on dynamic neuroimaging of craniocervical neuroanatomy in patients with FM.

scholarly journals CSF goes astray no matter what is in the way: occipital intradiploic pseudomeningocele and de novo syringomyelia following foramen magnum decompression for Chiari I malformation in a pediatric patient

2020 ◽  
Vol 25 (5) ◽  
pp. 529-534
Author(s):  
Arthur R. Kurzbuch ◽  
Shailendra Magdum
Keyword(s):  
Revision Surgery ◽  
De Novo ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Foramen Magnum Decompression ◽  
Dural Closure ◽  
First Case ◽  
Chiari I ◽  
Lost To Follow Up

Nontraumatic intradiploic pseudomeningoceles and de novo syringomyelia formation are very rare entities. The authors have previously reported the case of a 4-year-old girl who underwent foramen magnum decompression without dural closure for Chiari I malformation. Three years after the operation an intradiploic pseudomeningocele was documented, but the patient was lost to follow-up without undergoing revision surgery. Four years later, at the age of 11 years, the patient returned for treatment of intensifying symptoms. Radiological imaging then showed an increase in the size of the intradiploic pseudomeningocele and a new cervical syrinx. The patient underwent a first revision surgery in which a part of the internal layer of the occipital bone was removed and arachnoid scar lysis was performed. Two months later the syrinx had worsened, and in a second revision surgery a pseudomeningocele-peritoneal shunt was placed. Here, the authors describe what is to their knowledge the first case of an intradiploic pseudomeningocele and de novo syringomyelia formation following Chiari I decompressive surgery.

scholarly journals Surgical Experience in Pediatric Patients with Chiari-I Malformations Aged ≤18 Years

2019 ◽  
Vol 10 (01) ◽  
pp. 85-88 ◽  
Author(s):  
Ghanshyam Das Singhal ◽  
Shakti Singhal ◽  
Gunjan Agrawal ◽  
Deepti Singhal ◽  
Vipin Arora
Keyword(s):  
Pediatric Patients ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Neurological Deficits ◽  
Resonance Imaging ◽  
Surgical Experience ◽  
Limb Weakness ◽  
Lower Limb Weakness ◽  
Chiari I

ABSTRACT Objective: The objective of this study was to retrospectively study Chiari I malformation patients (<18 years) treated surgically. Materials and Methods: Chiari I malformation patients (<18 years) treated surgically at our institute were retrospectively studied. Results: During the study period between January 1999 and June 2011, fifty patients, aged ≤18 years with Chiari malformation, were treated surgically and formed the basis for this series. There were 21 female children (42%) and 29 male children (58%), with a female-to-male ratio of 1:1. At the last follow-up, oropharyngeal symptoms were improved in 33% (n = 3/9). Headache/neck/back pain improved in 69.56% of children (n = 16/23). Upper-extremity pain/weakness/numbness improved in 73.91% of children (n = 17/23). Ataxia improved in 66.66% of children (n = 4/6). Lower-limb weakness/hyperreflexia improved in 83.33% of children (n = 5/6). At follow-up, magnetic resonance imaging for patients with syrinx was available for 75% of patients (n = 30/50) and not available for 25% of patients (n = 10/40). Syrinx was diminished in size or resolved in 66.33% of patients (n = 19/30) and the remaining was same for 36.66% of patients (n = 11/30). Conclusions: The main goal of surgery is to arrest the progression of neurological deficits. Foramen magnum decompression with a lax duroplasty is the surgical procedure of choice.

scholarly journals Changes in cerebrospinal fluid flow assessed using intraoperative MRI during posterior fossa decompression for Chiari malformation

2015 ◽  
Vol 122 (5) ◽  
pp. 1068-1075 ◽  
Author(s):  
Aaron E. Bond ◽  
John A. Jane ◽  
Kenneth C. Liu ◽  
Edward H. Oldfield
Keyword(s):  
Posterior Fossa ◽  
Intraoperative Imaging ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Intraoperative Mri ◽  
Cine Mri ◽  
Posterior Fossa Decompression ◽  
Csf Flow ◽  
Chiari I ◽  
Adequate Decompression

OBJECT The authors completed a prospective, institutional review board–approved study using intraoperative MRI (iMRI) in patients undergoing posterior fossa decompression (PFD) for Chiari I malformation. The purpose of the study was to examine the utility of iMRI in determining when an adequate decompression had been performed. METHODS Patients with symptomatic Chiari I malformations with imaging findings of obstruction of the CSF space at the foramen magnum, with or without syringomyelia, were considered candidates for surgery. All patients underwent complete T1, T2, and cine MRI studies in the supine position preoperatively as a baseline. After the patient was placed prone with the neck flexed in position for surgery, iMRI was performed. The patient then underwent a bone decompression of the foramen magnum and arch of C-1, and the MRI was repeated. If obstruction was still present, then in a stepwise fashion the patient underwent dural splitting, duraplasty, and coagulation of the tonsils, with an iMRI study performed after each step guiding the decision to proceed further. RESULTS Eighteen patients underwent PFD for Chiari I malformations between November 2011 and February 2013; 15 prone preincision iMRIs were performed. Fourteen of these patients (93%) demonstrated significant improvement of CSF flow through the foramen magnum dorsal to the tonsils with positioning only. This improvement was so notable that changes in CSF flow as a result of the bone decompression were difficult to discern. CONCLUSIONS The authors observed significant CSF flow changes when simply positioning the patient for surgery. These results put into question intraoperative flow assessments that suggest adequate decompression by PFD, whether by iMRI or intraoperative ultrasound. The use of intraoperative imaging during PFD for Chiari I malformation, whether by ultrasound or iMRI, is limited by CSF flow dynamics across the foramen magnum that change significantly when the patient is positioned for surgery.

Osteopetrosis with Chiari I malformation: presentation and surgical management

2011 ◽  
Vol 7 (4) ◽  
pp. 369-374 ◽  
Author(s):  
Brian J. Dlouhy ◽  
Arnold H. Menezes
Keyword(s):  
Mr Imaging ◽  
Posterior Fossa ◽  
Autosomal Dominant ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Posterior Fossa Decompression ◽  
Imaging Data ◽  
Inherited Disorders ◽  
Chiari I ◽  
Autosomal Dominant Osteopetrosis

Osteopetrosis is a heterogeneous group of rare, inherited disorders of the skeleton that results in neurological manifestations due to restriction of growth of cranial foramina and calvarial thickening. A 25-year-old woman with a history of autosomal dominant osteopetrosis presented to the authors' institution with headache worsened by exertion and radiating from the occipital region forward with episodes of choking/coughing when eating and a loss of gag reflex on physical examination. On MR imaging, she was found to have severe posterior fossa calvarial thickening resulting in a small posterior fossa and tonsillar ectopia of 9 mm and compression and deformation of the brainstem. She underwent posterior fossa craniectomy, foramen magnum decompression, and partial C-1 laminectomy with external durotomy. The patient did well postoperatively with resolution of symptoms. This case describes a new neurological manifestation of autosomal dominant osteopetrosis. To the authors' knowledge, this report represents the first described case of extreme posterior fossa calvarial thickening from autosomal dominant osteopetrosis with associated Chiari I malformation (CM-I) requiring posterior fossa decompression and extradural decompression. Given previously published MR imaging data that demonstrate the association of osteopetrosis and CM may be more common than in this case alone, the authors discuss the need for further investigation of the incidence of CM-I in patients with autosomal dominant osteopetrosis. Additionally, they review osteopetrosis and other diagnoses of calvarial hyperostosis presenting as CM-I.

scholarly journals Obex position is associated with syringomyelia and use of posterior fossa decompression among patients with Chiari I malformation

2020 ◽  
Vol 26 (1) ◽  
pp. 45-52
Author(s):  
Gabe Haller ◽  
Brooke Sadler ◽  
Timothy Kuensting ◽  
Nivan Lakshman ◽  
Jacob K. Greenberg ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Area Under The Curve ◽  
Retrospective Chart Review ◽  
Severity Index ◽  
Basilar Invagination ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Posterior Fossa Decompression ◽  
Radiological Measurements ◽  
Chiari I

OBJECTIVEChiari I malformation (CM-I) has traditionally been defined by measuring the position of the cerebellar tonsils relative to the foramen magnum. The relationships of tonsillar position to clinical presentation, syringomyelia, scoliosis, and the use of posterior fossa decompression (PFD) surgery have been studied extensively and yielded inconsistent results. Obex position has been proposed as a useful adjunctive descriptor for CM-I and may be associated with clinical disease severity.METHODSA retrospective chart review was performed of 442 CM-I patients with MRI who presented for clinical evaluation between 2003 and 2018. Clinical and radiological variables were measured for all patients, including presence/location of headaches, Chiari Severity Index (CSI) grade, tonsil position, obex position, clival canal angle, pB-C2 distance, occipitalization of the atlas, basilar invagination, syringomyelia, syrinx diameter, scoliosis, and use of PFD. Radiological measurements were then used to predict clinical characteristics using regression and survival analyses, with performing PFD, the presence of a syrinx, and scoliosis as outcome variables.RESULTSAmong the radiological measurements, tonsil position, obex position, and syringomyelia were each independently associated with use of PFD. Together, obex position, tonsil position, and syringomyelia (area under the curve [AUC] 89%) or obex position and tonsil position (AUC 85.4%) were more strongly associated with use of PFD than tonsil position alone (AUC 76%) (Pdiff = 3.4 × 10−6 and 6 × 10−4, respectively) but were only slightly more associated than obex position alone (AUC 82%) (Pdiff = 0.01 and 0.18, respectively). Additionally, obex position was significantly associated with occipital headaches, CSI grade, syringomyelia, and scoliosis, independent of tonsil position. Tonsil position was associated with each of these traits when analyzed alone but did not remain significantly associated with use of PFD when included in multivariate analyses with obex position.CONCLUSIONSCompared with tonsil position alone, obex position is more strongly associated with symptomatic CM-I, as measured by presence of a syrinx, scoliosis, or use of PFD surgery. These results support the role of obex position as a useful radiological measurement to inform the evaluation and potentially the management of CM-I.

scholarly journals Surgical treatment of Chiari I malformation: indications and approaches

2001 ◽  
Vol 11 (1) ◽  
pp. 1-5 ◽  
Author(s):  
Tord D. Alden ◽  
Jeffrey G. Ojemann ◽  
T. S. Park
Keyword(s):  
Posterior Fossa ◽  
Clinical Presentation ◽  
Treatment Options ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Cerebrospinal Fluid Flow ◽  
Hindbrain Herniation ◽  
Chiari I ◽  
Symptoms And Signs ◽  
Exact Origin

Chiari I malformation is a well-described entity characterized by hindbrain herniation through the foramen magnum. Although the exact origin of congenital Chiari I malformation is unknown, it appears to be caused by a mismatch between the volume of the posterior fossa neural elements and the posterior fossa cranial content. Several theories have been proposed to describe the resultant pathophysiology of this mismatch. It is clear, however, that abnormal cerebrospinal fluid flow and velocity play a role in the symptoms and signs associated with this disorder. The authors will review the pathophysiology, clinical presentation, and treatment options for patients with Chiari I malformation.

Patients with “benign” Chiari I malformations require surgical decompression at a low rate

2019 ◽  
Vol 23 (4) ◽  
pp. 498-506 ◽  
Author(s):  
Tofey J. Leon ◽  
Elizabeth N. Kuhn ◽  
Anastasia A. Arynchyna ◽  
Burkely P. Smith ◽  
R. Shane Tubbs ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Posterior Fossa ◽  
Multivariate Logistic Regression Analysis ◽  
Large Cohort ◽  
Initial Evaluation ◽  
Published Data ◽  
Posterior Fossa Decompression ◽  
Signal Abnormality ◽  
Chiari I

OBJECTIVEThere are sparse published data on the natural history of “benign” Chiari I malformation (CM-I)—i.e., Chiari with minimal or no symptoms at presentation and no imaging evidence of syrinx, hydrocephalus, or spinal cord signal abnormality. The purpose of this study was to review a large cohort of children with benign CM-I and to determine whether these children become symptomatic and require surgical treatment.METHODSPatients were identified from institutional outpatient records using International Classification of Diseases, 9th Revision, diagnosis codes for CM-I from 1996 to 2016. After review of the medical records, patients were excluded if they 1) did not have a diagnosis of CM-I, 2) were not evaluated by a neurosurgeon, 3) had previously undergone posterior fossa decompression, or 4) had imaging evidence of syringomyelia at their first appointment. To include only patients with benign Chiari (without syrinx or classic Chiari symptoms that could prompt immediate intervention), any patient who underwent decompression within 9 months of initial evaluation was excluded. After a detailed chart review, patients were excluded if they had classical Chiari malformation symptoms at presentation. The authors then determined what changes in the clinical picture prompted surgical treatment. Patients were excluded from the multivariate logistic regression analysis if they had missing data such as race and insurance; however, these patients were included in the overall survival analysis.RESULTSA total of 427 patients were included for analysis with a median follow-up duration of 25.5 months (range 0.17–179.1 months) after initial evaluation. Fifteen patients had surgery at a median time of 21.0 months (range 11.3–139.3 months) after initial evaluation. The most common indications for surgery were tussive headache in 5 (33.3%), syringomyelia in 5 (33.3%), and nontussive headache in 5 (33.3%). Using the Kaplan-Meier method, rate of freedom from posterior fossa decompression was 95.8%, 94.1%, and 93.1% at 3, 5, and 10 years, respectively.CONCLUSIONSAmong a large cohort of patients with benign CM-I, progression of imaging abnormalities or symptoms that warrant surgical treatment is infrequent. Therefore, these patients should be managed conservatively. However, clinical follow-up of such individuals is justified, as there is a low, but nonzero, rate of new symptom or syringomyelia development. Future analyses will determine whether imaging or clinical features present at initial evaluation are associated with progression and future need for treatment.

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