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Pediatric Neurosurgery 2023The association between multisutural craniosynostosis with Chiari malformation (CM), venous hypertension, and hydrocephalus is widely described in the literature,...
INTRODUCTION
The association between multisutural craniosynostosis with Chiari malformation (CM), venous hypertension, and hydrocephalus is widely described in the literature, especially in children with paediatric craniofacial syndromes. Some efforts have been done in the last years to understand the complex pathogenetic mechanisms underlying this association, and several theories have been proposed. In particular, it is now accepted that the hypothesis of the overcrowding of the posterior fossa due to precocious suture fusion is the cause of the cerebellar herniation in syndromic and non-syndromic patients, against the theory of intrinsic cerebellar anomalies, ventriculomegaly, and venous hypertension. However, whatever the pathophysiological mechanism, it is still unclear what the best management and treatment of CM and hydrocephalus are in multisutural craniosynostosis patients. The aim of this study was to report our 25 years' experience in treating paediatric patients affected by these rare pathologies in order to propose a simple and effective therapeutic flow chart for their management.
MATERIALS AND METHODS
We retrospectively collected data of each patient who underwent a cranial vault remodelling (CVR) for complex multisutural craniosynostosis in our institution in the last 25 years, while monosutural craniosynostosis was excluded. We recorded data concerning type of craniosynostosis and craniofacial syndromes, presence of ventriculomegaly, and CM at presentation and clinical and radiological follow-up. Therefore, we evaluated the final outcomes (improved, stable, deteriorated) of these patients and created a practical flow chart that could help physicians choose the best surgical treatment when different pathological conditions, as Chiari malformation I (CMI) or hydrocephalus, affect complex craniosynostosis children.
RESULTS
Thirty-nine patients (39 out of 55; 70.9%), with an isolated multisutural craniosynostosis at presentation, underwent a two-step CVR as first surgery; 36 patients (92.3%) had an improved outcome, 2 patients (5.1%) had a stable outcome, and 1 patient (2.56%) had a deteriorated outcome. Other eight children (8 out of 55; 14.5%) had a radiological evidence of asymptomatic CMI at presentation. In this group, we performed CVR as first surgery. As for the final outcome, 7 patients had an improved outcome (87.5%) with good aesthetic result and stability or resolution of CMI. Finally, 7 patients (7 out of 55; 12.7%) presented a various combination of CMI and ventriculomegaly or hydrocephalus at presentation. Among them, 3 patients had an improved outcome (42.8%), and 4 patients had a deteriorated outcome (57.1%).
DISCUSSION
The prevalence of one pathological condition with associated symptoms over the others was the key factor leading our therapeutic strategy. When craniosynostosis is associated with a radiological CM, the assessment of clinical symptoms is of capital importance. When asymptomatic or pauci-symptomatic, we suggest a CVR as first step, for its efficacy in reducing tonsillar herniation and solving CM symptoms. When craniosynostosis is associated with ventricular enlargement, the presence of intracranial hypertension signs and symptoms forces physicians to first treat hydrocephalus with a ventriculo-peritoneal shunt or endoscopic third ventriculostomy. For patients with various degrees and severity of ventriculomegaly and associated CM, the outcomes were very heterogeneous, even when the same therapeutic strategy was applied to patients with similar starting conditions and symptoms. This is maybe the most unexpected and least clear part of our results. Despite the proposed algorithm comes from a clinical experience on 85% successfully treated patients with multiple craniosynostosis, more extensive and deep studies are needed to better understand CM and hydrocephalus development in such conditions.
Topics: Humans; Child; Syndrome; Retrospective Studies; Arnold-Chiari Malformation; Hydrocephalus; Craniosynostoses; Encephalocele; Hypertension; Magnetic Resonance Imaging
PubMed: 36720214
DOI: 10.1159/000529129 -
BMJ Case Reports Apr 2011Chiari in 1891 described a constellation of anomalies at the base of the brain inherited congenitally, the characteristic of which are (1) extension of a tongue of...
Chiari in 1891 described a constellation of anomalies at the base of the brain inherited congenitally, the characteristic of which are (1) extension of a tongue of cerebellar tissue posterior to the medulla and cord that extends into the cervical spinal canal; (2) caudal displacement of the medulla and the inferior part of the fourth ventricle into the cervical canal; and (3) a frequent but not invariable association with syringomyelia or a spinal developmental abnormality. Chiari recognized four types of abnormalities. Presently, the term has come to be restricted to Chiari's types I and II, that is, to cerebellomedullary descent without and with a meningomyelocele, respectively. The association of Arnold-Chairi malformation and high cervical cord infarction is unusual. The most common syndrome, anterior spinal artery syndrome (ASAS), is caused by interruption of blood flow to the anterior spinal artery, producing ischaemia in the anterior two-thirds of the cord, with resulting neurologic deficits. Causes of ASAS include aortic disease, postsurgical, sepsis, hypotension and thromboembolic disorders. The authors present an interesting case of cervical cord infarction due to anterior spinal artery thrombosis in a patient of type 1 Arnold-Chiari malformation without any of the above predisposing factors.
Topics: Adult; Anterior Spinal Artery Syndrome; Arnold-Chiari Malformation; Female; Humans; Magnetic Resonance Imaging; Paresis; Persistent Vegetative State; Respiratory Insufficiency
PubMed: 22701030
DOI: 10.1136/bcr.07.2010.3170 -
Turkish Neurosurgery 2021To examine foramen magnum morphometry and shape of Chiari malformation in comparison with a control group, and to contribute to the literature on whether Chiari...
AIM
To examine foramen magnum morphometry and shape of Chiari malformation in comparison with a control group, and to contribute to the literature on whether Chiari malformation affects foramen magnum morphometry.
MATERIAL AND METHODS
In this study, cranial magnetic resonance images of 71 people with Chiari malformation and 61 people as controls were examined. The anteroposterior diameter, transverse diameter, and area of the foramen magnum were measured, and the shape of the foramen magnum was determined.
RESULTS
In the Chiari malformation group, the anteroposterior diameter of the foramen magnum was 39.11 ± 4.29 mm, the transverse diameter was 34.25 ± 3.27 mm, and its area was 969.21 ± 199.57 mm < sup > 2 < /sup > . These results were statistically higher in female patients in the Chiari malformation group than in the control group. No significant difference was found between male patients in the Chiari malformation group and those in the control group. Round-shaped foramen magnum was predominant in both groups, but this was not statistically significant.
CONCLUSION
In Chiari malformation, the foramen magnum is affected. This effect can be a factor that influences development of a malformation. As a result of a malformation, the foramen magnum may have expanded due to the pressure effect. More detailed and extensive research is needed to explain this phenomenon.
Topics: Arnold-Chiari Malformation; Female; Foramen Magnum; Humans; Magnetic Resonance Imaging; Male; Skull
PubMed: 33978222
DOI: 10.5137/1019-5149.JTN.32021-20.3 -
Annals of Biomedical Engineering Jun 2021While the degree of cerebellar tonsillar descent is considered the primary radiologic marker of Chiari malformation type I (CMI), biomechanical forces acting on the...
While the degree of cerebellar tonsillar descent is considered the primary radiologic marker of Chiari malformation type I (CMI), biomechanical forces acting on the brain tissue in CMI subjects are less studied and poorly understood. In this study, regional brain tissue displacement and principal strains in 43 CMI subjects and 25 controls were quantified using a magnetic resonance imaging (MRI) methodology known as displacement encoding with stimulated echoes (DENSE). Measurements from MRI were obtained for seven different brain regions-the brainstem, cerebellum, cingulate gyrus, corpus callosum, frontal lobe, occipital lobe, and parietal lobe. Mean displacements in the cerebellum and brainstem were found to be 106 and 64% higher, respectively, for CMI subjects than controls (p < .001). Mean compression and extension strains in the cerebellum were 52 and 50% higher, respectively, in CMI subjects (p < .001). Brainstem mean extension strain was 41% higher in CMI subjects (p < .001), but no significant difference in compression strain was observed. The other brain structures revealed no significant differences between CMI and controls. These findings demonstrate that brain tissue displacement and strain in the cerebellum and brainstem might represent two new biomarkers to distinguish between CMI subjects and controls.
Topics: Adult; Arnold-Chiari Malformation; Biomechanical Phenomena; Brain; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Stress, Mechanical; Young Adult
PubMed: 33398617
DOI: 10.1007/s10439-020-02695-7 -
Revista de Neurologia Jun 2013Chiari malformations (CM) include a series of congenital anomalies involving the descent of the cerebellar tonsils below the foramen magnum, which may be associated with... (Review)
Review
INTRODUCTION
Chiari malformations (CM) include a series of congenital anomalies involving the descent of the cerebellar tonsils below the foramen magnum, which may be associated with compression in the brainstem, upper spinal cord, and cranial nerves, consequently altering the responses of brainstem auditory evoked potentials (BAEP) and somatosensory evoked potentials (SSEP). However, only a small number of authors have described the indications of these tests in CM, and study groups to date have been small and heterogeneous.
AIM
To review the results of BAEPs and SSEPs in published studies of patients with Chiari type 1 malformation (CM-1) or Chiari type 2 malformation (CM-2) as well as the indications of both tests in the diagnosis, treatment, and monitoring of both diseases, especially CM-1.
DEVELOPMENT
We present a review article analyzing data from all published studies in Medline starting in 1966, located through PubMed, using combinations of the following keywords: 'Chiari malformation', 'Arnold-Chiari malformation', 'Chiari type 1 malformation', 'Arnold-Chiari type 1 malformation', 'evoked potentials', 'brainstem auditory evoked potentials' and 'somatosensory evoked potentials' as well as records of patients with CM-1 from the neurosurgery and neurophysiology departments at the Hospital Universitari Vall d'Hebron.
CONCLUSIONS
Common findings of SSEP are a reduction in cortical amplitude from the posterior tibial nerve, a reduction or absence of cervical median nerve potential, and an increased N13-N20 interval. In BAEP, the most frequent findings are an increased I-V interval and a peripheral or cochlear auditory disturbance.
Topics: Adolescent; Adult; Age of Onset; Arnold-Chiari Malformation; Child; Child, Preschool; Cochlear Nerve; Decompression, Surgical; Encephalocele; Evoked Potentials, Auditory, Brain Stem; Evoked Potentials, Somatosensory; Humans; Magnetic Resonance Imaging; Median Nerve; Monitoring, Intraoperative; Reaction Time; Syringomyelia; Tibial Nerve; Young Adult
PubMed: 23744249
DOI: No ID Found -
Fluids and Barriers of the CNS Mar 2021Phase contrast magnetic resonance imaging, PC MRI, is a valuable tool allowing for non-invasive quantification of CSF dynamics, but has lacked adoption in clinical...
In vitro evaluation of cerebrospinal fluid velocity measurement in type I Chiari malformation: repeatability, reproducibility, and agreement using 2D phase contrast and 4D flow MRI.
BACKGROUND
Phase contrast magnetic resonance imaging, PC MRI, is a valuable tool allowing for non-invasive quantification of CSF dynamics, but has lacked adoption in clinical practice for Chiari malformation diagnostics. To improve these diagnostic practices, a better understanding of PC MRI based measurement agreement, repeatability, and reproducibility of CSF dynamics is needed.
METHODS
An anatomically realistic in vitro subject specific model of a Chiari malformation patient was scanned three times at five different scanning centers using 2D PC MRI and 4D Flow techniques to quantify intra-scanner repeatability, inter-scanner reproducibility, and agreement between imaging modalities. Peak systolic CSF velocities were measured at nine axial planes using 2D PC MRI, which were then compared to 4D Flow peak systolic velocity measurements extracted at those exact axial positions along the model.
RESULTS
Comparison of measurement results showed good overall agreement of CSF velocity detection between 2D PC MRI and 4D Flow (p = 0.86), fair intra-scanner repeatability (confidence intervals ± 1.5 cm/s), and poor inter-scanner reproducibility. On average, 4D Flow measurements had a larger variability than 2D PC MRI measurements (standard deviations 1.83 and 1.04 cm/s, respectively).
CONCLUSION
Agreement, repeatability, and reproducibility of 2D PC MRI and 4D Flow detection of peak CSF velocities was quantified using a patient-specific in vitro model of Chiari malformation. In combination, the greatest factor leading to measurement inconsistency was determined to be a lack of reproducibility between different MRI centers. Overall, these findings may help lead to better understanding for application of 2D PC MRI and 4D Flow techniques as diagnostic tools for CSF dynamics quantification in Chiari malformation and related diseases.
Topics: Arnold-Chiari Malformation; Cerebrospinal Fluid; Child, Preschool; Humans; Hydrodynamics; Magnetic Resonance Imaging; Meta-Analysis as Topic; Models, Anatomic; Reproducibility of Results; Review Literature as Topic
PubMed: 33736664
DOI: 10.1186/s12987-021-00246-3 -
Journal of Veterinary Internal Medicine 2014Although Chiari-like malformation (CM) and syringomyelia (SM) have been described in many small breed dogs, the prevalence and clinical manifestations of this complex...
BACKGROUND
Although Chiari-like malformation (CM) and syringomyelia (SM) have been described in many small breed dogs, the prevalence and clinical manifestations of this complex have not been documented in a large cohort of American Brussels Griffon (ABG) dogs.
OBJECTIVES
To characterize the clinical and magnetic resonance imaging (MRI) features of CM and SM in the ABG breed.
ANIMALS
Eighty-four American Kennel Club registered ABG dogs were recruited.
METHODS
Prospective study. Complete histories and neurologic examinations were obtained before MRI. Images were blindly reviewed and calculations were made by using OsiriX. All analyses were performed by Student's t-test, Spearman's correlation, ANOVA, and chi-square test where appropriate.
RESULTS
Chiari-like malformation and SM were present in 65% and 52% of dogs, respectively. Twenty-eight percent of dogs had neurologic deficits and 20% had neck pain. Mean central canal (CC) transverse height was 2.5 mm with a mean length of 3.6 cervical vertebrae. Neurologic deficits were significantly associated with a larger syrinx (P = .04, P = .08) and syrinx size increased with age (P = .027). SM was associated with a smaller craniocervical junction (CCJ) height (P = .04) and larger ventricles (P = .0001; P < .001).
CONCLUSIONS AND CLINICAL IMPORTANCE
Syringomyelia and CM are prevalent in American Brussels Griffon dogs. Syrinx size is associated with neurologic deficits, CM, larger ventricles, a smaller craniocervical junction height, neurologic deficits, and cerebellar herniation. Fifty-two percent of dogs with a SM were clinically normal.
Topics: Animals; Arnold-Chiari Malformation; Cerebellum; Dog Diseases; Dogs; Female; Magnetic Resonance Imaging; Male; Neuroimaging; Prevalence; Species Specificity; Syringomyelia
PubMed: 25145262
DOI: 10.1111/jvim.12421 -
BMJ Case Reports Jun 2021The Chiari I malformation (CM-I) is characterised by overcrowding of the posterior fossa and descent of the cerebellar tonsils and is associated with syringomyelia. With... (Review)
Review
The Chiari I malformation (CM-I) is characterised by overcrowding of the posterior fossa and descent of the cerebellar tonsils and is associated with syringomyelia. With the increasing availability of magnetic resonance imaging, CM-I is placing a growing burden on neurosurgical services. However, its natural history remains poorly understood, and the timing and nature of surgical intervention is controversial. We present a case of a significant, symptomatic CM-I with associated syrinx which underwent complete spontaneous resolution over a 4-year period. Spontaneous regression of Chiari malformation and syringomyelia is exceedingly rare; a literature review reveals 15 other cases and only one case which underwent complete resolution. The present case and literature review suggest a more benign natural history of CM-I and support a more conservative approach to its management. Further studies are required to determine whether any factors can predict resolution for certain patient cohorts.
Topics: Arnold-Chiari Malformation; Decompression, Surgical; Humans; Magnetic Resonance Imaging; Syringomyelia; Trachea
PubMed: 34155013
DOI: 10.1136/bcr-2021-241789 -
Journal of Clinical Sleep Medicine :... Feb 2020Arnold-Chiari malformations are structural defects in the base of the skull and cerebellum, when part of the cerebellar tonsils herniates through the foramen magnum into...
Arnold-Chiari malformations are structural defects in the base of the skull and cerebellum, when part of the cerebellar tonsils herniates through the foramen magnum into the upper spinal canal, compressing against the brainstem. This anatomical defect can be asymptomatic but often presents with symptoms such as headaches, stridor, dysphagia, and nystagmus. It also presents with a variety of sleep-related breathing disorders such as snoring, obstructive sleep apnea, central sleep apnea, bradypnea, and sleep hypoventilation. Sometimes these conditions can coexist in one patient. Although obstructive sleep apnea can be a manifestation of Arnold-Chiari malformation, identifying causality and the site of obstruction in these children can be a diagnostic challenge. We review the case of a 14-month-old male who presented with noisy breathing and obstructive sleep apnea diagnosed on sleep study that was refractory to initial upper airway surgery. Although a brain computed tomography scan done in the emergency room for altered mental status revealed a type 1 Arnold-Chiari malformation, and a flexible awake laryngoscopy was normal, subsequent drug-induced sleep endoscopy was helpful in justifying surgical decompression of the Arnold-Chiari malformation that resulted in complete resolution of the obstructive sleep apnea.
Topics: Adolescent; Arnold-Chiari Malformation; Endoscopy; Humans; Magnetic Resonance Imaging; Male; Pharmaceutical Preparations; Sleep; Sleep Apnea, Obstructive
PubMed: 31992420
DOI: 10.5664/jcsm.8194 -
BMJ Case Reports Nov 2014An 18-year-old female patient presented with left dominant neck pain after a motor vehicle collision. Her cervical spine MRI revealed syringomyelia with associated Type...
An 18-year-old female patient presented with left dominant neck pain after a motor vehicle collision. Her cervical spine MRI revealed syringomyelia with associated Type I Arnold-Chiari malformation. Some researchers have reported that these might be considered contraindications to spinal manipulation. Nevertheless, her benign and functional clinical examination suggested otherwise and she underwent four manipulative treatments in 2 weeks. By the end of the treatment plan and after 1-month follow-up, she was asymptomatic, no adverse effects were noted and her outcome assessment score decreased from 56% to 0%. This case illustrates that spinal manipulation may be a useful adjunctive treatment procedure for spinal pain, even in the presence of syringomyelia and Chiari malformation, which may not necessarily be a contraindication to spinal manipulation, when performed by a skilled and well-trained physician.
Topics: Adolescent; Arnold-Chiari Malformation; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Manipulation, Spinal; Neck Pain; Pain Measurement; Radiculopathy; Risk Assessment; Severity of Illness Index; Syringomyelia; Treatment Outcome
PubMed: 25385566
DOI: 10.1136/bcr-2014-207319