-
Journal of Clinical Medicine Oct 2023Arnold Chiari syndrome is a rare congenital disease of unknown prevalence and whose origin is still under study. It is encompassed within the posterior cranial... (Review)
Review
Arnold Chiari syndrome is a rare congenital disease of unknown prevalence and whose origin is still under study. It is encompassed within the posterior cranial malformations, showing a wide spectrum of symptomatology that can range from severe headache, dizziness, and paresthesia to complete asymptomatology. It is for this reason that early diagnosis of the disease is difficult, and it is usually diagnosed in adolescence. Treatment is based on remodeling and decompression of the malformed posterior cranial fossa, although the risk of residual symptoms after surgery is high. The aim of this review is to update all the existing information on this pathology by means of an exhaustive analysis covering all the scientific literature produced in the last 5 years. In addition, it has been carried out following the PRISMA model and registered in PROSPERO with code CRD42023394490. One of the main conclusions based on the results obtained in this review is that the origin of the syndrome could have a genetic basis and that the treatment of choice is the decompression of the posterior cerebral fossa.
PubMed: 37892831
DOI: 10.3390/jcm12206694 -
Neurology India 2022
Topics: Humans; Syringomyelia; Scoliosis; Arnold-Chiari Malformation; Magnetic Resonance Imaging
PubMed: 36352639
DOI: 10.4103/0028-3886.359263 -
Cephalalgia : An International Journal... Aug 2022Chiari I malformation typically presents with cough headache. However, migraine-like or tension-type-like headaches may also occur. There are limited publications on...
BACKGROUND
Chiari I malformation typically presents with cough headache. However, migraine-like or tension-type-like headaches may also occur. There are limited publications on Chiari I malformation-associated headache semiologies and the effect of foramen magnum decompression on different headache types.
METHODS
A retrospective analysis complemented by structured phone interviews was performed on 65 patients with Chiari I malformation, treated at our hospital between 2010 and 2021. Headache semiology (according to ICHD-3), frequency, intensity, and radiological characteristics were evaluated pre- and postoperatively.
RESULTS
We included 65 patients. 38 patients were female and 27 male. Mean age was 43.9 ± 15.7 years. Headache was predominant in 41 patients (63.0%). Twenty-one patients had cough headache and 20 had atypical headache (12 migrainous, eight tension-type headache-like). Thirty-five patients with headache underwent surgery. Frequency, intensity, and analgesic use was significantly reduced in cough headache ( < 0.001). Atypical headaches improved less ( = 0.004 to 0.176). Exploratory analysis suggested that larger preoperative tonsillar descent correlated with larger postoperative headache intensity relief ( = 0.025).
CONCLUSION
Decompression was effective in Chiari I malformation-related cough headache. Atypical headache responded less well, and the causal relation with Chiari I malformation remains uncertain. For atypical headache, decompression should only be considered after failed appropriate preventive therapy and within an interdisciplinary approach involving a neurologist.
Topics: Adult; Arnold-Chiari Malformation; Decompression, Surgical; Female; Headache; Headache Disorders, Primary; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Migraine Disorders; Retrospective Studies
PubMed: 35236163
DOI: 10.1177/03331024221079296 -
Romanian Journal of Anaesthesia and... Dec 2021Arnold Chiari malformation (ACM) is defined as protrusion of the meninges and brain components into a congenital defect in the cranium or in the vertebral column. It was...
Arnold Chiari malformation (ACM) is defined as protrusion of the meninges and brain components into a congenital defect in the cranium or in the vertebral column. It was originally described by Austrian pathologist Hans Chiari. Among the four types, type-III ACM is the rarest and may associate with encephalocele. We report a case of type-III ACM associated with large occipitomeningoencephalocele with herniation of dysmorphic cerebellum, vermis, kinking/herniation of medulla with cerebrospinal fluid, tethering of spinal cord with posterior arch defect of C1-C3 vertebrae. The anaesthetic challenge for such patients lies in the proper preoperative work up; proper positioning of the patient during intubation; safe anaesthetic induction; intraoperative management of intracranial pressure, normothermia, and fluid and blood loss; and postoperative planning of extubation to prevent aspiration are the prerequisites to overcome the dificult airway management and anaesthetic challenge in the management of type III ACM.
PubMed: 36844119
DOI: 10.2478/rjaic-2021-0009 -
Journal of Clinical Neuroscience :... Apr 2023Syringomyelia is a chronic, progressive disease of the spinal cord. Syringomyelia is an etiologically diverse affliction caused by disturbance of normal cerebrospinal... (Review)
Review
BACKGROUND
Syringomyelia is a chronic, progressive disease of the spinal cord. Syringomyelia is an etiologically diverse affliction caused by disturbance of normal cerebrospinal fluid flow dynamics. Lesions are characterized by the formation of tubular cavities in the gray matter of the spinal cord and gliosis; however, the etiology is unknown and treatment methods differ. Many existing studies have focused on the relationship between other diseases and syringomyelia. There is a lack of comprehensive and objective reports on the research status of syringomyelia. Therefore, this study aimed to conduct a bibliometric analysis to quantify studies on Syringomyelia and trending issues in the last 20 years.
METHODS
Articles were acquired from the Web of Science Core Collection database. We used the Library Metrology online analysis platform, BICOMB, gCLUTO, CiteSpace bibliometrics tools for analysis, VOSviewer 1.6.16 (Nees Jan van Eck and Ludo Waltman, 2010), and Microsoft Excel 2019 to perform bibliometric analysis and visualization. Individual impact and collaborative information were quantified by analyzing annual publications, journals, co-cited journals, countries/regions, institutions, authors, and co-cited authors. We then identified the trending research areas of syringomyelia by analyzing the co-occurrence of keywords and co-cited references.
RESULTS
From January 2003 to August 2022, 9,556 authors from 66 countries published a total of 1,902 research articles on syringomyelia in 518 academic journals. Most publications come from the United States, China, the United Kingdom, and Japan, with the United States dominating. Nanjing University and the University of Washington are the most active institutions, Dr. Claire Rusbridge has published the most papers, and Miholat has the most co-citations. The Journal of Neurosurgery has the highest number of co-cited articles, which are mainly in the fields of neurology, surgery, and biology. High-frequency keywords included syringomyelia, Chiari-I malformation, children, surgical treatment, and spinal cord.
CONCLUSIONS
The number of articles on syringomyelia has increased steadily over the past two decades. At present, research on syringomyelia is mainly focused on the age of onset, potential therapeutic interventions, surgical treatment, avoidance of recurrence, and delay of pain. The use of surgical treatment of the disease and the mechanism of further treatment are the current hot research topics. The correlation between trauma and congenital factors, translational application, the effect of surgical treatment, postoperative recurrence, and complications are further hot research areas. These may provide ideas for further research into a radical cure for syringomyelia.
Topics: Child; Humans; Syringomyelia; Arnold-Chiari Malformation; Bibliometrics; Cerebral Cortex
PubMed: 36822071
DOI: 10.1016/j.jocn.2023.01.004 -
Concurrent Presence of Thoracolumbar Scoliosis and Chiari Malformation: Is Operative Risk Magnified?Asian Spine Journal Aug 2023Retrospective review of Kids' Inpatient Database (KID).
STUDY DESIGN
Retrospective review of Kids' Inpatient Database (KID).
PURPOSE
Identify the risks and complications associated with surgery in adolescents diagnosed with Chiari and scoliosis.
OVERVIEW OF LITERATURE
Scoliosis is frequently associated with Chiari malformation (CM). More specifically, reports have been made about this association with CM type I in the absence of syrinx status.
METHODS
The KID was used to identify all pediatric inpatients with CM and scoliosis. The patients were stratified into three groups: those with concomitant CM and scoliosis (CMS group), those with only CM (CM group), and those with only scoliosis (Sc group). Multivariate logistic regressions were used to assess association between surgical characteristics and diagnosis with complication rate.
RESULTS
A total of 90,707 spine patients were identified (61.8% Sc, 37% CM, 1.2% CMS). Sc patients were older, had a higher invasiveness score, and higher Charlson comorbidity index (all p<0.001). CMS patients had significantly higher rates of surgical decompression (36.7%). Sc patients had significantly higher rates of fusions (35.3%) and osteotomies (1.2%, all p<0.001). Controlling for age and invasiveness, postoperative complications were significantly associated with spine fusion surgery for Sc patients (odds ratio [OR], 1.8; p<0.05). Specifically, posterior spinal fusion in the thoracolumbar region had a greater risk of complications (OR, 4.9) than an anterior approach (OR, 3.6; all p<0.001). CM patients had a significant risk of complications when an osteotomy was performed as part of their surgery (OR, 2.9) and if a spinal fusion was concurrently performed (OR, 1.8; all p<0.05). Patients in the CMS cohort were significantly likely to develop postoperative complications if they underwent a spinal fusion from both anterior (OR, 2.5) and posterior approach (OR, 2.7; all p<0.001).
CONCLUSIONS
Having concurrent scoliosis and CM increases operative risk for fusion surgeries despite approach. Being independently inflicted with scoliosis or Chiari leads to increased complication rate when paired with thoracolumbar fusion and osteotomies; respectively.
PubMed: 37226444
DOI: 10.31616/asj.2022.0077 -
Journal of Pediatric Rehabilitation... 2020Myelomeningocele (MMC) arises from an early neural developmental anomaly and results in a variety of structural abnormalities and associated functional neurologic... (Review)
Review
Myelomeningocele (MMC) arises from an early neural developmental anomaly and results in a variety of structural abnormalities and associated functional neurologic deficits. As such, neurologic issues are central to virtually all clinical problems. Neurosurgical intervention strives to correct or improve these defects and prevent secondary complications. These interventions include closure of the open myelomeningocele and management (across the life span) of hydrocephalus, the Chiari II malformation (C2M) and tethered spinal cord (TSC). The development of pre-natal closure techniques and reports of improved outcome with in-utero closure (IUMC) have revolutionized the neurosurgical approach to myelomeningocele. Controversies remain surrounding patient selection, maternal risks, technique of IUMC (endoscopic vs. open) and long-term outcomes. However, real gains include reduced rates of hydrocephalus, modestly improved motor capabilities and reduction in C2M morbidity. For many decades, the cornerstone of treatment of hydrocephalus for many decades has been the placement and support of ventricular shunts. Endoscopic third ventriculostomy (ETV) with or without choroid plexus coagulation (ETV/CPC) is an appealing alternate strategy that avoids the morbidity and complications associated with shunts. The exact criteria for ETV-CPC candidacy and best metrics for outcome analysis remain active areas of debate and controversy. Similarly, neurosurgical management C2M, has centered upon the indications and clinical thresholds for performing posterior fossa surgical decompression. Tethered spinal cord management incorporates the diagnosis and surgical management of adhesions formed at the initial closure site, the consequent longitudinal traction related stress on the cord and the resulting neurologic signs and symptoms.
Topics: Adolescent; Adult; Arnold-Chiari Malformation; Child; Child, Preschool; Female; Humans; Hydrocephalus; Infant; Infant, Newborn; Meningomyelocele; Neurosurgery; Neurosurgical Procedures; Pregnancy; Prenatal Care; Spinal Dysraphism; Young Adult
PubMed: 33325414
DOI: 10.3233/PRM-200782 -
Neurosurgery Clinics of North America Jan 2023Anatomic MRI, MRI flow studies, and intraoperative ultrasonography demonstrate that the Chiari I malformation obstructs CSF pathways at the foramen magnum and prevents... (Review)
Review
Anatomic MRI, MRI flow studies, and intraoperative ultrasonography demonstrate that the Chiari I malformation obstructs CSF pathways at the foramen magnum and prevents normal CSF movement through the foramen magnum. Impaired CSF displacement across the foramen magnum during the cardiac cycle increases pulsatile hindbrain motion, pressure transmission to the spinal subarachnoid space, and the amplitude of CSF subarachnoid pressure waves driving CSF into the spinal cord. Central canal septations in adults prevent syrinx formation by CSF directly transmitting its pressure wave from the fourth ventricle to the central canal.
Topics: Adult; Humans; Syringomyelia; Hydrodynamics; Arnold-Chiari Malformation; Subarachnoid Space; Cerebrospinal Fluid Pressure
PubMed: 36424067
DOI: 10.1016/j.nec.2022.08.007 -
Child's Nervous System : ChNS :... Oct 2021The knowledge of the development and the anatomy of the posterior cranial fossa (PCF) is crucial to define the occurrence and the prognosis of diseases where the surface... (Review)
Review
BACKGROUND
The knowledge of the development and the anatomy of the posterior cranial fossa (PCF) is crucial to define the occurrence and the prognosis of diseases where the surface and/or the volume of PCF is reduced, as several forms of craniosynostosis or Chiari type I malformation (CIM). To understand the functional and morphological changes resulting from such a hypoplasia is mandatory for their correct management. The purpose of this article is to review the pertinent literature to provide an update on this topic.
METHODS
The related and most recent literature addressing the issue of the changes in hypoplasic PCF has been reviewed with particular interest in the studies focusing on the PCF characteristics in craniosynostosis, CIM, and achondroplasia.
RESULTS AND CONCLUSIONS
In craniosynostoses, namely, the syndromic ones, PCF shows different degrees of hypoplasia, according to the different pattern and timing of early suture fusion. Several factors concur to PCF hypoplasia and contribute to the resulting problems (CIM, hydrocephalus), as the fusion of the major and minor sutures of the lambdoid arch, the involvement of the basal synchondroses, and the occlusion of the jugular foramina. The combination of these factors explains the variety of the clinical and radiological phenotypes. In primary CIM, the matter is complicated by the evidence that, in spite of impaired PCF 2D measurements and theories on the mesodermal defect, the PCF volumetry is often comparable to healthy subjects. CIM is revealed by the overcrowding of the foramen magnum that is the result of a cranio-cerebral disproportion (altered PCF brain volume/PCF total volume). Sometimes, this disproportion is evident and can be demonstrated (basilar invagination, real PCF hypoplasia); sometimes, it is not. Some recent genetic observations would suggest that CIM is the result of an excessive growth of the neural tissue rather than a reduced growth of PCF bones. Finally, in achondroplasia, both macrocephaly and reduced 2D and 3D values of PCF occur. Some aspects of this disease remain partially obscure, as the rare incidence of hydrocephalus and syringomyelia and the common occurrence of asymptomatic upper cervical spinal cord damage. On the other hand, the low rate of CIM could be explained on the basis of the reduced area of the foramen magnum, which would prevent the hindbrain herniation.
Topics: Arnold-Chiari Malformation; Cranial Fossa, Posterior; Craniosynostoses; Humans; Hydrocephalus; Magnetic Resonance Imaging; Syringomyelia
PubMed: 34169386
DOI: 10.1007/s00381-021-05193-w -
Neurological Sciences : Official... Feb 2022Chiari malformation type 1 (CM1) is a rare condition where agreed classification and treatment are still missing. The goal of this study is to achieve a consensus on the...
BACKGROUND
Chiari malformation type 1 (CM1) is a rare condition where agreed classification and treatment are still missing. The goal of this study is to achieve a consensus on the diagnosis and treatment of CM1 in children.
METHODS
A multidisciplinary panel formulated 57 provisional statements based on a review of the literature. Thirty-four international experts (IE) participated in a Delphi study by independently rating each statement on a 4-point Likert scale ("strongly disagree," "disagree," "agree," "strongly agree"). Statements that were endorsed ("agree" or "strongly agree") by < 75% of raters were re-formulated, or new statements were added, and another Delphi round followed (up to a maximum of three).
RESULTS
Thirty-five IE were contacted and 34 agreed to participate. A consensus was reached on 30/57 statements (52.6%) after round 1. Three statements were added, and one removed. After round 2, agreement was reached on 56/59 statements (94.9%). Finally, after round 3, which took place during the 2019 Chiari Consensus Conference (Milan, Italy), agreement was reached on 58/59 statements (98.3%) about four main sections (Definition and Classification, Planning, Surgery, Isolated Syringomyelia). Only one statement did not gain a consensus, which is the "definition of radiological failure 24 month post-surgery."
CONCLUSIONS
The consensus document consists of 58 statements (24 on diagnosis, 34 on treatment), serving clinicians and researchers following children with CM1. There is a clear need for establishing an international network and registry and to promote collaborative studies to increase the evidence base and optimize the long-term care of this patient population.
Topics: Arnold-Chiari Malformation; Child; Consensus; Delphi Technique; Humans; Italy; Syringomyelia
PubMed: 34097175
DOI: 10.1007/s10072-021-05317-9