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Clinical Case Reports Mar 2023A 6-year-old girl with persistent headaches and the visual problem was diagnosed as a delayed onset cranial pansynostosis with concurrent type 1.5 Arnold-Chiari...
A 6-year-old girl with persistent headaches and the visual problem was diagnosed as a delayed onset cranial pansynostosis with concurrent type 1.5 Arnold-Chiari malformation. She underwent multi-sutural reconstructive surgery and followed. The headache was greatly decreased and tonsillar-brain stem herniation and syrinx were resolved.
PubMed: 36891239
DOI: 10.1002/ccr3.6981 -
World Neurosurgery May 2023In patients with Chiari malformation type 1 (CMI) presenting with low intracranial compliance (ICC), foramen magnum decompression (FMD) often fails and the complication... (Review)
Review
BACKGROUND
In patients with Chiari malformation type 1 (CMI) presenting with low intracranial compliance (ICC), foramen magnum decompression (FMD) often fails and the complication rate may be higher. We therefore routinely perform a preoperative assessment of ICC from intracranial pressure measurement. Patients with low ICC are treated with ventriculoperitoneal shunt (VPS) before FMD. In this study, we assess the outcome of patients with low ICC, compared with patients with high ICC treated with FMD alone.
METHODS
We reviewed the clinical and radiologic data of all consecutive patients with CMI treated between April 2008 and June 2021. ICC was assessed by overnight measurement of the pulsatile intracranial pressure: mean wave amplitude (MWA) above a previously defined threshold for abnormality was considered a surrogate marker for low ICC. The outcome was scored by Chicago Chiari Outcome Scale.
RESULTS
Of 73 patients, 23 with low ICC (average MWA 6.8 ± 1.2 mm Hg) received VPS before FMD, whereas 50 with high ICC (average MWA 4.4 ± 1.0 mm Hg) received only FMD. After a mean follow-up of 78.7 ± 41.4 months, 96% of all patients subjectively improved. The mean Chicago Chiari Outcome Scale score was 13.1 ± 2.2. Enlargement of cerebrospinal fluid space in the foramen magnum was achieved in 95% and regression of syringomyelia in 74% of cases. We found no significant difference in outcome between the patients with low and high ICC.
CONCLUSIONS
By identifying patients with CMI associated with low ICC and tailoring their treatment by VPS before FMD, we achieved favorable clinical and radiologic outcomes comparable to those with high ICC.
Topics: Humans; Adult; Magnetic Resonance Imaging; Intracranial Pressure; Arnold-Chiari Malformation; Foramen Magnum; Decompression, Surgical; Treatment Outcome
PubMed: 36889634
DOI: 10.1016/j.wneu.2023.02.134 -
In Vivo (Athens, Greece) 2023There is increasing evidence that patients infected with SARS-CoV-2 develop neurological manifestations such as encephalitis. The purpose of this article was to present...
BACKGROUND/AIM
There is increasing evidence that patients infected with SARS-CoV-2 develop neurological manifestations such as encephalitis. The purpose of this article was to present a case of viral encephalitis associated with SARS-CoV-2 in a 14-year-old child with Chiari malformation type I.
CASE REPORT
The patient manifested frontal headache, nausea, vomiting, skin pallor, right side Babinski sign and was diagnosed with Chiari malformation type I. He was admitted with generalized seizures and suspected encephalitis. Brain inflammation and viral RNA in the cerebrospinal fluid suggested SARS-CoV-2 encephalitis. These findings indicate that the SARS-CoV-2 test in CSF of patients with neurological manifestations, confusion, and fever during the COVID-19 pandemic should be carried out even when there is no evidence of respiratory infection. To our knowledge, this presentation of encephalitis associated with COVID-19 has not yet been reported in a patient with a congenital syndrome such as Chiari malformation type I.
CONCLUSION
Further clinical data are needed to determine the complications of encephalitis due to SARS-CoV-2 in patients with Chiari malformation type I to standardize diagnosis and treatment.
Topics: Male; Humans; Child; Adolescent; COVID-19; Arnold-Chiari Malformation; SARS-CoV-2; Pandemics; Encephalitis
PubMed: 36881093
DOI: 10.21873/invivo.13165 -
Zhongguo Yi Xue Ke Xue Yuan Xue Bao.... Feb 2023Craniovertebral junction anomalies are a group of diseases characterized by the pathological changes of occipital bone,atlantoaxial bone,cerebellar tonsil,surrounding...
Craniovertebral junction anomalies are a group of diseases characterized by the pathological changes of occipital bone,atlantoaxial bone,cerebellar tonsil,surrounding soft tissue,and nervous system,which are caused by a variety of factors.Chiari malformation is a common type of craniovertebral junction anomalies,the conventional surgical therapy of which is posterior fossa decompression.Currently,scholars represented by Goel have proposed a new theory on the classification,pathogenesis,and treatment of Chiari malformation based on posterior atlantoaxial fixation (Goel technique).This article introduces the progress in Goel technique,aiming to provide reference for the clinical work.
Topics: Humans; Arnold-Chiari Malformation
PubMed: 36861162
DOI: 10.3881/j.issn.1000.503X.14637 -
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 -
Yonsei Medical Journal Mar 2023This study aimed to analyze the effect of foramen magnum decompression with C1 laminectomy (C1L) for Chiari malformation type 1 (CM-1) in terms of improving clinical...
PURPOSE
This study aimed to analyze the effect of foramen magnum decompression with C1 laminectomy (C1L) for Chiari malformation type 1 (CM-1) in terms of improving clinical symptoms, expanding posterior fossa volume, and decreasing syrinx volume.
MATERIALS AND METHODS
Between January 2007 and June 2019, 107 patients with CM-1 were included. The median patient age was 13±13 years (range: 9 months-60 years), female-to-male ratio was 1:1, and average length of tonsil herniation was 13±5 mm (range: 5-24 mm). Surgical techniques were divided into four groups based on duraplasty or C1L usage. Among the study subjects, 38 patients underwent duraplasty and had their syrinx volumes measured separately on serial magnetic resonance imaging. A three-dimensional visualization software was used to evaluate the syrinx-volume decrease rate.
RESULTS
Bony decompression exhibited a mere 20% volume expansion of the lower-half posterior fossa. C1L offered a 3% additional volume expansion, which rose to 5% when duraplasty was added (=0.029). There were no significant differences in complication rate when C1L was combined with duraplasty (=0.526). Syrinx volumes were analyzed in 38 patients who had undergone duraplasty. Among them, 28 patients who had undergone duraplasty without C1L demonstrated a 5.9% monthly decrease in syrinx volume, which was 7.5% in the remaining 10 patients with C1L (=0.040).
CONCLUSION
C1L was effective in increasing posterior fossa volume expansion, both with and without duraplasty. A more rapid decrease in syrinx volume occurred when C1L was combined with duraplasty.
Topics: Humans; Male; Female; Infant; Laminectomy; Decompression, Surgical; Dura Mater; Arnold-Chiari Malformation; Plastic Surgery Procedures; Syringomyelia; Magnetic Resonance Imaging; Treatment Outcome; Retrospective Studies
PubMed: 36825345
DOI: 10.3349/ymj.2022.0506 -
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 -
Cureus Jan 2023Arnold-Chiari malformation is a very uncommon array of deformities in the posterior part of the cranium and hindbrain caused due to abnormal extension of the posterior...
Arnold-Chiari malformation is a very uncommon array of deformities in the posterior part of the cranium and hindbrain caused due to abnormal extension of the posterior brain into the spinal canal. Chiari malformation is further divided into subtypes 1, 2, and 3. The latter two are more common in pediatric forms and present at birth. The severity of symptoms depends upon the extent of herniation of the hindbrain due to herniation of the cerebellum through the foramen of the cranium. Also, there have been instances of absence of cerebellum. Multiple associated disorders like hydrocephalus due to increased intracranial pressure, then encephalocele, syrinx, or spinal deformity in the form of scoliosis have been presented in many cases. All these factors thus become a challenge to anesthesiologists for such patients. Hence evidence-based knowledge along with multidisciplinary, well-planned approach is required for its management.
PubMed: 36819310
DOI: 10.7759/cureus.33848 -
Turkish Neurosurgery 2023To retrospectively evaluate the relationships between morphometric measurements in craniovertebral junction, and the possible genetic basis of the disease in Chiari 1...
AIM
To retrospectively evaluate the relationships between morphometric measurements in craniovertebral junction, and the possible genetic basis of the disease in Chiari 1 malformation patients undergoing surgical treatment.
MATERIAL AND METHODS
We included patients who underwent surgical treatment for Chiari 1 malformation with unknown etiology between 2013-2018 in the Neuosurgery Clinic of Eskişehir Osmangazi University. Morphometric and volumetric measurements of the posterior fossa from cranial and cervical radiological imagings of patients with Chiari 1 malformation were performed by a single experienced observer. The results were independently reviewed by two experienced observers who supervised the process and verified the calculations.
RESULTS
It was observed that basal angle was greater in the group of patients with Chiari 1 malformation (125.29° ± 9.522° Vs. 112.33° ± 9.09°), and a significant difference was observed (p=0.000). In patient group, it was also found that the basion-dens distance (3.51 mm ± 1.695mm Vs. 5.27 ± 1.695 mm) (p=0.000), and cerebellar hemispher height were shorter (58.47 ± 5.143 mm Vs. 52.05 ± 5,008 mm) (p=0.000). Tentorium angle was greater in the patient group (97.95° ± 11.321° vs. 87.79° ± 9.891°) (p=0.034). In patient group, dens height was found to be longer (13.9 ± 2.46 mm vs. 14.79 ± 1.631mm) (p=0.041). Sella volume was found to be smaller (41.4 mm3 Vs. 53.3 mm3) in the patient group compared to the control group (p=0.034), and foramen magnum plane was larger (64.6° vs. 31.1°) (p=0.000).
CONCLUSION
It is concluded that many parameters including BA, FMP, BDD, CHH, TA, DH, and cellar volume are abnormal, suggesting the need for focussing to the hormonal metabolism - related synchondral changes.
Topics: Humans; Retrospective Studies; Magnetic Resonance Imaging; Arnold-Chiari Malformation; Cerebellum; Skull; Cranial Fossa, Posterior
PubMed: 36799276
DOI: 10.5137/1019-5149.JTN.40088-22.4 -
Frontiers in Pediatrics 2022We present the case of a 13-year-old boy who unexpectedly needed to be resuscitated at home after an assumed uncomplicated otitis media. Imaging at our clinic showed...
We present the case of a 13-year-old boy who unexpectedly needed to be resuscitated at home after an assumed uncomplicated otitis media. Imaging at our clinic showed mastoiditis and a cystoid mass in the left cerebellopontine angle compressing the brainstem, as well as an Arnold-Chiari-Malformation. Both the laboratory examination of cerebrospinal fluid (CSF) and surgical biopsy with pathological evaluation of the mastoid supported the inflammatory etiology of the mass. Microbiologically, Streptococcus intermedius was detected in the blood culture and CSF. Due to brain death, which most likely already existed preclinically, the organs were released for donation during the course. Our case demonstrates a very rare lethal complication of acute otitis media on the basis of a cerebral malformation and emphasizes the need to stay alert when patients complain of symptoms after assumed resolution.
PubMed: 36756533
DOI: 10.3389/fped.2022.1013300