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Scientific Reports Jun 2024Chiari type 1 malformation is a neurological disorder characterized by an obstruction of the cerebrospinal fluid (CSF) circulation between the brain (intracranial) and...
Chiari type 1 malformation is a neurological disorder characterized by an obstruction of the cerebrospinal fluid (CSF) circulation between the brain (intracranial) and spinal cord (spinal) compartments. Actions such as coughing might evoke spinal cord complications in patients with Chiari type 1 malformation, but the underlying mechanisms are not well understood. More insight into the impact of the obstruction on local and overall CSF dynamics can help reveal these mechanisms. Therefore, our previously developed computational fluid dynamics framework was used to establish a subject-specific model of the intracranial and upper spinal CSF space of a healthy control. In this model, we emulated a single cough and introduced porous zones to model a posterior (OBS-1), mild (OBS-2), and severe posterior-anterior (OBS-3) obstruction. OBS-1 and OBS-2 induced minor changes to the overall CSF pressures, while OBS-3 caused significantly larger changes with a decoupling between the intracranial and spinal compartment. Coughing led to a peak in overall CSF pressure. During this peak, pressure differences between the lateral ventricles and the spinal compartment were locally amplified for all degrees of obstruction. These results emphasize the effects of coughing and indicate that severe levels of obstruction lead to distinct changes in intracranial pressure.
Topics: Arnold-Chiari Malformation; Cough; Humans; Hydrodynamics; Cerebrospinal Fluid; Computer Simulation; Cerebrospinal Fluid Pressure; Spinal Cord; Female
PubMed: 38830910
DOI: 10.1038/s41598-024-62374-8 -
Archives of Razi Institute Dec 2023Klippel-Feil Syndrome (KFS) is a rare genetic disorder characterized by the abnormal development of the cervical spine, leading to the fusion of two or more cervical...
Klippel-Feil Syndrome (KFS) is a rare genetic disorder characterized by the abnormal development of the cervical spine, leading to the fusion of two or more cervical vertebrae. The syndrome presents diverse symptoms, including limited neck movement, chronic pain, and neurological manifestations such as limb numbness or weakness. The severity of KFS can vary significantly, and treatment primarily focuses on symptom management and preventing complications such as scoliosis or spinal cord compression. Surgical interventions are often necessary for patients with complex forms of the syndrome. Interestingly, Chiari 1 malformation, a cranial anomaly affecting the brainstem, can coincide anatomically with KFS. In this case report, we present the case of a 9-year-old patient who sought medical attention due to persistent, unchanging neck pain. The patient's medical history was notable for developmental delays and cervical restraint observed during physical examination. Magnetic resonance imaging (MRI) findings revealed hydrocephalus and brainstem descent, indicating the presence of Chiari 1 malformation. Comprehensive MRI and CT scans were performed, and a management plan was formulated, primarily involving cranial surgery and physiotherapy. Implementation of the treatment approach resulted in significant improvement in the patient's symptoms. This case highlights the significance of considering Chiari 1 malformation as a potential comorbidity in patients diagnosed with KFS who present with persistent neck pain. Early detection and appropriate management of both conditions are crucial for achieving favorable outcomes and enhancing the quality of life for affected individuals. Understanding the complex interplay between KFS and Chiari 1 malformation is essential for providing comprehensive care and tailored treatment strategies. Further research is warranted to elucidate the underlying mechanisms linking these two conditions and to explore optimal management approaches for patients with dual pathology. By reporting this case, we contribute to the existing literature and increase awareness among healthcare professionals regarding the potential coexistence of KFS and Chiari 1 malformation. Continued efforts in identifying associated anomalies and optimizing therapeutic interventions will aid in improving patient outcomes and ensuring optimal care for individuals affected by these conditions.
Topics: Klippel-Feil Syndrome; Humans; Child; Magnetic Resonance Imaging; Arnold-Chiari Malformation; Male; Tomography, X-Ray Computed; Neck Pain; Female
PubMed: 38828178
DOI: 10.32592/ARI.2023.78.6.1868 -
Neurology India Mar 2024
Topics: Humans; Arnold-Chiari Malformation
PubMed: 38817188
DOI: 10.4103/neurol-india.Neurol-India-D-24-00173 -
Alpha Psychiatry Mar 2024Undiagnosed underlying medical conditions can cause many patients to be followed, for years, by a diagnosis of a primary psychiatric disorder and to receive...
BACKGROUND
Undiagnosed underlying medical conditions can cause many patients to be followed, for years, by a diagnosis of a primary psychiatric disorder and to receive inappropriate treatment. The aim of this study was to determine the prevalence of patients initially diagnosed with a primary psychiatric disorder but whose symptoms were later attributed to medical conditions. These patients' initial and final diagnoses were also examined.
METHODS
The records of 1843 patients hospitalized in Gazi University Faculty of Medicine Psychiatric Inpatient Clinic between 2015 and 2020 were examined in this retrospective and descriptive study. Thirteen patients were excluded from the study due to insufficient data. Descriptive statistics were performed on the data of 30 patients diagnosed with an underlying medical condition.
RESULTS
In follow-up, 49 patients' diagnoses changed. 19 patients had a diagnosis unrelated to an underlying medical condition, and 30 had a diagnosis related to an underlying medical condition. Five (16.7%) of the patients, previously known to have psychiatric disorders, were found to have Alzheimer's disease. Brain space-occupying lesions, frontotemporal dementia, epilepsy, Parkinson's disease, and Arnold-Chiari malformation followed Alzheimer's disease at 6.7% (n = 2). The mean time until the diagnosis of the patients was revised was 4.95 years (standard deviation [SD] = 7.78). It was observed that psychotropic medications (90%) were used more than non-psychotropic drugs until the diagnosis was revised.
CONCLUSION
During the diagnostic process, we believe that clinicians should be aware of potential underlying medical conditions and that the multidisciplinary work of psychiatry and neurology is also crucial.
PubMed: 38798811
DOI: 10.5152/alphapsychiatry.2024.231274 -
International Medical Case Reports... 2024Arnold-Chiari malformation is a displacement of the brain into the spinal canal, often leading to hemodynamic distress. Arnold-Chiari malformation type III (CM III) is a...
Arnold-Chiari malformation is a displacement of the brain into the spinal canal, often leading to hemodynamic distress. Arnold-Chiari malformation type III (CM III) is a rare and severe type that requires early detection to prevent complications. We aim to report this rare presentation of CM III. A 16-month-old girl with a massive tumor of the occiput suspected to be a meningoencephalocele with CM III was referred to the neurosurgery department. The patient was assessed with class 3 American Society of Anesthesiologist classification, posed challenges due to a difficult airway and the presence of a meningoencephalocele. The patient was scheduled for an elective surgery. Preoperative, the patient was lethargic with an abnormal heart rate and blood pressure. Induction was performed while preserving spontaneous ventilation, but was assisted by an oropharyngeal airway due to a short neck and obesity. The patient later developed laryngospasm thus, muscle relaxant and propofol were administered. A rescue laryngeal mask airway was inserted due to desaturation. Intubation was performed with video laryngoscope. When the surgeon opened the cele and removed the cerebrospinal fluid, the blood pressure began to decline. Resection of brain tissue caused the patient started to bleed, fluid and blood products were administered. At the end of surgery, the patient had stable hemodynamics and was transferred to the pediatric intensive care for monitoring. In patients with massive occipital meningoencephalocele and CM III, anesthetic management requires smooth intubation and prevention of excessive manipulation of the cervical joint to prevent increased intracranial pressure. The patient was not extubated because the brainstem could not be sufficiently reduced coupled with significant autonomic dysfunction.
PubMed: 38681994
DOI: 10.2147/IMCRJ.S453554 -
Neurosurgical Review Apr 2024Segmentation tools continue to advance, evolving from manual contouring to deep learning. Researchers have utilized segmentation to study a myriad of posterior...
BACKGROUND
Segmentation tools continue to advance, evolving from manual contouring to deep learning. Researchers have utilized segmentation to study a myriad of posterior fossa-related conditions, such as Chiari malformation, trigeminal neuralgia, post-operative pediatric cerebellar mutism syndrome, and Crouzon syndrome. Herein, we present a summary of the current literature on segmentation of the posterior fossa. The review highlights the various segmentation techniques, and their respective strengths and weaknesses, employed along with objectives and outcomes of the various studies reported in the literature.
METHODS
A literature search was conducted in PubMed, Embase, Cochrane, and Web of Science up to November 2023 for articles on segmentation techniques of posterior fossa. The two senior authors searched through databases based on the keywords of the article separately and then enrolled joint articles that met the inclusion and exclusion criteria.
RESULTS
The initial search identified 2205 articles. After applying inclusion and exclusion criteria, 77 articles were selected for full-text review after screening of titles/abstracts. 52 articles were ultimately included in the review. Segmentation techniques included manual, semi-automated, and fully automated (atlas-based, convolutional neural networks). The most common pathology investigated was Chiari malformation.
CONCLUSIONS
Various forms of segmentation techniques have been used to assess posterior fossa volumes/pathologies and each has its advantages and disadvantages. We discuss these nuances and summarize the current state of literature in the context of posterior fossa-associated pathologies.
Topics: Humans; Arnold-Chiari Malformation; Cranial Fossa, Posterior; Magnetic Resonance Imaging
PubMed: 38637466
DOI: 10.1007/s10143-024-02366-4 -
Case Reports in Endocrinology 2024We present a case of acromegaly associated with Arnold-Chiari 1 malformation and a literature review regarding this association, mainly focusing on the importance of a...
We present a case of acromegaly associated with Arnold-Chiari 1 malformation and a literature review regarding this association, mainly focusing on the importance of a clear distinction between Chiari malformation and herniation of cerebellar tonsils (CTH). Indeed, in many clinical cases, this distinction has not been properly made and a better description of the radiological findings could be important for the clinical management of these patients. In fact, Arnold-Chiari 1 malformation, as a congenital disease, is not caused by acquired growth hormone (GH) excess, but the latter could worsen pre-existing CTH or even induce it . Therefore, awareness of this condition in the clinical management of acromegaly appears crucial.
PubMed: 38550571
DOI: 10.1155/2024/4733399 -
World Neurosurgery May 2024Chiari malformation is characterized by inferior displacement of the cerebellar tonsils through the foramen magnum, frequently resulting in strain related headaches, and...
BACKGROUND
Chiari malformation is characterized by inferior displacement of the cerebellar tonsils through the foramen magnum, frequently resulting in strain related headaches, and motor/sensory dysfunction. Chiari decompression technique varies significantly, possibly contributing to frequent revisions. We reviewed revision Chiari decompressions at our institution to determine the primary indications for revision and outcomes after revision.
METHODS
We retrospectively reviewed patients who underwent revision of Chiari decompression at our institution from 2005 to 2020. Demographics, indications for revision surgery, operative techniques, imaging findings, and preoperative/postoperative symptoms were collected. χ test was performed to determine statistical significance using a P < 0.05. Independent predictors of operative outcomes were identified.
RESULTS
A total of 46 patients (91% females, mean age 38.8 years) were included for analysis. The median time to revision surgery was 69.1 months (range 0-364 months) with headache (n = 37, 80%) being the most commonly recurring symptom. Large craniectomy (n = 28, 61%) was the most frequent indication for revision surgery. Thirty-two (70%) patients underwent cranioplasty, 20 (43%) required duraplasty, 15 (33%) required arachnoid dissection, and 15 (33%) required tonsillar reduction during revision surgery. Postrevision follow-up (at 8.9 ± 5.2 months average, range 1-18 months), revealed an average reduction in all Chiari-related symptoms relative to symptoms before the revision.
CONCLUSIONS
The most common indication for revision Chiari decompression was a large craniectomy resulting in cerebellar ptosis. We found that tonsillar reduction paired with modest craniectomy achieved near-complete resolution of symptoms with minimal complications. For patients with recurrent or persistent sequelae of Chiari malformation after decompression, revision may reduce symptom severity.
Topics: Humans; Arnold-Chiari Malformation; Female; Male; Reoperation; Adult; Decompression, Surgical; Retrospective Studies; Middle Aged; Treatment Outcome; Young Adult; Cohort Studies; Decompressive Craniectomy; Postoperative Complications
PubMed: 38490446
DOI: 10.1016/j.wneu.2024.03.026 -
Radiology Case Reports May 2024Chiari malformations are structural defects in the cerebellum that are characterized by the downward displacement of one or both cerebellar tonsils through the foramen...
Chiari malformations are structural defects in the cerebellum that are characterized by the downward displacement of one or both cerebellar tonsils through the foramen magnum. A case report of a female neonate with clinical features of Chiari II malformation is presented. The diagnosis of this condition was made through a combination of clinical and radiological features. In this case, the diagnosis was confirmed by a noncontrast computed tomography (CT) scan of the brain and multiplanar brain magnetic resonance imaging (MRI).
PubMed: 38434787
DOI: 10.1016/j.radcr.2024.01.061 -
Acta Neurochirurgica Feb 2024The purpose of our study was to examine the long-term outcomes of operated Chiari malformation type 1 (CM1) patients and evaluate whether different duraplasty techniques...
PURPOSE
The purpose of our study was to examine the long-term outcomes of operated Chiari malformation type 1 (CM1) patients and evaluate whether different duraplasty techniques affected outcome after surgery in Kuopio University Hospital catchment area.
METHODS
In this retrospective study, a total of 93 patients were diagnosed with CM1 and underwent posterior fossa decompression surgery with or without duraplasty between 2005 and 2020. All patients' medical records were examined for baseline characteristics, surgical details, and long-term follow-up data after operation.
RESULTS
The mean age of CM1 patients was 25.9 years (SD 19.2 years), with female preponderance 69/93 (73.4%). The mean clinical follow-up time was 26.5 months (SD 33.5 months). The most common presenting symptoms were headache, symptoms of extremities, and paresthesia. Posterior fossa decompression with duraplasty was performed in 87 (93.5%) patients and bony decompression in 6 (6.5%) patients. After surgery, preoperative symptoms alleviated in 84.9% (79/93) and the postoperative syringomyelia regression rate was 89.2% (33/37) of all patients. The postoperative complication rate was 34.4% (32/93), with aseptic meningitis being the most common, 25.8% (24/93). Revision surgery was required in 14% (13/93) of patients. No significant correlation between postoperative outcome and extent of dural decompression, or type of duraplasty performed was found.
CONCLUSION
This is the largest reported series of surgically treated CM1 patients in Finland. Posterior fossa decompression is an effective procedure for CM1 symptomology. Duraplasty technique had no significant difference in complication rate or long-term outcomes.
Topics: Humans; Female; Adult; Finland; Retrospective Studies; Arnold-Chiari Malformation; Headache; Hospitals, University
PubMed: 38416251
DOI: 10.1007/s00701-024-05999-y