Arnold-Chiari Malformation: Core Concepts

2021 ◽  
Vol 40 (5) ◽  
pp. 313-320
Author(s):  
Kristina Fons ◽  
Amy J. Jnah
Keyword(s):  
Chiari Malformation ◽  
Foramen Magnum ◽  
Severe Form ◽  
Arnold Chiari Malformation ◽  
Health Care Burden ◽  
Downward Displacement ◽  
Core Concepts ◽  
Improve Patient ◽  
Chiari Malformations

Arnold-Chiari malformation (ACM), a defect that involves downward displacement of the hindbrain and herniation of the cerebellar vermis, tonsils, pons, medulla, and fourth ventricle through the foramen magnum, is the most complex of the 4 types of Chiari malformations. Unique to the other types of Chiari malformations, approximately 95 percent of infants with ACM also present with an associated myelomeningocele (MMC), the most severe form of spina bifida. Among affected infants, those with symptomatic comorbidities incur a significantly higher morbidity and mortality risk. Prompt identification and diagnosis of ACM, as well as evidence-based postnatal and postsurgical nursing and medical care, is critical. Early surgical intervention can repair an existing MMC and restore proper cerebrospinal fluid circulation, which can dramatically improve patient outcomes and quality of life, and reduce disease and health care burden.

Quality of Life in Individuals Affected by Arnold Chiari Malformation: Comparison and Validation of a Measurement Instrument

2018 ◽  
Vol 18 (4) ◽  
pp. 388-396 ◽  
Author(s):  
Antonio Martinez-Sabater ◽  
Maria Luisa Ballestar-Tarin ◽  
Miriam Vazquez-Seoane ◽  
Lucia Mari-Avargues ◽  
Carlos Saus-Ortega ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Chiari Malformation ◽  
Measurement Instrument ◽  
Arnold Chiari Malformation

The Association of Chiari Type I Malformation and Neurofibromatosis Type 1

1993 ◽  
Vol 32 (3) ◽  
pp. 189-190 ◽  
Author(s):  
Joseph Dooley ◽  
Daniel Vaughan ◽  
Michael Riding ◽  
Peter Camfield
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Chiari Malformation ◽  
Neurofibromatosis Type ◽  
Foramen Magnum ◽  
Type I ◽  
Chiari Type ◽  
Chiari Type I Malformation ◽  
Chiari Type I ◽  
Chiari Malformations

The association of neurofibromatosis type 1 (NF1) with Chiari malformations of the cerebellum and brain stem has been reported on only two previous occasions.1,2 The pathogenesis of both conditions has remained unclear, although the Chiari type I malformation is most likely due to hypoplasia of the posterior fossa with subsequent extension of the cerebellum through the foramen magnum.3 NF1 is also associated with a variety of cerebral dysplasias.4 We present a patient with both of these dysplastic lesions whose Chiari malformation was asymptomatic.

Symptomatic Arnold-Chiari malformation: review of experience with 22 cases

1987 ◽  
Vol 66 (6) ◽  
pp. 812-816 ◽  
Author(s):  
William O. Bell ◽  
Edward B. Charney ◽  
Derek A. Bruce ◽  
Leslie N. Sutton ◽  
Luis Schut
Keyword(s):  
Chiari Malformation ◽  
Complete Resolution ◽  
Older Patients ◽  
Surgical Decompression ◽  
Complete Relief ◽  
Content Type ◽  
Arnold Chiari Malformation ◽  
Feeding Difficulty ◽  
Symptoms And Signs ◽  
Chiari Malformations

✓ Twenty-two patients with closed myelomeningoceles, shunted hydrocephalus, and symptomatic Arnold-Chiari malformations were studied retrospectively. Seventeen of the 22 patients were aged 6 months or younger; five patients were aged 3 to 23 years. Patients in the younger group presented with stridor, apnea, and/or feeding difficulty; those in the older group presented with hemiparesis, quadriparesis, oscillopsia, nystagmus, or opisthotonos. Fourteen of the 17 younger patients underwent surgical decompression of the Arnold-Chiari malformation: 10 within 18 days following the onset of symptoms and four on Day 19 or later. Of the 10 infants with early treatment, five eventually died secondary to continued symptoms and five survived. Of those surviving, three were asymptomatic and two had continuing symptoms but were improved over their preoperative state. Among the four patients undergoing surgery later, two died, one had lessening of stridor, and one had complete relief of symptoms. Of the three infants not undergoing decompression, two died and one eventually became asymptomatic. All five of the older patients underwent decompression and all had complete resolution of their preoperative symptoms and signs. The authors conclude that while decompression of a symptomatic Arnold-Chiari malformation may be effective in children and adults, this treatment does not always improve the clinical condition of infants. This may be the result of ischemic/hypoxic effects on the infant's brain stem, which may not be organized normally at birth.

Suboccipital decompression during posterior cranial vault remodeling for selected cases of Chiari malformations associated with craniosynostosis

2013 ◽  
Vol 12 (2) ◽  
pp. 166-170 ◽  
Author(s):  
William W. Scott ◽  
Jeffrey A. Fearon ◽  
Dale M. Swift ◽  
David J. Sacco
Keyword(s):  
Chiari Malformation ◽  
Demographic Data ◽  
Foramen Magnum ◽  
Cranial Vault ◽  
Direct Result ◽  
Suboccipital Decompression ◽  
Combined Technique ◽  
Cranial Vault Remodeling ◽  
Chiari Malformations

Object The optimal management of Chiari malformations in the setting of craniosynostosis is not well established. In this report the authors describe their outcomes with the combined technique of simultaneous suboccipital decompression (SOD) during posterior cranial vault remodeling (PCVR). Methods A retrospective review was performed of all patients undergoing PCVR and simultaneous SOD. Demographic data, diagnosis, imaging studies, operative intervention, and clinical follow-up were evaluated. Results Thirty-four patients were identified as having undergone a simultaneous PCVR/SOD for Chiari malformation associated with craniosynostosis. Eighty-eight percent of these patients had syndromic, multisutural craniosynostosis, and the remaining patients had unilateral lambdoid craniosynostosis. There were no postoperative complications as a direct result from this combined procedure. Two patients required a subsequent direct approach for decompression of the Chiari malformation. The interval between these subsequent surgeries was 3 years and 19 months. Conclusions Chiari malformations are commonly associated with syndromic, complex craniosynostosis and isolated lambdoid craniosynostosis. In appropriately selected patients, a combined posterior cranial vault enlargement and SOD of the foramen magnum is associated with a low complication rate and appears to be an effective procedure.

The quality of life and psychiatric morbidity in patients operated for Arnold–Chiari malformation type I

2013 ◽  
Vol 17 (4) ◽  
pp. 259-263 ◽  
Author(s):  
Bahadir Bakim ◽  
Burcu Goksan Yavuz ◽  
Adem Yilmaz ◽  
Oguz Karamustafalioglu ◽  
Meral Akbiyik ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Chiari Malformation ◽  
Psychiatric Morbidity ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Arnold Chiari Malformation

Major Depressive Disorder Comorbid Severe Hydrocephalus Due to Arnold Chiari Malformation in an Apathetic Patient

2017 ◽  
Vol 41 (S1) ◽  
pp. S627-S627
Author(s):  
A. Kandeger ◽  
H.A. Guler ◽  
O. Guler
Keyword(s):  
Major Depressive Disorder ◽  
Depressive Disorder ◽  
Chiari Malformation ◽  
Brain Magnetic Resonance Imaging ◽  
Foramen Magnum ◽  
Psychiatric Evaluation ◽  
Major Depressive ◽  
Arnold Chiari Malformation ◽  
Severe Hydrocephalus

ObjectiveArnold Chiari Malformation (ACM) is a disorder of embryologic development that is characterized of herniation of the cerebellar structures through the foramen magnum by four types. ACM type 1 (ACM 1) consists in cerebellar tonsil herniation, which is sometimes associated with other abnormalities, including syringohydromyelia, hydrocephalus and skull base alterations. To date, five cases of psychiatric disorders comorbid with ACM-I have been reported. We here present an apathetic patient have delayed diagnosis ACM-I and severe hydrocephalus and comorbid major depressive disorder.CaseA 36-year-old, male patient who is married and two children, was admitted to hospital with don’t want to make anything, despondency, thoughts of have an incompetency, uselessness and want to death, tiredness, weakness complaints which are increased day by day last 2 months. He was diagnosed with major depression after the psychiatric evaluation and hospitalised. He has unwillingness, tiredness and headache complaints which are started when he was 20 years old and he used antidepressant, anxiolytic, and low dose anthipsycotic drugs under psychiatrist control at this years. In radiologic evaluation, Arnold Chiari type 1 and severe hydrocephalus was detected in brain magnetic resonance imaging.DiscussionACM 1 is related to hydrocephalus as a result of posterior fossa hypoplasia and causes spinal injury by obstruction to cerebrospinal fluid (CSF) flow at the foramen magnum. Apathy is a common yet often overlooked symptom in hydrocephalus. This symptom may be a significant obstacle for cognition and quality of life and is associated with increased level of depression.Disclosure of interestThe authors have not supplied their declaration of competing interest.

Abductor vocal fold palsy as a manifestation of type one Arnold Chiari malformation

2000 ◽  
Vol 114 (3) ◽  
pp. 221-223 ◽  
Author(s):  
G. M. Allsopp ◽  
A. Karkanevatos ◽  
R. C. Bickerton
Keyword(s):  
Aspiration Pneumonia ◽  
Chiari Malformation ◽  
Vocal Fold ◽  
Rare Case ◽  
Arnold Chiari Malformation ◽  
Vocal Fold Palsy ◽  
Laryngeal Sensation ◽  
Chiari Malformations

We report a rare case of type 1 Chiari malformation which presented to the Otolaryngology department with abductor vocal fold palsy following an aspiration pneumonia secondary to absent laryngeal sensation. Chiari malformations and related cases are discussed.

scholarly journals Chiari Malformation and Attention Deficit Hyperactivity Disorder

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Anaïs DuBow ◽  
Aurélie Mourot ◽  
Smadar Valérie Tourjman
Keyword(s):  
Attention Deficit Hyperactivity Disorder ◽  
Ct Scan ◽  
Attention Deficit ◽  
Chiari Malformation ◽  
Foramen Magnum ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Hyperactivity Disorder ◽  
Chiari Malformations

Attention deficit hyperactivity disorder (ADHD) is a neurodevelopmental condition characterized by inattention, hyperactivity, and impulsivity. Chiari malformations (CM), first described approximately a hundred years ago, refer to a spectrum of hindbrain malformations characterized by cerebellar herniation through the foramen magnum. We present the case of a 28-year-old woman with ADHD and concurrent Chiari malformation type I (CM-I) that was diagnosed by CT scan. There is growing evidence supporting the role of the cerebellum and its associated structures in the pathophysiology of ADHD. Thus, a cerebellar malformation such as CM may impact neurological circuitry in a manner favoring the development of a neuropsychiatric disorder such as ADHD. Our case highlights the need for further studies pertaining to the role of the cerebellum in the pathophysiology of ADHD and the importance of considering the presence of CM when evaluating a patient with ADHD.

Arnold-Chiari malformation

1983 ◽  
Vol 58 (2) ◽  
pp. 183-187 ◽  
Author(s):  
Kamal S. Paul ◽  
Richard H. Lye ◽  
F. Alexander Strang ◽  
John Dutton
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Significant Proportion ◽  
Foramen Magnum ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Cerebellar Syndrome ◽  
Adult Type ◽  
Arnold Chiari Malformation ◽  
Central Cord Syndrome

✓ The natural history of symptomatic adult Type I Arnold-Chiari malformation (ACM) is variable, and the value of surgery in the management of this disease is difficult to assess. A series of 71 patients in whom a diagnosis of Type I ACM was confirmed at operation is presented, and the progress of the patients following posterior fossa decompression is analyzed. The length of history varied greatly. Pain was the commonest symptom (69% of patients); other symptoms included weakness (56%), numbness (52%), and unsteadiness (40%). The presenting physical signs consisted of a foramen magnum compression syndrome (22%), central cord syndrome (65%), or a cerebellar syndrome (11%). Myelography was performed in 69 patients, and was the most useful investigation. Only 23% of plain radiographs were abnormal. In addition to tonsillar descent, the operative findings included arachnoid adhesions (41%) and syringomyelia (32%). All patients underwent suboccipital craniectomy and C1–3 laminectomy. Respiratory depression was the most frequent postoperative complication (14%), and one patient died from sleep apnea. Early postoperative improvement of both symptoms (82%) and signs (70%) was followed by later relapse in 21% of patients, showing an initial benefit following surgery. None of the patients with a cerebellar syndrome deteriorated, whereas 56% of patients with evidence of foramen magnum compression and 66% of those with a central cord syndrome maintained their initial improvement. The authors conclude that posterior fossa decompression appears to benefit some patients, although a significant proportion might be expected to relapse within 2 to 3 years after operation, depending upon the presenting syndrome.

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