Clinical diagnosis—part II: what is attributed to Chiari I

Novegno, Federica

doi:10.1007/s00381-019-04192-2

Cited by 16 publications

(7 citation statements)

References 82 publications

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“…Firstly, it is important for the differential diagnosis between CM1 headache and migraine or headaches resulting from other conditions. Inadequate and inaccurate assessment of headache will result in a wrong indication for surgery [ 37 , 38 ]. Several studies have attempted to address this topic and proposed criteria to correctly diagnose the Chiari headache (cough headache, Valsalva headache, occipital or suboccipital, lasting a few minutes) [ 39 , 40 ].…”

Section: Resultsmentioning

confidence: 99%

Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document

et al. 2021

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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.

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Section: Resultsmentioning

confidence: 99%

Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document

et al. 2021

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“…Thus, the results of the study show that Arnold-Chiari malformation Type I can affect the cochlear and vestibular nuclei, leading to abnormal audio-vestibular results [14,15]. A detailed audio-vestibular evaluation can identify the possible site of the lesion and the pathophysiology of the audio-vestibular symptoms.…”

Section: Discussionmentioning

confidence: 84%

Audio-Vestibular Findings in an Adult With Arnold–chiari Malformation

Prabhu¹,

Anish²,

Vijayan³

et al. 2020

J Hear Sci

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Background: Arnold-Chiari malformation is defined as a congenital neuroanatomic deformity of the craniocervical junction, which is characterized by herniation of contents from the posterior cranial fossa through the foramen magnum into the cervical spinal canal. There is limited literature on audiological and vestibular evaluation on individuals with Arnold-Chiari malformation. Thus, the present study reports the results of a detailed audio-vestibular evaluation in an adult female diagnosed with Arnold-Chiari malformation.Case report: A detailed audio-vestibular evaluation was carried out in an adult female diagnosed with Arnold-Chiari malformation. Pure tone audiometry, immittance evaluation, otoacoustic emissions, auditory brainstem response (ABR), cervical vestibular evoked myogenic potential (cVEMP), ocular vestibular evoked myogenic potential (oVEMP), and video head impulse test (vHIT) were recorded, and the results were analyzed. The results of the study showed an abnormal prolongation of inter-peak latencies in the ABR, which was seen in both ears. Also, cVEMP and oVEMP was absent in both ears. The results suggest possible compression of the cerebellar tonsils on the cochlear and vestibular nuclei. Conclusions:The study suggests that individuals with Arnold-Chiari malformation should undergo a detailed audio-vestibular evaluation to understand the pathophysiology of the disorder, which can guide further management.

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“…The incidence of CM1 is estimated as <1%, but an increasing number of ectopic cerebellar tonsils are now discovered because of the diffusion of MRI. 2 , 4 , 5 There is general agreement that surgery should be reserved for symptomatic patients, 4 , 6 but the correct assessment of preoperative symptoms may not be simple. For instance, headache is very frequent in patients with CM1, but the differential diagnosis of headache may be quite difficult.…”

Section: Discussionmentioning

confidence: 99%

Chiari malformation type 1: are we doing less with more? Illustrative case

Talamonti¹,

Ferrari²,

D’Aliberti³

2021

Journal of Neurosurgery: Case Lessons

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BACKGROUNDClassic treatment of Chiari malformation type 1 consists of foramen magnum decompression. Selected patients may require occipitocervical fixation, transoral odontoidectomy, tonsillectomy, and so forth. Treatment standardization does not yet exist, and some patients risk being overtreated.OBSERVATIONSA 20-year-old man with headache and Chiari malformation type 1 underwent extradural bone decompression. One year later, he was managed with the extradural section of his filum terminale. Eighteen months later, the patient underwent monitoring of intracranial pressure, occipitocervical stabilization, transoral odontoidectomy, minimally invasive subpial tonsillectomy, and occipital cranioplasty. His headache never changed, and he progressively developed hemiparesis and swallowing and respiratory disturbances. Two years later, a new magnetic resonance imaging scan showed extended syringomyelia with scarce peritonsillar subarachnoid space. The umpteenth operation consisted of the removal of a constricting epidural scar, arachnoid dissection, total tonsillectomy, creation of a wide subarachnoid space, and dural sac augmentation. The patient’s initial postoperative course was smooth, and his headache improved. However, 8 days after surgery, the patient acutely presented with vegetative disturbances and died because of malignant brainstem edema of unknown origin.LESSONSThe story of this patient is not so uncommon. He underwent all the possible surgical treatments rather than a timely adequate osteodural decompression. Probably, he received less with more.

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Clinical diagnosis—part II: what is attributed to Chiari I

Cited by 16 publications

References 82 publications

Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document

Diagnosis and treatment of Chiari malformation type 1 in children: the International Consensus Document

Audio-Vestibular Findings in an Adult With Arnold–chiari Malformation

Chiari malformation type 1: are we doing less with more? Illustrative case

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