Natural history of Chiari I malformation in children: a retrospective analysis

The Chiari I malformation (CM-I) is characterised by overcrowding of the posterior fossa and descent of the cerebellar tonsils and is associated with syringomyelia. With the increasing availability of magnetic resonance imaging, CM-I is placing a growing burden on neurosurgical services. However, its natural history remains poorly understood, and the timing and nature of surgical intervention is controversial. We present a case of a significant, symptomatic CM-I with associated syrinx which underwent complete spontaneous resolution over a 4-year period. Spontaneous regression of Chiari malformation and syringomyelia is exceedingly rare; a literature review reveals 15 other cases and only one case which underwent complete resolution. The present case and literature review suggest a more benign natural history of CM-I and support a more conservative approach to its management. Further studies are required to determine whether any factors can predict resolution for certain patient cohorts.

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Subpial Aspirated Cerebellar Tonsils in Pediatric Chiari I Malformation: anatomopathological study

JBNC - JORNAL BRASILEIRO DE NEUROCIRURGIA ◽

10.22290/jbnc.v29i3.1781 ◽

2019 ◽

Vol 29 (3) ◽

pp. 501-505

Author(s):

Bruna Lisboa do Vale ◽

Maurus Marques de Almeida Holanda ◽

Diogo Berto Campos ◽

Moisés Dantas Cartaxo de Abreu Pereira ◽

Érico Samuel Gomes Galvão da Trindade

Keyword(s):

Natural History ◽

Purkinje Cells ◽

Chiari I Malformation ◽

Surgical Removal ◽

Type I ◽

Negative Consequences ◽

Loss Of Function ◽

History Of ◽

Chiari I ◽

Cerebellar Tonsils

Introduction: Chiari Malformation (CM) is a condition in which ectopy or herniation of components of the posterior cranial fossa to theforamen magnum are present, and can be divided into four types. Type I (CM-I) occurs when there is protrusion of only the cerebellartonsils into the cervical spinal canal. One of the available therapeutic options is subpial aspiration. Previous anatomopathologicalstudies showed that the main alterations in the aspirated tonsils were the loss of Purkinje cells, Bergmann gliosis, cortical atrophyand neuronal changes due to hypoxia; all of them secondary to the aggression to the nervous tissue over time. These changes leadto gradual loss of function of the tissue, with compensation by other structures. Therefore, when subpial aspiration of the herniatedtonsil is performed in adults, there are no negative consequences to the patient in the future, as concluded by many studies targetingadults with CM. However, studies in children with CM are scarce, which means that we cannot affirm in which age the histologicalchanges occur. Hence, we cannot determine whether or not the surgical removal of this tissue in childhood leads to future losses tothe patient. Objective: To analyze the main histological aspects of cerebellar tonsils in children with Chiari I Malformation whounderwent subpial aspiration, to help clarify the natural history of this pathology, as well as to help determine the prognosis of thispopulation when treated with this technique. Method: We analyzed the histopathology of the tonsils removed with subpial aspirationtechnique, in several hospitals of João Pessoa, Brazil, from children diagnosed with Chiari I malformation. Results: The slides of alltonsils showed the same histological changes: Bergmann gliosis, loss of Purkinje cells, atrophy of the cerebellar cortex and meningealfibrosis. The obtained results were similar to the ones found in previous studies carried out in adults. Conclusion: The cerebellaranatomopathological alterations secondary to Chiari I Malformation, which leads to loss of tissue function, seem to appear early inlife, suggesting the safety of the subpial aspiration in pediatric patients. However, more extensive studies and long-term follow-up ofpatients are needed to establish with more precision the natural history of the disease.

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Faculty Opinions recommendation of Natural history of imatinib-naive GISTs: a retrospective analysis of 929 cases with long-term follow-up and development of a survival nomogram based on mitotic index and size as continuous variables.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.13356067.14726207 ◽

2011 ◽

Author(s):

Richard Riedel

Keyword(s):

Natural History ◽

Retrospective Analysis ◽

Mitotic Index ◽

Continuous Variables ◽

Long Term Follow Up ◽

History Of

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Retrospective Analysis of the Natural History of a Case of Biliary Cystadenocarcinoma

The Japanese Journal of Gastroenterological Surgery ◽

10.5833/jjgs.44.541 ◽

2011 ◽

Vol 44 (5) ◽

pp. 541-548 ◽

Cited By ~ 1

Author(s):

Takanori Kyokane ◽

Hiroaki Shibahara ◽

Junichi Takamizawa ◽

Hayato Nakamura ◽

Shingo Kuze ◽

...

Keyword(s):

Natural History ◽

Retrospective Analysis ◽

Biliary Cystadenocarcinoma ◽

History Of

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Sa1160 – The Natural History of Low Grade Dysplasia in Patients with Barrett's Esophagus: 12 Year Retrospective Analysis from a Tertiary Centre

Gastroenterology ◽

10.1016/s0016-5085(19)37542-0 ◽

2019 ◽

Vol 156 (6) ◽

pp. S-289-S-290

Author(s):

Mohamed Hussein ◽

Vinay Sehgal ◽

Cormac Magee ◽

Sarmed S. Sami ◽

Matthew Banks ◽

...

Keyword(s):

Natural History ◽

Retrospective Analysis ◽

Barrett’S Esophagus ◽

Barrett's Esophagus ◽

Low Grade ◽

Tertiary Centre ◽

History Of ◽

Low Grade Dysplasia

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A retrospective analysis on the natural history of incidental small paraclinoid unruptured aneurysm

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2013-305019 ◽

2013 ◽

Vol 85 (3) ◽

pp. 289-294 ◽

Cited By ~ 27

Author(s):

J. S. Jeon ◽

J. H. Ahn ◽

W. Huh ◽

Y.-J. Son ◽

J. S. Bang ◽

...

Keyword(s):

Natural History ◽

Retrospective Analysis ◽

Unruptured Aneurysm ◽

History Of

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Natural history of Rathke's cleft cysts: A retrospective analysis of a two centres experience

Clinical Endocrinology ◽

10.1111/cen.13744 ◽

2018 ◽

Vol 89 (2) ◽

pp. 178-186 ◽

Cited By ~ 6

Author(s):

Elisa Sala ◽

Justin M. Moore ◽

Alvaro Amorin ◽

Giulia Carosi ◽

Hector Martinez ◽

...

Keyword(s):

Natural History ◽

Retrospective Analysis ◽

History Of ◽

Rathke's Cleft Cysts ◽

Rathke's Cleft

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CONVERSION TO SYMPTOMATIC CHIARI I MALFORMATION AFTER MINOR HEAD OR NECK TRAUMA

Neurosurgery ◽

10.1227/01.neu.0000325498.04975.c0 ◽

2008 ◽

Vol 63 (4) ◽

pp. 748-753 ◽

Cited By ~ 41

Author(s):

Michael J. Wan ◽

Hiroshi Nomura ◽

Charles H. Tator

Keyword(s):

Health Care Professionals ◽

Foramen Magnum ◽

Chiari I Malformation ◽

Senior Author ◽

Minor Trauma ◽

Inclusion Criteria ◽

Neck Trauma ◽

Asymptomatic Patients ◽

History Of ◽

Chiari I

ABSTRACT OBJECTIVE The Chiari I malformation is a rare disorder characterized by downward herniation of the brainstem and cerebellar tonsils through the foramen magnum. Many individuals with the Chiari I malformation do not become symptomatic until adulthood, and the factors that contribute to the onset of symptoms have not been well characterized. The purpose of this systematic chart review was to determine the incidence and validity of minor head or neck trauma as precipitating factors for the onset of symptomatic Chiari I malformation. METHODS The charts of all patients seen by the senior author (CHT) between January 1985 and July 2006 were reviewed to identify patients who had presented with symptomatic Chiari I malformation after minor head or neck trauma. Specific inclusion criteria were used to determine whether the onset of symptoms could be reliably attributed to the minor trauma. RESULTS Of the 85 patients with symptomatic Chiari I malformation seen by the senior author during this time, 11 (12.9%) had a history of minor head or neck trauma preceding the onset of symptoms. Of these, there were 3 patients (3.5%) in whom the onset of symptoms could be attributed to the trauma based on strict inclusion criteria. CONCLUSION Minor head or neck trauma can precipitate the onset of symptoms in a small number of previously asymptomatic patients with Chiari I malformation. Health care professionals must be aware that neurological symptoms that persist or worsen after minor head or neck trauma could indicate an underlying Chiari I malformation.

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Natural history of generalized motor seizures: A retrospective analysis

Seizure ◽

10.1016/j.seizure.2020.05.019 ◽

2020 ◽

Vol 80 ◽

pp. 109-112 ◽

Cited By ~ 1

Author(s):

Neishay Ayub ◽

Sharon Chiang ◽

Robert Moss ◽

Daniel Goldenholz

Keyword(s):

Natural History ◽

Retrospective Analysis ◽

History Of

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The Chiari I malformation

Journal of Neurosurgery Pediatrics ◽

10.3171/2019.5.peds18382 ◽

2019 ◽

Vol 24 (3) ◽

pp. 217-226 ◽

Cited By ~ 3

Author(s):

Samuel G. McClugage ◽

W. Jerry Oakes

Keyword(s):

Surgical Intervention ◽

Chiari I Malformation ◽

Proper Patient Selection ◽

Time Period ◽

Treatment Paradigm ◽

History Of ◽

Chiari I ◽

Degenerative Disorder ◽

Short Time ◽

Chiari Malformations

As with many pathologies, the course of our understanding of the Chiari I malformation (CIM) has developed extensively over time. The early descriptions of the Chiari malformations by Hans Chiari in 1891 opened the door for future classification and research on this topic. However, even over a long timeframe, our understanding of the pathophysiology and, more importantly, treatment, remained in its infancy. As recently as the 1970s, CIM was not discussed in popular neurology textbooks. Syringomyelia is listed as a degenerative disorder with no satisfactory treatment. Radiation therapy was considered an option in treatment, and surgery was thought to play no role. During the last 40 years, equivalent to the duration of a neurosurgical career, our understanding of the pathophysiology and natural history of CIM, coupled with modern MRI, has improved the treatment paradigm for this patient population. More importantly, it has given us evidence confirming that CIM is a disorder responsive to surgical intervention, giving patients once thought to be destined for lifelong disability a comparatively normal life after treatment. The purpose of this article is to offer a review of CIM and its important associated entities. The authors will discuss the evolution in understanding of the Chiari malformation and, importantly, distinguish between symptomatic CIM and asymptomatic tonsillar ectopia, based on imaging and presenting symptomatology. They will discuss techniques for surgical intervention, expected outcomes, and complications after surgery. Proper patient selection for surgery based on appropriate symptomatology is tantamount to achieving good surgical outcomes in this population, separating those who can be helped by surgery from those who are unlikely to improve. While our knowledge of the Chiari malformations continues to improve through the efforts of clinical and basic science researchers, surgeons, and patients, our current understanding of these entities represents a monumental improvement in patient care over a relatively short time period.

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