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Acute presentation of Chiari 1 malformation in children
Giuseppe Talamonti1, Eleonora Marcati2, Giulia Gribaudi2
1Department of Neurosurgery, ASST Niguarda, P.za Ospedale Maggiore 3, 20162, Milan, Italy. tala_nch@yahoo.it.
Insights
Acute Chiari 1 malformation in children is rare, often presenting with unusual symptoms. Prompt diagnosis and treatment are crucial, even if severe outcomes are infrequent.
Area of Science:
- Neurology
- Pediatric Neurosurgery
- Medical Literature Review
Background:
- Chiari 1 malformation is increasingly detected due to advanced imaging.
- Understanding acute presentations in children is critical for timely intervention.
Purpose of the Study:
- To review literature on acute Chiari 1 malformation in children.
- Focus on acute cervical cord syndromes and cortico-spinal tract impairment.
- Analyze precipitating factors and pathophysiology of acute onset.
Main Methods:
- Literature review.
- Illustrative case presentation.
Main Results:
- Few documented cases of acute Chiari 1 malformation in pediatric populations.
- Common presentations include oropharyngeal dysfunction, cranial nerve deficits, increased intracranial pressure, and motor deficits.
- Acute cervical long tract impairment is rare, with only 16 prior cases of quadriparesis or hemiparesis reported.
Conclusions:
- Severe manifestations can occur in previously asymptomatic patients.
- The rarity of acute deterioration does not support routine prophylactic surgery.
- Prompt diagnosis is vital for potentially life-altering cranio-cervical decompression, despite unusual presentations.
Purpose:
To review the literature about the acute presentation of Chiari 1 malformation in children, with a focus on acute cervical cord syndromes with impairment of the cortico-spinal tract. To analyze the possible precipitating factors and the pathophysiology of the acute onset.
Method:
Illustrative case presentation with literature review.
Results:
The literature includes just a few dozens of children with acute presentation of Chiari 1 malformation. The more frequent presentations consist of oropharyngeal dysfunction, cranial nerve impairment, high intracranial pressure, peripheral motor deficit. Acute impairment of cervical long tracts is very rare and we could find only 16 previously reported cases of cervical cord impairment with quadriparesis or hemiparesis.
Conclusions:
Nowadays, a lot of asymptomatic Chiari 1 malformations are frequently observed owing to the wide diffusion of magnetic resonance imaging. This raised the question about the management of these patients. Despite severe and even lethal manifestations being reported in previously asymptomatic patients, the absolute rarity of acute deterioration does not justify prophylactic surgery. The diagnosis of Chiari malformation may be initially difficult in patients with isolated, acute, and unusual presentations but physicians should bear in mind its possibility, because prompt cranio-cervical decompression may be decisive.
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