- •Chiari 1 malformations have variable presentation and demographics, however key patterns exist.
- •The unusually aggressive Chiari I malformation presentation in a 3-month-old is discussed.
- •Emergency surgical intervention due to the acute onset lead to full patient recovery.
- •Knowledge of unique cases may improve recognition and aid diagnosis.
Quadriparesis and cardiovascular instability are not symptoms commonly associated with Chiari I malformation diagnoses, less so in a 3-month-old toddler. Chiari I malformations have a wide array of symptoms, varying with regard to both the nature of onset and the age of the patient presenting. This variation is illustrated in this report, presenting a novel description of a set of symptoms in a particularly young patient, a case lacking the common insidious nature of Chiari I malformation symptoms. Acute onset of cardiovascular instability, global hypertonia, hyperreflexia, and proximal upper limb weakness in a 3-month-old toddler are discussed. Parallels between the presented case and previous case reports are few, however some key features are distinguishable among similar cases. A successful posterior fossa craniectomy allowed the acutely unwell toddler to become a healthy, fully functional child. We believe this case contributes to elucidating the rare presentations of a curious pathology.
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- Disorders of segmentation of the neural tube: agenesis of selective neuromeres.Handb Clin Neurol. 2008; 87: 105-113
- Unusual presentation of Chiari I in toddlers: case reports and review of the literature.Childs Nerv Syst. 2012; 28: 1965-1970
- Chiari malformation Type I in children younger than age 6 years: presentation and surgical outcome.J Neurosurg Pediatr. 2010; 5: 554-561
- The pediatric Chiari I malformation: a review.Childs Nerv Syst. 2007; 23: 1239-1250
- Clinical presentation of Chiari I malformation and syringomyelia in children.Neurosurg Clin N Am. 2015; 26: 509-514
- Institutional experience with 500 cases of surgically treated pediatric Chiari malformation Type I.J Neurosurg Pediatr. 2011; 7: 248-256
- Acute presentation of Chiari I malformation with hemiparesis in a pediatric patient.World Neurosurg. 2016; 85: e1-e4
- Clinical features of Chiari I malformations.Childs Nerv Syst. 2004; 20: 329-331
- Chiari I malformation in the very young child: the spectrum of presentations and experience in 31 children under age 6 years.Pediatrics. 2002; 110: 1212-1219
Published online: August 22, 2018
Accepted: August 12, 2018
Received: January 17, 2018
Crown Copyright © 2018 Published by Elsevier Ltd. All rights reserved.