Related Experiment Video
Updated: Apr 3, 2026
Author Spotlight: Advancing Pediatric Epilepsy Surgery in Children Through Novel Biomarkers and Enhanced Localization
Published on: September 20, 2024
Acute Presentation of Chiari I Malformation with Hemiparesis in a Pediatric Patient
Stephen P Miranda1, Kristopher T Kimmell2, Howard J Silberstein2
1University of Rochester School of Medicine & Dentistry, Rochester, New York, USA.
Insights
Chiari I malformation (CM-I) can present acutely in children with neurological deficits. This case shows a 3-year-old boy with hemiparesis due to CM-I and spinal cord edema, highlighting the need for awareness of acute presentations.
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Medical Imaging
Background:
- Chiari I malformation (CM-I) involves cerebellar tonsil herniation through the foramen magnum.
- CM-I typically causes chronic symptoms like headaches and dizziness.
- Acute neurological deficits in pediatric CM-I are rarely reported.
Observation:
- A 3-year-old boy presented with acute onset of difficulty walking, ataxia, and right-sided hemiparesis.
- MRI revealed cerebellar tonsillar ectopia consistent with CM-I and significant spinal cord edema from C1 to C3.
- The patient had a recent upper respiratory viral infection and a history of coughing fits.
Findings:
- The patient was diagnosed with Chiari I malformation associated with spinal cord edema and syringomyelia.
- Surgical decompression via suboccipital craniectomy and C1 laminectomy was performed.
- Postoperative recovery was uneventful, with complete symptom resolution at 1-month follow-up.
Implications:
- This case demonstrates an atypical acute presentation of CM-I in a pediatric patient.
- Spinal cord edema may be a significant factor in acute neurological deficits associated with CM-I.
- Clinicians should consider CM-I in the differential diagnosis of acute neurological deficits in children, even without typical chronic symptoms.
Background:
Chiari I malformation (CM-I) is defined by cerebellar tonsillar herniation through the foramen magnum. Patients typically present with chronic complaints, including headache, dizziness, and numbness, although there are few reports in the literature of pediatric patients presenting acutely with neurological deficit caused by CM-I. We report a child who presented acutely with hemiparesis and magnetic resonance imaging findings consistent with CM-I and spinal cord edema.
Case Description:
A 3-year old boy with normal development presented with difficulty walking and increased drooling. His mother stated he was running into objects and had balance issues for several days. Neurological examination showed ataxia with falling to the right after a few steps and weakness of the right arm and leg. His medical history was remarkable only for mild asthma, although he had recently been treated for an upper respiratory viral infection. Computed tomography of the head demonstrated no brainstem mass. Magnetic resonance imaging of the head and cervical spine showed tonsillar ectopia approximately 2 cm below the craniocervical junction with increased T2 signal in the spinal cord from C1 to C3 consistent with syringomyelia and cord edema. The patient underwent suboccipital craniectomy with removal of the posterior arch of C1 and dural patch graft. His postoperative course was unremarkable, with complete resolution of his symptoms at his 1-month follow-up visit.
Conclusions:
This case highlights an unusual presentation of CM-I with neurological deficit related to spinal cord edema, possibly precipitated by the "water-hammer" effect of this patient's coughing fits. Providers should be aware of the acute presentations of CM-I.
