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Urgent surgical intervention in pediatric patients with Chiari malformation type I. Report of two cases
John C Wellons1, R Shane Tubbs, Cuong J Bui
1Division of Neurosurgery, Section of Pediatric Neurosurgery, Children's Hospital, Birmingham, Alabama 35233, USA.
Insights
Chiari malformation Type I (CM-I) can present acutely in children with rapid neurological decline. Prompt surgical decompression led to significant recovery in two pediatric cases.
Area of Science:
- Neurology
- Pediatric Neurosurgery
Background:
- Chiari malformation Type I (CM-I) typically presents with chronic symptoms.
- Acute presentations of CM-I are rarely documented, especially in pediatric populations.
Observation:
- Two children presented with rapidly progressing neurological deficits, including hypopnea, dysphagia, hemiparesis, gait disturbance, and anisocoria.
- One patient also had a holocord syrinx associated with CM-I.
- Symptoms developed over a period of less than 48 hours to a few days.
Findings:
- Urgent posterior fossa decompression was performed for both patients.
- Both children showed rapid neurological improvement within 24 hours post-surgery and continued to improve subsequently.
Implications:
- Clinicians should consider CM-I in the differential diagnosis for pediatric patients with acute brainstem or long tract signs.
- This case series highlights the potential for acute CM-I presentation in children and the efficacy of surgical intervention.
Abstract:
Patients with Chiari malformation Type I (CM-I) most commonly present with chronic symptoms. A search of the current medical literature revealed scant information regarding acute presentations of CM-I in either pediatric or adult patients. The authors report on two children who presented with rapidly worsening neurological symptoms attributable to a previously undiagnosed CM-I. One patient became profoundly hypopneic with dysphagia and right hemiparesis over a less than 48-hour period. The second patient presented with a few days of worsening right hemiparesis, gait disturbance, and anisocoria. In addition to a CM-I, magnetic resonance imaging in the second patient revealed a holocord syrinx. Following urgent posterior fossa decompression, both patients rapidly improved in the 24 hours immediately following surgery and continued to improve in the subsequent weeks. Few reports detail acute symptoms due to CM-I and those that do exist almost exclusively involve adult patients. Although seemingly rare, the clinician should consider CM-I in the differential diagnosis in pediatric patients presenting with acute brainstem or long tract signs.