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[Symptomatic Chiari type II malformation]
J F Salomão1, A R Bellas, R D Leibinger
1Departamento de Cirurgia Pediátrica, Instituto Fernandes Figueira-Fundação Oswaldo Cruz (M.S.-Fiocruz), Rio de Janeiro.
Insights
Chiari type II malformation in infants with myelomeningocele presents differently by age. Prompt diagnosis and surgery improve outcomes, but mortality remains high in infants under six months.
Area of Science:
- Neurology
- Pediatrics
- Neurosurgery
Context:
- Chiari type II malformation is a primary cause of mortality in infants with myelomeningocele.
- This study examines 17 cases of symptomatic Chiari type II malformation in two age-dependent groups.
Purpose:
- To analyze the distinct clinical presentations and surgical outcomes of Chiari type II malformation in neonates/infants versus older children.
- To highlight the importance of timely diagnosis and surgical intervention.
Summary:
- Group I (infants <1 year) presented with cranial nerve/brainstem dysfunction (e.g., vocal cord paralysis, apnea).
- Group II (older children) presented insidiously with neck pain and cerebellar signs.
- Surgical treatment (shunt, posterior fossa decompression) showed better outcomes in older children; infants <6 months had a 46.1% mortality rate.
Impact:
- Early surgical intervention is crucial for favorable outcomes in pediatric Chiari type II malformation.
- Understanding age-specific presentations can guide prompt diagnosis and treatment strategies.
- This research underscores the critical need for timely management to reduce mortality in affected infants.
Abstract:
The Chiari type II malformation is the leading cause of death in infants with myelomeningocele. The authors report 17 cases of symptomatic Chiari type II malformation occurring in two distinct age dependent population. In Group I, 13 neonates and infants in the first year of life presented with cranial nerve and brain stem dysfunction characterized by vocal cord paralysis, apnea, dysphagia and laryngeal stridor. In Group II, 4 patients developed signs and symptoms after the first year of life. In this group, the presentation was more insidious and included neck pain and cerebellar manifestations. The surgical treatment consisted initially in shunt implantation or revision and when there was no improvement, posterior fossa decompression was performed. The response to the surgical treatment differed considerably between the two groups: older patients improved promptly after surgery and there was no casualties; in newborn and infants, especially those under 6 months of age, the mortality rate was 46.1%. The authors stress that prompt diagnosis and surgical intervention should be performed in order to produce a favorable outcome.