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[Chiari type II malformation with brain stem paroxystic dysfunction. What can we do?]
J López-Pisón1, R Cabrerizo de Diago, A Ramírez Gómara
1Sección de Neuropediatría. Unidad de Cuidados Intensivos Pediátricos. Hospital Infantil Universitario Miguel Servet. Zaragoza. España. jlopezp@hmservet.insalud.es
Anales De Pediatria (Barcelona, Spain : 2003)
|April 12, 2003
Summary
Chiari type II malformation causes death in children with myelomeningocele due to respiratory issues. This study highlights the unpredictable and often fatal outcomes, even with intensive care, for affected infants.
Area of Science:
- Pediatric Neurology
- Developmental Biology
- Congenital Malformations
Background:
- Chiari type II malformation is a primary cause of mortality in myelomeningocele patients.
- Respiratory dysfunction is the usual cause of death.
- Effective cures for this condition are currently unavailable.
Observation:
- Episodic brain stem dysfunction is a frequent symptom in children with Chiari type II malformation.
- A case is presented of an infant with Chiari type II malformation and brain stem dysfunction requiring tracheotomy and nasogastric tube feeding.
- The infant experienced episodic symptoms at 8 months and died at 9 months from respiratory dysfunction despite intensive care.
Findings:
- The study reviewed 12 years of experience with five patients diagnosed with Chiari type II malformation and episodic brain stem dysfunction.
- Patients requiring mechanical ventilation experienced fatal outcomes.
- Neither surgical decompression nor intensive care interventions could prevent mortality in severe cases, suggesting an unpredictable and inevitable course.
Implications:
- The prognosis for Chiari type II malformation with brain stem dysfunction appears grim, particularly for those requiring respiratory support.
- Less severe cases, not necessitating mechanical ventilation, may have a more favorable prognosis.
- Further research is needed to understand and potentially alter the fatal trajectory of this condition.