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Apnea in patients with myelomeningocele
D D Cochrane1, R Adderley, C P White
1Department of Pediatric Neurosurgery, British Columbia's Children's Hospital, Vancouver, Canada.
Insights
Infants with Chiari II malformation can experience obstructive apnea, which may improve with treatment. However, central expiratory apnea with cyanosis (PEAC) is a severe complication that can be fatal.
Area of Science:
- Pediatric Neurology
- Neuroscience
- Respiratory Medicine
Background:
- Chiari II malformation is associated with significant complications in infants.
- Apnea is a critical complication, manifesting in obstructive and central forms.
- Brainstem dysfunction is implicated in the pathogenesis of these apneic events.
Purpose of the Study:
- To characterize the spectrum of apnea in infants with Chiari II malformation.
- To investigate the relationship between brainstem dysfunction and apnea subtypes.
- To evaluate the response of different apnea types to medical and surgical interventions.
Main Methods:
- Observation of 9 infants with Chiari II malformation and apnea.
- Clinical assessment of obstructive apnea causes (e.g., abductor palsy, laryngomalacia).
- Evaluation of central apnea, specifically cyanotic expiratory apnea of central origin (PEAC).
Main Results:
- Obstructive apnea may be linked to hydrocephalus and cervical compression, with potential for reversal.
- Cyanotic expiratory apnea of central origin (PEAC) is a progressive, treatment-refractory condition.
- Five out of six patients with PEAC experienced sudden death, some despite resuscitation.
Conclusions:
- Apnea in Chiari II malformation may represent a continuum of brainstem dysfunction.
- Optimal hydrocephalus management and/or cervical decompression can improve obstructive apnea.
- PEAC is a severe, life-threatening complication necessitating careful monitoring and reporting of treatment outcomes for both apnea types.
Abstract:
Apnea is a known complication of the Chiari II malformation presenting in infancy. Obstructive apnea secondary to bilateral abductor palsy or laryngomalacia and centrally mediated expiratory apnea with cyanosis can occur. Observations of 9 patients suggest that these forms of apnea may represent stages in a continuum of brain stem dysfunction due to the combined effects of the hindbrain malformation and its compression, hydrocephalus and progressive arachnoiditis. Obstructive apnea in some patients may be reversed by optimal control of hydrocephalus with or without cervical decompression. These patients may also develop episodes of cyanotic expiratory apnea of central origin (PEAC). This form of apnea does not respond to surgical or medical treatment and may show progressive worsening over time. Five of 6 patients with this form of apneic spell died suddenly, 2 of these died despite full recuscitative efforts. It is recommended that reports of treatment address results for both forms of apnea.