From the Guidelines
Central sleep apnea (CSA) in pediatric patients is primarily caused by conditions such as Prader-Willi syndrome (PWS), which increases the risk of CSA, particularly in infants, with a reported prevalence of 43% in this age group 1.
Causes of Central Sleep Apnea
The causes of CSA in pediatric patients can be multifaceted, including:
- Genetic syndromes like Prader-Willi syndrome, which not only increases the risk of CSA but also may involve other sleep-disordered breathing due to factors like scoliosis or spinal abnormalities 1
- Poor ventilatory control, as seen in PWS, contributing to the development of CSA
- Other potential causes, though not directly mentioned in the provided evidence, may include congenital central hypoventilation syndrome, brainstem abnormalities, neuromuscular disorders, prematurity, and certain medications
Diagnosis and Management
Diagnosis of CSA in pediatric patients, especially those with PWS, should involve polysomnography (PSG) at the time of diagnosis and potentially at regular intervals, such as every 6 months, for those with central adrenal insufficiency 1.
Treatment Approaches
Treatment for CSA in pediatric patients can vary based on the underlying cause and age of the patient, with:
- Supplemental oxygen being an effective treatment for infants with CSA
- Positive airway pressure therapy being the standard of care for older individuals 1 It's crucial to address CSA promptly to prevent complications such as developmental delays, cognitive impairment, and cardiovascular issues, emphasizing the need for a multidisciplinary approach in management.
From the Research
Pediatric Causes of Central Sleep Apnea (CSA)
The causes of central sleep apnea (CSA) in children can be attributed to various underlying conditions. Some of the key causes include:
- Neurosurgical pathologies, such as Arnold-Chiari malformation 2, 3, 4
- Genetic diseases, including Prader-Willi syndrome, achondroplasia, and Down syndrome 4
- Isolated fourth ventricle compressing the brainstem 5
- Type I Chiari malformation 2, 3
- Syringomyelia 2
- Thoracolumbar myelomeningocele and secondary hydrocephalus 5
Prevalence and Diagnosis
CSA is thought to occur in about 1-5% of healthy children 6. The diagnosis of CSA in children can be made using polysomnography (PSG) and brain magnetic resonance imaging (MRI) 2, 4. The management of CSA needs to be tailored to the patient and may include medication, non-invasive ventilation, and surgical intervention 6, 4.
Treatment and Outcome
Treatment of underlying conditions, such as posterior fossa decompression surgery, can effectively treat central breathing disorders 2, 3. Individualized management with neurosurgery and/or chemotherapy, continuous positive airway pressure, or noninvasive ventilation can improve CSA and the clinical presentation in most patients 4.