Bi-level positive airway pressure (BiPAP) ventilation in an infant with central hypoventilation syndrome
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M P Villa1, A Dotta, D Castello
1TV Cattedra di Clinica Pediatrica, University La Sapienza, Rome, Italy.
Insights
Central hypoventilation syndrome in an infant was successfully treated with nocturnal bi-level positive airway pressure (BIPAP). This intervention resolved respiratory events, normalized blood gases, and reversed cardiac and growth abnormalities.
Area of Science:
- Pediatric Pulmonology
- Sleep Medicine
- Critical Care
Background:
- Central hypoventilation syndrome (CHS) is a rare, life-threatening disorder characterized by impaired ventilatory control during sleep.
- Infantile-onset CHS presents with severe respiratory compromise, including apnea, hypopnea, and hypercapnia, often requiring mechanical ventilation.
- Early diagnosis and management are crucial to prevent long-term complications such as cardiovascular abnormalities and developmental delays.
Observation:
- A 4-month-old infant presented with progressive aphonia, dyspnea, dysphagia, cyanosis, apnea, and hypopnea during sleep.
- Polysomnography revealed severe obstructive sleep apnea (apnea-hypopnea index 47.1), hypercapnia (mean end-tidal PCO2 89 mmHg), and significant arterial desaturation (mean SaO2 91%, with 68% of sleep time below 93%).
- The infant exhibited an absent ventilatory response to CO2, absent cardiac responses to apnea during sleep, and developed right ventricular hypertrophy.
Findings:
- Nocturnal nasal bi-level positive airway pressure (BIPAP) initiated at 6 cmH2O and titrated to 16 cmH2O effectively eliminated sleep-related respiratory abnormalities.
- Following BIPAP initiation, end-tidal PCO2 normalized to 39 mmHg, and oxygen saturation (SaO2) improved to 94%.
- After 6 months of nocturnal BIPAP, right ventricular hypertrophy reversed, arrested growth normalized, and hypotonia resolved.
Implications:
- Nocturnal nasal BIPAP is a highly effective treatment for infants diagnosed with central hypoventilation syndrome.
- Early and consistent application of non-invasive ventilation can prevent or reverse significant secondary complications of CHS, including cardiovascular and developmental issues.
- This case highlights the importance of polysomnography in diagnosing CHS and the potential for long-term, successful management with non-invasive ventilatory support.
Abstract:
A 4-month-old baby girl, after a period of apparent good health, began to have aphonia, dyspnea, difficulties with swallowing, cyanosis, apnea, and hypopnea during sleep that resulted in admission to an intensive care unit for intubation and mechanical ventilation. At the age of 9 months she was admitted to our hospital with a possible diagnosis of central hypoventilation syndrome. A polysomnographic study showed apnea and hypopnea (apnea + hypopnea index = 47.1), hypercapnia (mean end-tidal PCO2 89 +/- 15.0 mmHg), and arterial desaturation (mean SaO2 91 +/- 1.7%; lowest SaO2 < 50%; 68% of total sleep time at SaO2 below 93%); the study also showed an absent ventilatory response to CO2, absent cardiac responses to apnea during sleep, and right ventricular hypertrophy. Nocturnal nasal bi-level positive airway pressure (BIPAP), applied initially at 6 cmH2O and gradually increased to 16 cmH2O, caused the sleep-related abnormal respiratory events to disappear. End-tidal PCO2 decreased to 39 mmHg, and SaO2 increased to 94%. After 6 months of nocturnal BiPAP ventricular right hypertrophy reversed and arrested growth and hypotonia normalized. The child has tolerated and has remained on BiPAP support up to her current age of 3 years and continues to use this form of ventilatory assistance without difficulties.