Paeds Cases · respiratory-sleep-and-airway
Central sleep apnoea and hypoventilation syndromes — structured clinical encounter
Structured encounter testing the approach to a term neonate who breathes adequately while awake but hypoventilates and desaturates during sleep with a structurally normal heart and clear lungs and cannot wean from support: the recognition of a central control-of-breathing disorder, the ventilate-not-oxygenate principle, PHOX2B confirmation of congenital central hypoventilation syndrome, the ladder of lifelong home ventilation, the multisystem surveillance, and the conversation with the family about a technology-dependent future.
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Station 1 — recognition
The neonatal team asks why this baby cannot wean when his heart and lungs are normal. I explain that a baby who breathes adequately awake but hypoventilates in sleep, with a normal heart and lungs, has a central disorder of the control of breathing, because sleep withdraws the wakefulness drive and exposes a deficient chemoreceptor drive. The constipation since birth raises associated Hirschsprung disease within the autonomic spectrum. [1] [3]
References15ShowHide
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