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. 2014 Mar 1;9(1):105-118.
doi: 10.1016/j.jsmc.2013.10.005.

Central Hypoventilation Syndromes

Christopher Cielo  1 , Carole L Marcus  2
Affiliations

Central Hypoventilation Syndromes

Christopher Cielo et al. Sleep Med Clin. .
No abstract available

Keywords: Autonomic dysfunction; CCHS; Central hypoventilation; Children; Home ventilation; Noninvasive ventilation; PHOX2B; ROHHAD.

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Figures

Fig. 1
Fig. 1
One hundred twenty-second polysomnogram epoch of a mechanically ventilated 20-year-old patient with CCHS, 20/26 genotype. When briefly disconnected from the ventilator, there is a dramatic decrease in airflow and the patient desaturates to 77% and ETCO2 increases by 10 mm Hg. Y-axis parameters: time axis, clock time (in s) with the epoch number superimposed; C3-A2, O1-A2, C4-A1, and O2-A1 are EEG leads; LOC-A2 and ROC-A1 are left and right electrooculograms, respectively. ABDM, abdominal wall motion; CAP, end-tidal Pco2 waveform; CHEST, chest wall motion; CHIN, submental EMG signal; ETCO2, end-tidal Pco2 value; PNEUMOFLOW, airflow measured with a pneumotachograph; PWF, oximeter pulse waveform; SAO2, arterial oxygen saturation.
Fig. 2
Fig. 2
Growth curve of a patient with ROHHAD from birth to 36 months. (From Katz ES, McGrath S, Marcus CL. Late-onset central hypoventilation with hypothalamic dysfunction: a distinct clinical syndrome. Pediatr Pulmonol 2000;29(1):62–8. Copyright John Wiley & Sons; with permission.)
Fig. 3
Fig. 3
Sixteen-month-old boy with CCHS. Many children with central hypoventilation syndromes, including those dependent on positive pressure ventilation, can lead happy and fulfilling lives. Note that this child has a tracheostomy tube with flexible tube extension for greater mobility when ventilated and heat moisture exchanger to provide humidification when not connected to a ventilator. (Courtesy of Brianne Elizabeth.)
See this image and copyright information in PMC

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