Further Outpatient Care
Periodic follow-up is necessary and is usually more frequent in younger children with congenital central hypoventilation syndrome (CCHS). Follow-up incorporates multidisciplinary approaches, aiming to determine that all areas receiving care are addressed. In addition, adequacy of ventilatory support must be established based on an overnight sleep study in the laboratory.
The CCHS family network provides community support.
Further Inpatient Care
Because of an absent or negligible respiratory drive, monitor pulse oximetry and end-tidal carbon dioxide in any individual with congenital central hypoventilation syndrome (CCHS), particularly during asleep states, because they may develop profound hypoxemia and hypercarbia whenever they are hospitalized for any reason.
The major complications of congenital central hypoventilation syndrome (CCHS) include death due to hypoxemia during sleep and pulmonary hypertension and cor pulmonale due to recurrent hypoxemia either from delayed diagnosis or inadequate ventilatory support.
There can also be complications associated with procedures (eg, tracheostomy, gastrostomy tube, colostomy).
About 5-10% of patients may develop neural crest–derived tumors (eg, ganglioneuroma, neuroblastoma, ganglioneuroblastoma).
Overall, the prognosis of patients with congenital central hypoventilation syndrome (CCHS) is excellent if the diagnosis is prompt and medical management is appropriate; however, neurocognitive deficits of varying severity, stunted growth, cor pulmonale, and/or seizure disorders are frequent in older patients who may not have benefited from prompt recognition or intervention. Long-term prognosis is variable, but ventilator support is lifelong, as disease does not improve with age.
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