Noninvasive ventilation in a child affected by achondroplasia respiratory
difficulty syndrome.
OTTONELLO G, VILLA G, MOSCATELLI A, DIANA MC, PAVANELLO M.
Paediatr Anaesth 2007;17(1):75-9.
Paediatric Intensive Care Unit, G. Gaslini Children's Hospital, Genoa, Italy.
giancarloottonello@ospedale-gaslini.ge.it
Abstract:
Achondroplasia can result in respiratory difficulty in early infancy, from
anatomical abnormalities such as mid-facial hypoplasia and/or
adenotonsillar hypertrophy, leading to obstructive apnea, or to
pathophysiological changes occurring in nasopharyngeal or glossal muscle
tone, related to neurological abnormalities (foramen magnum and/or
hypoglossal canal problems, hydrocephalus), leading to central apnea. More
often, the two respiratory components (central and obstructive) are both
evident in mixed apnea. Polysomnographic recording should be used during
preoperative and postoperative assessment of achondroplastic children and
in the subsequent follow-up to assess the adequacy of continuing home
respiratory support, including supplemental oxygen, bilevel positive
airway pressure, or assisted ventilation.