Five cases of central congenital hypoventilation, Ondine's curse, were
studied. The diagnostic criteria were: lack of respiratory autonomy d
uring sleep, abnormal CO2 test results and abnormal respiratory monito
ring results during sleep. This hypoventilation induced chronic compli
cations, such as a pulmonary arterial hypertension and psychomotor and
/or growth retardation. The other conditions frequently associated wit
h Ondine's curse were: brainstem disturbances, Hirschsprung disease an
d neuroblastoma. Since other brainstem dysfunctions are sometimes asso
ciated with hypoventilation and because complications can arise, these
children routinely underwent complementary investigations. Treatment
was symptomatic. Assisted mechanical ventilation was initiated as soon
as possible and carefully monitored. The prognosis for these children
has greatly improved and some of them lead a normal life with nightly
assisted ventilation at home.