Table 2

Clinical features of CCHS/Hirschsprung patients

1 2 3 4 5
Gestation (weeks)39 3935>4040
Birth weight (kg)3.38 3.442.763.53.085
Length of aganglionosisSigmoid?TCATCA?SigmoidSigmoid
Age at presentation of HSCRDay 2 Day 3Day 5Day 2Day 3
Age ventilated30 minutes 12 hoursBirth3 hoursBirth
Respiratory responsesNo response to  raised CO2 No response to  asphyxia or CO2 110  mm Hg when asleepRaised CO2, no  polygraphy  performedRapid O2 desaturation  when asleepNo response to CO2 50 mm Hg,  and decreased O2, in quiet sleep
AliveNo Yes: on home  ventilationNoYes: on home ventilationYes: on home ventilation
  • CCHS = congenital hypoventilation syndrome; HSCR = Hirschsprung’s disease.