Homozygous alpha-thalassaemia: clinical presentation, diagnosis and management. A review of 46 cases.

Forty-six hydropic infants with homozygous alpha-thalassaemia born during a period of 10 years have been reviewed. The incidence was 1:1550 total births, and accounted for 81% of all non-immune hydrops. The male to female ratio was 1:1.4. There was increased incidence of anaemia, pregnancy induced hypertension, antepartum haemorrhage, malpresentation, prematurity, fetal distress, difficult vaginal delivery, caesarean section, retained placenta, postpartum haemorrhage and congenital abnormalities. Antenatal diagnosis by DNA hybridization with subsequent abortion of the affected fetuses is the best method to decrease maternal morbidity and to reduce the incidence of hydrops fetalis in couples at risk. For those with no previous history, but with early onset hypertension and/or polyhydramnios, sonography is useful in making an earlier diagnosis, and in reducing avoidable morbidity, because DNA analysis can be done before caesarean section and aggressive neonatal management is instituted.