Bart's Hydrops Fetalis — Clinical Presentation and Management — An Analysis of 25 Cases

EDITORIAL COMMENT: It is interesting to compare the maternal hazards of hydrops fetalis due to alpha thalassaemia to those due to erythroblastosis resulting from rhesus isoimmunization, and also other causes of nonimmunological hydrops fetalis. All causes of hydrops may result in dystocia and difficult delivery of the distended fetal abdomen, with postpartum haemorrhage being the other main problem because of a very large placenta. It is noteworthy that none of the 25 patients in this series had severe preeclampsia. This is not the case with other causes of non‐immunological hydrops where the incidence of preeclampsia is about 40%, the figure increasing to 60% in patients with idiopathic hydrops. (I) In Macafee 's series (I) 8 of 13 patients with idiopathic hydrops had preeclampsia, including 3 cases of severe preeclampsia (proteinuria and diastolic pressure above 100 mmHg) and 1 of eclampsia. Most cases of idiopathic hydrops are diagnosed when ultrasonography is performed because of polyhydramnios and the further management of the patient depends upon fetal maturity, the presence of fetal anomalies and whether or not they are correctable, and finally the presence of preeclampsia which may necessitate immediate delivery regardless of fetal condition. In the editor's experience ‘the syndrome’ of preeclampsia seen in some patients with a hydropic erythroblastosis fetus due to rhesus isoimmunization also occurs in cases of idiopathic hydrops fetalis. Figure A shows such a patient who was a 39‐year‐old mother of 4, admitted to hospital, comatose and moribund, at 31 weeks' gestation, with polyhydramnios, gross generalized oedema, oliguria and hypertensive cardiac failure (blood pressure 170/110, urinary protein 6 g/l). The patient made a complete recovery, after temporary intellectual impairment, but required Caesarean section for delivery because of deteriorating condition, when amniotomy failed to bring her into labour. Figure B shows the hydropic infant (birth‐weight 3,160 g) and placenta (1,390 g); the infant died 30 minutes after birth. Summary: Twenty‐nine hydropic infants were born in the Kandang Kerbau Hospital between 1980 and 1985, during which there were 131,658 deliveries, giving an incidence of 1 in 4,540 total births. Twenty‐five of these cases were confirmed to be due to homozygous alpha thalassaemia. No case of fetal hydrops due to Rh isoimmunization was detected. The mean age of the mothers was 28.86 ± 4.05 years (±SD). Eight patients had delivered 1 hydropic baby previously while 1 had a history of 2 babies with hydrops fetalis; 92% of the patients had been followed antenatally while 8% were first seen when they were admitted in labour; 25% of the patients had anaemia, 52% had polyhydramnios, 20% developed hypertension and 64% had bilateral lower limb oedema. None of the patients had concomitant hypertension, generalized oedema and proteinuria. In 4 cases of recurrent hydrops, serial ultrasound scans were performed from early pregnancy but ultrasonic features of hydrops fetalis were only seen from 27 weeks' gestation. Spontaneous labour occurred in 75% of patients at a mean of 32.3 ± 3.3 weeks (± SD). All delivered vaginally and only 1 patient required abdominal decompression. Four patients required Caesarean section, 2 for failure to progress after induction of labour, 1 for major placenta praevia and the fourth for fetal distress; in the last case, diagnosis of hydrops fetalis was only made after delivery of the baby. All the babies in the series died within one hour of delivery. Homozygous alpha thalassaemia is the commonest cause of hydrops fetalis in Singapore and is an invariably fatal condition. It is associated with an increased incidence of maternal anaemia, polyhydramnios and prematurity.