A healthy, non-smoking 34-year-old pregnant woman was monitored with fetal ultrasound scans at 2 weekly intervals due to a previous intrauterine death at 35 weeks of unknown cause.
A fetal ultrasound at 32 weeks showed a normal singleton pregnancy but at 34 weeks showed severe hydrops with normal liquor volume. The mother had no associated systemic symptoms. Due to the history of previous intrauterine death a decision was made to perform an emergency caesarean section.
A severely oedematous baby girl was born at 34+5 weeks weighing 2220 g. She cried at birth but then required a brief period of bag mask ventilation due to poor respiratory effort and bradycardia. Apgar scores were 5 at 1 min, 8 at 5 min and 9 at 10 min. Due to increasing respiratory effort and associated hydrops the baby was intubated at 15 min of age.
She received 2 doses of Survanta within the first 24 h for associated surfactant deficiency but despite this became increasingly difficult to ventilate requiring high pressures (30 mm Hg).
Platelet counts were low from birth (51). On day 5 she developed an extensive ecchymotic rash () associated with a decreasing platelet count (31) and received a single transfusion of platelets. Following this her platelets remained above 150.
On day 7 she was transferred to a tertiary neonatal unit for possible drainage of pleural effusions. Chest ultrasound performed in the tertiary centre revealed only small effusions and no drains were inserted. Following transfer her ventilatory requirements slowly improved and on day 14 she was successfully extubated to continuous positive airway pressure (CPAP) support. She received CPAP for 2 days and low-flow oxygen for a further 7 days.
Following extubation the oedema gradually improved and she was discharged home on day 34 of life.