A 1 day old male presented to local hospital in collapsed state following normal vaginal delivery at 41 weeks gestation. The baby had sudden onset of limpness at home. On arrival at hospital he was making no spontaneous movement had slowly reactive pupils, oxygen saturation was 76% in air. He was intubated and blood was noted on his vocal cords. His haemoglobin was 2.3g/dl and coagulation grossly abnormal with APTT of >100seconds. There was no family history of bleeding diathesis. The baby was transferred to neonatal intensive care unit at this hospital.
On arrival at this hospital the baby was ventilated, had received both FFP and blood transfusion and an additional dose of Vitamin K whilst clotting factor assays were awaited. Abdominal distention was noted and an ultra sound of the abdomen revealed a large collection of blood in the abdominal cavity. Severe vWD (FVIII 8iu/dl and VWF Rapid 5iu/dl post FFP) was diagnosed. Treatment with Wilate 60iu/kg/dose was initiated and continued for 7 days during which time there was no further bleeding.
The baby was ventilated for 72 hours, a central line was inserted for venous access and administration of Wilate. Subsequent factor assays (with out FFP) reveal FVIII <1 iu/dl and vWF <1 iu/dl. The baby was discharged from hospital aged 2 weeks and has had no further major bleeds but has been treated with Wilate for minor bleeding following trauma. He is now 7 months old and is thriving and well. Genetic analysis confirms severe vWD inherited in an autosomal recessive manner.
The FVIII/VWF concentrat, Wilate, proved effective in achieving lasting haemostatsis in the management of this acutely unwell neonate with significant spontaneous intra-abdominal bleeding.
Asterisk with author names denotes non-ASH members.