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Factor V deficiency and antenatal intraventricular haemorrhage
Archives of Disease in Childhood
|October 1, 1984
Summary
A rare bleeding disorder, factor V deficiency, complicated a pregnancy, leading to hydrocephalus requiring surgical intervention. This case highlights the importance of identifying and managing rare coagulation disorders during pregnancy.
Area of Science:
- Obstetrics and Gynecology
- Neonatal Neurology
- Hematology
Background:
- Congenital factor V deficiency is a rare inherited bleeding disorder.
- Hydrocephalus is a condition characterized by excess cerebrospinal fluid in the brain's ventricles.
Observation:
- Serial obstetric ultrasounds revealed asymmetrical ventricular dilatation between 28 and 32 weeks' gestation.
- Postnatal progressive ventricular dilatation necessitated a ventriculo-atrial shunt in the neonate.
Findings:
- Postoperative bleeding into the cerebral ventricles was consistently associated with extremely low factor V levels (2%).
- This suggests a direct link between factor V deficiency and intraventricular hemorrhage in this case.
Implications:
- Highlights the critical need for antenatal diagnosis and management of rare bleeding disorders.
- Underscores the importance of considering coagulation status in neonatal neurological complications.
- Informs obstetric and pediatric management strategies for pregnancies complicated by factor V deficiency.