Haemorrhagic problems in obstetrics and gynaecology in patients with congenital coagulopathies.
OBJECTIVE
To review the obstetric and gynaecological problems in women with congenital coagulopathies.


METHODS
Retrospective review.


METHODS
Regional Adult Haemophilia Unit, Glasgow Royal Infirmary.


METHODS
All women in contact with the Unit over a period of 30 years, comprising eight with von Willebrand's disease, 18 obligate carriers of haemophilia A and five obligate carriers of Christmas disease. Each woman was interviewed and details of their obstetric and gynaecological histories were obtained and their case records were reviewed.


METHODS
Haemostatic changes associated with pregnancy and gynaecological problems.


RESULTS
In 14 pregnancies in seven patients with von Willebrand's disease, there were four primary and four secondary post-partum haemorrhages and a large perineal haematoma complicating an episiotomy. These problems arose despite the endogenous rise in factor VIIIc seen with pregnancy. All women seen with von Willebrand's disease complained of menorrhagia and had been referred to gynaecologists. Treatment included danazol, tranexamic acid and the contraceptive pill. Diagnostic curettage resulted in severe haemorrhage in one woman and two women with pelvic pain and dyspareunia were found to have spontaneous broad ligament haematomas, one requiring surgery. In 43 pregnancies in obligate carriers of haemophilia A and Christmas disease there were five post-partum haemorrhages and a large perineal haematoma.


CONCLUSIONS
In von Willebrand's disease it should be noted that adequate laboratory correction of factor VIIIc levels does not ensure clinical haemostasis; hence platelet function should also be measured. Patients with congenital coagulopathies pose particular problems for the obstetrician and gynaecologist and should be managed in close association with the local haemophilia centre.
Greer
I A
IA
University Department of Obstetrics & Gynaecology, Centre for Reproductive Biology, Edinburgh.
Lowe
G D
GD
Walker
J J
JJ
Forbes
C D
CD
D001778:Blood Coagulation Disorders / Q000151:congenital*; D004107:Dilatation and Curettage / Q000009:adverse effects; D005260:Female; D006406:Hematoma / Q000209:etiology; D006467:Hemophilia A / Q000150:complications / Q000235:genetics; D002836:Hemophilia B / Q000150:complications / Q000235:genetics; D006470:Hemorrhage / Q000209:etiology; D006579:Heterozygote; D006801:Humans; D008595:Menorrhagia / Q000209:etiology*; D010502:Perineum; D006473:Postpartum Hemorrhage / Q000209:etiology*; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic; D014842:von Willebrand Diseases / Q000150:complications