Severe von Willebrand's Disease During Labor and Delivery

Lipton, Richard A.; Ayromlooi, Jahangir; Coller, Barry S.

doi:10.1001/jama.1982.03330110051027

Cited by 19 publications

(13 citation statements)

References 11 publications

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“…Because of the great variability of haemostatic response of von Willebrand's disease to pregnancy between individuals and even in different pregnancies in the same individual (as seen in one of our patients) regular monitoring of coagulation factors is required. Von Willebrand factor activity has been considered 17 to be the best predictor for risk of bleeding in pregnant women with von Willebrand's disease and bleeding may still occur in women with normal factor VIII procoagulant activity. Therefore, it is essential to monitor both von Willebrand factor antigen and activity levels, together with factor VIII procoagulant activity during pregnancy.…”

Section: Discussionmentioning

confidence: 99%

Pregnancy in women with von Willebrand's disease or factor XI deficiency

Kadir¹,

Lee

Sabin

et al. 1998

BJOG

271

360

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Objective To assess the obstetric outcome in women with von Willebrand's disease or factor XI Setting Haemophilia Centre and Haemostasis Unit, The Royal Free Hospital.Population Women with von Willebrand's disease (n = 3 1) and with factor XI deficiency (n = 11) registered at the Royal Free Hospital Haemophilia Centre who had had a pregnancy within the previous 17 years (1980)(1981)(1982)(1983)(1984)(1985)(1986)(1987)(1988)(1989)(1990)(1991)(1992)(1993)(1994)(1995)(1996), including 84 in women with von Willebrand's disease and 28 in women with factor XI deficiency.Methods Women were interviewed and details of the obstetric history were obtained. The records of the Haemophilia Centre and the women's maternity records were also reviewed.Results Threatened miscarriage occurred in 33% and 14% of pregnancies with von Willebrand's disease and factor XI deficiency, respectively. Excluding recwent miscarriages, 14/68 (21 %) of pregnancies with von Willebrand's disease and one pregnancy with factor XI deficiency miscarried spontaneously. There was an increased incidence of primary and secondary post-abortal bleeding complications. Factor VIJI and von Willebrand factor antigen and activity levels increased significantly in pregnancy in all women apart from those with severe von Willebrand's disease. Factor XI, however, did not show any significant change. No neonatal haemorrhagic complications in association with the birth process were reported, although ventouse and difficult forceps deliveries were avoided. Extensive perineal bruising and haematoma was reported in three women with von Willebrand's disease; two of these were associated with forceps delivery. The incidence of primary postpartum haemorrhage was 185% in von Willebrand's disease and 16% in factor XI deficiency. Blood transfusion was required in six cases of von Willebrand's disease and two cases of factor XI deficiency. Ten of fourteen instances of primary postpartum haemorrhage occurred when maternal factor levels were < 50 IU/dL with no prophylactic treatment for labour. The incidence of secondary postpartum haemorrhage was 20% in von Willebrand's disease and 24% in factor XI deficiency. None of the women who had prophylactic treatment during labour or the puerperium suffered any significant bleeding complications. There were three neonatal bleeding complications.Conclusion Pregnancy, labour and the puerperium are associated with significant bleeding problems in women with von Willebrand's disease or factor XI deficiency, but these are largely preventable. SDecialist obstetric care in close liaison with the haemophilia centre is essential to minimise maternal deficiency.and neonatal complications.

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Section: Discussionmentioning

confidence: 99%

Pregnancy in women with von Willebrand's disease or factor XI deficiency

Kadir¹,

Lee

Sabin

et al. 1998

BJOG

271

360

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“…Other pregnancy‐associated bleeding reported in women with VWD includes extensive bruising and hematomas at intramuscular injection, episiotomy and surgical wound sites, which may require drainage in rare cases. Delayed acute bleeding from episiotomy wounds, severe enough to require transfusion, may also occur [13,18,21,38]. Most of these complications occurred in the absence of, or after discontinuation of, prophylactic therapy.…”

Section: Other Bleeding Complicationsmentioning

confidence: 99%

von Willebrand disease and pregnancy

Kujovich

2005

Journal of Thrombosis and Haemostasis

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“…Only a few reports exist about the management of pregnancy and delivery in women with VWD type 3. Bleeding problems predominantly occur during delivery and in the postpartum period [3, 14–17]. For delivery, prophylactic substitution with plasma products is recommended and under these circumstances vaginal delivery is considered safe [3, 14, 17].…”

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confidence: 99%