von Willebrand's disease and pregnancy: Management during delivery and outcome of offspring

Chediak, Juan; Alban, G.; Maxey, Betty

doi:10.1016/0002-9378(86)90290-5

Cited by 90 publications

(68 citation statements)

References 12 publications

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“…To prevent postpartum haemorrhage after delivery, women routinely receive oxytocin, which also causes fluid retention. Administration of DDAVP, combined with litres of fluids and oxytocin, may result in lifethreatening hyponatraemia [70]. A single dose of DDAVP immediately prior to epidural catheter placement in labour, however, has not been associated with adverse events [71].…”

Section: Management Of Pregnancy and Childbirthmentioning

confidence: 99%

Women and bleeding disorders

James

2010

Haemophilia

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Summary. While women are rarely affected by haemophilia, they are equally as likely as men to have other bleeding disorders. Menorrhagia, or heavy menstrual bleeding, is the most common symptom that they experience. Not only is menorrhagia more prevalent among women with bleeding disorders, but bleeding disorders are more prevalent among women with menorrhagia. Although menorrhagia is the most common reproductive tract manifestation of a bleeding disorder, it is not the only manifestation. Women with bleeding disorders appear to be at an increased risk of developing haemorrhagic ovarian cysts and possibly endometriosis. Women suspected of having a bleeding disorder or being a carrier of haemophilia should be offered diagnostic testing before getting pregnant to allow for appropriate preconception counselling and pregnancy management. During pregnancy, women with bleeding disorders may be at an increased risk of bleeding complications. At the time of childbirth, women with bleeding disorders appear to be more likely to experience postpartum haemorrhage, particularly delayed or secondary postpartum haemorrhage. As women with bleeding disorders grow older, they may be more likely to manifest gynaecological conditions which present with bleeding. Women with bleeding disorders are more likely to undergo a hysterectomy and are more likely to have the operation at a younger age. While women with bleeding disorders are at risk for the same obstetrical and gynaecological problems that affect all women, women with bleeding disorders are disproportionately affected by conditions that manifest with bleeding. Optimal management involves the combined expertise of haemostasis experts and obstetrician-gynaecologists.

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Section: Management Of Pregnancy and Childbirthmentioning

confidence: 99%

Women and bleeding disorders

James

2010

Haemophilia

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“…They reach their maximum between 29 and 35 weeks [40,43]. Factor IX and XI levels usually do not change significantly during pregnancy [42,43].…”

Section: Pregnancy In Patients With Bleeding Disordersmentioning

confidence: 98%

“…Factor VIII and vWF antigen and activity usually increase significantly during pregnancy in women with type 1 vWD [40][41][42] and in hemophilia A43 carriers. They reach their maximum between 29 and 35 weeks [40,43].…”

Section: Pregnancy In Patients With Bleeding Disordersmentioning

confidence: 99%

Gynaecological and obstetric management of women with inherited bleeding disorders

2006

Intl J Gynecology & Obste

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1. Inherited bleeding disorders should be considered in the differential diagnosis of all patients presenting with menorrhagia (II-2B). The graphical scoring system presented is a validated tool which offers a simple yet practical method that can be used by patients to quantify their blood loss (II-2B). 2. Because underlying bleeding disorders are frequent in women with menorrhagia, physicians should consider performing a hemoglobin/hematocrit, platelet count, ferritin, PT (INR) and APTT in women with menorrhagia. In women who have a personal history of other bleeding or a family history of bleeding, further investigation should be considered, including a vWD workup (factor VIII, vWF antigen, and vWF functional assay) (II-2B). 3. Treatment of menorrhagia in women with inherited bleeding disorders should be individualized (III-B). 4. An inherited bleeding disorder is not a contraindication to hormonal therapy (oral contraceptives [II-1B], depot medroxyprogesterone acetate (DMPA) [II-3B], danazol [II-2B], GnRH analogs [II-3B]) or local treatments (levonorgestrel-releasing IUS [II-1B]) and non-hormonal therapy (antifibrinolytic drug tranexamic acid [II-1B]) as well as desmopressin (II-1B). These therapies represent first line treatment. Blood products should not be used for women with mild bleeding disorders (III-A). 5. In women who no longer want to preserve their fertility, conservative surgical therapy (ablation) and hysterectomy may be options (III-B). Clinicians may consult the "SOGC Clinical Practice Guideline: Guidelines for the Management of Abnormal Uterine Bleeding" for an in-depth discussion of the available therapeutic modalities, both medical and surgical. To minimize the risk of intraoperative and post-operative hemorrhage, coagulation factors should be corrected preoperatively with post-operative monitoring (II-1B). 6. Girls growing up in families with a history of vWD or other inherited bleeding disorders should be tested pre-menarchally to determine whether or not they have inherited the disease to allow both the patient and her family to prepare for her first and subsequent menstrual periods (III-C). 7. In adolescents presenting with menorrhagia, an inherited bleeding disorder should be excluded (III-B). When possible, investigation should be undertaken before oral contraceptive therapy is instituted, as the hormonally induced increase in factor VIII and vWF may mask the diagnosis (II-B). 8. Pregnancy in women with inherited bleeding disorders may require a multidisciplinary approach. A copy of their recommendations should be given to the patient and she should be instructed to present it to the health care provider admitting her to the birthing centre. Women with severe bleeding disorders or with a fetus at risk for a severe bleeding disorder should deliver in a hospital (level three) or where there is access to consultants in obstetrics, anesthesiology, hematology, and pediatrics (III-C). 9. Vacuum extraction, forceps, fetal scalp electrodes, and fetal scalp blood sampling should be avoided if th...

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“…However, during pregnancy, synthesis of FVIII:C and vWF increases markedly and often corrects the deficit observed in type 1 von Willebrand disease. 2,4,5 There are few data in the literature concerning outcome of epidural analgesia during labor in these patients, but it seems that epidural puncture is safe when hematological abnormalities are corrected. [5][6][7][8] Thus, we reviewed the clinical files of pregnant patients with type 1 von Willebrand disease in our institution in order to reassess the time profile of hemostasis abnormalities as well as technical features and any complication of epidural analgesia when performed.…”

Section: Introductionmentioning

confidence: 94%