Management of Inherited Bleeding Disorders in Pregnancy
Scope and Conditions Covered
Women with inherited bleeding disorders are at increased risk of bleeding at miscarriage, termination, invasive antenatal procedures, and delivery, and require individualised, multidisciplinary care. The guideline covers:
- Von Willebrand disease (VWD) — the most common inherited bleeding disorder in women
- Haemophilia A and B carriers — X-linked; carriers may have clinically low factor levels
- Factor XI deficiency
- Rare clotting factor deficiencies (e.g. factor VII, X, XIII)
- Fibrinogen disorders
- Platelet function disorders — Bernard–Soulier syndrome, Glanzmann's thrombasthenia, and others
Antenatal Planning
- Care should be led by a multidisciplinary team — obstetrician, haematologist, and, where relevant, obstetric anaesthetist and neonatologist — ideally with joint antenatal review early in pregnancy
- A written peripartum management/birth plan should be agreed and documented in the notes before labour, covering anticipated factor levels, delivery unit, mode of delivery, anaesthetic options, and neonatal plan
- Factor VIII and von Willebrand factor levels rise physiologically through pregnancy (often normalising by the third trimester in VWD type 1), but factor IX levels and platelet function disorders do not show the same compensatory rise — levels should be checked in the third trimester (around 34–36 weeks) to guide delivery planning
- Where the fetus/carrier status is unknown or the fetus may be affected (e.g. possible haemophilia), delivery should be planned as if the fetus is affected until proven otherwise
- Delivery should take place in a unit with on-site haematology support, blood bank access, and the ability to administer clotting factor concentrate or desmopressin (DDAVP) promptly
- Invasive prenatal diagnostic testing (CVS, amniocentesis) in a fetus at risk of a bleeding disorder carries additional haemorrhage/haematoma risk and should be discussed with haematology
Delivery and Anaesthetic Considerations
- Mode of delivery: vaginal delivery is not contraindicated by a maternal bleeding disorder alone, but the mode should be individualised jointly with haematology
- Instrumental delivery: ventouse extraction and mid-cavity (rotational) forceps are contraindicated where the fetus has known or suspected haemophilia (or another significant bleeding disorder), because of the recognised risk of neonatal intracranial and extracranial haemorrhage; low-cavity/outlet forceps may be considered if clinically necessary
- Invasive fetal monitoring: fetal scalp electrodes and fetal blood sampling should be avoided in fetuses expected to have moderate or severe haemophilia; in fetuses at risk of only mild haemophilia, judicious use may be considered to facilitate vaginal birth and avoid unnecessary caesarean section
- Regional anaesthesia (epidural/spinal): coagulation factor levels should be checked before siting a neuraxial block. A widely applied minimum threshold — consistent with general UK obstetric anaesthesia guidance for coagulation abnormalities — is a factor level (e.g. FVIII, VWF activity, FIX) of ≥50 IU/dL (0.5 IU/mL) before regional anaesthesia is considered safe; levels below this favour general anaesthesia or alternative analgesia. Exact thresholds should be individualised with haematology and anaesthetic input rather than applied rigidly
- Third stage of labour: active management is recommended given the elevated postpartum haemorrhage (PPH) risk; PPH risk is highest in the immediate and delayed postpartum period (up to several weeks) as clotting factor levels fall back to baseline after delivery
- Intramuscular injections and non-steroidal anti-inflammatory drugs should generally be avoided in women with bleeding disorders around the time of delivery
Neonatal Management
- A cord blood sample should be taken at delivery for a coagulation screen and factor VIII/IX assay where relevant, avoiding maternal blood contamination; if the result is uncertain, a venous sample should be taken from the baby
- Intramuscular vitamin K should be withheld in babies at risk of a bleeding disorder until coagulation results are known; if there is likely to be significant delay, oral vitamin K should be given instead
- Cranial ultrasound should be considered before discharge in all neonates with confirmed severe or moderate haemophilia, to screen for intracranial haemorrhage
- Female carrier fetuses can, rarely, have clinically low factor levels through extreme lyonisation and should not automatically be assumed low-risk
High-Yield Exam Points
- GTG 71 is a joint RCOG/UKHCDO guideline (first edition, 2017) — know this covers VWD, haemophilia A/B carriers, factor XI deficiency, rare factor deficiencies, fibrinogen disorders, and platelet function disorders (Bernard–Soulier, Glanzmann's)
- Factor VIII and VWF rise in pregnancy; factor IX and platelet function disorders do not — check levels in the third trimester
- Ventouse and mid-cavity forceps are contraindicated in fetuses with known/suspected haemophilia
- Avoid fetal scalp electrodes and fetal blood sampling where moderate/severe haemophilia is suspected
- Regional anaesthesia is generally considered safe once factor levels are ≥50 IU/dL — below this, avoid neuraxial blockade
- Withhold IM vitamin K in at-risk neonates until coagulation results are back; use oral vitamin K if delay is likely
- Cranial ultrasound pre-discharge for neonates with severe/moderate haemophilia
- PPH risk remains elevated postpartum, not just intrapartum, as factor levels fall after delivery — active management of the third stage is recommended
- Manage a fetus of unknown carrier/affected status as if affected until proven otherwise
Source: RCOG Green-top Guideline No. 71 (April 2017 (first edition — joint RCOG/UK Haemophilia Centre Doctors' Organisation (UKHCDO) guideline, published BJOG 2017;124:e193–e263, replacing the 2006 UKHCDO guidance; no subsequent RCOG revision identified as of this review — check rcog.org.uk for a current edition before relying on numeric thresholds))
Read the original on rcog.org.uk
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