Factor XI deficiency presenting in pregnancy: diagnosis and management

Abstract

A 34 year old primagravidae had an uneventful dichorionic diamniotic twin pregnancy, with normal fetal growth on ultrasound and both twins in a cephalic presentation. Labour was induced at 40 +5 weeks of gestation with vaginal prostaglandin and an epidural catheter was sited. The first twin was delivered by rotational forceps delivery for delay in the second stage of labour secondary to deep transverse arrest, and a right mediolateral episiotomy was made just before delivery. The second twin was delivered 19 minutes later by ventouse extraction for fetal distress using a silicone cup. The third stage of labour was actively managed and the uterus contracted well; however, following delivery of the second twin, profuse vaginal bleeding was noticed from the episiotomy site and two bilateral vaginal tears that extended to one-third of the length of the vagina. No other genital trauma was present and the vaginal lacerations and episiotomy were repaired without delay. The estimated blood loss was 1500 mL. A coagulation screen showed an isolated prolonged activated partial thromboplastin time of 61 seconds (control 34 seconds, normal range 36 -50 seconds), corrected to 48 seconds in vitro by a 50:50 mix with normal plasma. The haemoglobin concentration was 6.5 g/dl and the woman received a transfusion of five units of blood. The activated partial thromboplastin time following transfusion was 49 seconds. She had no history of bleeding following trauma or surgery, which included fractured tibia and fibula, spinal surgery for lumbar vertebral fracture and appendicectomy. There was no family history of a bleeding tendency. Clotting studies later indicated a low level of factor XI of 0.16 U/mL (normal range 0.7 -1.5 U/mL) and normal levels of factors VIII, IX and XII. This confirmed a diagnosis of factor XI deficiency. Lupus anticoagulant and antiphospholipid antibodies were not present. Postpartum recovery of the mother and both babies was uneventful with no neonatal bleeding complications and both infants had normal levels of factor XI. Case 2A 34 year old woman had an uneventful pregnancy. She was admitted with a nine-hour history of spontaneous rupture of the membranes at 39 weeks of gestation. Her previous delivery at another hospital was an emergency lower segment caesarean section for failed induction of labour following spontaneous rupture of membranes at 37 weeks of gestation. This was reported to be complicated by excessive blood loss although coagulation studies were not performed at the time. An appendicectomy was complicated by delayed wound haemorrhage on the second post-operative day. The wound required resuturing. She returned to theatre because of secondary haemorrhage following tonsillectomy and required a blood transfusion. Laser cone biopsy and diagnostic laparoscopy had been uneventful: her sister has bled following a diagnostic laparoscopy.A repeat caesarean section was planned when labour has not begun 24 hours after admission. A coagulation screen performed because of her bleeding history showed an i...

Changes in the levels of factor VIII and von Willebrand factor in the puerperium

“…None of the cases were complicated by epidural/ spinal haematoma. Similarly, in 12 case reports and series [6,12,[34][35][36][37][38][39][40][41][42][43][44] describing the use of obstetric block in 60 women with inherited bleeding disorders, none reported any complications.…”

The obstetric experience of women with factor XI deficiency

“…PPH can affect both women with severe or partial deficiency, especially those with a positive bleeding history. A small number of case report/series have shown that the risk of PPH can be minimized by prophylactic treatment with fresh frozen plasma (FFP) or FXI concentrate [8], [14–16]. In our unit, intrapartum prophylaxis with FXI concentrate is recommended in all women with severe FXI deficiency for all modes of delivery [10], [17].…”

The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization

“…Regional anaesthesia. Epidural anaesthesia has been carried out without complication in FXI-deficient patients [54,97]; however, the consequences of a spinal haematoma with compression of the spinal cord makes it an unacceptable risk and epidural should be avoided in severe cases or women with significant bleeding history. If epidural is necessary, it should be covered with FXI concentrate.…”