Background
Neuraxial anesthesia, as the standard of care for Cesarean deliveries (CD), is associated with decreased blood loss1. However, parturients with inherited bleeding disorders are at increased risk for epidural hematomas. A small retrospective study2 has shown that parturients with known factor deficiencies can safely undergo neuraxial anesthesia once the specific factors are replenished. We present the anesthetic management for a CD in a patient with an unspecified inherited bleeding disorder.
Case Description
A 39-year-old G6P3 95 kg woman at 37 6/7 weeks gestational age presented for a repeat CD. She reported easy bruising, gingival bleeding, and menorrhagia. She also had compartment syndrome from hemorrhage after ankle surgery, persistent menorrhagia after her first two CD, and intra-abdominal hemorrhage after her third CD. Additionally, her mother had menorrhagia and her grandmother died from postpartum hemorrhage. An extensive hematological workup prior to her most recent operation, a cervical spine surgery, was normal for von Willebrand factor (VWF) parameters, fibrinogen, platelet function, and rare coagulation factors. She was then diagnosed with an unspecified inherited bleeding disorder, with a differential diagnosis of a rare VWF dysfunction or undetected defects in fibrin, fibrinolysis, or platelet function. She was successfully treated for her cervical spine surgery with prophylactic fresh frozen plasma (FFP), cryoprecipitate, platelets, and antifibrinolytics.
On the day of her CD, the patient prophylactically received 2 units of FFP, 10 units of cryoprecipitate, and 2 units of platelets before placement of her routine spinal anesthetic. Immediately after delivery of a healthy infant, aminocaproic acid was given. Uterine tone remained poor after routine oxytocin administration, but improved with methylergonovine and misoprostol. Blood loss was an estimated 1.5 liters. As a precaution, 2 units of platelets were given postoperatively. Postpartum, the patient continued antifibrinolytics and had an uneventful recovery.
Discussion
This patient had a considerably increased risk of peripartum bleeding due to an unspecified inherited bleeding disorder. Her previous deliveries were complicated by hemorrhage. Despite the lack of a specific diagnosis, hematology provided detailed recommendations for blood product administration, enabling a safe spinal anesthetic followed by CD. A spinal anesthesia was preferred, despite being the fourth repeat CD, over a combined spinal epidural to decrease the risk of an epidural hematoma. Communication between obstetric and anesthesia teams was crucial to expedite the start of surgery after the spinal placement and to aim for its timely completion.
In summary, the careful planning among obstetric, anesthesia, and hematology teams helped achieve an uneventful CD in this patient with an extremely high bleeding risk.
References
1Guay J. (2006) J Clin Anesth. 18(2):124-8.
2Chi C et al. (2009) Thromb Haemost. 101(6):1104-11.
