Anesthetic Management of a Parturient with
May-Hegglin Anomaly
Background
•May-Hegglin Anomaly (MHA) is an inherited macrothrombocytopenia caused by an autosomal dominant mutation in the MYH9 gene.
•This provides a challenge during the peripartum period due to increased risk of bleeding and hematoma formation with neuraxial anesthesia.
The Case
35-year-old G1P0010 female with May-Hegglin anomaly (MHA) presenting at 39 weeks gestation for scheduled cesarean delivery (C/S) under general anesthesia (GA).
Hematology consult preop
-Avoidance of NXA given plt count, possible dysfunction, and genetic variant
-1g TXA prior to delivery and q8h during admission
-Transfuse plts for any evidence of excessive bleeding regardless of pltcount
Anesthetic management
-C/S under GA
-Induction complicated by difficult airway requiring 2 attempts
-2 PIV
-2u plts available in the OR, but no transfusion necessary
-EBL 800
- TAP block and PCA postoperatively
Teaching Points
•MHA is an inherited macrothrombocytopenia caused by an autosomal dominant mutation in the MYH9 gene presenting with bruising, gum bleeding, epistaxis, and menorrhagia.
•Patients with MHA traditionally deliver via C/S under GA
•Most deliveries in women with MHA proceed without severe bleeding complications regardless of delivery mode 1
•There are case reports documenting successful vaginal deliveries and neuraxial anesthesia without complication 2,3,4,5
•TXA often used as surgical prophylaxis to help strengthen clot formation