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LABOR ANALGESIA IN A PARTURIENT WITH MAY-HEGGLIN ANOMALY
Abstract Number: F-59
Abstract Type: Case Report/Case Series
May-Hegglin anomaly is an uncommon genetic condition manifested by significant thrombocytopenia and large platelets with preserved function.  Its inheritance pattern is autosomal dominant, but severity of thrombocytopenia and bleeding may vary; most heterozygotes are asymptomatic.  Despite thrombocytopenia, spontaneous bleeding may be mild.  Frequently, patients are misdiagnosed as having immune thrombocytopenia (ITP).  We present a case of a patient with known May-Hegglin anomaly.
A 20 year old G1P0 diagnosed with May-Hegglin anomaly at an outside hospital presented for evaluation prior to delivery. Medical records and complete blood count demonstrated her baseline platelet level ranged 20,000-30,000. She reported occasional nosebleeds, but denied spontaneous bleeding or bruising. She had received a platelet transfusion and had an uneventful appendectomy. Her mother endorsed similar history.
After evaluation, a multidisciplinary meeting was held to discuss delivery plans and labor analgesic options. Hematologists confirmed her diagnosis and excluded ITP. The timing and indication for platelet transfusion was discussed, prophylactic vs therapeutic. The safety of neuraxial anesthesia, with or without platelet transfusion, given thrombocytopenia but normal platelet function, was heavily debated.
The patient and MFM team preferred vaginal delivery, given the reduced risk for bleeding. The anesthesia team counseled the patient about her options, risks and benefits during a preoperative visit, regarding general anesthesia for cesarean section and neuraxial anesthesia-analgesia, after transfusion of platelets. After counseling, patient opted against neuraxial anesthesia.
She was admitted for induction at 38 weeks due to increased blood pressures at term. Per recommendations of the hematologists desmopressin acetate was given when active labor began. Platelet count was 45,000 upon admission, transfusion was unnecessary. Analgesia for delivery was with a fentanyl PCA, which patient considered adequate. Labor was unremarkable and patient delivered within 48 hours of admission, oxytocin 40 units and citotec were administered after delivery, estimated blood loss was 350mL. Newborn was evaluated in the NICU to rule out bleeding and thrombocytopenia. Both were discharged three days after delivery. Team work and multidisciplinary management and counseling of patient were demonstrated in this case.
Several cases of both vaginal and Cesarean delivery in patients with May-Hegglin anomaly have been described. Neuraxial techniques have been performed in such patients both with and without platelet transfusion, and no neurologic consequences have been reported. We present the clinical considerations as well as pros and cons of various labor analgesia management options for patients with May-Hegglin anomaly.
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