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Anesthetic Management of a Parturient with Systemic Mastocytosis
Abstract Number: T 51
Abstract Type: Case Report/Case Series
We describe the anesthetic management of a parturient with systemic mastocytosis, a rare disorder of increased mast cells in extradermal tissues that predisposes to type 1 immediate hypersensitivity reactions and anaphylaxis.
A 20 year old G1P0 at 39 weeks gestation with a history of systemic mastocytosis presented for induction of labor. Her disease was characterized by episodes of flushing, pruritus, chest tightness, tongue swelling, tachycardia, hypotension, and abdominal cramping that was responsive to diphenhydramine and IM epinephrine. No triggers were identified. Prior to pregnancy, treatments included oral contraceptives, prednisone, fexofenadine, and montelukast without improvement. She had a history of at least 20 anaphylactic episodes. Pregnancy caused increased flushing that was worsened by stress, rapid temperature changes and uterine contractions. An exacerbation at 8 weeks gestation required multiple doses of epinephrine for resolution.
An L4-5 epidural was placed prior to induction in order to blunt the sympathetic response to uterine contractions. It was tested with lidocaine 1.5% with epinephrine 1/200K and maintained with bupivacaine 0.125% and fentanyl 2 mcg/ml patient controlled epidural analgesia. Labor was induced with a foley bulb followed by oxytocin. Throughout the labor course, the patient was asymptomatic from her disease and vaginally delivered a vigorous infant.
Symptoms of mastocytosis can occur either from mast cell release of histamine, leukotrienes, prostaglandins, and cytokines resulting in anaphylaxis or from infiltration of mastocytes into the bone marrow leading to anemia or thrombocytopenia. This case is unique in that the patient’s triggers included uterine contractions, increasing the risk of anaphylaxis during delivery. Few reports have been published regarding the management of obstetric patients with mastocytosis. While two have reported adverse effects and fetal compromise secondary to the disease (1, 2), Worbec et al described nine women with cutaneous or systemic mastocytosis who had a total of eleven successful deliveries (3).
The anesthetic management of the parturient with systemic mastocytosis focuses on reducing stressors, which are known triggers for mediator release. In some cases, patients are treated prophylactically with glucocorticoids or histamine antagonists; however efficacy has not been established. Providers should be prepared to treat an episode of mast cell degranulation with intravenous access, epinephrine, and availability of personnel to treat cardiovascular collapse.
1. Donahue JG, Lupton JB, Golichowski AM. Cutaneous mastocytosis complicating pregnancy. Obstet Gynecol 1995;85:813–54.
2. Watson KD, Arendt KW, Watson WJ, Volcheck GW. Systemic mastocytosis complicating pregnancy. Obstet Gynecol. 2012 Feb;119(2 Pt 2):486-9.
3. Worobec AS, Akin C, Scott LM, Metcalfe DD. Mastocytosis complicating pregnancy. Obstet Gynecol 2000;95:391-5