Anesth Analg 2004;98:1471-1472
© 2004 International Anesthesia Research Society
doi: 10.1213/01.ANE.0000108132.95279.DD
OBSTETRIC ANESTHESIA
General Anesthesia for Cesarean Delivery in a Patient with Paroxysmal Nocturnal Hemoglobinuria and Thrombocytopenia
Klaus Kjaer, MD,
Michele Comerford, MD, and
Farida Gadalla, MD
From the Department of Anesthesiology, Weill Medical College of Cornell University, New York, New York
Address correspondence and reprint requests to Klaus Kjaer, MD, Weill Cornell Medical Center, Department of Anesthesiology, 525 E. 68th St. Suite M-325, New York, NY 10021. Address email to kkjaer{at}hotmail.com
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Abstract
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Pregnancy in patients with paroxysmal nocturnal hemoglobinuria (PNH) increases the risk of complement activation, hemolysis, and thrombosis. We describe the anesthetic management of a patient with PNH who underwent general anesthesia for cesarean delivery. Steroids, heparin, and blood products were administered early to minimize the likelihood of a hematologic emergency.
IMPLICATIONS:Pregnancy in patients with paroxysmal nocturnal hemoglobinuria increases the risk of hemolysis, thrombosis, anemia, and thrombocytopenia. We describe the anesthetic management of a patient with paroxysmal nocturnal hemoglobinuria and thrombocytopenia who underwent general anesthesia for cesarean delivery.
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Introduction
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Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia characterized by activation of the complement system resulting from loss of cell surface protein expression in hematopoietic stem cells (1). Maternal mortality estimates range from 5.8% to 20.8% (2,3). The most common cause of death is venous thromboembolism. Previous reports have described regional labor analgesia for parturients with PNH and general anesthesia for abdominal surgery in nonpregnant patients with PNH (4,5). However, this is the first English language report describing the anesthetic and hematologic management of cesarean delivery using general anesthesia in a PNH patient. At age 41, this patient is also the oldest patient with PNH known to have had a successful pregnancy.
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Case Report
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A 41-yr-old primigravida with a 10-yr history of PNH was admitted at 28 wk gestation with pre-term labor. Her pregnancy was complicated by 2 episodes of symptomatic anemia; both were treated with transfusion of 2 U of packed red blood cells. Her outpatient medications included prednisone 25 mg PO qod. On admission she was started on terbutaline and heparin 7500 IU bid.
At 31 wk gestation, the patient’s membranes ruptured and it was decided to proceed with urgent cesarean delivery. Her laboratory data were hematocrit 35.2, platelets 52,000/µL, partial thromboplastin time (PTT) 28.4, and INR 1.05. Her last heparin dose was administered 6 h earlier. A large-bore IV and a radial arterial line were placed, and general anesthesia was induced with fentanyl, thiopental, and succinylcholine. The patient was tracheally intubated easily and ventilated with O2 50%, N2O 50%, and isoflurane 0.4%. A healthy male infant was delivered 13 min after induction. Apgar scores were 2 and 6. On delivery of the placenta, the uterus bled profusely and the patient was transfused immediately with 6 U of platelets and 2 U of packed red blood cells. Oxytocin 40 IU/L was infused simultaneously along with hydrocortisone 100 mg. Hemostasis gradually improved. The estimated blood loss was 1000 mL.
After tracheal extubation, a heparin infusion was started immediately to maintain the PTT between 60 and 80 s. Postoperative laboratory values included hematocrit 31.7 and platelets 84,000/µL. She remained pain-free on IV morphine patient-controlled analgesia until the next day, when she complained of abdominal discomfort and developed dark urine. She was transfused 2 U of packed red blood cells, and within 6 h the urine cleared. On the third postoperative day she complained of incisional discomfort and developed fever to 38.8°C. Her hematocrit had decreased to 19.7 and platelets to 67,000/µL. She was transfused 2 U of packed red blood cells. The heparin infusion was stopped and the dosage changed to 5000 IU bid to avoid expansion of a hematoma found in the anterior rectus sheath on computed tomographic (CT) scan. The hematoma resolved and she was discharged on the ninth postoperative with hematocrit 26.9 and platelets 60,000/µL.
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Discussion
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This case is notable in that hematologic management was highly anticipatory. Steroids, heparin, and blood products were administered early to minimize the likelihood of a hematologic emergency characterized by fulminant hemolysis and thrombosis. It is well recognized that such an emergency may be triggered by pregnancy, stress, excessive physical exercise, infection, or surgery.
Both general anesthesia and regional anesthesia have been described in patients with PNH (4–7). Regional anesthesia was not chosen for this patient because of severe thrombocytopenia. Adding to this patient’s risk of spinal hematoma was the possible need for immediate anticoagulation in case of an acute exacerbation of PNH (8). Neuraxial block would have been the most effective way to inhibit the physiologic stress response associated with surgical stimulation. Large-dose opioid anesthesia was an alternative that we avoided because of its respiratory depressant effects on the neonate (9). It has been suggested that N2O should be avoided in PNH because of its myelodepressant effects (10). However, we chose to use N2O because the duration of N2O exposure was expected to be short and because we felt that the risk of aggravating bone marrow hypoplasia was smaller than the risk of uterine atony at larger concentrations of isoflurane.
A radial arterial line was placed to facilitate frequent monitoring of hematocrit and platelet counts. The blood products requested from the blood bank included platelets and unwashed leukocyte depleted packed red blood cells, as washed cells are no longer considered necessary (11). To eliminate the risk of a transfusion reaction before delivery of the fetus while at the same time promoting intraoperative hemostasis as early as possible, platelet infusion was started just after delivery of the placenta. IV access with a single large-bore IV was deemed adequate because of the bleeding risk associated with central line placement in the setting of thrombocytopenia. For postoperative pain control, IV morphine patient-controlled analgesia was chosen despite its associated respiratory acidosis. Although respiratory acidosis can serve as a trigger for hemolysis in PNH, the physiologic stress associated with inadequate pain control may also serve as a trigger.
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References
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Accepted for publication October 29, 2003.
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Abstract
Introduction
