2. CASE PRESENTATION
A 38-year-old woman at 39 weeks 6 days gestation (G1P0, height 159 cm, weight 66 kg, 52 kg prepregnancy) presented to our hospital with labor pains. She had a long history of mastocytosis, with the development of pale brown chromatosis and hives from the back to the buttocks starting at 26 years old. Her symptoms worsened at 29 years old, and a wheal appeared while she was wearing and removing clothes. At the age of 32, the skin eruption worsened, and she was diagnosed with urticaria pigmentosa by a skin biopsy. She developed anaphylaxis twice with generalized skin rash and dyspnea in response to ibuprofen and diclofenac, and she was transferred to the emergency department and treated with intravenous betamethasone both times. Six months later, a skin biopsy and bone marrow biopsy performed at another hospital revealed systemic mastocytosis. She experienced convulsions after the use of an over-the-counter cold remedy and developed systemic erythema and dyspnea after the use of loxoprofen. Before fertility treatment, an allergy test was performed to identify the drugs that could be used during delivery, but no tests were performed for the drugs that can be used for labor analgesia. She became pregnant by in vitro fertilization, and her pregnancy progressed smoothly. She was on famotidine and ebastine for systemic mastocytosis before pregnancy and took additional fexofenadine hydrochloride when her condition worsened. She regularly took antihistamines and fewer oral steroids after the pregnancy.
During her pregnancy, the obstetricians, dermatologists, immunologists, hematologists, and anesthesiologists discussed various delivery plans and opted for epidural analgesia to avoid mast cell degranulation and anaphylaxis. The obstetrician planned a vaginal delivery without induction, based on evidence; systemic mastocytosis itself is not an indication for cesarean section. The patient received a prenatal anesthetic evaluation and was given an explanation of the anesthesia plan: epidural analgesia would be placed to avoid stress, which could trigger mast cell degranulation.
At 39 weeks 6 days of gestation, she complained of strong pain at the time she was admitted to the hospital. At admission, a rash and general cold sweat were found on her anterior chest wall and back. We suspected labor pain and psychological stress as the triggers for those symptoms and administered antihistamines and performed a combined spinal-epidural analgesia (CSEA). Before anesthesia, there were no abnormal laboratory data. The CSEA was placed at the L3-L4 level, and 1.5 mg 0.5% isobaric bupivacaine with fentanyl 15 µg was injected into the subarachnoid space, then a catheter was introduced into the epidural space at a cervical dilation of 4 cm and a pain NRS (Numerical Rating Scale) of 10. After placing the CSEA, her NRS became 0, and her sensory analgesia level was confirmed on both sides from Th5 to S5. Two hours after placing the CSE, her labor analgesia was switched to a programmed intermittent epidural bolus (PIEB) with 0.1% ropivacaine, and fentanyl 2 µg/mL was set at 8 mL with a 60-minute interval. Her analgesia level was kept at NRS 3–4, and her anesthesia level was kept at Th 8.9–S3 area until delivery. When the cervix was fully dilated, a rash and pruritus were noted on her abdomen, but sufficient pain relief was obtained with an NSR score of 3. H1 and H2 antihistamines were administered, the pruritus was suppressed, and vaginal delivery was performed as scheduled. The female infant weighed 3246 g and had Apgar scores of 7 and 9 at 1 and 5 minutes, respectively. The patient and the infant had an uncomplicated postpartum course and were discharged on postpartum day 5.