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.