Pregnancy management of IVF-ET pregnancies in a patient with classical 21-hydroxylase deficiency: A case report and review of the literature

•Repeated pregnancies in patients with classical 21-hydroxylase deficiency are rare, especially with Assisted Reproductive Technology.•We report a case of a woman with classical 21-hydroxylase deficiency who had twice singleton pregnancies with live births after IVF-ET.•We monitor the patient's pregnancy, individualized glucocorticoid therapy during pregnancy, glucocorticoid stress dose at delivery, management of systemic metabolism to prevent maternal and infant complications, and new-born screening are realized.•Reviewing the relevant literature, it is suggested that spontaneous and assisted reproductive pregnancies in patients with classical 21-OHD are feasible and safe, while intensive perinatal monitoring is essential. Perinatal management of 21-OHD patients still need to be standardized. To report a rare case of a woman with classical 21-hydroxylase deficiency who twice had singleton pregnancies with live births after in vitro fertilization and embryo transfer (IVF-ET). Design: Case report and literature review. Patient: A 35-year-old woman with classical 21-hydroxylase deficiency underwent external genital plastic surgery during adolescence and achieved second pregnancy after IVF-ET with long-term glucocorticoid replacement therapy. During regular antenatal testing, we focus on monitoring patients' weight gain, blood pressure, increasing trend of uterine height and abdominal circumference, and fetal growth trend. Individualized glucocorticoid therapy during pregnancy, glucocorticoid stress dose at delivery, management of systemic metabolism to prevent maternal and infant complications, and newborn screening are realized. In the second pregnancy, the glucocorticoid dosage was not increased. 17-hydroxyprogesterone and testosterone tended to increase in late pregnancy, but they were lower than in the first pregnancy. Blood pressure and blood glucose were normal, but lipids were abnormally elevated, D-dimer also showed a sharp rise under labor stress. A mature male infant was delivered by cesarean at 33+4 weeks of gestation due to placental abruption. Repeated pregnancies in patients with classical 21-hydroxylase deficiency are rare, especially with Assisted Reproductive Technology. We present a case including a comparison of her two pregnancy monitoring indicators, glucocorticoid medication and delivery to achieve a successful delivery. We review the available literature to analyze pregnancies with classical 21-hydroxylase deficiency.