Pregnancy outcome in a woman with congenital adrenal hyperplasia: a rare case report

Mamatha Swamy; Divya Ganesh; Ankitha C. R.

doi:10.18203/2320-1770.ijrcog20254302

Authors

Mamatha Swamy Department of Obstetrics and Gynecology, Ramaiah Medical College and Hospital, Bangalore, Karnataka, India
Divya Ganesh Department of Obstetrics and Gynecology, Ramaiah Medical College and Hospital, Bangalore, Karnataka, India
Ankitha C. R. Department of Obstetrics and Gynecology, Ramaiah Medical College and Hospital, Bangalore, Karnataka, India

DOI:

https://doi.org/10.18203/2320-1770.ijrcog20254302

Keywords:

Congenital adrenal hyperplasia, Pregnancy, Steroid replacement

Abstract

Congenital adrenal hyperplasia (CAH) refers to family of inherited disorders of adrenal steroidogenesis caused by mutations in genes encoding steroidogenic enzymes involved in glucocorticoid synthesis/in cofactor enzyme P450 oxidoreductase that serves as electron donor to CYP21A2 and CYP17A1 with autosomal recessive inheritance pattern. A 24-year-old primigravida with simple virilizing CAH who underwent reconstructive surgery in childhood. During pregnancy, she developed severe preeclampsia requiring preterm emergency caesarean delivery at 30+6 weeks period of gestation. Stress steroid supplementation was administered intrapartum. Both mother and neonate had a favourable outcome with close multidisciplinary management. Early diagnosis, timely reconstructive surgery, optimized steroid replacement, and multidisciplinary care are crucial for favourable maternal and neonatal outcomes in CAH pregnancies.

Metrics

Metrics Loading ...

References

Keely E, Malcolm J. Congenital adrenal hyperplasia in pregnancy: approach depends on who is the ‘patient’. Obstetric Med. 2012;5(4):154-60. DOI: https://doi.org/10.1258/om.2012.120015

Antal Z, Zhou P. Congenital Adrenal Hyperplasia: Diagnosis, Evaluation, and Management. Pediat Rev. 2009;30(7):e47-59. DOI: https://doi.org/10.1542/pir.30.7.e49

Guo X, Zhang Y, Yu Y, Zhang L, Ullah K, Ji M, et al. Getting pregnant with congenital adrenal hyperplasia: Assisted reproduction and pregnancy complications. A systematic review and meta-analysis. Front. Endocrinol. 2022;13:982953. DOI: https://doi.org/10.3389/fendo.2022.982953

Cera G, Locantore P, Novizio R, Maggio E, Ramunno V, Corsello A, et al. A Pregnancy and Prenatal Management of Congenital Adrenal Hyperplasia. J Clin Med. 2022;11:6156. DOI: https://doi.org/10.3390/jcm11206156

Ogilvie CM, Rumsby G, Kurzawinski T.Outcome of bilateral adrenalectomy in congenital adrenal hyperplasia: one unit’s experience. Eur J Endocrinol. 2006;154:405-8. DOI: https://doi.org/10.1530/eje.1.02096

Shorakae S, Teede H. Congenital adrenal hyperplasia and pregnancy. Case Rep. 2013;2013:bcr2013010299. DOI: https://doi.org/10.1136/bcr-2013-010299

Nigel Pereira, Karen Lin-Su. Reproductive Function and Fertility in Women with Congenital Adrenal Hyperplasia: EMJ Repro Health. 2018;4(1):101-7. DOI: https://doi.org/10.33590/emjreprohealth/10314092

Witchel SF. Management of CAH during pregnancy: optimizing outcomes. Curr Opin Endocrinol Diabetes Obes. 2012;19:489-96. DOI: https://doi.org/10.1097/MED.0b013e32835a1a2e