375- What are the appropriate timings for genital reconstruction surgery in a girl
with genital ambiguity due to CAH?
In girls with CAH who have severe genital virilization (Prader stage ≥3), clito-
ral recession should be considered along with perineal reconstruction during
infancy. Vaginal reconstruction in these children will depend on the position of
vaginal confl uence; if high vaginal confl uence is present, surgery may be
delayed till puberty, whereas with low vaginal confl uence, surgery may be done
simultaneously along with clitoroplasty. Children with Prader stage 1 and 2
should be kept under close surveillance as clitoromegaly may regress after
intensive treatment with glucocorticoids.Further Readings
- DeGroot L, Jameson J. Endocrinology. Philadelphia: Saunders/Elsevier; 2010.
- Han TS, Walker BR, Arlt W, Ross RJ. Treatment and health outcomes in adults with congenital
adrenal hyperplasia. Nat Rev Endocrinol. 2014;10:115–24.
Fig. 10.20 Siblings with
CAH due to
21 α-hydroxylase
defi ciency ( a ) 46,XY ( b )
46,XX reared as male
10 Congenital Adrenal Hyperplasia