345
- How to assess the degree of virilization in a 46,XX newborn?
The degree of virilization in a 46,XX newborn can be assessed by using Prader
staging, which is shown in the fi gure given below (Fig. 10.5 ).
Stage 1: Normal female genitalia with clitoromegaly
Stage 2: Partial posterior labial fusion with clitoromegaly
Stage 3: Labioscrotal fusion so that there is a single opening in the perineum, and
clitoromegaly
Stage 4: Fusion of labioscrotal folds so that the single opening is at the base of the
phallic structure
Stage 5: Appearance like a male external genitalia
ab
c
d
Fig. 10.4 Varying degree of labioscrotal fusion in children with CAH due to 21α-hydroxylase
defi ciency. ( a ) Isolated clitoromegaly. ( b ) Posterior labial fusion with clitoromegaly. ( c )
Labioscrotal fusion with penoscrotal hypospadias. ( d ) Complete labioscrotal fusion and apparently
male genitalia with absent testes
10 Congenital Adrenal Hyperplasia