The external genitalia of patients with Androgen Insensitivity Syndrome (AIS) may range from phenotypically male to female and anywhere in-between.
Clinical Appearance
Grade
normal masculinization
1
male phenotype with mild defect (isolated hypospadia)
2
male phenotype with severe defect (small penis, perineoscrotal hypospadias, bifid scrotum, and/or cryptorchidism)
3
severe genital ambiguity (phallus intermediate between clitoris and penis, labioscrotal folds, single perineal orifice)
4
female phenotype, with posterior labial fusion, mild clitoromegaly and separate vaginal and urethral orifices
5
female phenotype with pubic and axillary hair after puberty
6
female phenotype with no pubic or axillary hair after puberty
7
Prior to puberty a patient with a female phenotype is classified as Grade 6/7.
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Specialty: Urology, Pedatrics
ICD-10: ,
