TABLE 1.

The systematization of 114 females with various disorders of sex development (DSD).

Systematization of Intersex Disorders
Nosology	Karyotype	Gonadal morphology	Internal genital anatomy	External genital anatomy	Surgical treatment	N
Congenital adrenal hyperplasia	46,XX	ovary	uterus and vagina	ambiguous genitalia	feminizing plasty: clitororeduction, introitoplasty	29
Turner syndrome (45,X)	45,X	fibrous streak gonad	PMDS	female	colpopoesis	7
Turner syndrome (45,X/46,XX)	45,X/46,XX	ovarian dysgenesis	uterus and vagina normal	female	laparoscopy gonadal biopsy	8
Turner syndrome (45,X/46,XY)	45,X/46,XY	fibrous streak, testicular dysgenesis, ovotestis	PMDS	ambiguous genitalia	laparoscopy gonadectomy; feminizing plasty: clitororeduction, introitoplasty; colpopoesis	5
46,XY gonadal dysgenesis, incomplete form	46,XY	testicular dysgenesis	PMDS, hypoplastic uterus	ambiguous genitalia	laparoscopy gonadectomy; feminizing plasty: clitororeduction, introitoplasty; colpopoesis	19
46,XY gonadal dysgenesis, complete form	46,XY	testicular dysgenesis	uterus and vagina (normal or hypoplastic)	female	laparoscopy gonadectomy	12
Androgen insensitivity syndrome, complete form	46,XY	testicles	PMDS	female	laparoscopy gonadectomy, colpopoesis	14
Androgen insensitivity syndrome, incomplete form	46,XY	testicles	PMDS	ambiguous genitalia	laparoscopy gonadectomy; feminizing plasty: clitororeduction, introitoplasty; colpopoesis	14
Ovotesticular DSD	46,XY 46,XX/46,XY	ovotestis	PMDS	ambiguous genitalia	laparoscopy gonadectomy; feminizing plasty: clitororeduction, introitoplasty;colpopoesis	6