Saiby Khan, Gynae
Saiby Khan, Gynae
Saiby Khan, Gynae
Presented by:-
Saiby Khan
96542
(B.U.M.S Final Year)
CERTIFICATE
This is to certify that Saiby Khan (Student of
B.U.M.S Final Prof.) has completed the project
report on
Under my guidance.
Signature of lecturer:-
Saiby Khan
96542
SEX AND INTERSEXUALITY
Defining Sex and Gender
Gender identity (Psychological sex)
Inner sense of owns maleness/ femaleness.
• Sex of rearing
• Gender role
• Sexual identity (Organic sex)
• The biologic sexual differentiation
• Chromosomal sex
• Gonadal sex
• Internal genital sex
• External genital sex
• Hormonal sex
Testes OVARY
Internal genital organs development
External genital organs development
Male Development
Testis
Leydig Sertoli
cells Cells
Male external
genitalia
Male internal
genital organs
Female development
Neutral development
OVARY
• Most of upper
Absence of androgen vagina
exposure • Cervix and uterus
• Fallopian tubes
Summary of Normal Sex Differentiation
• genetic sex is determined at fertilization.
• testes develop in XY fetus, ovaries develop
in XX fetus.
• XY fetus produces MIS and androgens
and XX fetus does not.
• XY fetus develops Wolffian ducts and
XX fetus develops Mullerian ducts.
• XY fetus masculinizes the female
genitalia to make it male and the XX
fetus retains female genitalia.
INTERSEX
An individual in whom there is discordance between
chromosomal, gonadal, internal genital, and
phenotypic sex or the sex of rearing.
INTERSEXUALITY:
Discordance between any two
of the organic sex criteria
TRANSSEXUALITY:
Discordance between organic sex and psychological se
x components
CLASSIFICATION OF INTERSEXUALITY
1. Virilization of genitically female foetus
Female pseudohemaphroditism
2. Incomplete musculinization of genitically male
foetus
Male pseudohermaphroditism (XV-FEMALE)
3. The presence of both ovarian and testicular tissue
in the same individual
True hermaphroditism
4. Chromosomal abnormality
Mixed gonadal dysgenesis ( 45,X0 / 46,XY)
21-hydroxylase Cortisol
Androgen
s
Cortisol Androgen
s
Congenital adrenal
hyperplasia
• The commonest cause of
genital ambiguity at birth
• 21-Ohas deficiency is most
common form
• Autosomal reccessive
• Salt wasting form may be
lethal in neonates
• SERUM 170H-progesterone
(210Hase)
• SERUM deoxycorticosterone,
11-deoxycotisol
(11- OHase)
• Treatment: cortisol
replacement and Surgery
Streak Gonads
-NO MIF
(uterus +)
-No SEX
Female Female
external Internal
genitalia genitalia
Mixed Gonadal Dysgenesis
• Combined features of Turner's
syndrome and male
pseudohermaphroditism
• Short stature
• Streak gonad on one side with a testis
on the other
• Unicornuate uterus & fallopian tube-
side of streak gonad
• Karyotype 46XY / 45X0
• Considrable variation in the sexual
phenotype
True Hermaphroditism
DYSEMBROGENESIS genital
ambiguity with associated
anomalies
• Can occur in both genitic males and
genitic females
• Most common genital malformation :
Penoscrotal transposition Agenesis of
phallus in a genitic male
• Coexistence of other caudal or urologic
abnormalities should strongly suggest
dysembryogenisis
CLINICAL PRESENTATION
OF INTERSEXUALITY
• AT BIRTH
Ambiguous genitalia
• DURING CHILDHOOD
Heterosexual features
• AT ADOLESSCENCE
Delayed or Heterosexual
Puberty
DIAGNOSIS
• History: pregnancy; family
• Detailed examination: abdomen; pelvis;
external genitalia; urethral and anal openings.
Investigations
• Rule out cong. Adrenal hyperplasia:
Serum electrolytes; 17-OHP level and
urinary levels of 17-ketosteroids
• Karyotype (buccal smear; blood)
• Pelvic US and sometimes MRI or
Genitogram
• Skin biopsy; fibroblast culture to
measure 5alpha-reductase activity or
dihydrotestosterone binding
• Laparoscopy
• Gonadal biopsy (laparotomy)
TA US Genitogram
Sex assignment
General guidelines
• Sexassignment should be decided after
detailed assessment, investigations and
accurate diagnosis
• Complete gender assignment by age 18
months
Sex assignment
• Male gender assignment :
- stretched phallus > 2 cm
-erectile tissue
-lack of severe hypospadias
Surgical Considerations
• Phallic / clitoral reduction if the assigned
sex is female, before 3 years of age
• Removal of intra-abdominal gonads /
streaks in newborns carrying Y
chromosome
• Vaginal construction / repair is better
performed around puberty
Before Surgery After Surgery
Ambiguous genitalia
• Neglected congenital adrenal
hyperplasia
• Mixed gonadal dysgenesis
• Partial androgen resistance
• Congenital anorchia (Late)
• Testicular enzymatic failure
• Leydig cell agenesis (incomplete)
• True hermaphrotidism
MANAGEMENT OF INTERSEXUALITY
PRESENTING AT ADOLESCENCE
• Clitoral reduction
• Removal of gonads in the presence of Y chr
omosome
• Vaginal repair and construction
Before surgery After surgery
Gonadectomy
Vaginal Creation
Vaginal dilatation
McIndoe Vaginoplasty
William's vulvo-
vaginoplasty
Colovaginoplasty
Transsexualism
• Transsexualism occurs when a person strongl
y believes that he or she belong to the opposit
e sex.
• This is typically a lifelong feeling and results i
n varied degrees of physical/external changes
• These patients should be referred
to the psychiatrist