EUGONADISM

1.Anatomical causes
Mullerian agenesis
Impersonate hymen
Transverse vaginal septum
2.Androgen insensitivity syndrome
Mullerian Agenesis
◦ It is caused by embryological underdeveloped of mullerian duct with resultant
agenesis or atresia of vagina,uterus or both.
◦ The vaginal canal is markedly shortened and may appear as a dimple below
urethra.
◦ The ovaries and fallopian tubes are present
◦ Normal 46XX chromosome with normal external genitalia are present.
◦ 47% have associated urinary tract abnormalities, and 12% skeletal anomalies
◦ Clinical features
◦ Uterine anomalies are often an incidental diagnosis while seeing the patient for
different complaints like, pelvic pain, dysmenorrhoea, abnormal vaginal
bleeding
◦ Investigations
◦ Physical examination
◦ Gynecological USG
◦ Pelvic MRI
◦ Hyseterosal pinography
◦ Laproscopy and/ hysteroscopy
◦ Treatment
◦ Psychological counseling
◦ Vaginoplasty
◦ Excision of uterine reminant( if it has functioning endometrium)
◦ Vaginal dilators
Imperforate hymen
◦ It is a congenital disorder where a hymen without an opening completely
obstructs the vagina
◦ Caused by failure of hymen to perforate during fetal development
◦ It is the most common obstructive anomaly of the female reproductive tract.
◦ It is most often diagnosed in adolescent girls when menstrual blood
accumulates in vagina and sometimes in the uterus
◦ It is treated by surgical incision of the hymen
◦ Signs and symptoms
◦ In newborns my present with acute urinary retention
◦ In adolescent the most common symptoms are, cyclic pelvic pain and
amenorrhea
◦ Other symptoms associated with hematocolpos include urinary retention,
constipation ,
◦ Back pain, nausea and diarrhea
◦ Complications
◦ Peritonitis
◦ Mucometrocolpos
◦ Hematometrocolpos
◦ UTI
◦ Urinary retention, constipation
◦ Management
◦ Before surgical intervention in adolescent, symptoms can be relieved
by the combination of OCP taken continuous to suppress the menstrual
cycle
◦ NSAIDS to relieve pain
◦ Surgical treatment of the Imperforate hymen by hymenotomy
Transverse vaginal septum
◦ A transverse vaginal septum forms during embryological development when
the tubes that eventually become a vagina don’t fuse together properly
◦ The cause of this genetically abnormality is unknown
◦ Pathology
◦ Vertical fusion defect
1. Failure in resolution of the tissue between the vaginal plate and the caudal
aspect of the fused mullerian duct
2. A Transverse vaginal septum can be either perforate or imperforate
Symptoms

◦ Unusual abdominal swelling

◦ Amenorrhea
◦ Periods that last beyond the normal 4-7 day cycle
◦ Abdominal pain from blood collecting in upper vagina
Diagnosis
◦ Antenatal: In utero when there is obstruction causing significant amount of
hydrocortisone
◦ MRI
◦ Treatment by surgical excision of obstructed septum through a perineal
approach
Androgen insensitivity syndrome
Genetic condition where affected people have male chromosomes and male
gonadotropin with complete or partial feminization of the external genitals
An inherited X linked recessive disease with a mutation in the Androgen
receptor gene resulting in:
◦ Functioning in Y sex chromosome
◦ Abnormality on X sex chromosome
Clinical features: maldeveloped testis, typically tall and feminine individual,
external genitalia looks female in male, pubic and axillary hairs are scanty and
absent, infertility, short and blind vagina, signs of virilization in a girl with
primary amenorrhea, lack of virilization.
Investigation
◦ Chromosomal study: karyotype is 46XY
◦ USG: ovaries, uterus, upper 2/3rd of vagina and tunes are absent
◦ Confirmation of diagnosis is by gonadal biopsy
◦ DNS sequencing for AR gene is also possible
◦ Treatment
◦ Medical
1. Hormone replacement therapy
2. Psychological care
◦ Surgical
1. Gonadectomy