1) Congenital anomalies of the female genital tract refer to any physical abnormalities present at birth. The causes are mostly unknown but may involve hormone deficiencies, excesses, genetic factors or exposure to harmful substances. 2) Some common external anomalies include perineal or vestibular anus, ectopic ureters, and hymen abnormalities like imperforate hymen. Internal anomalies affect the vagina, uterus and ovaries. 3) Uterine anomalies are classified by the American Fertility Society and include conditions like arcuate uterus, bicornuate uterus, septate uterus and DES-related abnormalities. Clinical features may include infertility, dyspareunia and menstrual or obstet

1. CONGENITAL
MALFORMATIONS OF
FEMALE GENITAL TRACT
PRESENTED BY
ABHILASHA VERMA
LECTURER
( M.Sc. NURSING OBG. GYNAE)

2. DEFINITION
“ Congenital anomaly of the genitalia is a
medical term referring to any physical
abnormality of the male and female internal
or external genitalia present at birth”

3. CAUSES
• Exact cause is unknown
• Hormone deficiency and excess
• Teratogenic effects
• Genetic determination

4. DEVELOPMENTAL ANOMALIES OF
EXTERNAL GENITALIA
 PERINEAL OR VESTIBULAR ANUS-
• Detected at time of birth.
• The anal opening is situated either close to posterior end
vestibule or in vestibule.
• Rarely it is situated in vagina ( congenital recto vaginal
fistulla)
• Future reproduction not a problem, generally ceaserean
section advised.
 ECTOPIC URETER-
• Additional ureter opening is usually in vestibule close to
vagina and urethra.
• Symptom – Uncontrollable wetness
• Ureterectomy or implantation of ectopic ureter into
bladder may be done

5. VAGINAL ABNORMALITIES
NARROW INTROITUS-
• Revealed after marriage because “dyspareunia”
may be the first complaint.
It may be detected during investigation of infertility
• Surgical enlargement is treatment.

6. HYMEN ABNORMALITY-
• Imperforate hymen is important abnormality .
• Always unnoticed until 14-16 years of age.
• Uterine functioning is normal menstrual blood
is pent up inside vagina behind
hymen.(Cryptomenorrhoea)
• Depending upon amount of blood accumulated first
descend into vagina ( Hematocolpos)
• The uterus is next involved and cavity dialated (
Hematometra)
• If late and neglected, tube also distended after
fimbrial ends and becoming closed by
adhesions.(Hematosalpinx)

8. Symptopms-
• Periodic lower abdominal pain, continuous
nature.
• Primary amenorrhoea.
• Dysuria and retention of urine due to
elongation of urethra.
• On examination-
– Abdominal exam. : Suprapubic swelling
– Vulval inspection: Tense bulging membrane of
blue discoloration.
– Rectal exam. : Bulged vagina

9. TEATMENT –
• Incision made in hymen, quadrants of the
hymen partially excised, not too close to
vaginal mucosa.
• Spontaneous escape of dark tarry colored
blood following incision.
• Antibiotic should be given

10. VAGINAL MALDEVELOPMENTS-
Agenesis of vagina –
• It is a birth defect when vagina does not develop
fully. (1:5000)
Septate vagina-
• Complete or incomplete longitudinal septum.
• Transverse vaginal septum.
• May be asymptomatic or may represent
dyspareunia or obstructed delivery.
• Treatment – septum to be excised.

11. Partial atresia of vagina-
• Segment of vagina may be atretic , it may be
associated with cervical atresia.
• Primary amenorrhoea, periodic pain in lower
abdomen .
• Excision of atretic portion and suturing the margins
of vaginal wall.
Complete atresia of vagina-
• It is mainly associated with absence of uterus.
• Absence or abnormal narrowing of vaginal
opening .

12. ASSOCIATED ABNORMALITIES
• Vesico-vaginal fistula- Abnormal opening
between vagina and bladder.
• Recto-vaginal fistula- Abnormal opening
between rectum and vagina.

13. UTERINE ANOMALIES
• American Fertility Society ( AFS) classification-
Class I: Mullerian agenesis/ Hypoplasia
Class II: Unicornuate uterus with or without
rudimentory horn.
Class III: Uterus didelphys
Class IV: Bicornuate uterus
Class V: Septate uterus
Class VI: Arcuate uterus
Class VII: Diethylstilbesterol (DES) related anomalies

14. MULLERIAN AGENESIS
Failure of development of one or both
Mullerian ducts:
• Absence of both ducts leads to absence
of uterus including oviduct and absence
or vagina as well.
Primary amenorrhoea is chef complaint.
• Absence of one duct leads to unicornuate
uterus with single oviduct.

15. ARCUATE UTERUS (18%)
• Fundus looks concave with heart shaped
cavity outline.
• This looks more like a normal uterus, except it
has a deep or slight indentation at the top.
• It is a common abnormality, affecting 1:25 in
general population
• It doesn’t usually make conceiving difficulty.

17. UTERUS DIDELPHYS ( 8%)
• Complete lack of fusion of the Mullerian ducts
with a double uterus and double vagina.
• Uterus has two inner cavities, each cavity may
leads to own cervix and vagina, so there are
two cervix and two vaginas.
• It is uncommon affecting 1:350 women.
• It is possible to concieve and have a straight
forward pregnancy.

19. UTERUS BICORNIS (26%)
There are varying degrees of fusion of muscle
walls of two ducts.
 Uterus bicornis bicollis: There are two
uterine cavities with double cervix with or
without vaginal septum.
 Uterus bicornis unicollis: There are two
uterine cavities with one cervix. Horns may be
equal or one horn may be rudimentary and
have no communication with developed horn.

21. SEPTATE UTERUS (35%)
• Two Mullerian ducts fused together but there
is persistence of septum in between two
either partially or completely

23. UNICORNUATE UTERUS (10%)
• Failure of development of one Mullerian duct

26. DES–RELATED ABNORMALITIES
It is due to DES exposure during intra uterine
life, variety of mal formations are included-
 Vagina: Adenosis, adenocarcinoma
 Cervix : Cockscomb cervix, cervical collar.
 Uterus : Hypoplasia, T-shaped cavity, uterine
synechiae.
 Fallopian tube: Cornual budding, abnormal
fimbriae.

27. CLINICAL FEATURES
GYNAECOLOGICAL-
• Infertility and dyspareunia (vaginal septum)
• Dysmenorrhoea in bicornuate uterus or due to
cryptomenorrhoea ( pent up menstrual blood
in rudimentary horn)
• Menstrual disorders like menorrhagia ( due to
increased surface area in bicornuate uterus),
cryptomenorrhoea

28.  OBSTETRICAL-
• Midtrimester miscarriage
• Rudimentary horn pregnancy.
• Cervical incompetence
• Increased risk of mal presentation.
• Pre-term labor, IUGR,IUD.
• Prolonged labor ( d/t inco-ordinated uterine
actions)
• Obstructed labor ( obstruction due to non gravid
horn of bicornuate or rudimentary horn)
• Retained placenta or PPH due to implantation on
septum .

29. INVESTIGATIONS
• Hysterography
• Hysteroscopy
• Laparoscopy
• TVS USG.
• MRI

30. TREATMENT
Reproductive outcome:
• Septate uterus (86%)
• Bicornuate uterus (50%)
• Unicornuate uterus ( 40%) poor
• Uterus didelphys has best possibilities of
successful pregnancy. (64%)

31. TREATMENT:
• Rudimentary horn excision to reduce ectopic
pregnancy risk.
• Unification operation in bicornuate and
septate uterus.
• Hysteroscopic metroplasty – resection of an
intrauterine septum maintaing symmertry of
uterine cavity.( 80-90% success rate)

32. OVIDUCT ABNORMALITIES
• Elongated fallopian tube
• Absent of one side tube.
• Gonadal agenesis.
• Presence of accessory ovary
• Ovaries may be present n broad ligaments.
OVARY ABNORMALITIES

33. OTHER ABNORMALITIES
• LABIA MINORA- Labia fusion due to
developmental defect.
• LABIA MAJORA- Hyperplastic,hypoplastic,
abnormal fusion
• CLITORAL ABNORMALITY- Clitoral duplication,
clitoral megally
• PERINEUM- Anal agenesis, imperforate anus,
anal stenosis, rectal fistulla.

34. THANK YOU