This document discusses congenital malformations of the female genital tract. It begins by outlining the normal development of the Mullerian ducts and Wolffian ducts. It then describes various types of malformations that can occur due to abnormalities in this development, including aplasia, hypoplasia, atresia, nonfusion, hermaphroditism and others. Specific conditions like vaginal atresia, imperforate hymen, and uterine fusion defects are explained. The clinical features, diagnosis and treatment of some of these congenital anomalies are provided.

1. CONGENITAL
MALFORMATIONS
FEMALE GENITAL TRACT

2. DEVELOPMENT OF FEMALE GENITAL TRACT
• GONADAL DEVELOPMENT IS SEPARATE FROM THAT
OF THE DUCTS. NORMAL OVARY MAY BE PRESENT
WITH ABSENT UTERUS & TUBES.
• MULLERIAN & WOLFIAN DUCTS ARE CLOSELY
RELATED . HENCE ASSOCIATION WITH
MALFORMATIONS OF THE RENAL TRACT
• SEVERE CONGENITAL ANAMOLIES MAY BE
ASSOCIATED WITH CHROMOSOMAL
ABNORMALITIES

3. DEVELOPMENT OF FEMALE GENITAL TRACT
 EARLY EMBRYO – INTERMEDIATE CELL MASS FORMS
MEDIAL PART OF UROGENITAL RIDGE FORMS URINARY TRACT
& GENITAL TRACT ; except Bladder, Urethra,Vagina,Vulva.
 Initially all embryos are undifferentiated. Hence INDIFFERENT
Gonad . Cells from Coelom migrate to medial part of
urogenital ridge . Development depends on the XY Genotype
Chromosomal expression of H-Y Factor which promotes
Testicular differentiation and secretion of ANTI-MULLERIAN
FACTOR which causes regression of the Mullerian system
while the Wolfian Ducts grow in response to Testosterone
secreted from testes and differentiate into the
retetubules, Epididymis, Vas, Prostrate, Seminal Vesicles.
 Absence of Y genes, leads to Gonad forming into Uterus and
since no AMF is secreted so Mullerian ducts differentiate
while the Wolfian ducts regress in XX Genotype.

4. DEVELOPMENT OF FEMALE GENITAL TRACT
• MULLERIAN DUCTS ARE SOLID COLUMNS/CORDS OF
CELLS, which later CANALISES.
• THEY ARE INITIALLY SEPERATE CEPHALIC part but join
up Caudally and then open into the Urogenital sinus as
mullerian tubercles.
• Upper part forms Fallopian Tubes, Lower part forms the
Uterus,Cervix,Upper2/3 of Vagina
• The cavity (lumen) is formed by canalisation.
• SINO-VAGINAL BULBS from the Mesodermal thickening
forms the lower 1/3 of VAGINA.
• The membrane seperating upper & lower vagina
disappears.
• WOLFIAN DUCT remnants may form cysts
Epoopheron, Paraoopheron, Gartner’s Duct

5. DEVELOPMENT OF FEMALE GENITAL TRACT
• VULVA develops in the area between the coccyx and
the umbilicus from 2 Genital tubercles.
• The tubercles fuse to form a single tubercle the
Clitoris. Posteriorly two genital swellings are present
which form the Labia Majora ; medial to this are 2
folds which form the Labia minora

6. DEVELOPMENT OF FEMALE GENITAL TRACT
• APLASIA- Nondevelopment of Organs.
Complete aplasia is associated with severe malformations of the
Urinary system, may not be compatible with life.
APLASIA OF OVARY RARE. May be found in TURNER’S Syndrome
XO Genotype, Ovarian tissue replaced by a fibrous band without
germinal epithelium. Associated absence of tubes.
• HYPOPLASIA- Poor development of Organs.
• ATRESIA- Noncanalisation of part / whole of the system.
Commonest Vagina Part / Complete Atresia
• NONFUSION or Imperfect fusion of Mullerian Ducts.
• HERMAPHRODITISM TRUE- Should have both Gonads - Ovary &
Testes as well as both external genetalia CLINICALLY NOT
POSSIBLE.
• HERMAPHRODITISM PSEUDO- FEMALE : Gonad Ovary and External
Genetalia Male
• URINARY TRACT DEVELOPMENT ANAMOLIES HYPOSPADIASIS &
ECTOPIA VESICAE often associated.

11. CONGENITAL MALFORMATIONS
• VAGINAL ATRESIA : Partial / Complete .
Ovary,Tubes,Uterus are normal. No outlet for OVA
or Menstrual blood. Blood collects in Vagina.
1.H/o Primary Amenorrhoea Cryptomenorrhoea.
2.Internal collection of blood
- Pain and discomfort
- Haematocolpos
- Haematrometra
- Haematosalpinx
- Haematoperitoneum
3.Urinary Retention

12. CONGENITAL MALFORMATIONS
• IMPERFORATE VAGINA (HYMEN)
Bluish discoloration of hymen
Rx Drainage (i)Excision of Hymen(ii)Canalisation of
Vagina(iii)Allow blood to drain by gravity
Do not place drain or press abdomen. PV after a
month on followup . Antibiotics in case of infection

13. CONGENITAL MALFORMATIONS
• FUSION DEFECTS
1.UTERUS DIADELPHIS :2 Uterii, 2 Cervix, 2 Vaginas
2.UTERUS BICORNUATE , BICOLLIS :2 Uterii, 2
Cervix, septate vagina
3.UTERUS BICORNUATE , UNICOLLIS : 2 Uterii, Septate
Cervix & Vagina
4.SEPTATE / SUBSEPTATE UTERUS
5.SEPTATE VAGINA
6.ARCUATE UTERUS : Fundus not well developed
7.UNICORNUATE : One side Mullerian duct develops
8.RUDIMENTARY HORN : One horn develops normally
other does not develop. Types – Communicating/
Noncommunicating
9.UNILATERAL HAEMATOCOLPOS - Cryptomenorrhoea

14. CONGENITAL MALFORMATIONS
• CLINICAL FEATURES :-
1.Symptomless – accidental discovery on USG/Exam
2.Menstrual problems – Menorrhagia
3.Unilateral dysmenorrhoea – abnormal contraction of one horn
of uterus
4.Coital difficulty – Pain or Bleeding (septate vagina)
5.Obstetrical Problems –
Infertility (coitus in rudimentary half of vagina)
Ectopic Pregnancy (rudimentary horn)
Habitual Abortions / Premature Labour
Malpresentations – Transverse lie / Breech presentation in
Septate Uterus & Bicornuate/Unicornuate Uterus
During Labour – Dysfunctional Labour, Obstructed Labour
(Rudimentary horn), Postpartum Haemorrhage /Retained
Placenta

15. CONGENITAL MALFORMATIONS
 DIAGNOSIS :
H/O Clinical Features
OBSTETRICAL HISTORY
CLINICAL EXAM
MAY MISTAKE TWO CORNU FOR FIBROID UTERUS
 INVESTIGATIONS :
ULTRASONOGRAPHY
SALINE SONOGRAPHY
HYSTEROSALPINGOGRAPHY
INTRAVENOUS PYELOGRRAPHY / UROGRAPHY
DIAGNOSTIC LAPROSCOPIC
COMPUTERISED TOMOGRAPHY
MAGNETIC RESONANCE IMAGING
POSITIVE EMISSION TOMOGRAPHY

16. CONGENITAL MALFORMATIONS
• PROBLEMS ON UNDIAGNOSED CASES
1. DYSMENORRHOEA – dilatation done on one side
other side produces pain
2. INTRODUCTION OF IUCD – IUCD placed in opposite
horn, pregnancy develops in undetected horn
3. CAESAREAN Section due to nondilatation of Cervix
4. MTP / MR Suction Evacuation – Pregnancy may
continue as procedure done in opposite horn
5. CARCINOMA CERVIX may be missed

17. CONGENITAL MALFORMATIONS
• TREATMENT OPERATIVE
UNIFICATION OF HORNS – METROPLASTY
May be done by Abdominal Route STRASSMAN’S
Hysteroscopic Resection of septae
EXCISION OF VAGINAL SEPTA
EXCISION OF RUDIMENTARY HORN to prevent risk
of Ectopic Pregnancy