Growth anomalies of the female genital tract

Growth Anomalies Of The Female Genital Tract

Invagination of coelomic mesothelium occurs close to the primitive gonad.

Two on either sides

Grow caudally

Cross Wolffian duct

meet in the midline

Mullerian ducts

Mullerian tubercle

Sinovaginal bulbs

Intervening septum

Uterovaginal canal

Growth continues…

Aplasia : Failure of developmentHypoplasia : Organs are rudimentaryAtresia : Partial or complete failure of

canalisationMullerian Duct Anomalies : Assymetric

development, failure of fusion, failure of disappearance of intervening septum

Hermaphroditism and Pseudohermaphroditism : Abnormal development of gonads

Developmental Defects : Defective development of urinogenital sinus

Mullerian Anomalies

May involve the whole part of the Genital Tract in the female.

1. Ovary Ovarian agenesis appearing as a streak

gonad.

2. Fallopian Tube rudimentary tubes with abnormal ostia and

fimbriae

3. Uterus Complete agenesis only with concurrent non

development of the urinary system A unilateral development of the Mullerian

duct is known to occur.

ATRESIA

4. Vagina

Incidence is reported to be 1:400 to 1: 5000 women.

Familial tendancy, multy factorial in origin.

Failure of the vagina to canalize may be partial or

complete

Functioning uterus present occasionaly in 7 to 8 %

Testicular Feminising Syndrome, RKH syndrome

(Rokitanski-Kuster-Hauser syn.)

Development of secondary sexual characters is normal

Menarche fails to occur

Sexual intercourse is not possible

Frank’s Technique / Construction of and artificial vaginaMcIndoe Operation : space created between

the urinary bladder and the rectum by incising the dimple vagina transversely. Later lined with a split skin graft (from thigh) or fresh amnion

William’s vaginoplasty : creating a pouch out of Labia majora. Leaves grossly distorted external genitalia

Frank’s dilatation

Management

1. More common than aplasia

2. C/F:

Rudimentary horn

Pubescent or infantile uterus

Infantile cervix with a pin point os

Uterocervical length less than

Endometrial lining is scanty

The fallopian tubes appear thin, long and wavy with

poorly developed Plicae.

Underdeveloped external genitalia

HYPOPLASIA

Resection of the rudimentary horn to prevent development or spread of endometriosis and occurrence of ectopic pregnancy.

Uterine unification can be done in patients with communicating horn.

Management

3. Presenting features:

Infertility

Hypomenorrhoea or Oligomenorrhoea

Dysmenorrhoea and Dyspareunia

4. A non canalizing rudimentary horn may not

cause any problem. but can be mistaken for

a fibroid.

5. A functioning horn may cause symptoms like

Haematometra (communicating horn)

Endometriosis and ectopic pregnancy (non

communicating)

1. May involve a part or whole of the tract

2. Fallopian tube atresia : Partial/ Complete

Partial atresia : Hematometra

Atresia at the junction of Mullerian Tubercle :

Imperforate Hymen or Vaginal septum

Mucocolpos or Hematocolpos

ATRESIA

1. Imperforate Hymen may be the cause

2. C/F :

Colicky abdominal pain which is often cyclic

Menarche has not yet achieved.

Vagina gets progressively filled with blood

Pressure symptoms

Difficulty in micturition Urinary retention

HAEMATOCOLPOS

3. O/E : A suprapubic bulge producing discomfort on

palpation On separation of the labia, a bluish bulging

membrane is recognized

4. Complications: Progress beyond hematocolpos to

hematometra and hematosalpinx Development of pelvic endometriosis Constitutional symptoms : Low grade fever,

tachycardia, malaise, pallor etc USS establishes any associated renal

abnormality

1. These result from defective fusion, canalization or absorption of the medial septum of the female reproductive septum during embryonic development

2. Usually asymptomatic. Symptoms include reproductive failures (Abortions, Preterm birth, LBW), malpresentations (transverse lie/oblique lie/breech)

MULLERIAN DUCT ABNORMALITIES

Two Mullerian Ducts

fails to fuse along the

whole of their lengths

Develop normally and

remain separate

Two vaginas open at the

vulva

A vaginal Septum can be

seen

Two cervices seen

1. Uterus Didelphys

Two Mullerian ducts fuses at the body of the uterus

Uterus bicornis bicollis: Vagina is single but the two cornua remain separated and two cervices project into the vagina.

Uterus bicornis unicollis: Vagina is single with a single cervix

2. Uterus Bicornis

Although the two Mullerian ducts have fused, a median septum passes from the fundus of the uterus to the cervix and may extend into the vagina

In Uterus subseptus, the septum is retricted to the body of the uterus

3. Uterus septus/subseptus

There is no actual septum in the region of the fundus

Instead of the usual dome-shaped convexity of the fundus, there is a shallow concave depression

Arcuate Uterus

Seen in 1% cases and is associated with renal abnormality on the same side.

Recognized by the absence of round lig. And fallopian tube on the opposite side

Can cause abortion, preterm labor and IUGR

Uterus unicornis

Class I Segmental Mullerian agenesis or hypoplasia

a) Vaginalb) Cervicalc) Fundald) Tubale) Combined

Class II Unicornuate uterus a) With Rudimentary horn

• With a communicating endometrial cavity

• With a non communicating cavity

• With no cavitya) Without any

rudimentary horn

Buttram and gibbons classification

Class III Uterus didelphys

Class IV Bicornuate uterus a) Complete upto the internal os

b) Partialc) Arcuate

Class V Septate uterus a) With a complete septum

b) With an incomplete septum or Uterus subseptus

Class VI Uterus with internal luminal changes

Dyspareunia due to a coexisting vaginal septum

Pap smear must be taken separately from each cervix for gynaec. Examination

Use of separate IUDs for each hornBicornuate uterus cause the following gynaec.

Problems.Habitual abortions due to myometrial weakness or

septal implantationPreterm labourPersistent malpresentations Retained adherent placenta

Clinical Aspects

Ultrasound of the pelvis as well as the renal region

HysterogramHysteroscope : the gold standard in the

diagnosis as well as managementLaparoscopy

Investigations

1. Strassman utriculoplasty with a transverse fundal incision; in women with bicornuate uterus

2. Jones’ operation of wedge resection of a part of fundus with the septum followed by uniting the two horns vertically; in women with septate uterus

3. Hysteroscopic resection using a resectoscope, has a higher pregnancy rate that other invasive procedures

4. Excision of the rudimentary horn to avoid heamatometra and ectopic pregnancy

Management

True hermaphroditism : Glands of both sexes must be present in the same individual.

In most cases the accessory sex gland is atrophic and shows no evidence of functional activity

In other cases the functional glands are partly of ovarian and partly of testicular tissue

Along with Ovarian follicles and corpus luteum spermatogonia and spermatocytes are seen

HERMAPHRODITISM

In this case, the sex glands are of one sex while the external genitalia will be of opposite sex

Ovaries may descend within the inguinal canal to lie n the labia majora, with a hypertrophied clitoris and fused labiosacral folds rescemble a rudimentary scrotum (female pseudohermaphroditism)

Testis fails to descend, the penis is ill developed and the external genitalia rescembles that of a female (Male hermaphroditism)

PSEUDOHERMAPHRODITISM

Epispadias: Rare anomaly presenting with genital prolapse and urinary incondinence

Ectopia vesicae: Due to the defective development of the lower abdominal wall and the anterior wall of the urinary bladder. The red mucous membrane of the interior of the bladder remain exposed and two ureteric orifices are visible.

Hypospadias

DEVELOPMENTAL DEFECTS OF THE UROGENITAL SINUS

Imperforate anus:Results from the failure of the claocal membrane to divide below the terminal intestine

Atresia recti:Lower part of the rectum fails to develop. Much more serious than imperforate anus.

Congenital rectovaginal Fistula:Imperfect separation between rectum and the urogenital sinus. It can result in a perineal anus or vaginal anus

MALFORMATIONS OF RECTUM AND ANAL CANAL

The upper part of the Wolffian duct may at times dilate to form a Para ovarian cyst and the lower portion forms a Gartner Cyst

Usually diagnosed by a laparotomy, when the ovary is normal and the cyst lies in the broad ligament

A small gartner’s cyst can be left alone, but if it causes dyspareunia, excision is required.

WOLFFIAN DUCT ANOMALIES

A double ureter is rarely encountered. Its recognition by laparotomy is necessary to avoid any injury

An ectopic ureter sometimes communicates with vagina

Diagnosis confirmed by a pyridine test and an intravenous pyelogram (IVP)

RENAL TRACT ABNORMALITIES

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