Growth anomalies of the female genital tract
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Transcript of 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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