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Aplasia : Failure of development Hypoplasia : Organs are rudimentary Atresia : Partial or complete failure of canalisation Mullerian 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
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 fimbriae3. Uterus Complete agenesis only with concurrent non development of the urinary system A unilateral development of the Mullerian duct is known to occur.
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 vagina McIndoe 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
1. More common than aplasia2. 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
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.
3. Presenting features: Infertility Hypomenorrhoea or Oligomenorrhoea Dysmenorrhoea and Dyspareunia4. 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 tract2. Fallopian tube atresia : Partial/ Complete Partial atresia : Hematometra Atresia at the junction of Mullerian Tubercle : Imperforate Hymen or Vaginal septum Mucocolpos or Hematocolpos
1. Imperforate Hymen may be the cause2. 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
3. O/E : A suprapubic bulge producing discomfort on palpation On separation of the labia, a bluish bulging membrane is recognized4. 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 development2. Usually asymptomatic. Symptoms include reproductive failures (Abortions, Preterm birth, LBW), malpresentations (transverse lie/oblique lie/breech)
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
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
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
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
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
Class I Segmental Mullerian a) Vaginal agenesis or hypoplasia b) Cervical c) Fundal d) Tubal e) CombinedClass II Unicornuate uterus a) With Rudimentary horn • With a communicating endometrial cavity • With a non communicating cavity • With no cavity a) Without any rudimentary horn
Class III Uterus didelphysClass IV Bicornuate uterus a) Complete upto the internal os b) Partial c) ArcuateClass V Septate uterus a) With a complete septum b) With an incomplete septum or Uterus subseptusClass 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 horn Bicornuate uterus cause the following gynaec. Problems. Habitual abortions due to myometrial weakness or septal implantation Preterm labour Persistent malpresentations Retained adherent placenta
Ultrasound of the pelvis as well as the renal region Hysterogram Hysteroscope : the gold standard in the diagnosis as well as management Laparoscopy
1. Strassman utriculoplasty with a transverse fundal incision; in women with bicornuate uterus2. Jones’ operation of wedge resection of a part of fundus with the septum followed by uniting the two horns vertically; in women with septate uterus3. Hysteroscopic resection using a resectoscope, has a higher pregnancy rate that other invasive procedures4. Excision of the rudimentary horn to avoid heamatometra and ectopic pregnancy
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
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)
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
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
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.
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)