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CONGENITAL MALFORMATIONS OF THE ♀ GENITAL TRACT

CONGENITAL MALFORMATIONS OF THE ♀ GENITAL TRACT. OBJECTIVES. You should understand the clinical presentations, complications and management of the following malformations: 1-Mullerian agenesis 2-Disorders of lateral fusion of the mull ducts A-uterus didelphus B- bicornuate uterus

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CONGENITAL MALFORMATIONS OF THE ♀ GENITAL TRACT

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  1. CONGENITAL MALFORMATIONS OF THE ♀ GENITAL TRACT

  2. OBJECTIVES You should understand the clinical presentations, complications and management of the following malformations: 1-Mullerian agenesis 2-Disorders of lateral fusion of the mull ducts A-uterus didelphus B-bicornuate uterus C-septate uterus D-unicornuate uterus E-unicurnuate with rudimentary horn 3-Disorders of vertical fusion of the mullerian duct A- vaginal septum B-cx AGENISIS / DYSGENISIS 4-Unusual configuration of vertical/lateral fusion defects 5-Defects of the external genitalia A-Ambiguous genitalia B-Defects of the clitoris C-Imperforate hymen

  3. 1-MULLERIAN AGENISIS • Mayer –Rokitansky-Kuster-Huser syndrome • Etiology ? • Failure of mullerian duct development  absence of the upper vagina, cx & uterus (uterine reminants may be found) • The ovaries & fallopian tubes are present • Normal 46XX ♀ with normal exrenal genitalia • Pt present with 1ry amenorroea • 47% have asociared urinary tract anomalies • 12% skeletal anomalies • Rx  psychological counseling surgical - vaginoplasty - excision of utrinereminant (if it has functioning endometrium) -vaginal dilators

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  12. 2-DISORDERS OF LATERAL FUSION OF THE MULL DUCTS Incidence ? 0.1-2% 4% of infertile pt 6-10% recurrent abortion pt Most pt can conceive without difficulty  Incidence of : ◊ recurrent abortions ◊ premature birth ◊ fetal loss ◊ fetal malpresentation ◊ C S ◊ cx incompetence

  13. 2-DISORDERS OF LATERAL FUSION OF THE M D CLINICAL PRESENTATION ♣Shortly after menarche if there is obstruction to uterine blood flow ♣Difficulty in intercorse  longitudinal vaginal septum ♣Dysmenorrhea or menorrhagia ♣Abnormality detected on D&C ♣U/S, laparoscopy or laparotomy ♣Palpable mass ♣Complications of pregnancy ♣HSG  during infertility or RFL investigations

  14. NON OBSTRUCTIVE MALFORMATIONS OF THE MULLERIANE DUCTS

  15. 2-DISORDERS OF LATERAL FUSION OF THE MULL DUCTS A-Uterus didelphus • Complete duplication of uterus, cx & vagina (due to failure of fusion of the two Mull ducts) • ?  pregnancy wastage • Dx HSG or at laparoscopy / laparotomy • Rx  If affecting pregnancy outcome  surgical correction (metroplasty) B-Bicornuate uterus • Incomplete fusion of the two Mull ducts •  pregnancy wastage • Dx HSG or at laparoscopy / laparotomy • Rx  If affecting pregnancy outcome  surgical correction (metroplasty)

  16. 2-DISORDERS OF LATERAL FUSION OF THE MULL DUCTS C-Septate uterus External contour of the uterus is normal but there is intrauterine septum of varying length & thickness Worst pregnancy outcome Dx both HSG & laparoscopy Rx  Hystroscopic excision of the septum D-Unicornuate uterus Due to development of only one Mull duct Almost all pt have associated single kidney Pregnancy outcome  similar to pt with didelphic uteri Dx  HSG or surgery Rx  NO corrective surgery  if pt has associated cx incompetence  cx cerclage

  17. 2-DISORDERS OF LATERAL FUSION OF THE MUL DUCTS E-Unicurnuate with rudimentary horn Noncommunicating horn 90% Present with cyclic pelvic pain , mass, ectopic pregnancy in the rud horn or endometriosis Rx  surgical excision Communicating horn Present with ectopic pregnancy in the rud horn or  pregnancy wastage

  18. 3-DISORDERS OF VERTICALE FUSION OF THE MULLERIAN DUCTS A- VAGINAL SEPTUM • Faults in the junction between the Mull. Tubercle & the urogenital sinus  could be very thick or thin • 85% in upper two third of the vagina • Pt present 1ry amenorrhea, hematocolpus, mass or cyclic abdominal pain •  incidence of endometriosis • Rx  surgical exision B-Cx AGENISIS / DYSGENISIS • V rare • Difficult, unsuccessful surgical correction • Rx  hysterectomy

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  21. Long vag septum S 62 S 61

  22. 4-UNUSUAL CONFIGRATION OF VERTICAL/LATERAL FUSION DEFECTS • Combined lateral & verticle defects • Do not fit in other categories • EXAMPLE, double uterus with obstructed hemivagina

  23. Double uterus with vaginal obstruction A-Complete vaginal obstruction B-Incomp vag obst C-Comp obst with comm double uterus

  24. 5-DEFECTS OF THE EXTERNAL GENITALIA • Ambiguous genitalia  congenital adrenal hyperplasia hermaphrodites • Defects of the clitoris  uncommon  bifid clitoris hypertrophied  androgen effect • IMPERFORATE HYMEN Hymen is formed at the junction of the urogenital sinus & sinovaginal bulbs Pt presents with 1ry amenorrhea with cyclic abdominal pain or hematocolpus /hematometra Rx cruciate incision

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