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Congenital malformation of female
genital tract
Dr Abenezer G.(R-II)
Moderator: Dr Amenu(urogynecology and
pelvic reconstructive surgeon)
2/8/2024 Congenital malformation of female
genital tract
1
OUTLINE
• Introduction
• Common anomalies
• Diagnosis
• Managment
2/8/2024 Congenital malformation of female
genital tract
2
• There are four common developmental defects of the mullerian
system to consider:
 Agenesis of both ducts
 Unilateral maturation of one müllerian duct
 Absent/ faulty midline fusion of the ducts; or
 Defective canalization.
• Include Agenesis(MRKH) and specific parts.
2/8/2024 3
Congenital malformation of female
genital tract
DEVELOPMENTAL DEFECTS:
ANOMALIES OF THE HYMEN
• Results from incomplete degeneration of central
portion of the hymen.
• Anatomic variants include imperforate, microperforate,
septate, and cribriform hymens
• Include imperforate hymen and incomplete hymenal
fenestration.
2/8/2024 Congenital malformation of female genital tract 4
Imperforate hymen
• Most common obstructive lesions of the genital tract.
• Presentation: bulging introitus & cyclic pain and
hematocolpos
• Timing: At birth, infants or adolescent girl.
• Rx: Hymectomy/Cruciate incision
Timing: Newborn, postpubertal, or premenarchal.
Give certificate to child
2/8/2024 Congenital malformation of female genital tract 6
Incomplete hymenal fenestration
• Incomplete fenestration of the hymenal opening.
• Presentation: inability to insert tampons, douches, or
vaginal creams, or because of difficulty with coitus.
• Rx: Resection of the excess hymenal tissue.
2/8/2024 Congenital malformation of female genital tract 8
Anomalies of vagina
• May be isolated or associated with other müllerian anomalies.
• Include MRKH, MURCS (müllerian duct aplasia, renal aplasia,
and cervico-thoracic somite dysplasia).
• Obstetrical significance depends on degree of obstruction.
• Complete vaginal agenesis, unless corrected surgically,
precludes pregnancy by vaginal intercourse.
2/8/2024 Congenital malformation of female genital tract 9
1. Transverse vaginal septum
2. Longitudinal vaginal septum
3. Obstructed hemi-vagina
4. Agenesis of vagina
5. Agenesis of lower vagina
6. Vaginal cyst
2/8/2024 10
Congenital malformation of female
genital tract
cont.....
Transverse vaginal septum:
Definition:
• Failure of fusion and/or canalization of the
urogenital sinus and müllerian ducts.
Depending on the site:
• Upper vagina(46%)
• Middle portion(35-40%)
• Lower vagina(15-20%)
11
2/8/2024 12
Congenital malformation of female
genital tract
Anomalies of Cervix
• Include agenesis, duplication,or a longitudinal dividing
septum.
• Complete agenesis is incompatible with pregnancy.
• IVF with gestational surrogacy or with transmyometrial
embryo transfer are options.
• Uterovaginal anastomosis??
2/8/2024 Congenital malformation of female genital tract 13
Anomalies of uterus
2/8/2024 14
Congenital malformation of female
genital tract
• Prevalence, 0.4 to 10 percent.
• Arcuate->septate->bicornuate->didelphic->unicornuate.
• Pose greater risk for miscarriage, malpresentation,
preterm birth, and poor fetal growth.
 Sonography, HSG, MRI, laparoscopy, and hysteroscopy
may also be diagnostic.
2/8/2024 15
Congenital malformation of female
genital tract
16
2/8/2024 17
Congenital malformation of female
genital tract
2/8/2024 18
Congenital malformation of female
genital tract
Müllerian Agenesis (Class I)
• Caused by müllerian hypoplasia or agenesis as
• Can affect the vagina, cervix, uterus, or fallopian tubes
• Can also be isolated or may coexist with other müllerian
anomalies.
Unicornuate Uterus (Class II)
• Incidence of 1 case in 4000 women.
• Detected accidentally during fertility evaluation by HSG.
• Include Main horn and Rudimentary horn.
• Can be communicating & Non-communicating.
• Main obstetrical significance is cornual px,
• Rx: surgical removal of rudimentary horn and in situ
pregnancy.
2/8/2024 Congenital malformation of female genital tract 19
Uterine Didelphys (Class III)
• Results from incomplete fusion.
• Suspected on PV by identification of longitudinal vaginal
septum and two cervices.
• HSG: shows two separate endocervical canals.
• Each separate cavities have a solitary fallopian tube.
• Rx: Surgical Metroplasty.
- Scheduled delivery is recomended after Rx.
2/8/2024 Congenital malformation of female genital tract 20
Bicornuate Uterus (Class IV)
• Results in two hemiuteri.
• Central myometrium runs either partially or completely
to the cervix.
• Can be bicornuate unicollis or bicornuate bicollis.
• Radiological discrimination of a bicornuate from a septate
uterus is challenging.
2/8/2024 Congenital malformation of female genital tract 21
Septate Uterus (Class V)
• Results from resorption defect with persistent complete
or partial longitudinal uterine septum.
• Many Dxsed during evaluation of infertility or RPL.
• 3D TVS or MR imaging is required to differentiate
septate and bicornuate uteri.
• Rx: Hysteroscopic septal resection.
2/8/2024 Congenital malformation of female genital tract 23
Arcuate Uterus (Class VI)
• Mild deviation of normally developed uterus.
• Most consider this anomaly benign
• Obstetrical significance : 2nd Px losses, preterm labor,
and malpresentation.
2/8/2024 Congenital malformation of female genital tract 24
Cesarean Delivery
• Failed TOLAC or risk of rupture may stem from
Smaller than normal cavity size,
Abnormal propagation of myometrial action potentials
Weaker scar site due to altered vascular anatomy.
• ECV is reasonable to avoid primary C/S.
2/8/2024 Congenital malformation of female genital tract 25
Treatment with Cerclage
• Indication:
• RPL after 1st TM.
• Partial cervical atresia or hypoplasia.
• Candidacy for cervical length is similar with general population.
2/8/2024 Congenital malformation of female genital tract 26
DES-related Abnormalities (Class VII)
• Exposed women show a cervix or vagina with
transverse septum.
• Uteri are potentially smaller or have a T-shaped cavity.
2/8/2024 Congenital malformation of female genital tract 27
Anomalies of Fallopian Tube
• Develop from unpaired distal ends of the müllerian ducts.
• Includes accessory ostia, complete or segmental tubal
agenesis, and several embryonic cystic remnants.
• The most common—the hydatid of Morgagni.
2/8/2024 Congenital malformation of female genital tract 28
Obstetric complications
• Miscarriage,
• Prematurity,
• IUGR
• APH &PPH
• Cervical
incompetence
• Malpresentati
on
• HDP
• Cesarean delivery
• RPL
• Preterm delivery
• Uterine rupture
 Most common, uterine
septum and least common,
arcuate uterus.
2/8/2024 29
Congenital malformation of female
genital tract
Diagnosis
• Hx: Pain, Pelvic mass, Dysmenorrhea, Dyspareunia,
Infertility, or recurrent pregnancy loss.
• Ix: Ultrasound
• Hysterosalphingography
• MRI
• Laparascopy
• Hysteroscopy
2/8/2024 Congenital malformation of female genital tract 30
Managment
• Directs to type of Anomalies part.
• Can be
» Surgical or
» Non-surgical.
2/8/2024 Congenital malformation of female genital tract 31

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Female genital tract(Congenital malformation).pptx

  • 1. Congenital malformation of female genital tract Dr Abenezer G.(R-II) Moderator: Dr Amenu(urogynecology and pelvic reconstructive surgeon) 2/8/2024 Congenital malformation of female genital tract 1
  • 2. OUTLINE • Introduction • Common anomalies • Diagnosis • Managment 2/8/2024 Congenital malformation of female genital tract 2
  • 3. • There are four common developmental defects of the mullerian system to consider:  Agenesis of both ducts  Unilateral maturation of one müllerian duct  Absent/ faulty midline fusion of the ducts; or  Defective canalization. • Include Agenesis(MRKH) and specific parts. 2/8/2024 3 Congenital malformation of female genital tract DEVELOPMENTAL DEFECTS:
  • 4. ANOMALIES OF THE HYMEN • Results from incomplete degeneration of central portion of the hymen. • Anatomic variants include imperforate, microperforate, septate, and cribriform hymens • Include imperforate hymen and incomplete hymenal fenestration. 2/8/2024 Congenital malformation of female genital tract 4
  • 5.
  • 6. Imperforate hymen • Most common obstructive lesions of the genital tract. • Presentation: bulging introitus & cyclic pain and hematocolpos • Timing: At birth, infants or adolescent girl. • Rx: Hymectomy/Cruciate incision Timing: Newborn, postpubertal, or premenarchal. Give certificate to child 2/8/2024 Congenital malformation of female genital tract 6
  • 7.
  • 8. Incomplete hymenal fenestration • Incomplete fenestration of the hymenal opening. • Presentation: inability to insert tampons, douches, or vaginal creams, or because of difficulty with coitus. • Rx: Resection of the excess hymenal tissue. 2/8/2024 Congenital malformation of female genital tract 8
  • 9. Anomalies of vagina • May be isolated or associated with other müllerian anomalies. • Include MRKH, MURCS (müllerian duct aplasia, renal aplasia, and cervico-thoracic somite dysplasia). • Obstetrical significance depends on degree of obstruction. • Complete vaginal agenesis, unless corrected surgically, precludes pregnancy by vaginal intercourse. 2/8/2024 Congenital malformation of female genital tract 9
  • 10. 1. Transverse vaginal septum 2. Longitudinal vaginal septum 3. Obstructed hemi-vagina 4. Agenesis of vagina 5. Agenesis of lower vagina 6. Vaginal cyst 2/8/2024 10 Congenital malformation of female genital tract cont.....
  • 11. Transverse vaginal septum: Definition: • Failure of fusion and/or canalization of the urogenital sinus and müllerian ducts. Depending on the site: • Upper vagina(46%) • Middle portion(35-40%) • Lower vagina(15-20%) 11
  • 12. 2/8/2024 12 Congenital malformation of female genital tract
  • 13. Anomalies of Cervix • Include agenesis, duplication,or a longitudinal dividing septum. • Complete agenesis is incompatible with pregnancy. • IVF with gestational surrogacy or with transmyometrial embryo transfer are options. • Uterovaginal anastomosis?? 2/8/2024 Congenital malformation of female genital tract 13
  • 14. Anomalies of uterus 2/8/2024 14 Congenital malformation of female genital tract • Prevalence, 0.4 to 10 percent. • Arcuate->septate->bicornuate->didelphic->unicornuate. • Pose greater risk for miscarriage, malpresentation, preterm birth, and poor fetal growth.  Sonography, HSG, MRI, laparoscopy, and hysteroscopy may also be diagnostic.
  • 15. 2/8/2024 15 Congenital malformation of female genital tract
  • 16. 16
  • 17. 2/8/2024 17 Congenital malformation of female genital tract
  • 18. 2/8/2024 18 Congenital malformation of female genital tract Müllerian Agenesis (Class I) • Caused by müllerian hypoplasia or agenesis as • Can affect the vagina, cervix, uterus, or fallopian tubes • Can also be isolated or may coexist with other müllerian anomalies.
  • 19. Unicornuate Uterus (Class II) • Incidence of 1 case in 4000 women. • Detected accidentally during fertility evaluation by HSG. • Include Main horn and Rudimentary horn. • Can be communicating & Non-communicating. • Main obstetrical significance is cornual px, • Rx: surgical removal of rudimentary horn and in situ pregnancy. 2/8/2024 Congenital malformation of female genital tract 19
  • 20. Uterine Didelphys (Class III) • Results from incomplete fusion. • Suspected on PV by identification of longitudinal vaginal septum and two cervices. • HSG: shows two separate endocervical canals. • Each separate cavities have a solitary fallopian tube. • Rx: Surgical Metroplasty. - Scheduled delivery is recomended after Rx. 2/8/2024 Congenital malformation of female genital tract 20
  • 21. Bicornuate Uterus (Class IV) • Results in two hemiuteri. • Central myometrium runs either partially or completely to the cervix. • Can be bicornuate unicollis or bicornuate bicollis. • Radiological discrimination of a bicornuate from a septate uterus is challenging. 2/8/2024 Congenital malformation of female genital tract 21
  • 22.
  • 23. Septate Uterus (Class V) • Results from resorption defect with persistent complete or partial longitudinal uterine septum. • Many Dxsed during evaluation of infertility or RPL. • 3D TVS or MR imaging is required to differentiate septate and bicornuate uteri. • Rx: Hysteroscopic septal resection. 2/8/2024 Congenital malformation of female genital tract 23
  • 24. Arcuate Uterus (Class VI) • Mild deviation of normally developed uterus. • Most consider this anomaly benign • Obstetrical significance : 2nd Px losses, preterm labor, and malpresentation. 2/8/2024 Congenital malformation of female genital tract 24
  • 25. Cesarean Delivery • Failed TOLAC or risk of rupture may stem from Smaller than normal cavity size, Abnormal propagation of myometrial action potentials Weaker scar site due to altered vascular anatomy. • ECV is reasonable to avoid primary C/S. 2/8/2024 Congenital malformation of female genital tract 25
  • 26. Treatment with Cerclage • Indication: • RPL after 1st TM. • Partial cervical atresia or hypoplasia. • Candidacy for cervical length is similar with general population. 2/8/2024 Congenital malformation of female genital tract 26
  • 27. DES-related Abnormalities (Class VII) • Exposed women show a cervix or vagina with transverse septum. • Uteri are potentially smaller or have a T-shaped cavity. 2/8/2024 Congenital malformation of female genital tract 27
  • 28. Anomalies of Fallopian Tube • Develop from unpaired distal ends of the müllerian ducts. • Includes accessory ostia, complete or segmental tubal agenesis, and several embryonic cystic remnants. • The most common—the hydatid of Morgagni. 2/8/2024 Congenital malformation of female genital tract 28
  • 29. Obstetric complications • Miscarriage, • Prematurity, • IUGR • APH &PPH • Cervical incompetence • Malpresentati on • HDP • Cesarean delivery • RPL • Preterm delivery • Uterine rupture  Most common, uterine septum and least common, arcuate uterus. 2/8/2024 29 Congenital malformation of female genital tract
  • 30. Diagnosis • Hx: Pain, Pelvic mass, Dysmenorrhea, Dyspareunia, Infertility, or recurrent pregnancy loss. • Ix: Ultrasound • Hysterosalphingography • MRI • Laparascopy • Hysteroscopy 2/8/2024 Congenital malformation of female genital tract 30
  • 31. Managment • Directs to type of Anomalies part. • Can be » Surgical or » Non-surgical. 2/8/2024 Congenital malformation of female genital tract 31