Doç. Dr. Ulun Uluğ

1. Surgical Management of Genital
Abnormality

2. Uterine anomalies are more common than
generally recognized by many practicing
clinicians.
Prevalence in general population :1 in 201
(0.5%)
According to “Acien,” 2-3% of fertile women
and 3% of infertile women and 5-10% of those
with repeated miscarriages.
Distribution: 7% arcuate, 34% septate, 39%
bicornuate, 11% didelphic, 5% unicornuate, 4%
hypoplastic/asplastic/solid and other forms

3. Clinical symptoms varies from cosmetic disturbances (clitoromegaly) to
life threatening conditions (congenital adrenal hyperplasia)
1. Dysparaneu
2. Dismenorhea
3. Pelvic mass
4. Infertility
5. Poor obstetric outcome

5. • Outflow tract abnormalities
1. Imperforate hymen
2. Longitudinal vaginal septum
3. Transverse vaginal septum
4. Vaginal agenesis
• Mullerian abnormalities
1. Unicornuate
2. Didelphys
3. Bicornuate
4. Septate
5. Arcuate

6.  Congenital genital abnormalities encompass
embrionic maldevelopment of paramesonephric
ducts and malapproximation with urogenital sinüs
 Therefore congenital genital abnormalities carry
out disorders of internal and external genitalia
and sometimes both
 Underlying etiologies are genetic, hormonal and
usually multifactorial

7. Introduction
 True incidence and prevalence are difficult to
assess. Why?
Selection bias:
0.4% in nonobstetric cases
8-10% in recurrent pregnancy loss
Nonstandardized classification systems
Lack of standards in acquired diagnostic data

8. Why is this important?
 The majority have no problem conceiving,
but have higher rates of:
 1. Spontaneous Abortion
 2. Premature Delivery
 3. Infertility
 4. Abnormal Fetal Lie
 5. Dystocia at delivery
 6. Dysmenorrhea, endometriosis
 7. Cervical incompetence

9. Mullerian anomalies are usually associated with
malformations of kidney and skeleton.
They are often diagnosed as late as puberty when
menarche does not occur or sexual activity is not
possible or even later if infertility is the only
symptom.

10. There are 2 types of classification, which both
needs clarification. A new revised standart system
is needed
Oppelt et al
• Vulvar cervical uterin adnexal
and associated malformations
(VCUAM)
AFS classification

11. American Fertility Society Classification
Mullerian Anomalies

12. Congenital Malformation of The Vagina
 remnant cyst
 obstructive outflow tract disorders
1. Imperforate hymen
2. Transverse vaginal septa
3. Longitudinal vaginal septum
 congenital absence of the vagina

13. Mullerian Agenesis (MRKH Syndrome)
 Expected Menarche
 Difficult to differentiate from imperforate hymen
 No uterus on exam, U/S, MRI, Laparoscopy, IVP
 Confused with Androgen Resistance Syndrome
with shallow pouch and no uterus.
 Determine karyotype.
 47% have associated urinary tract anomalies
 12% Skeletal Anomalies

14. MRKH Syndrome: Treatment
Goal is to restore normal sexual function through creation of
neovagina.
Nonsurgical:
1. Frank technique: Pressure to perineal dimple.
2. Ingram method: Bicycle seat stool.
Surgical: Create neovagina
1. Surgical creation of the neovaginal space
(McIndoe)
2. Bowel vaginoplasty
3. Neovaginoplasty using peritoneum(Davydov )
4. Vecchietti operation: transabdominal approach.
5. Vaginoplasty using skin flaps

15. Imperforate Hymen: Diagnosis/ Treatment
 Cyclic pelvic pain due to hematocolpos
hematometria, or hematosalpinx
 Bulging hymeneal membrane or a blind-ending
pouch on exam.
 Pelvic/Rectal exam, U/S, MRI
 Rarely urologic anomalies.
 Tx: Cruciate incision

16. Transverse Vaginal Septum:
Presentation/Diagnosis
Cyclical pain due to hematocolpos
or hematometria.
Blind-ending pouch.
No bulging at outlet, hydromucocolpos or
hematocolpos, rectal exam or U/S, MRI.
Incidence 1 in 2100 to 1 in 72000.
Thickness varies and site varies in vaginal canal.
19% lower 1/3, 35% middle third, 46% upper third.

17. Transverse Vaginal Septum
Treatment
Aspirate fluid
Transverse incision and identify
cervix
Indwelling stent
Normal coital function after excision
Pregnancy rates: More likely 40-50% especially if
septum in lower or middle third of vagina.
Goal is to relieve cyclic abdominal pain and prevent
development of endometriosis

18. Longitudinal vaginal septum
lateral fusion defects lead to longitudinal septa
less likely to lead to obstruction
may not be found until the initiation of sexual
activity or pelvic examinations.
Surgical Correction
If dyspareunia is present
To minimize the risk of major trauma during
delivery

19. Mullerian Anomaly??
How common?
Is further screening required for other disorders?
How does this abnormality occur?
What is the likelihod of having an affected child with
the same anomalies?
What is the probability of delivering a healthy child?
What are the specific obstetric challenges?
Would medical or surgical interventions improve the
outcome?
Decreased fertility?

20. The presence of a malformed uterus
increases the incidence of:
Spontaneous abortion
First trimester bleeding
Preterm labor
Premature rupture of membrane
Abnormal fetal positioning
Plasental abruption
Fetal growth restriction
Fetal death
(Grimbizis et al.,2001)

21. Uterine anomalies and pregnancy outcome
 105 women with uterine anomaly vs 182
women with normal shaped uterus:
 Highest incidence of early spontaneous
abortion in septate uterus
 Highest incidence of preterm labor in arcuate
and bicornuate uterus
(Zlopasa G. 2007)

22. tr= trimester, pr= pregnancy(Zlopasa G, 2007)
Pregnancy outcome before and after hysteroscopic
treatment of anomaly in 25 women
Outcome Before
Treatment
After
treatment
1st tr loss 34 (77.3) 18 (34.6) <0.001
2nd tr loss 7 (15.9) 3 (5.8)
Total pr loss 41 (93.2) 21 (40.4) <0.001
Term
Deliveries
0 23 (44.2)

23. Class II Abnormality (Unicornuate Uterus)
 Accounts 20% for all mullerian abnormalities
 Usually on the right side
 Rudimentary horn are detected in 70%
 About 50% of rudimentary horns have
endometrial tissue
 Primary symptom may be dysmenorrhea
 Associated with spontaneous abortion and
preterm delivery

24. 20 studies consisting of 290 women with
unicornuate uterus are examined
Occurs in 1:4020 women in general population
More common in infertile women and in
women with repeated poor outcomes
(Reichman et al., 2009)

26. Main etiologies explaining poor obstetrical
outcomes:
Small cavity
Diminished muscle mass
Abnormal uterine blood flow
Cervical incompetence
Unicornuate Uterus
(Reichman et al., 2009)

27.  Expectant management
 Cervical length measurement
 Cervical suturing in selected cases
 Remove rudimentary horn if present
Unicornuate Uterus
Management

28. Uterus Didelphys
Non-obstructed failure of lateral fusion involving both
uterus and vagina
5%-7% of müllerian defects
Reproductive outcomes are slightly better than
unicornuate uterus

29. (DeviWold et al., 2006)
Spontaneous abortion rate: 43%
Premature birth rate: 38%
Fetal survival rate: 54%
Uterus Didelphys

30. (Propst AM., 2000)
 Surgical intervention: Unclear.
 Surgical metroplasty should be reserved for
selected patients suffering from recurrent
pregnancy loss or preterm births.
Uterus Didelphys

31. (Reichman et al., 2010)
Class IV (Bicornuate Uterus)
 Is a result of incomplete fusion of the uterine horns at the
level of fundus
 Represents 10% of müllerian duct anomalies
 Obstetrical outcomes are reported to be better than
unicornuate uterus.

32. (Sinha et al., 2006; Lolis et al., 2005)
Spontaneous abortion 36%
Premature birth 21%
Fetal survival 60%
Bicornuate Uterus

33. Class V (Septate Uterus)
 The most common
uterine anomaly is
septate uterus with a
mean incidence of
35%
(Tulandi et al., 1980; Acien, 1993; Raga et al., 1997; Woelfer et al., 2001, Zlopasa, 2009)

34. Uterine Septum
Reproductive outcome is poor
Spontaneous abortion 26%-94%
Premature labor 9%-33%
Fetal survival 10%-75%
Spontaneous abortion after
metroplasty 5.9%
(Toriano et al., 2004)

35. Reproductive outcome after hysteroscopic
metroplasty
(Homer et al., 2000)

36. Reproductive outcome H/S
metroplasty
(Homer et al., 2000)

37. Reproductive outcome after hysteroscopic
metroplasty
61 patients with septate uterus and otherwise
unexplained infertility
After hysteroscopic metroplasty (mean 11.2
months)
41% (n:25) conceived
Of these, 18 (29.5 % of the total group) had
live births
7 (11.5 % of the total group) spontaneous
abortions.
(Pabuçcu & Gomel, 2004)

38. Grup A
N=44
Septa + Unexplained infertility
Group B
N=132
Unexplained infertility
Hysteroscopic
metroplasty
Ekspectant
management
All women were followed-up for 1 year
without any intervention
(Mollo et al., 2009)
Hysteroscopic resection of the septum improves
the pregnancy rate of women with unexplained
infertility: a prospective controlled trial

39. Pregnancy and live birth rate are significantly
higher in patients who had undergone
hysteroscopic metroplasty
(Mollo et al., 2009)

40. Recurrent abortions
Preterm delivery
Infertility
1. unexplained infertility
2. 35 years old
3. Those who will have LS or HS for other
reasons
4. Those who will have ART
Dysmenorrea and dysparunia
When should we operate uterine septum?

41. Class VI (Arcuate Uterus)
The near complete resorption
of the uterovaginal septum may
lead to a mild concave
indentation of the cavity, giving
the uterus an arcuate
configuration.

42. Arcuate Uterus
In a retrospective case series of 176 patients
45% early abortion rate was reported.
Acien et al, Hum Reprod 1993
In contrast, another study reported only 13%
early miscarriage rate.
Raga F et al, Hum Reprod 1997

43. Most of studies report an increase in adverse
reproductive outcomes, mostly second
trimester loss.
Tulandi et al., 1980; Acien, 1993; Raga et al.,
1997; Woelfer et al., 2001, Zlopasa, 2009.
Arcuate Uterus

44. The treatment is usually expectant.
Reconstructive procedures do not improve
pregnancy outcomes.
Arcuate Uterus
Management

45. Conclusion
 Genital anomalies consist of a wide range of
defects that may vary from patient to patient.
Therefore, their management must also be
individual, taking anatomical and clinical
characteristics into consideration, as well as the
patient's wishes.