Surgical Management of Genital Abnormality

Ulun ULUĞ, M.D.Bahceci Women Health Group, Istanbul, Turkey

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

Clinical symptoms varies from cosmetic disturbances (clitoromegaly) to life threatening conditions (congenital adrenal hyperplasia)1. Dysparaneu2. Dismenorhea3. Pelvic mass4. Infertility5. Poor obstetric outcome

• Outflow tract abnormalities1. Imperforate hymen2. Longitudinal vaginal septum3. Transverse vaginal septum4. Vaginal agenesis

• Mullerian abnormalities1. Unicornuate2. Didelphys3. Bicornuate4. Septate5. Arcuate

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

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 systemsLack of standards in acquired diagnostic data

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

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.

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

American Fertility Society Classification Mullerian Anomalies

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

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

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 neovagina1. 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

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

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.

Transverse Vaginal Septum Treatment

Aspirate fluidTransverse incision and identify cervixIndwelling stentNormal coital function after excisionPregnancy 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

Longitudinal vaginal septum

lateral fusion defects lead to longitudinal septaless likely to lead to obstructionmay 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

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?

The presence of a malformed uterus increases the incidence of:

Spontaneous abortionFirst trimester bleedingPreterm laborPremature rupture of membraneAbnormal fetal positioningPlasental abruptionFetal growth restrictionFetal death

(Grimbizis et al.,2001)

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)

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)

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

20 studies consisting of 290 women with unicornuate uterus are examinedOccurs in 1:4020 women in general populationMore common in infertile women and in women with repeated poor outcomes

(Reichman et al., 2009)

Higher frequency of abortion, preterm labor, cervical shortening

Main etiologies explaining poor obstetrical outcomes:

Small cavityDiminished muscle massAbnormal uterine blood flowCervical incompetence

Unicornuate Uterus

(Reichman et al., 2009)

Expectant management

Cervical length measurement

Cervical suturing in selected cases

Remove rudimentary horn if present

Unicornuate UterusManagement

Uterus Didelphys

Non-obstructed failure of lateral fusion involving both uterus and vagina5%-7% of müllerian defectsReproductive outcomes are slightly better than unicornuate uterus

(DeviWold et al., 2006)

Spontaneous abortion rate: 43%Premature birth rate: 38%Fetal survival rate: 54%

Uterus Didelphys

(Propst AM., 2000)

Surgical intervention: Unclear. Surgical metroplasty should be

reserved for selected patients suffering from recurrent pregnancy loss or preterm births.

Uterus Didelphys

(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.

(Sinha et al., 2006; Lolis et al., 2005)

Spontaneous abortion 36%

Premature birth 21%

Fetal survival 60%

Bicornuate Uterus

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)

Uterine Septum

Reproductive outcome is poorSpontaneous abortion 26%-94% Premature labor 9%-33%Fetal survival 10%-75%Spontaneous abortion after metroplasty 5.9%

(Toriano et al., 2004)

Reproductive outcome after hysteroscopic metroplasty

(Homer et al., 2000)

Reproductive outcome H/S metroplasty

(Homer et al., 2000)

Reproductive outcome after hysteroscopic metroplasty

61 patients with septate uterus and otherwise unexplained infertilityAfter hysteroscopic metroplasty (mean 11.2 months)

41% (n:25) conceivedOf these, 18 (29.5 % of the total group) had live births 7 (11.5 % of the total group) spontaneous abortions.

(Pabuçcu & Gomel, 2004)

Grup AN=44

Septa + Unexplained infertility

Group BN=132

Unexplained infertility

Hysteroscopic metroplasty

Ekspectantmanagement

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

Pregnancy and live birth rate are significantly higher in patients who had undergone

hysteroscopic metroplasty

(Mollo et al., 2009)

Recurrent abortionsPreterm deliveryInfertility

1. unexplained infertility2. 35 years old 3. Those who will have LS or HS for other reasons4. Those who will have ART

Dysmenorrea and dysparunia

When should we operate uterine septum?

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.

Arcuate Uterus

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

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

The treatment is usually expectant.Reconstructive procedures do not improve pregnancy outcomes.

Arcuate UterusManagement

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.