CONGENITAL MALFORMATIONS OF THE GENITAL TRACT AND PREGNANCY

Presented by Click to edit Master subtitle style Abhinay Bhugoo

Embryologicalmechanism

ClassificationafterMusset(1964)

ClassificationafterAmericanFertilitySociety AFS(1988)

Bilateral Completelethal IncompleteMayer-Rokitansky-KsterHausersyndrome

TypeI

AplasiaoragenesisoftheMllerian ducts
Unilateral Completetrueunicornuateuterus Incompletepseudounicornuateuterus TypeII Unicornuateuterus IIa:withcommunicatingrudimentaryhorn IIb:withnoncommunicatingrudimentaryhorn IIc:rudimentaryhornwithoutcavity IId:withoutrudimentaryhorn

Didelphysuterusorbicornuatebicervicaluterus

TypeIII Uterusdidelphys TypeIV Bicornuateuterus IVa:complete IVb:partial

Anomalyofthefusion

Bicornuateunicervicaluterus Total Subtotal Fundic Septateuterus Total Sub-total Corporal(onlytheuterinebody) Fundal

TypeV Uterusseptate Va:complete Vb:partial

Anomalyoftheresorption

Arcuateuterus

TypeVI Arcuate uterus according withAFS this form can be consideredasapartiallyseptateuterus(fundal) TypeVII DES(diethylstilbestrol)uterus(iatrogenic)

Hypoplasia

Uterinehypoplasia DES(diethylstilbestrol)syndrome

Uterine fusion anomalies

A. Normal uterus

Unicornuate uterus

Arcuate uterus

Septate uterus

Bicornuate uterus

Didelphic uterus with a septate vagina

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)

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

DISORDERS OF LATERAL FUSION OF THE MUL DUCTS E-Unicornuate 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

Obstetrical risk

Midtrimester abortion which may be recurrent Cornual pregnancy with inevitable rupture around 16th week-if pregnancy occurs in the rudimentary horn Increased incidence of malpresentationtransverse lie in arcuate or subseptate, breech in the bicornuate, unicornuate or complete septate uterus Preterm labour, IUGR, IUD Prolonged labour-date to incordinate uterine action Obstructed labour-obstruction by the non gravid horn of the bicornuate uterus or

Ute

Very reduced volume of uterushypoplastic uterus

T-shaped uterus

Unic

Difficult to differentiate from the normal uterus by USG. Suspected when the uterus appears small & laterally positioned.

Bicornua

Bicornuate Bicollis Uterus

Bicornuate uterus

Gravid bicornuate uterus, pregnancy in right horn (RH).

Bicornuate uterus

Gravid bicornuate uterus, pregnancy in right horn (RH).

Uterine Didelphis
Sonography of the separate patient. This is an extreme example of complete failure of fusion of the 2 halves of the uterus (Mullerian ducts) during the fetal stage cervices uterus and

shows 2 separate uteri, 2 separate vagina in this female

Uterine Didelphis

Double cavity uterus

completely separate right and left cavities

Septate uterus

Pregnant Septate uterus

Fundal septum is seen in this patient with bicornuate uterus. The placenta is implanted posteriorly within the right horn and attaches onto the septal wall.

Septate uterus

Longitudinal section of the fetus showing its location belong the uterine septum within the amniotic cavity that had spread to the both uterine horns.

Pregnant Septate uterus

The fetal head situated in the right uterine horn.

Management

Hysteroscopic metroplasty Assisted reproductive techniques