mullerian anomalies

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Department of Obstetrics & Gynaecology
Obstetrics & Gynaecology RKMSP & VIMS, Kolkata
Date-30 Apr 2020
EMBRYOLOGY: MULLERIAN DUCT
of dorsal celomic epithelium.
corresponding Wolffian ducts.
the fellow from opposite side.
• Upper vertical part lateral to Wolffian duct,
forms part of fallopian tubes.
• Middle horizontal part forms remaining of
fallopian tubes.
forms uterus, cervix and upper 2/3rd of vagina.
• HoxA9 gene» fallopian tube
Contd…
MECHANISM
three (3) phases of development:
1. ORGANOGENESIS
2. FUSION
eg. uterine agenesis or hypoplasia, unicornuate
uterus.
segment of paired Mullerian duct fuse eg. uterine
didelphys, bicornuate uterus, arcuate uterus.
Vertical fusion: Fusion of ascending
sinovaginal bulb with descending
mullerian duct - transverse vaginal
uterocervical cavity - septate uterus.
• Genes affecting: WT1; Pax 2; WNT 2; PBX 1; HOX
• Associated renal anomalies (30–50% cases)
Unilateral renal agenesis
Severe renal hypoplasia
CLASSIFICATION
• In classic mullerian agenesis, a shallow vaginal pouch (1-2
inches) remains.
• Genotype: 46XX
• Phenotype: female
renal.
• Secondary sexual characters normal.
• Vaginal vault may be completely absent or short vaginal port can be present.
• Hormonal profile: normal
MRI
Coexisting renal abnormality can be identified.
Contd…
Treatment:
FRANK method (1938): Sequential application of
graduated hard glass dilators.
INGRAM’S passive dilatation (1981): Dilators affixed to a
bicycle seat mounted upon a stool; 30 mins – 2 hrs daily.
Silicon dilators.
between bladder and rectum, lining with split thickness
skin graft from buttock or thigh.
Modified McINDOE methods: Use of buccal mucosa,
human amnion, absorbable adhesion barriers as
neovaginal lining.
from the pelvis into newly created vaginal space and then
to the introitus.
create an apparatus for passive vaginal dilatation.
UTERINE TRANSPLANTATION: HARVEST AND
females.
• Types:
• Embryologically, the urogenital sinus fails to contribute its expected caudal
portion of vagina (Simpson, 1999).
• Lower portion of vagina (1/5th to 1/3rd of total length) is replaced by 2–3 cm
fibrous tissue.
• Diagnosis:
2. USG: Displays upper reproductive tract organs
3. MRI: Length of atresia, amount of upper vaginal dilatation, identify cervix.
Contd…
• Diagnosis made from history and imaging.
Treatment:
Niver (1980) and others reported creation of an epithelialized
endocervical tract and vagina.
reproduction options (Doyle, 2009).
fails to develop or develop incompletely.
• Types:
unicornuate uterus
unicornuate uterus
Without any rudimentary horn.
TYPES OF UNICORNUATE UTERUS
• Risk of spontaneous abortion and preterm labour is more due to:
Reduced uterine capacity
Associated cervical incompetence.
• Pregnancy may occur in the rudimentary horn (both communicating and
non-communicating)
preconceptionally.
Contd…
ducts.
each with an endometrial cavity and uterine cervix.
• Good reproductive prognosis due to improved
blood supply from collateral connections between
two horns.
BICORNUATE UTERUS
Mullerian ducts.
• Can be confused with septate uterus, but
discrimination is important as septate
uterus can be treated more easily.
• Treatment: STRASSMAN Metroplasty.
segment to regress.
• Types: 1. Partial
Partial or complete implantation on the avascular septum.
Distorted uterine cavity
• Diagnosis done by USG, HSG and diagnostic hysteroscopy.
• Treatment:
because:
Chances of adhesions subsequent infertility.
• 87% live birth following hysterescopic resection.
• 70% live birth following metroplasty.
Contd…
• Slight midline septum within a broad fundus,
sometimes with a minimal fundal cavity indentation.
• Most clinicians reported no impact in reproductive
outcome.
• HSG:
indentation.
• MRI:
similar to myometrium.
absence of other causes of RPL.
Contd…
• Affects gene regulation: suppresses WNT4 gene and
alters Hox gene expression.
adenocarcinoma of vagina and cervix; transverse
septa; circumferential ridges involving cervix and
vagina; cervical collars or “cockscomb cervix”.
IMAGING MODALITIES
• Intercornual distance:
o Septate uterus: <75*.
• T- shaped uterus: Only anomaly where HSG plays a significant role.
• A hypoplastic, irregular, T shaped uterine cvity: in-utero DES exposure.
Contd…
ULTRASONOGRAPHY
• Also diagnoses associated renal anomalies.
Hypoplasia / Agenesis:
Unicornuate uterus:
Banana-shaped uterus
Laterally positioned
similar to myometrium.
Contd…
Concave fundus with fundal cleft >1 cm.
Increased intercornual distance >4 cm.
Intervening septum echogenicity similar to myometrium.
Septate uterus:
Contd…
• The coronal plane shows the entire endometrial canal and its
relation to myometrium and serosa.
• Accurately analyses uterine structure, contour of fundus,
Muscular thickness, septal length.
• Also important to evaluate concomitant renal anomaly.
Unicornuate uterus with hematometra in
rudimentary horn

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