Mullerian Duct Anomalies

- By Dr. Poonam J NerlekarRadiology Resident (1styr),

Holy Spirit Hospital, Andheri (E), Mumbai.

EMBRYOLOGY

Embryology of the ovaries and vagina

• Ovaries develop from a gonadal ridge• Bottom third of vagina develops from the urogenital sinus

– Following this, there is fusion of upper 2/3 and lower 1/3 of vagina with degeneration of transverse septum

• Rupture of the hymen at birth

Stage 1 – Weeks 6 to 7• Stage one:

– The two separate endometrial, cervical and vaginal components

Normal

• Arrest in stage 1: Non-development– Agenesis or hypoplasia

• Class I

– Unicornuate• Class II• 20%

• 10%

Stage Two – Weeks 10 to 13• By the end of stage two:

– The two tubes fuse

Normal

• Arrest in stage 2: Non-fusion– Didelphys: complete non-fusion

• Class III

– Bicornuate: partial non-fusion• Class IV

• 5% of MDA• 75% assoc.

w/ vag septum

• 10%• 25% assoc.

w/ vag septum

Stage Three – Weeks 13 to 20 • The midline portions dissolve

Normal

• Arrest in stage 3: Non-degeneration– Septate and arcuate

• Class V and VI

• 55%• May be assoc.

w/ vag septum

ANATOMY

MULLERIAN DUCT ANOMALIES AFS CLASSIFICATION OF MULLERIAN DUCT ANOMALIES

CLASS I- UTERINE AGENESIS

• there is complete absence of uterine tissue above the vagina

CLASS II UNICORNUATE UTERUS

• There is failure of one Mullerian duct to elongate while the other develops normally. It may or may not have rudimentary horn.

CLASS III UTERUS DIDELPHYS

• there is complete duplication of uterine horns as well as duplication of the cervix, with no communication between them.

CLASS IV BICORNUATE UTERUS

• There is partial failure of fusion of the ducts.

1) Bicornuate bicollis2) Bicornuate

unicollis

CLASS V SEPTATE UTERUS

• Results from partial or complete failure of resorption of the uterovaginalseptum after fusion of the ducts

CLASS VI ARCUATE UTERUS

• There is mild indentation of the endometrium at the uterine fundus as the result of near complete resorption of the uterovaginal septum.

CLASS VII T SHAPED UTERUS

• It is the most commonly associated abnormality from in utero diethylstilbestrol (DES) exposure

ASSOCIATED ANOMALIES

• RENAL AGENESIS / RENAL ECTOPIA

• SKELETAL ABNORMALITIES = SCOLIOSIS

• MAYER ROKITANKSY KUSTER – HAUSER SYNDROME

• ABSENCE OR HYPOPLASIA OF VAGINA

TAKE HOME MESSAGE

DETAIL KNOWLEDGE OF EMBRYOLOGY IS MUST TO EVALUATE ALL DEVELOPMENTAL ANOMALIES

References:-• Troiano, R. Mullerian Duct Anomalies: Imaging and Clinical Issues.

Radiology. 2004;233(1):19-34.• Grimbizis GF. Congenital malformations of the female genital tract:. Fertil

Steril. 2010;94(2):401-407.• Simon C, Martinez L, Pardo F, et al. Mullerian defects in women with

normal reproductive outcome. Fertil Steril. 1991;56(6):1192-3.• Agrawal, G. et al. Evaluation of Uterine Anomalies: 3D FRFSE Cube versus

Standard 2D FRFSE. Am. J. Roentgenol. 2009;193:558-562.• Troiano, R. Mullerian Duct Anomalies: Imaging and Clinical Issues.

Radiology. 2004;233(1):19-34• Gray, Henry (1858), Anatomy Descriptive and Surgical, London: John W.

Parker and Son, retrieved 16 October 2011 Online- and PDF versions of the 1st edition at Open Library/Internet Archive.

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