Mullerian Anomalies Flashcards
What is the incidence of mullerian anomalies in the general population?
2-3%
In the pop’n with RPL, the incidence is 5-10%
and in the pop’n with recurrent T2 loss, it is 25%
Choose all correct answers:
a) the mullerian ducts develop into the uterus, tubes, and upper 1/3 of the vagina
b) the mullerian ducts develop medial to the wolffian ducts which ultimately gives rise to the male reproductive organs (under the influence of testosterone) and the kidneys/ureters
c) the external genitalia and lower vagina develop from the urogenital sinus
d) MIS AKA AMH leads to the regression of the wolffian system
e) gene mutations in WNT are associated with Meyer-Rokitansky-Kuster-Hauser syndrome
a) true
b) false lateral
c) true
d) false -leads to regression of mullerian system in males
e) true -mutation leads to mullerian agenesis aka MRKH as this gene is response for the initiation of mullerian duct formation
Choose all correct answers:
a) the expression of specific HOX genes determines the tissue identity for structures in the mullerian duct
b) DES alters HOX gene expression leading to mullerian anomalies
c) embryos initially develop either a mullerian system or wolffian system based on the presence/abscence of MIS on Y chromosome
a) true
b) true
c) MIS causes mullerian regression in males but both systems develop initially
What’s the diagnosis?
a) normal telarche, sparse pubic hair, blind vaginal pouch
b) amenorrhea, no pain, blind vaginal pouch, normal telarche/adrenarche
c) amenorrhea, progressive monthly pain
a) androgen insensitivity syndrome
b) mullerian agenesis
c) transverse anatomic anomaly (septum, imperforate hymen, cervical agenesis, etc)
Pick all correct answers:
a) renal anomalies are associated with mullerian anomalies in 10% of cases. Most common is unilateral renal agenesis.
b) 1st line imaging to rule out renal anomaly is IV pyelogram or renal ultrasound
a) 30% of cases: anomalies can include ipsilateral renal agenesis, horseshoe shaped kidney, duplex collecting system, renal duplication
b) true
List implications for pregnancy with mullerian anomalies:
- recurrent T1 loss
- recurrent T2 loss
- inc ectopic risk
- inc risk PTD
- inc risk malpresentation
List the ASRM classification of mullerian anomalies:
- agenesis
- unicornuate (can be communicating or not)
- didelphys
- bicornuate
- septet
- arcuate
- DES exposure (T shaped)
What associated anomalies must be ruled out in a patient with Mayer-Rokitansky-Kuster-Hauser syndrome?
renal, cardiac, skeletal, and auditory
these are present in 58% of patients
What counselling would you do around fertility for a patient with mullerian agenesis/MRKH?
normal ovaries, can use gestational carrier with own eggs
can make functional vagina (90% non-surgical with dilators), primary surgical option is Abbe-McIndoe procedure
in presence of functional mullerian remnant, recommend laparoscopic removal
How is a unicornuate uterus formed?
failure of one mullerian duct to fully elongate and meet the urogenital sinus (can be communicating or non-communicating with or without functional endometrium in rudimentary horn)
How does a didelphys uterus happen?
mullerian ducts fail to fuse leading to two completely separate uterine cavities
complete or partial longitudinal vaginal septum associated with didelphys in 75% of cases
What is OHVIRA
associated with uterine didelphys: obstructed hemi-vagina and ipsilateral renal anomaly syndrome
-associated with progressive pain from hematocolpos on obstructed side
How does a bicornuate uterus form? At what gestational age does this happen?
Incomplete fusion of the the mullerian ducts at the level of the uterine fundus at 9th week of gestation
How does a septate uterus form?
Failure of resorption of septum between mullerian ducts. Most common type of mullerian anomaly. Can be resected hysteroscopically to improve fertility rate/decrease SA rate. Septoplasty shown to increase rate of term deliveries.
How does an arcuate uterus form?
Near complete resorption of utero-vaginal septum. Appears as heart shaped endometrial contour. Appears normal from outside. Asymptomatic with no effect on pregnancy outcomes. No surgical intervention required.
