Grand Rounds Mullerian Anomalies Sara Schaenzer, PGY-3 9/26/18
Background Congenital uterine anomalies occur in 2-4% of women Three times more common in women with recurrent pregnancy loss True incidence likely understated as 57% of women with uterine malformations will have successful fertility and pregnancy 25% of women with uterine anomalies will have poor pregnancy outcomes Anomalies can cause dysmenorrhea and dyspareunia
Embryology Mullerian ducts form the fallopian tubes, uterus, cervix, and upper 2/3 of the vagina Phases of normal development Organogenesis: formation of both mullerian ducts Failure results in uterine agenesis or unicornuate uterus Fusion: fusion of the ducts to form the uterus Failure results in bicornuate or didelphys Septal resorption: resorption of central septum once ducts have fused Failure results in septate or arcuate uterus
Mullerian agenesis Congenital absence of uterus, vagina, or both Common form: Mayer- Rokitansky-Kuster-Hauser syndrome Shallow vaginal pouch (1-2 inches) Normal ovaries Steinkampt, M. Treatment of Vaginal Agenesis. Alabama Fertility.
Mullerian Agenesis Incidence: 1 per 4,500 to 5,000 females May have rudimentary mullerian structures These structures may have endometrial activity causing cyclic pelvic pain Evaluate for renal and skeletal anomalies 50-60% of women have secondary malformations 15-36% with urinary defects, 12% with scoliosis
Treatment of mullerian agenesis Goal is for creation of a functional vagina Vaginal dilators Surgical creation of neovagina
Unicornuate Uterus Failure of one mullerian duct to develop and elongate
Evaluation of Unicornuate Uterus Hysterosalpigogram followed by MRI and/or sono Renal sono 40% of patients have renal agenesis
Pregnancy outcomes in Unicornuate Uterus Miscarriage Rate: 36% Preterm birth rate: 16% Other complications: breech presentation, IUGR, dysfunctional labor, c-section
Uterine Didelphys Failed fusion of mullerian ducts Two separate uterine horns, each with an endometrial cavity and cervix and often a vaginal septum between the cervices
Evaluation HSG to rule out communication between uteri MRI to confirm diagnosis Pregnancy Pregnancy develops in one horn Same complications as unicornuate, but slightly better outcomes
Bicornuate Uterus Due to incomplete fusion of mullerian ducts Results in two separate but communicating endometrial cavities and a single cervix Fundal cleft greater than 1 cm deep
Evaluation HSG followed by MRI and/or pelvic sono ACOG CREOG Quiz #358
Surgical Metroplasty Consider only with recurrent spontaneous abortions and no other cause Goal is unification of endometrial cavities
Septate Uterus A result of failure of the medial segments to regress following fusion of mullerian ducts Results in some degree of septum within the uterine cavity Most common of uterine anomalies
Pregnancy Outcomes Associated with a large increase in spontaneous abortion Inadequate blood supply Distortion of cavity Live birth rates are improved with hysteroscopic resection of septum
Arcuate Uterus Considered a mild deviation from normal Result of near complete absorption of uterovaginal septum
A 29 yo female presents with her partner for a history of 3 miscarriages between 6-10 weeks. You perform a work-up for recurrent pregnancy loss and a hystersalpingogram is suspicious for a uterine septum versus a bicornuate uterus. What is the next best step in management to differentiate between the possibilities? A. Pelvic MRI B. Repeat HSG C. Pelvic x-ray D. Laparoscopy with direct visualization E. Pelvic Exam
Which of the following uterine anomalies is caused by a partial lack of fusion of the two mullerian ducts? A. Unicornuate Uterus B. Bicornuate Uterus C. Uterine didelphus D. Septate Uterus E. T-shaped uterus
A 19 yo G0 is incidentally found to have a Mullerian anomaly. Which of the following will significantly increase her risk of spontaneous abortion in future A. Arcuate Uterus B. Bicornuate Uterus C. Septate Uterus D. Unicornuate Uterus E. Uterine didelphus pregnancies?
References Chandler et al. Mullerian duct anomalies: from diagnosis to intervention. Br J Radiology. 2009 Dec; 82(984): 1034-1042. Hoffamn et al. Williams Gynecology. 2 nd ed. McGraw Hill. 2012. Müllerian agenesis: diagnosis, management, and treatment. ACOG Committee Opinion No. 728. American College of Obstetricians and Gynecologists. Obstet Gynecol 2018;131:e35 42. Speroff L, Fritz MA. Clinical gynecologic endocrinology and infertility. 8 th ed. Philadelphia: Lippincott, Williams, Wilkins. 2011.