Benign Disorders of the Upper Genital Tract

• Müllerian anomalies


  Reproductive structures that arise from the müllerian system, except the ovaries and lower one-third of the vagina
• Formation of reproductive structures


  Fusion of the paramesonephric (müllerian) ducts forms the superior vagina, cervix, uterus, and fallopian tubes
• Types of müllerian anomalies

  • Presence of simple septa
  • Bicornuate uterus
  • Complete duplication of the entire female reproductive system
• The most common müllerian anomaly is the septate uterus due to malfusion of the paramesonephric ducts
• Müllerian anomalies may be associated with inguinal hernias and urinary tract anomalies
• The incidence of müllerian anomalies is estimated to be 0.5% (1 in 201) of the female population
• There is an increased incidence of müllerian anomalies in women exposed in utero to diethylstilbestrol (DES) from 1940 to 1971
• Symptoms associated with uterine anomalies

  • Menstrual abnormalities
  • Dysmenorrhea
  • Dyspareunia
  • Cyclic and noncyclic pelvic pain
  • Infertility
  • Recurrent miscarriage
• Uterine septa


  Positioned vertically, composed of collagen fibers, often lack adequate blood supply to facilitate placentation and maintain a growing pregnancy
• 25% of women with uterine septa may suffer from recurrent first-trimester pregnancy loss
• Bicornuate uterus


  More commonly complicated by the limited size of the uterine horn rather than by blood supply, associated with second-trimester pregnancy loss, malpresentation, and preterm labor and delivery
• Müllerian agenesis or hypoplasia (Mayer-Rokitansky-Kuster-Hauser syndrome)


  Absence of the vagina with variable uterine development, presents as primary amenorrhea
• Diagnostic tools for uterine abnormalities

  • Pelvic ultrasound
  • CT
  • MRI
  • Sonohistogram
  • Hysterosalpingogram
  • Hysteroscopy
  • Laparoscopy
• Uterine septa and bicornuate uteri may appear identical on hysteroscopic evaluation, better distinguished using MRI or laparoscopy
• There is an increased incidence of renal anomalies in the setting of a congenital Müllerian anomaly
• Uterine septa


  Can be excised with operative hysteroscopy once bicornuate uterus has been ruled out
• Bicornuate uterus


  Many women are able to carry a pregnancy to fruition, although preterm labor and delivery is a significant risk. Viable pregnancies have been achieved with surgical unification procedures, requiring delivery via cesarean section
• Classification of Müllerian Anomalies

  • Segmented müllerian agenesis or hypoplasia
  • Unicornuate uterus
  • Uterus didelphis
  • Bicornuate uterus
  • Septate uterus
  • Uterus with internal luminal changes
• Uterine leiomyomas (fibroids)


  Benign proliferations of smooth muscle cells of the myometrium, typically occur in women of childbearing age and regress during menopause
• Approximately one-third of all hysterectomies performed are for uterine fibroids
• Pathogenesis of uterine leiomyomas


  Benign monoclonal tumors, genetic predisposition, steroid hormone factors, growth factors, and angiogenesis may play a role in their formation and growth
• Fibroid classification by location

  • Submucosal (beneath the endometrium)
  • Intramural (in the muscular wall of the uterus)
  • Subserosal (beneath the uterine serosa)
• Intramural leiomyomas are the most common type, and submucosal fibroids are commonly associated with heavy or prolonged bleeding
• Parasitic leiomyoma


  A pedunculated fibroid that becomes attached to the pelvic viscera or omentum and develops its own blood supply
• Fibroids contain a large quantity of extracellular matrix and are surrounded by a pseudocapsule, which distinguishes them from adenomyosis
• It is unclear whether fibroids have any malignant potential, but leiomyosarcomas are thought to represent separate new neoplasias rather than a degeneration of an existing benign fibroid
• The lifetime risk of developing leiomyoma is 70% in whites and greater than 80% of African American women by age 50
• Risk factors for uterine fibroids

  • African American heritage
  • Nonsmoking
  • Early menarche
  • Nulliparity
  • Perimenopause
  • Increased alcohol use
  • Hypertension
• Low-dose oral contraceptive pills are generally protective against the development of new fibroids but may stimulate existing fibroids, except in women who start OCPs between ages 13-16
• The risk of fibroids decreases with increasing parity, with oral contraception use, and injectable depot medroxyprogesterone acetate use
• Symptoms of uterine fibroids

  • Abnormal uterine bleeding
  • Pelvic pain
  • Pressure-related symptoms (pelvic pressure, constipation, hydronephrosis, venous stasis)
• Abnormal uterine bleeding from fibroids

  Increasingly heavy periods of longer duration (menorrhagia), spotting after intercourse (postcoital spotting), bleeding between periods (metrorrhagia), or heavy irregular bleeding (menometrorrhagia)
• Blood loss from fibroids can lead to chronic iron-deficiency anemia, dizziness, weakness, and fatigue
• Submucosal fibroids can impact implantation, placentation, and ongoing pregnancy
• Fibroids


  • Can cause spotting after intercourse (postcoital spotting)
  • Can cause bleeding between periods (metrorrhagia)
  • Can cause heavy irregular bleeding (menometrorrhagia)
  • Can lead to chronic iron-deficiency anemia, dizziness, weakness, and fatigue
• Pelvic pain is not usually part of the symptom complex of fibroids unless vascular compromise is present
• Patients may experience secondary dysmenorrhea with menses, particularly when menorrhagia or menometrorrhagia are present
• Fibroids


  • Can cause pressure-related symptoms like constipation, urinary frequency, or urinary retention as the space within the pelvis becomes more crowded
• Submucosal fibroids


  • Can impact implantation, placentation, and ongoing pregnancy
• Resection of submucosal fibroids in patients diagnosed with infertility does lead to increased conception rates