If the vagina is absent or short, progressive dilation is usually successful in making it functional (70,73). If dilation fails or the patient is unable to perform dilation, the McIndoe split thickness graft technique may be performed (62,74,75). Controversy exists as to the best technique (60). The initial use of vaginal dilators is required to maintain a functional vagina.
5.
In patients with complete androgen insensitivity, the testes should be removed after pubertal development is complete to prevent malignant degeneration (76). In patients with testes, 52% develop a neoplasia, most often a gonadoblastoma. Almost one-half of the testicular neoplasms are malignant (dysgerminomas), but transformation usually does not occur until after puberty (77). In patients who develop virilization and have an XY karyotype, the testes should be removed immediately to preserve the female phenotype and to promote female gender identity. Bilateral laparoscopic gonadectomy is the preferred procedure for removal of intra-abdominal testes.
6.
Adhesions in the cervix and uterus (Asherman syndrome) can be removed using hysteroscopic resection with scissors or electrocautery. It is reasonable to place a pediatric Foley catheter in the uterine cavity for 7 to 10 days postoperatively (along with systemic administration of broad-spectrum antibiotic therapy). A 2-month course of high-dose estrogen therapy with monthly progestogen withdrawal is used to prevent reformation of adhesions. Eighty percent of patients thus treated achieve pregnancy, but complications including miscarriage, preterm labor, placenta previa, and placenta accreta can occur (78). Cervical stenosis can be treated by cervical dilation.
Amenorrhea with Secondary Sexual Characteristics and Normal Pelvic Anatomy
Although the complete list of potential causes is long, as noted below, the most common causes of amenorrhea in women with normal secondary sexual characteristics and normal pelvic are pregnancy, polycystic ovarian syndrome, hyperprolactinemia, primary ovarian insufficiency (also known as premature ovarian failure), and hypothalamic dysfunction. Pregnancy must be considered in all women of reproductive age with amenorrhea.
PCOS is a medical condition associated with hyperandrogenism, ovulatory dysfunction, and polycystic ovaries (79). All definitions of PCOS exclude patients with significantly elevated prolactin, significant thyroid dysfunction, adult-onset congenital adrenal hyperplasia, and androgen-secreting neoplasms from being classified as PCOS. The National Institutes of Health (NIH) 1990 criteria included hyperandrogenism and oligomenorrhea or amenorrhea as required for PCOS diagnosis. The Rotterdam 2003 criteria required two of three of the following for PCOS diagnosis: hyperandrogenism, oligomenorrhea or amenorrhea, polycystic ovaries by ultrasound (80). Although insulin resistance is noted among women with PCOS, it is not included in any of the diagnostic criteria. Obesity is common, but approximately 20% of women with PCOS are not obese. Women with PCOS are often subfertile caused by infrequent or absent ovulation. PCOS can have other general health implications, including increased risk for endometrial hyperplasia and cancer, diabetes, and possibly cardiovascular disease.
Even though PCOS usually causes irregular bleeding rather than amenorrhea, it remains one of the most common causes of amenorrhea (2). The etiology of PCOS remains largely unknown.