This patient's clinical presentation is consistent with Asherman's syndrome.
Asherman's syndrome is characterized by endometrial fibrosis and adhesions, usually as a result of prior intrauterine instrumentation. Patients are particularly at risk for scarring when vigorous curettage is performed during low-estrogen states. The risk of Asherman's syndrome is roughly 16% after a single curettage, but increases to >30% after 3 procedures. Patients with mild to moderate disease may become fertile after removal of adhesions, but pregnancy in the presence of adhesions increases the risk of placental abnormalities.
Master-Hunter and Heiman discuss the evaluation of amenorrhea. They note that Asherman's syndrome is the most common cause of outflow obstruction in secondary amenorrhea. Hysterosalpingography, sonohysterography, and hysteroscopy can all be useful for establishing the diagnosis.
March reviews the management of Asherman's syndrome. He notes that miniature scissors should be used for lysis of uterine adhesions, and a balloon stent can be placed in the uterus immediately after the procedure. In addition, estrogen therapy in the post-operative period promotes regrowth of the endometrium. Subsequent pregnancies should be monitored for complications including placenta accreta, cervical incompetence, and intrauterine growth restriction.
Figure A shows a hysterosalpingogram of a patient with Asherman's syndrome, with noted uterine filling defects. Illustration A shows a normal hysterosalpingogram, with complete filling of the uterus and bilateral fallopian tubes. Illustration B shows uterine adhesions typical of Asherman's syndrome, as seen via intrauterine laparoscopy. Illustration C describes the uterus and ovaries in several causes of infertility.
Answer 1: Kallmann syndrome is caused by abnormal cellular migration during development, leading to deficiency of hypothalamic neurons involved in GnRH secretion, as well as diminished or absent olfactory sense. Patients with this syndrome have a uterus, but have an increased rate of uterine malformations, compared to the normal population.
Answer 2: Swyer syndrome is associated with the 46, XY genotype with gonadal dysgenesis, and usually results from mutations in the SRY gene. Patients with this syndrome have a normal uterus.
Answer 4: Turner's syndrome is associated with the 45, X genotype, and is associated with infertility as a result of early ovarian failure. Patients with this syndrome have a normal or underdeveloped uterus.
Answer 5: Androgen insensitivity syndrome occurs when embryos with a 46, XY karyotype are unable to respond to androgens during development, leading to female external genitalia with male internal sex organs. Patients with this syndrome do not have a uterus.