QUICK RECERTIFICATION SERIES
Amenorrhea Renee Andreeff, EdD, MS, MPAS, PA-C
GENERAL FEATURES • Amenorrhea is the absence or abnormal cessation of menstrual cycles in a woman of reproductive age, and can be classified as primary or secondary. • Primary amenorrhea, a rare disorder that occurs in 1% to 2% of women, is the absence of menstrual bleeding by age 16 in women who exhibit signs of secondary sexual characteristics. Causes can be divided into three main categories: ° End-organ disorders including chromosomal abnormalities (such as Turner syndrome) that cause gonadal dysgenesis (about 50% of patients with primary amenorrhea), Mullerian agenesis (about 15% of patients), and ovarian failure. ° Outflow tract obstruction such as imperforate hymen, transverse vaginal septum, testicular feminization, and vaginal agenesis or atresia. ° Central regulatory disorders including hypothalamic and pituitary disorders. Examples include Kallmann syndrome, pituitary tumors, and congenital gonadotropin-releasing hormone (GnRH) deficiency. All causes of secondary amenorrhea also can cause primary amenorrhea. • Secondary amenorrhea occurs in 3% to 5% of women. This condition is defined as cessation of menstrual cycles for three or more consecutive cycles in women with previously normal menstrual cycles or cessation of menstrual cycles for more than 6 months in women with previously irregular menstrual cycles. ° The most common cause is pregnancy. ° Other causes can be divided into four main categories: t Anatomic abnormalities, including Asherman syndrome (intrauterine adhesions) or cervical os stenosis t Ovarian dysfunction such as polycystic ovary syndrome or ovarian failure due to torsion, postsurgical
Renee Andreeff is an academic coordinator and a clinical assistant professor in the PA program at D’Youville College in Buffalo, N.Y. The author has disclosed no potential conflicts of interest, financial or otherwise. Dawn Colomb-Lippa, MHS, PA-C department editor DOI: 10.1097/01.JAA.0000453871.15689.a2
infection, radiation, chemotherapy, or premature ovarian failure t Pituitary dysfunction caused by pituitary adenomas, primary hypothyroidism, or medications such as haloperidol, metoclopramide, estrogen, phenothiazine, monoamine oxidase inhibitors, tricyclic antidepressants, and opioids t Central nervous system or hypothalamic disorders such as sellar masses and functional hypothalamic GnRH deficiency t Functional hypothalamic GnRH deficiency excludes pathologic disease and is characterized by a decrease in hypothalamic GnRH secretion. Causes include eating disorders such as anorexia nervosa,
QUESTIONS 1. A 28-year-old woman presents to the office complaining that her menstrual cycle is late. She has not had a menstrual period for 3 months and is concerned she may be pregnant, although she takes oral contraceptives and a urine pregnancy test was negative. Her cycle is usually every 30 days and has been so since she was 14. She states that she started a new job 6 months ago and has been working 14 to 16 hours a day. Her appetite is normal but she gets 6 hours of sleep each night. She denies any fatigue, weight gain, hot flashes, insomnia, and polydipsia. She takes no medications other than the oral contraceptives. What other factor might be contributing to her late menstrual cycle? a. stress b. inadequate caloric intake c. menopause d. hypothyroidism 2. Uterine surgical procedures such as curettage can cause scarring. This is known as Asherman syndrome and is a cause of which type of amenorrhea? a. primary b. secondary
Copyright © 2014 American Academy of Physician Assistants
50
www.JAAPA.com
Volume 27 • Number 10 • October 2014
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Amenorrhea
JAAPA Journal of the American Academy of Physician Assistants
COMPLICATIONS • Infertility • Psychosocial developmental delays • Osteoporosis and fractures, if hypoestrogenic.
Answers
DIAGNOSIS • Pregnancy testing via beta human chorionic gonadotropin in all women is the first step, even if the patient denies sexual activity. • Evaluation of primary amenorrhea for a patient with secondary sexual characteristics begins with a pelvic ultrasound or MRI to evaluate anatomic abnormalities of the vagina, cervix, and uterus. ° If the uterus is abnormal or absent, obtain a karyotype analysis.
TREATMENT • Directed toward underlying pathology and helping a woman to achieve fertility if desired. • Induction of ovulation can occur for most women with the use of medications (dopamine agonist, clomiphene citrate, insulin-secreting agents, and gonadotropins) except for women with premature ovarian failure.
1. A, stress. Functional hypothalamic GnRH deficiency can be caused by emotional stress among other causes. This patient states that her appetite is fine and she does not have symptoms of hypothyroidism such as weight gain and fatigue. She is young to be entering menopause (which would be called premature ovarian insufficiency). She does not have symptoms of menopause.
CLINICAL ASSESSMENT • Evaluation of primary amenorrhea should begin at age 15 years in girls who exhibit secondary sexual characteristics and at age 14 years in girls who do not exhibit secondary sexual characteristics. • History questions important to ask for primary amenorrhea include completion of Tanner stages (breast development), growth spurts, and family history of delayed or absent puberty. Also rule out a history of neonatal crisis, short stature, and hyperandrogenism. • History for primary and secondary amenorrhea should also include questions relating to chance of pregnancy, and any procedures or infections that could have scarred the endometrium. Ask about changes in weight, stress, illness, or use of medications. Also ask about weight gain, acne, hirsutism, libido, galactorrhea, headaches, visual field changes, fatigue, polyuria, and polydipsia. Include questions about estrogen deficiency, such as hot flashes, vaginal dryness, and poor sleep. • History for secondary amenorrhea should include questions pertaining to the patient’s risk for endometrial hyperplasia and/or cancer. Risk factors include obesity, age over 40 years, history of polycystic ovarian syndrome, and previous history of hyperplasia. • Physical examination of primary amenorrhea should focus on Tanner staging, Turner syndrome features, and the presence or absence of the vagina and uterus. • Physical examination for secondary amenorrhea should focus on assessment of hyperandrogenism (hirsutism, acne, and hyperseborrhea), dental erosions, galactorrhea, and vaginal anatomic defects along with signs of estrogen deficiency. Also assess cranial nerves and visual fields. • Record the patient’s body mass index (BMI) and note if it is over 30 kg/m2 in patients with polycystic ovarian syndrome, or low in patients with eating disorders.
If the uterus is present and normal, evaluate for outflow tract obstruction; if outflow tract is normal, evaluate for secondary causes of amenorrhea. • Evaluation of primary amenorrhea for a patient without secondary sexual characteristics begins with measuring follicle-stimulating hormone (FSH) and luteinizing hormone levels. ° If levels are low (less than 5 IU/L) diagnose hypogonadotropin hypogonism disorders. ° If levels are high (FSH above 20 IU/L and luteinizing hormone above 40 IU/L), consider hypergonadotropic hypogonadism disorders and obtain a karyotype analysis. • Evaluation of secondary amenorrhea and a negative pregnancy test begins with checking thyroid-stimulating hormone (TSH) and prolactin levels. ° If TSH and prolactin levels are normal, perform a progesterone challenge test. ° If prolactin level is normal and TSH is abnormal, thyroid disease can be diagnosed. ° If TSH is normal and prolactin levels are abnormal: t Prolactin greater than 100 ng/mL—perform MRI of brain to evaluate for pituitary adenoma t Prolactin less than or equal to 100 ng/mL—consider causes such as liver disease, renal disease, teratoma, or medication adverse reactions. °
2. B, Asherman syndrome. This condition is a cause of secondary amenorrhea.
exercise, nutritional deficiencies, severe illness, and emotional stress.
www.JAAPA.com
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
51
Amenorrhea Renee Andreeff, EdD, MS, MPAS, PA-C
GENERAL FEATURES • Amenorrhea is the absence or abnormal cessation of menstrual cycles in a woman of reproductive age, and can be classified as primary or secondary. • Primary amenorrhea, a rare disorder that occurs in 1% to 2% of women, is the absence of menstrual bleeding by age 16 in women who exhibit signs of secondary sexual characteristics. Causes can be divided into three main categories: ° End-organ disorders including chromosomal abnormalities (such as Turner syndrome) that cause gonadal dysgenesis (about 50% of patients with primary amenorrhea), Mullerian agenesis (about 15% of patients), and ovarian failure. ° Outflow tract obstruction such as imperforate hymen, transverse vaginal septum, testicular feminization, and vaginal agenesis or atresia. ° Central regulatory disorders including hypothalamic and pituitary disorders. Examples include Kallmann syndrome, pituitary tumors, and congenital gonadotropin-releasing hormone (GnRH) deficiency. All causes of secondary amenorrhea also can cause primary amenorrhea. • Secondary amenorrhea occurs in 3% to 5% of women. This condition is defined as cessation of menstrual cycles for three or more consecutive cycles in women with previously normal menstrual cycles or cessation of menstrual cycles for more than 6 months in women with previously irregular menstrual cycles. ° The most common cause is pregnancy. ° Other causes can be divided into four main categories: t Anatomic abnormalities, including Asherman syndrome (intrauterine adhesions) or cervical os stenosis t Ovarian dysfunction such as polycystic ovary syndrome or ovarian failure due to torsion, postsurgical
Renee Andreeff is an academic coordinator and a clinical assistant professor in the PA program at D’Youville College in Buffalo, N.Y. The author has disclosed no potential conflicts of interest, financial or otherwise. Dawn Colomb-Lippa, MHS, PA-C department editor DOI: 10.1097/01.JAA.0000453871.15689.a2
infection, radiation, chemotherapy, or premature ovarian failure t Pituitary dysfunction caused by pituitary adenomas, primary hypothyroidism, or medications such as haloperidol, metoclopramide, estrogen, phenothiazine, monoamine oxidase inhibitors, tricyclic antidepressants, and opioids t Central nervous system or hypothalamic disorders such as sellar masses and functional hypothalamic GnRH deficiency t Functional hypothalamic GnRH deficiency excludes pathologic disease and is characterized by a decrease in hypothalamic GnRH secretion. Causes include eating disorders such as anorexia nervosa,
QUESTIONS 1. A 28-year-old woman presents to the office complaining that her menstrual cycle is late. She has not had a menstrual period for 3 months and is concerned she may be pregnant, although she takes oral contraceptives and a urine pregnancy test was negative. Her cycle is usually every 30 days and has been so since she was 14. She states that she started a new job 6 months ago and has been working 14 to 16 hours a day. Her appetite is normal but she gets 6 hours of sleep each night. She denies any fatigue, weight gain, hot flashes, insomnia, and polydipsia. She takes no medications other than the oral contraceptives. What other factor might be contributing to her late menstrual cycle? a. stress b. inadequate caloric intake c. menopause d. hypothyroidism 2. Uterine surgical procedures such as curettage can cause scarring. This is known as Asherman syndrome and is a cause of which type of amenorrhea? a. primary b. secondary
Copyright © 2014 American Academy of Physician Assistants
50
www.JAAPA.com
Volume 27 • Number 10 • October 2014
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Amenorrhea
JAAPA Journal of the American Academy of Physician Assistants
COMPLICATIONS • Infertility • Psychosocial developmental delays • Osteoporosis and fractures, if hypoestrogenic.
Answers
DIAGNOSIS • Pregnancy testing via beta human chorionic gonadotropin in all women is the first step, even if the patient denies sexual activity. • Evaluation of primary amenorrhea for a patient with secondary sexual characteristics begins with a pelvic ultrasound or MRI to evaluate anatomic abnormalities of the vagina, cervix, and uterus. ° If the uterus is abnormal or absent, obtain a karyotype analysis.
TREATMENT • Directed toward underlying pathology and helping a woman to achieve fertility if desired. • Induction of ovulation can occur for most women with the use of medications (dopamine agonist, clomiphene citrate, insulin-secreting agents, and gonadotropins) except for women with premature ovarian failure.
1. A, stress. Functional hypothalamic GnRH deficiency can be caused by emotional stress among other causes. This patient states that her appetite is fine and she does not have symptoms of hypothyroidism such as weight gain and fatigue. She is young to be entering menopause (which would be called premature ovarian insufficiency). She does not have symptoms of menopause.
CLINICAL ASSESSMENT • Evaluation of primary amenorrhea should begin at age 15 years in girls who exhibit secondary sexual characteristics and at age 14 years in girls who do not exhibit secondary sexual characteristics. • History questions important to ask for primary amenorrhea include completion of Tanner stages (breast development), growth spurts, and family history of delayed or absent puberty. Also rule out a history of neonatal crisis, short stature, and hyperandrogenism. • History for primary and secondary amenorrhea should also include questions relating to chance of pregnancy, and any procedures or infections that could have scarred the endometrium. Ask about changes in weight, stress, illness, or use of medications. Also ask about weight gain, acne, hirsutism, libido, galactorrhea, headaches, visual field changes, fatigue, polyuria, and polydipsia. Include questions about estrogen deficiency, such as hot flashes, vaginal dryness, and poor sleep. • History for secondary amenorrhea should include questions pertaining to the patient’s risk for endometrial hyperplasia and/or cancer. Risk factors include obesity, age over 40 years, history of polycystic ovarian syndrome, and previous history of hyperplasia. • Physical examination of primary amenorrhea should focus on Tanner staging, Turner syndrome features, and the presence or absence of the vagina and uterus. • Physical examination for secondary amenorrhea should focus on assessment of hyperandrogenism (hirsutism, acne, and hyperseborrhea), dental erosions, galactorrhea, and vaginal anatomic defects along with signs of estrogen deficiency. Also assess cranial nerves and visual fields. • Record the patient’s body mass index (BMI) and note if it is over 30 kg/m2 in patients with polycystic ovarian syndrome, or low in patients with eating disorders.
If the uterus is present and normal, evaluate for outflow tract obstruction; if outflow tract is normal, evaluate for secondary causes of amenorrhea. • Evaluation of primary amenorrhea for a patient without secondary sexual characteristics begins with measuring follicle-stimulating hormone (FSH) and luteinizing hormone levels. ° If levels are low (less than 5 IU/L) diagnose hypogonadotropin hypogonism disorders. ° If levels are high (FSH above 20 IU/L and luteinizing hormone above 40 IU/L), consider hypergonadotropic hypogonadism disorders and obtain a karyotype analysis. • Evaluation of secondary amenorrhea and a negative pregnancy test begins with checking thyroid-stimulating hormone (TSH) and prolactin levels. ° If TSH and prolactin levels are normal, perform a progesterone challenge test. ° If prolactin level is normal and TSH is abnormal, thyroid disease can be diagnosed. ° If TSH is normal and prolactin levels are abnormal: t Prolactin greater than 100 ng/mL—perform MRI of brain to evaluate for pituitary adenoma t Prolactin less than or equal to 100 ng/mL—consider causes such as liver disease, renal disease, teratoma, or medication adverse reactions. °
2. B, Asherman syndrome. This condition is a cause of secondary amenorrhea.
exercise, nutritional deficiencies, severe illness, and emotional stress.
www.JAAPA.com
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
51
