Amenorrhea

An-Najah National University Dr.Ahmad Abu Khaizaran

Amenorrhea

Primary

Absence of menses by age 16 with normal secondary sexual characteristics Absence of menses by age 14 without secondary sexual development
Absence of menses for 6 months in a previously menstruating female

Secondary

Events of Puberty

Thelarche (breast development) Requires estrogen Pubarche/adrenarche (pubic hair development) Requires androgens Menarche Requires: GnRH from the hypothalamus FSH and LH from the pituitary Estrogen and progesterone from the ovaries Normal outflow tract

Are there secondary sexual characteristics?

Primary Amenorrhea

Is there normal development of secondary sexual characteristcs? NO Think hypogonadism or hypogonadotropism

Amenorrhea with Immature Secondary Characteristics

FSH Serum level

Low / normal
Hypogonadotropic hypogonadism

High
Gonadal dysgenesis

Primary Amenorrhea

Hypogonadism

30% have genetic abnormality

Gonadal dysgenesis, Turners syndrome, mosaicism

Enzyme deficiencies Kallmanns syndrome, CNS tumors Irradiation Chemotherapy Galactosemia

Primary Amenorrhea with Immature Sexual Characteristics

Hypogonadism (gonadal failure)

Gonadal dysgenesis Irradiation Chemotherapy Galactosemia

Note: gonadotropins (FSH/LH) will be high, similar to menopause

Gonadal Dysgenesis
Chromosomally abnormal - Classic turners syndrome (45XO) - Turner variants (45XO/46XX),(46X-abnormal X) - Mixed gonadal dygenesis (45XO/46XY) Chromosomally normal - 46XX (Pure gonadal dysgeneis) - 46XY (Swyers syndrome)

Primary Amenorrhea with Immature Sexual Characteristics

Hypogonadotropism

Hypothalamic dysfunction

Kallmann syndrome Anorexia nervosa Space-occupying lesion of CNS Marijuana use

Pituitary damage (surgery/radiation) Constitutional delay

Are there secondary sexual characteristics?

Primary Amenorrhea

Is there normal development of secondary sexual characteristics? YES Think

Pregnancy Mullerian anomaly Androgen insensitivity

Primary Amenorrhea with Normal Secondary Characteristics

Mullerian Anomalies

Mullerian agenesis (Mayer-Rokitansky-KusterHauser syndrome) Imperforate hymen Transverse vaginal septum

Mayer-Rokitansky-Kuster-Hauser Syndrome (utero-vaginal agenesis)

15% of primary amenorrhea Normal secondary development & external female genitalia Normal female range testosterone level Absent uterus and upper vagina & normal ovaries Karyotype 46-XX 15-30% renal, skeletal and middle ear anomalies

Imperforate Hymen

Androgen Insensitivity

Normal breasts but no sexual hair Normal looking female external genitalia Absent uterus and upper vagina Karyotype 46, XY Male range testosterone level Treatment : gonadectomy after puberty + HRT

Primary Amenorrhea

Evaluation

Pregnancy test Physical exam to determine presence of uterus FSH Karyotype

Primary Amenorrhea

Treatment

Cyclic estrogen/progestin Remove gonadal streaks if XY or mosaic

Increased (52%) risk of gonadoblastomas, dysgerminomas, and yolk sac tumors

Pulsatile GnRH for ovulation induction in select patients Surgical resection of intrauterine, cervical, and vaginal adhesions/septa

Secondary Amenorrhea

Pregnancy! CNS disorders Pituitary gland Thyroid Ovary Uterus Systemic disorders

Renal failure, liver disorders, DM

Medications: anti-psychotics, reserpine

Secondary Amenorrhea

CNS disorders

Chronic hypothalamic anovulation

Stress Increased exercise levels Anorexia nervosa

Head trauma Space-occupying lesions

Secondary Amenorrhea

Pituitary disorders

Hyperprolactinemia

Prolactinoma Medications PCOS Renal failure Pituitary resection Sheehans syndrome

Hypoprolactinemia

Thyroid disorders

Hyper- or hypothyroidism

Secondary Amenorrhea

Ovulation disorders

Polycystic ovarian syndrome Premature ovarian failure

Ashermans syndrome Cervical stenosis Hormonal contraceptives GnRH analogues

Uterine abnormalities

Drug-induced amenorrhea

Ashermans Syndrome

Secondary Amenorrhea
History

Nutrition/exercise habits, weight change Sexual/contraceptive practice History of uterine/cervical surgery Height/weight Hirsutism Galactorrhea Estrogen status of tissues
BhCG PRL & TSH progesterone challenge FSH if high karyotype

Physical exam

Laboratory

Negative Pregnancy.test

TSH ,PROLACTIN, Progesterone challenge test

withdrawal bleeding without withdrawal bleeding hypoestrogenic +ve.est/progest challenge test compromised outflow tract -ve.est/progest challenge test Normal FSH FSH>30-40 repeat HSG OR hysteroscopy Ashermans PreOv Failure

anovulation

FSH norm.

Repeat+serum estrogen level

hypothalamicpituitary failure

Secondary Amenorrhea

Treatment goals

Discovery and treatment of underlying disorder Hormone replacement Menses every 1-3 months Pregnancy

Ovulation induction GnRH pump FSH/LH