Malformation of Female Reproductive System

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Unit 2: Malformations of female

Reproductive Organs and Menstrual


irregularities and AUB

Prepared by:
Kriti Banstola
Malformation of the female external genitalia
Congenital uterine abnormalities result from abnormal formation, fusion
or resorption of the mullerian duct during fetal life. These anomalies
have been associated with an increase rate of miscarriage, preterm
delivery and other adverse fetal outcomes. Neither uterine size, shape
nor position is permanently fixed.
Imperforated hymen:
A hymen is a membrane that surrounds or covers the opening of the
vagina. The hymenal tissue is a circular form of tissue, which has a hole
within the center. When there is no opening in the hymen, a membrane
covers the area called an imperforate hymen.
An imperforate hymen is a congenital disorder where a hymen without
an opening completely obstructs the vagina. It is caused by a failure of
the hymen to perforate during fetal development. It is most often
diagnosed in adolescent girls when menstrual blood accumulates in the
vagina and sometimes also in the uterus.
Causes
A congenital imperforate hymen is a result of the failure of completion
of the cannulization of the epithelial plug that fills the vagina.

Signs and symptoms


• Asymptomatic before puberty.
• Primary amenorrhea with periodic lower abdominal pain.
• Possible urinary retention, frequency, dysuria.
• Possible palpable lower abdominal mass.
• Perineal examination: tense, bulging, bluish membrane in the vulva.
• Other symptoms associated with hematocolpos include urinary
retention, constipation, low back pain, nausea and diarrhea.
• Other vaginal anomalies can have similar symptoms to an imperforate
hymen.
• A strong urge to defecate has been observed in a few women.
Diagnosis
• An imperforate hymen is most often diagnosed in adolescent girls
after the age of menarche with otherwise normal development.
• Amenorrhea and cyclic pelvic pain is the typical presentation in
adolescent girls of menarcheal age.
• On examination, there is typically a bulging obstruction of the vagina.
• An imperforate hymen in visible on vaginal inspection as a buldging
blue membrane.
• Transabdominal, transperineal or transrectal ultrasound
Management
• Symptomatic management by the combined oral contraceptive pill
taken continuously to suppress the menstrual cycle or NSAIDs to
relieve pain.
• Hymenotomy: making cruciate incision of hymen, excising segments
of hymen from their bases, and draining the vaginal canal and uterus.
• Prophylactic antibiotics should be given postoperatively.
Complications:
• Peritonitis or endometriosis
• Mucometrocolpos
• hematometrocolpos
Incomplete hymenal fenestration
Partial obstruction of the hymen is referred to as incomplete fenestration
of the hymenal opening and the pattern of obstruction varies, including
micro-perforated and septed.
Diagnosis
• Asymptomatic
• Malodorous discharge
• Inability to insert tampon
Treatment
Surgical management: resection of the excess hymena; tissue to create a
normal diameter hymenal ring.
Complication
Tubo-ovarian abscess or systematic infection
Septate hymen
A septed hymen is when the hymenal membrane has extra tissue in the
middle, causing two small vaginal openings as opposed to one. This
could interfere with the ability to wear a tampon or to take a tampon out
after it has filled with blood.
Treatment
A septate hymen does not need to be surgically removed and is typically
torn during sexual intercourse. Possible side effects include pain,
discomfort or bleeding. This can be corrected via a simple surgical
approach that removes the septate hymen.
Vaginal agenesis
• Vaginal agenesis is a condition that develop before birth where the
vagina fails to fully develop. It occurs when the caudal portion of the
vagina, contributed by urogenital sinus fails to form. The caudal
portion of vagina is replaced with fibrous tissue.
• Vaginal agenesis can occur as isolated developmental defects or as a
part of complex of anomalies. The disorders that are usually coupled
with vaginal agenesis are Rokitansky-Mayer-Kuster-Hauser Syndrome
(MRKH), Bardet-Biedl Syndrome, Fraser Syndrome. Most common
being the MRKH syndrome.
• MRKH Syndrome: This condition causes the vagina and uterus to be
underdeveloped or absent. Women with MRKH syndrome have female
sex chromosome pattern (46 XX) and normally functioning ovaries.
There occurs the failure of Mullerian ducts to develop.
• There are several variation of MRKH such as lack of a vagina and a
uterus, or no vagina, a single midline uterus, and no cervix.
Cause
Exact cause is unknown. Typically the creation of vaginal canal is
completed within fetus by 20th weeks of gestation. Mullerian ducts do
not develop properly resulting into vaginal agenesis.
Signs and Symptoms:
• Cyclical abdominal pain
• Amenorrhea
• Abdominal- pelvic mass
• Small pouch or dimple where a vaginal opening should be
• Difficult voiding
• Dyspareunia and infertility
• Marked distention of vagina may result in back pain
Diagnosis
• History taking and physical examination
• Laboratory studies: Includes evaluation of levels of LH, FSH,
Prolactin, Estradiol, Progesterone
• USG ( reveal if the uterus and ovaries are present or not and their
location)
• MRI ( show the more detailed picture of reproductive tract)
• Laparoscopy to evaluate the uterus and adnexal structures.
• Cystourethrography
Management:
• Non-surgical treatment: Self- dilation of vagina
• Surgical treatment: vaginoplasty

Complications
• Rectovaginal fistula
• Vesicovaginal fistula
• Bleeding
• Infection
• Dyspareunia
Vaginal atresia
Vaginal atresia is a condition in which the vagina is abnormally closed
or absent. The main causes can either be complete vaginal hypoplasia,
or a vaginal obstruction, often caused by an imperforated hymen, or less
commonly, a transverse vaginal septum.
Clinical features
• Asymptomatic before puberty
• Primary amenorrhea
• Infertility
• Dyspareunia
• Vaginal dimple on examination
Treatment: Vaginoplasty
Transverse vaginal septum
• A transverse vaginal septum results when there is a failure of fusion
and/or canalization of the urogenital sinus and Mullerian duct. A
transverse vaginal septum is a horizontal collection of the tissue that
forms in the embryo. It essentially creates a blockage of the vagina.
This can occur at different level of vagina. Some women have a small
hole in the septum called fenestration.
• During a menstrual period, blood could take longer to flow, causing
periods to last longer than four to seven days. If there is no hole and
the teenage girls may well present with primary amenorrhea and
dysmenorrhea and in these circumstances, clinical examination is all
that is required to make the diagnosis.
Clinical features
• Asymptomatic before puberty
• Primary amenorrhea
• Infertility
• Periodic lower abdominal pain
• Possibly, palpable lower abdominal mass
• Perineal examination: normal vulva and external genitalia
Diagnosis
• Abdominal palpation may reveal a mass arising from the pelvis if the
hematocolpos is large enough.
• A mass may be palpated above the examining finger on recto-
abdominal examination.
• USG\MRI

Treatment
• First line: dilation over 6-12 months using graduated vaginal dilators
• Second line: vaginoplasty
Anastomosis
Vertical or complete vaginal septum
A vertical or complete vaginal septum is a condition where a wall of
tissue runs vertically up and down the length of the vagina, dividing it
into two cavities. While this condition may cause no symptoms, the
client could experience pain when removing or inserting a tampon, or
pain during intercourse.
Longitudinal septa are typically associated with uterine anomalies, such
as the septate uterus and uterus didelphys. The septum that divides the
vagina may be partial or complete.
Clinical features
• Asymptomatic
• Dyspareunia
• Persistent bleeding despite use of tampons

Diagnosis
• Physical examination
• Imaging of upper reproductive tract to determine if there is a single uterus
or two uterine structure.

Treatment
• Complete resection of the septum
Uterine anomalies/malformation
A uterine malformation is a type of female genital malformation
resulting from abnormal development of the mullerian duct(s) during
embryogenesis. Symptoms ranges from amenorrhea, infertility,
recurrent pregnancy loss and pain, to normal functioning depends on the
nature of the defect.
Uterine anomalies may result from three mechanism:
Failure of one or both of the 2 mullerian ducts to form.
Failure of the 2 ducts to fuse completely
Failure of the 2 fused mullerian duct to dissolve the septum
Types:
Uterine anomalies have been divided into 7 types by the American
Fertility Society (1988).
1. Hypoplasia (Mullerian agenesis)
2. Unicornuate uterus
3. Didelphys uterus
4. Bicornuate uterus
5. Septated uterus
6. Arcuate uterus
7. Diethylstilbesterol
1. Hypoplasia/Mullerian agenesis
Mullerian agenesis is a congenital malformation characterized by a
failure of the mullerian duct to develop, resulting in a missing uterus
and variable degree of vaginal hypoplasia of its upper portion.
Mullerian agenesis is the cause of primary amenorrhea. Because most of
the vagina does not develop from the Mullerian duct, instead of
developing from the urogenital sinus, along with the bladder and
urethra, it is present even when the mullerian duct is completely absent.
Because ovaries do not develop from the mullerian ducts, affected
women might have normal secondary sexual characteristics but are
infertile due to the lack of a functional uterus.
Clinical features
• Asymptomatic before puberty
• Dysmenorrhea
• Primary amenorrhea
• Infertility and dyspareunia
Increased risk of the following obstetric complications:
• Ectopic pregnancy
• Mid-trimester miscarriage
• Malpresentation
• Retained placenta
• Cervical impotence
• Preterm labor
• Obstructed labor
• Postpartum hemorrhage
• Diagnosis
- Internal examination - Screening tests
- Transvaginal or abdominal ultrasound - Hysterosalphingography
- Hysteroscopy - Confirmatory test: MRI

• Treatment
- Mullerian duct anomaly is not an indication for surgical therapy. In a
patient with infertility and congenital uterine anomalies, its is important to
rule out other causes of infertility before surgical therapy is considered.
- Metroplasty ( it is a reconstructive surgery used to repair congenital
anomalies of the uterus, including septate uterus and Bicornuate uterus.
The surgery entails removing the abnormal tissue that separates the cornua
of the uterus, then using several layers of stitches to create a normal shape)
2. Unicornuate uterus
A Unicornuate uterus represents a uterine malformation where the
uterus is formed from only one of the paired mullerian duct while the
other Mullerian duct does not develop or only in a rudimentary fashion.
A Unicornuate uterus has a single cervix and vagina. Associated defects
may affect the renal system, and less common, the skeleton.
Sign and symptom
• May be asymptomatic and normal pregnancy may occur
• Ectopic pregnancy
• Miscarriage
• Preterm delivery
3. Uterine didelphys
Uterine didelphys is a condition where a women has two uterine bodies.
Each uterus has a cervix. Uterus didelphys, with completely separate
uterine cavities, are also frequent. The cervices are externally united and
the uterine fundi are externally separate. In most patient the vagina is
septate, causing a double vagina. The halves of such a uterus are often
of different sizes.
Signs and symptoms
• Dysmenorrhea
• Dyspareunia
• Premature delivery
• Breech presentation
4. Bicornuate uterus
A Bicornuate uterus or bicornate uterus is a type of mullerian anomaly
in the human uterus, where there is a deep indentation at the fundus
(top) of the uterus.
Bicornuate uterus develops when the proximal portion os the
paramesonephric ducts does not fuse, but the distal portion that
develops into the lower uterine segment, cervix, and upper vagina fuses
normally.
Clinical features
• Pelvic pain (cyclic or non-cyclic)
• Abnormal vaginal bleeding
• Uterine rupture during pregnancy
• Dysmenorrhea
• Vaginal pain
• Recurrent pregnancy loss
• The patient may have a concurrent renal abnormalities
Complications:
• Recurrent miscarriage • Uterine rupture due to poor
• Preterm birth development
• Abortion • Disruption to fetal growth
• Weak uterine action • Premature rupture of membrane
• Postpartum hemorrhage • Placenta Previa
• Adhesion of the placenta • Retained placenta
• Malpresentation • Cesarean delivery
• Prolonged or obstructed labor
Treatment
• Bicornuate uterus typically requires no treatment
• Metroplasty : People who have recurrent miscarriage with no other
explanation may benefit from surgery.
5. Septate uterus
• A septate uterus results from a problem in stage 2 or 3 uterine
development. The two mullerian duct fused normally. However, there
was a failure in degeneration of the median septum.
• If the failure was complete, a median septum persists in the entire
uterus, separating the uterine cavity into two single-horned uteri that
share one cervix.
• If the failure was partial, resorption of the lower part of the median
septum occurred in stage 2 but the top of the septum failed to dissolve
in stage 3. Thus, there is a single cervix and uterine cavity at the
bottom, but at the top that cavity divides into two distinct horns.
• Because this uterine anomaly occurs later in uterine development, after
complete duct fusion the external shape of the uterus is a normal-
appearing single unit. This is distinct from the Bicornuate uterus,
which can be seen branching into two distinct horns when viewed
from the outside.
• Preterm delivery and Malpresentation are common with pregnancy.
6. Arcuate uterus
This type of uterus is essentially normal in shape with a small midline
indentation in the uterine fundus, which results from failure to dissolve
the median septum completely. It is given a distinct classification
because it does not seem to have any negative effects on pregnancy in
regard to preterm labor or Malpresentation.
7. DES uterus (Diethylstilbestrol)
The daughter of mother exposed to diethylstilbestrol (DES) during
pregnancy are predisposed to uterine abnormalities and clear cell
carcinoma of the vagina.
Two-thirds have abnormalities, including a small, incompletely formed
uterus (“hypoplastic”) and/or a T-shaped cavity and 50% have cervical
defects, for example, an incompletely formed cervix that predisposes to
cervical insufficiency. The mechanism by which DES disrupts normal
uterine development is unknown.
Cervical Agenesis
Cervical agenesis is a congenital disorder of the female genital system
that manifests itself in the absence of cervix, the connecting structure
between the uterus and vagina. Milder forms of the condition, in which
the cervix is present but deformed and nonfunctional, are known as
cervical atresia or cervical dysgenesis. Patient with cervical agenesis
typically present in early adolescence, around the time of menarche,
with primary amenorrhea and cyclic pelvic pain caused by the
obstruction of menstrual flow from the uterus.
Cervical agenesis arises during fetal development, during which time
the paramesonephric duct (mullerian duct) fails to canalize into the
cervix.
Diagnosis
• Ultrasound can be done to identify a hematoma secondary to cervical
agenesis
• MRI can detect the absence of a cervix (agenesis), it is unable to show
cervical dysgenesis (where the cervix is present but malformed).
Management
• The first line of therapy after diagnosis typically involves the
administration of the combined oral contraceptive pill,
medroxyprogesterone acetate or gonadotropin releasing hormone
agonist to suppress menstruation and thereby relieve pain.
• Surgically, cervical agenesis has historically been treated through
hysterectomy (removal of the uterus) to relieve symptoms caused by
hematocolpos (the accumulation of menstrual fluid in the vagina).
• Other surgical methods of management involve the creation of
anastomotic connection between the uterus and vagina by
vaginoplasty or recanalization of the cervix.
• Cervical agenesis occurs when a woman is born without a cervix. This
means there could be the absence of a uterus and a vagina. If a uterus
is present, the doctor may suggest medications to control retrograde
menstruation (the backward movement of menstrual fluid). Perform a
surgical procedure that fuses the uterus to a vagina.
• Outcomes in these cases are generally poor, since the natural functions
of the cervix such as mucus production and providing a barrier against
ascending infection.
Cervical duplication
Cervical duplication occurs when a women is born with two cervixes.
Symptoms
• Abnormal pain before a period
• Abnormal bleeding
• Infertility issue
• Dysmenorrhea
Diagnosis
• Complete medical history, history of clinical features, obstetrical
history.
• Physical examination
• Other diagnostic procedure includes:
‾ Hysterosalpingogram (HSG)
‾ Ultrasound
‾ Diagnostic laparoscopy
‾ Ct scan
‾ MRI
Treatment
Congenital uterine abnormalities are treated through surgical
procedures. If the doctor determines the need of treatment after
assessing the location, severity, symptoms or concern about a successful
pregnancy, the following surgical techniques may be used:
• Laparoscopy: Operative laparoscopy involves the use of a thin, lighted
tube inserted through a small incision in the patient abdomen. This
procedure is minimally invasive.
• Hysteroscopy: Operative hysteroscopy is a less invasive surgical
procedure used to remove polyps, tumor or abnormalities through
cauterizing.
Fallopian tube anomalies
• Rarely, the absence of one or both tubes may occur and is almost
always associated with the absence of the uterus as well as with other
anomalies. Localized factors may result in an incomplete tube.
• The tubes may be unduly elongated; may have accessory ostia or
diverticula.
• If the tube is long, thin hypoplastic structure and responds to
appropriate endocrine therapy, it is classified as infantile.
• Occasionally, ostia are duplicated or an accessory tube may be present.
• Most anomalies as such do not require any treatment.
Retroversion of the uterus
Definition
Retroversion of the uterus occurs when a woman’s uterus (womb) tilts
backward rather than forward . It is commonly called a “tipped uterus”.
The incidence of uterine retroversion in pregnancy is about 15%. In most
cases, retroversion resolves spontaneously by 14 weeks when the gravid uterus
grows into the abdominal cavity. Rarely the uterus remains retroverted and
becomes fixed in the pelvic cavity as it grows.
Causes
• Retroversion of the uterus is common. One in five women has this condition.
• The problem may also occur due to weakening of the pelvic ligamnets at the
time of menopause. An enlarged uterus can also be caused by pregnancy or a
tumor.
• Scar tissue in the pelvis (pelvic adhesions) can also hold the uterus in a
retroverted position.
• Scarring may come from:
Endometriosis
Pelvic inflammatory disease
Pelvic surgery
Symptoms
• Retroversion of the uterus almost never causes any symptoms.
• Rarely, it may cause pain or discomfort
• Dyspareunia
• Dysmenorrhea
• UTI
Treatment
• Underlying disorders such as endometriosis or adhesions should be
treated as needed.
Retroflexion of the uterus
A retroflexed uterus is oriented in a backward-tilting position when
compared to a normal uterus. In this condition the top of the uterus
points towards the back of the pelvic region rather than tilting towards
the bladder.
Causes
• Complication of endometriosis, fibroids, PID, multiparty, lack of
abdominal tone, genetics, previous abdominal surgeries.
Symptoms
• Painful menstruation
• Dyspareunia
• UTI
Treatment
• Treatment of the underlying cause.
• Surgery is performed to suspend a retroflexed uterus.
• Knee-chest position for ten minutes, three times per day.
• Kegel’s exercise
• Use of pessary
Menstrual irregularities and AUB
Abnormal Uterine Bleeding (AUB)
Menstruation is considered normal when uterine bleeding occurs
every 21 to 35 (average 28) days and is not excessive (blood
loss<80ml). The normal duration of menstrual bleeding is between
two and seven days (average 4days).
Abnormal uterine bleeding is excessive or prolonged or frequent
bleeding episodes.
Total Menstrual Blood loss >80ml
When Bleeding persists for >7days
Cycles <21 days

AUB is vaginal bleeding from the uterus that is abnormally frequent,


last excessively long, is more than normal, or is irregular.
Causes
PALM-COEIN is a useful acronym provided by the International
Federation of Obstetrics and Gynecology (FIGO) to classify the
underlying etiologies of abnormal uterine bleeding. The first portion,
PALM, describes structural issues. The second portion, COEI, describes
non-structural issues. The N stands for “not otherwise classified”.
Structural causes
• P: Polyp
• A: Adenomyosis
• L: Leiomyoma
• M: Malignancy and hyperplasia
Non-structural cause
• C: Coagulopathy
• O: Ovulatory dysfunction
• E: endometrial disorders
• I: Iatrogenic
• N: not otherwise classified
Clinical types
• Amenorrhea: Absence of menses for >6 months.
• Polymenorrhea: frequent but regular episodes of uterine bleeding
occurring at intervals of 21 days or less.
• Oligomenorrhea: Menstrual periods at interval of more than 35 days.
• Hypomenorrhea: Scanty menstruation. Uterine bleeding that is regular
but decreased in amount.
• Intermenstrual bleeding: bleeding of variable amounts occurring
between regular menstrual period.
• Menorrhagia: Uterine bleeding excessive in both amount and duration
of flow, but occurring at regular intervals.
• Metorrhagia: uterine bleeding occurring at irregular interval, the
amount is variable.
• Menometorrhagia: Uterine bleeding usually excessive and prolonged
occurring at irregular interval.
• Postmenopausal bleeding: Uterine bleeding that occurs more than 1
year after the last menses in a woman with ovarian failure.
Diagnosis of AUB
• History taking
• Physical examination
• Investigation/diagnostic test:
⁻ The pregnancy test is routine if the women is premenopausal
⁻ A blood count may be done to rule out anemia resulting from
excessive blood loss
⁻ Pap smear is also done to rule out cervical cancer
⁻ Pelvic ultrasound is often performed based on the women’s medical
history and pelvic examination
⁻ Investigation to rule out blood clotting disorder
• Blood sample can be tested to evaluate thyroid function, liver function
or kidney function abnormalities.
• Blood test for progesterone levels.
• If the doctor suspects that the ovaries are falling, such as with
menopause, blood levels of FSH my be tested.
• Additional blood hormones tests are done if the doctor suspects
polycystic ovary, or if excessive hair growth is present.
Treatment
Treatment goal includes:
• To find the cause of bleeding
• To control bleeding
• To prevent recurrence
• To preserve fertility
• Correct associated conditions
• Induce ovulation in patients who want to conceive
Management
Hormonal therapy:
• Intravenous conjugated equine estrogen
• Combined oral contraceptive pills
• Oral progestin's
Tranexamic acid prevents fibrin degradation and can be used to
treated acute AUB.
Based on the PALM-COEIN acronym for etiologies of chronic AUB,
specific treatment options for each category are listed below:
• Polyps are treated through surgical resection
• Adenomyosis is treated via hysterectomy. Less often,
adenomyomectomy is performed.
• Leiomyomas (fibroids) can be treated through medical or surgical
management depending on the patient’s desire for fertility, medical
comorbidities, pressure symptoms, and distortion of the uterine
cavity. Surgical options include uterine artery embolization,
endometrial ablation, or hysterectomy. Medical management
options include a levonorgestrel-releasing intrauterine device
(IUD), GnRH agonists, systematic progestin's, and tranexamic acid
with non-steroidal anti-inflammatory drugs (NSAIDs).
• Coagulopathies leading to AUB can be treated with tranexamic
acid or desmopressin (DDAVP).
• Ovulatory dysfunction can be treated through lifestyle modification
in women with obesity, PCOS, or other conditions in which
anovulatory cycles are suspected. Endocrine disorders should be
corrected with the use of appropriate medications.
• Iatrogenic causes of AUB should be managed based on the
offending drug and/or drugs.
• Endometritis can be treated with antibiotics.
Complications:
• Severe anemia
• Hypotension
• Infertility
• Endometrial cancer
Polymenorrhea
Polymenorrhea is defined as a cyclic bleeding where the cycle is
reduced to an arbitrary limit of fewer than 21 days and remains constant
at that frequency. If the frequent cycle is associated with excessive or
prolonged bleeding, is called epimenorrhagia.
Causes
• This condition usually occurs following childbirth and abortion,
during adolescence and premenopausal period and in pelvic
inflammatory disease.
• The follicular development is speeded up with resulting shortening of
the follicular phase. This is probably due to hyper stimulation of the
follicular growth by FSH.
• Rarely, the luteal phase may be shortened due to premature lysis of the
corpus luteum. Sometimes, it is related to stress-induces stimulation.
Treatment
• Treated by hormone: progestin, estrogen, danazol, mifepristone
• Antifibrinolytic agent e.g. tranexamic acid
• Surgical management:
- Uterine curettage
- Endometrial ablation
- Hysterectomy
Oligomenorrhea
Menstrual bleeding occurring more than 35 days apart and which
remains constant at that frequency is called Oligomenorrhea.
Causes
• Age related: it my be met in adolescence and preceding menopause
• Stress and exercise related
• Endocrine disorder:
- Hyperprolactinemia
- Prolactinomas
- Thyrotoxicosis
• Androgen producing tumors
• PCOS
• Anorexia nervosa
• Tubercular endometritis

Treatment
Drugs: Phenothiazine's, cimetidine, methyldopa
Treatment according to the cause
Metorrhagia
• A type of abnormal bleeding that occur at irregular intervals and with
variable amounts. The bleeding occurs between periods or is unrelated
to periods.
• Bleeding has no cycle, it is either irregular in occurance or is
continuous.
• Painless bleeding or other features may be associated with periods
• Intermenstrual bleeding often light or sometimes heavy
Causes
1. Uterine causes
2. Non-uterine causes
Uterine causes
• Fibroids especially sub-mucous type.
• Intrauterine mucus polyp, Dysfunctional uterine bleeding.
• Carcinoma body of the uterus.
• Retained products of the conception following childbirth, abortion.
• Ovulation bleeding.
• Erosion of the cervix.
• Carcinoma of cervix.
• Polyps of cervix.
• Use of IUCD or birth control pills.
Non-uterine causes
• Acute pelvic inflammation due to salphingitis.
• Complication of ovarian tumors
• Estrogen withdrawal bleeding
• Thrombocytopenia.
• Stress
• IUCD in uterus
Diagnosis
• History
• Obtain complete medical and menstrual cycles.
• Bleeding and non-bleeding days, heavy bleeding should be collected.
• Clinical examination
• Special investigations:
-Complete blood count,
-Thyroid function studies
-diagnostic curettage.
• Endometrial biopsy
• Hysteroscopy
• Hysterosalphingography
• USG of uterus, ovaries and pelvis.
Treatment
• Treatment is directed to the underlying pathology. Malignancy is
to be excluded prior to any definitive treatment.
• When a pelvic lesion is detected, this should be treated appropriately
according to cause.
• When no lesion is found, general measures are adopted;
✓Bed rest.
✓Sedatives
✓Drug: ergometrine to minimize the bleeding.
✓Hormones: commonly using hormones are estrogen,
progesterone and testosterone.
Surgery:
• Curettage, especially for retained products of conception,
endometritis and metropathia (abnormal excessive often continuous
uterine bleeding due to persistence of the follicular phase of menstrual
cycle).
• Hysterectomy: not responding to any form of treatment.
• Radiation: hemorrhage may be stopped by using either x-ray radiation
or by radium.
Dysmenorrhea
• Dysmenorrhea is defined as severe cramping pain in the lower
abdomen that occurs just before or during menses.
• Menstrual pain that interferes with daily activities.
• It is a painful menstruation of sufficient magnitude so as to
incapacitate day-to-day activities.
• Menstrual pain is any pain during menstruation whether it is normal or
abnormal.
Types
• Primary dysmenorrhea
• Secondary dysmenorrhea
Other types
• Membranous dysmenorrhea
• Neuralgic dysmenorrhea
• Obstructive or congenital or mechanical dysmenorrhea
• Ovarian dysmenorrhea
Primary dysmenorrhea
• Primary dysmenorrhea is defined as painful menses in women with
normal pelvic anatomy, usually begins during adolescence. It is
characterized by crampy pelvic pain beginning shortly before or at the
onset of menses and lasting one to three days.
• Dysmenorrhea is thought to be caused by the release of prostaglandins
in the menstrual fluid, which causes uterine contractions and pain.
• Vasopressin also may play a role by increasing uterine contractility
and causing ischemic pain as a result of vasoconstriction.
• Elevated vasopressin levels have been reported in women with
primary dysmenorrhea.
Causes
• Uterine contractions or ischemia
• Psychological factors
• Mostly confined to adolescents
• Almost always confined to ovulatory cycles
• Pain is like labor usually cured following pregnancy and vaginal
delivery.
1. Psychosomatic factors
• Pain is due to tension and anxiety during adolescence, presents with or
shortly after menarche.
• May starts within 6 months after menarche.
• Lower pain threshold is often aggravating factors.
• Perception of pain
2. Abnormal anatomical and functional aspects of uterus
 Uterine contraction
• Contraction abnormalities
• Elevated basal tone
• Elevated active pressure
• Non-rhythmic or in coordinate uterine contraction
• These abnormalities lead to poor uterine perfusion and oxygenation
thus giving rise to pain.
 Uterine blood flow
The strong and abnormal uterine contractions in dysmenorrheic
women reduce uterine blood flow and cause myometrium
ischemia, resulting pain.
Stenosis of the internal os or narrowing of the cervical canal
may lead to difficult for the menstrual blood to escape and cause
strong uterine contraction's and pain.
Unequal development of mullerian duct causes the pain due to
unequal muscular contractions.
3. Prostaglandins

• Prostaglandin (PG F2α) release from endometrial cells uterine


smooth muscle contraction, increased intra-uterine pressure & some
degree of uterine ischemia.
• PG production ↑ during the 1st 48-72 hrs of menses
• PG may also cause hypersensitization of pain terminals to physical
& chemical stimuli
4. Vasopressin

• Produce dysrhythmic uterine contraction that can reduce uterine blood


flow and cause uterine hypoxia and pain.
• Still controversial
Risk factors
• Age < 20 years
• Attempts to lose weight
• Depression/anxiety
• Disruption of social networks
• Heavy menses
• Nulliparity
• Smoking
Clinical features
1. Patient profile
• Predominantly confined to adolescence girls
• Usually appears within 2 years of menarche.
2. Symptoms
• Spasmodic fluctuating pain occurs few hours before onset of
menstrual flow; lasts for 2-3 days.
• Intense pain on the first and second day [24-36h]
• Located to suprapubic region
• Radiates into the inner thigh.
• Cramps are frequently accompanied by backache, nausea,
vomiting, diarrhea, headache, fatigue, pallor, cold, sweats.
• With severe pain absent from school or work.
Diagnosis
• History and physical examination
- Proximity of the onset of primary dysmenorrhea, menarche,
onset of symptoms with the onset of menstrual flow, duration,
regularity, time of pain, its characteristics etc.
- Perform only an abdominal examination in young adolescents
with a typical history who have never been sexually active
• Pelvic examination[ usually does not reveal any abnormal
findings]
• Ultrasonography
• laparoscopy or laparotomy with biopsy
Management
• Pharmacological approach
• Non-pharmacological approach
• Surgical
Pharmacological approach
1. NSAID: ibuprofen, sodium naproxen, nimesulide better pain relief
by inhibiting endometrial prostaglandins production.
2. Cyclooxygenase II inhibitors: Prostaglandin synthetase inhibitors
reduce intrauterine pressure & have direct analgesics effect.
[continue for 3-6 cycles]
3. Oral contraceptive: combined oral contraceptive pills with medium
dose estrogen and progesterone.
• These drugs reduce menstrual blood flow volume & prostaglandins to
within or even below or normal range.
• Oral pills may also lower the elevated vasopressin levels.
• Continue for 3-6 cycles.
4. Glyceryl trinitrate: diminished levels of nitric oxide induce
myometrial contractions while nitric oxide causes uterine
relaxation.
5. Calcium antagonists: Nifidine, a calcium channel blocker inhibits
myometrium contractibility.
6. Vitamin B
7. Vitamin E
8. herbs: rose tea
Non Pharmacological Approaches

1. Warmth
2. Transcutaneous Electrical nerve Stimulation [TENS]
3. Acupuncture & Acupressure
4. Self-help therapy: exercises, behavioral therapy
Surgical approaches

1. Dilatation of cervical canal


2. Nerve ablation
- Laproscopic presacral neuroctomy
- Laproscopic uterosacral nerve ablation
3. Spinal manipulation
Secondary dysmenorrhea
• It is caused by organic pelvic pathology and it usually has its onset
many years after the menarche.
• Any woman who develops secondary dysmenorrhea should be
considered to have organic pathology in the pelvis until proved
otherwise.
• It is a painful menses secondary to pathology pain may begin before
bleeding and may last for entire duration.
• The mechanism of pain in secondary dysmenorrhea is due to pelvic
congestion which is more marked in the premenstrual period.
• Pain increases in its severity as menstruation approaches and is
relieved by the onset of menstrual flow, due to the diminution of
pelvic congestion. [diminution; reduction in the size]
Causes
1. Endometriosis:
Pieces of the uterine lining or endometrium grow outside the uterus
in other parts of the pelvic cavity i.e. fallopian tubes, ovaries etc.
The tumors respond to the hormonal changes & bleed with periods.
Bleeding from endometriosis stays in the body & women with
endometriosis suffer from menstrual pain.
2. Uterine fibroids tumors:
3. Scar tissue[adhesions] can cause organs to stick together and resulting
pain.
4. Pelvic Inflammatory Disease
5. An ovarian cyst and tumor
6. Mullerian anomalies, endometrial polyp, pelvic congestion
Clinical features
1. Patient profile
- Patient are usually in between the age of 30-40 years.
- More often parous women occurs many years of relatively painless
menstruation.
2. Symptoms
- Pain starts 3-5 days before the onset of menses.
- Pain is dull aching lower abdominal continuous pain accompanied by
backache without any radiation.
- The intermenstrual period may have back and lower abdominal
discomforts.
- May associated with other symptoms as dyspareunia, infertility,
menorrhagia, white discharge.
3. Signs
- Patient is healthy except being slightly anemia.
- May be primary lesion felt like uterine fibroids.
- There may be any type of pelvic pathology in uterus as some
inflammatory s/s may be present.
Management
Treatment of secondary dysmenorrhea is to treat the cause rather than
symptoms. The type of treatment depends on the severity, age & parity
of the patient.
1. Management of Cx stenosis:
• Cervical stenosis increases the pressure during menses and scanty
menstrual flow and severe cramping throughout the menstrual cycle.
Cervical stenosis may be congenital or secondary to cervical injury
due to electrocautery, cryocautery and infection.
• Dilatation and curettage is done to treat the cervical stenosis, the
problem frequently recurs repeat the procedure.
2. Endometriosis
- Cauterization of endometriotic spots.
- Analgesics
- Gonadotropin releasing hormone agonists
3. Pelvic infections & adhesions
- Antibiotics
- Surgery: release of adhesions, TAH with BSO
4. Pelvic congestion syndrome
- Medroxyprogesterone acetate
- TAH with BSO
5. Correction of the anatomic abnormalities
6. Uterine artery embolization (UAE) may helpful to treat
dysmenorrhea caused by fibroids. In this procedure, the blood
vessels to the uterus are blocked with small particles, stopping the
blood flow that allows fibroids to grow.
7. Hysterectomy
Dysfunctional Uterine Bleeding
Definition
• DUB is defined as “a state of abnormal uterine bleeding without any
clinically detectable organic, systemic, and iatrogenic cause.” [Pelvic
pathology i.e. tumor, inflammation or pregnancy is excluded]
• Dysfunctional uterine bleeding (DUB) can be defined as “excessive
uterine bleeding (excessively heavy, prolonged or frequent), which is
not due to demonstrable pelvic disease, complications of pregnancy or
systemic disease”.
The prevalence varies widely but an incidence of 10-20% usually at
extremes of reproductive life and has a negative impact on the quality of
life of affected women, whether young or old. Twenty-percent of cases
of DUB occur in adolescence, and 40% of cases occur in patients over
age 40. The bleeding may be abnormal in frequency, amount or duration
or combination of any three.
• Bleeding patterns in DUB
• Excessive or heavy menstrual loss (menorrhagia)
• Irregular bleeding (metrorrhagia)
• Frequent bleeding with shortened cycle (polymenorrhoea).
• Prolonged bleeding
Types of DUB
• Ovular bleeding
• Anovular bleeding
Ovulatory
• 10% of cases occur in women who are ovulating. In ovulatory DUB,
progesterone secretion is prolonged; irregular shedding of the
endometrium results, probably because estrogen levels remain low, near
the threshold for bleeding (as occurs during menses). This causes
irregular shedding of the uterine lining and break-through bleeding. Some
evidence has associated ovulatory DUB with more fragile blood vessels
in the uterus.
• The major proposed mechanism of ovulatory dysfunctional uterine
bleeding is impaired hemostatic mechanism.
• A shift in the ratio of an endometrial vasoconstrictor (PGF2) to
vasodilator (PGE2) and an increase in total endometrial prostaglandins
have been demonstrated in ovulatory dysfunction uterine bleeding
patients.
• Platelet and plug formation are poor due to prolonged vasodilation. In
addition, a potent vasodilator parathyroid hormone-related protein and
high proteolytic lysosomal enzyme activity are increased in women
with ovulatory dysfunctional uterine bleeding. As a result, in ovulatory
dysfunctional uterine bleeding, treatment with prostaglandin
synthetase inhibitors are more effective than hormonal treatment.
Anovulatory
• About 90% of DUB events occur when ovulation is not occurring.
During an anovulatory cycle, the corpus luteum does not form. Thus,
the normal cyclic secretion of progesterone does not occur, and
estrogen stimulates the endometrium unopposed.
• Without progesterone, the endometrium continues to proliferate,
eventually outgrowing its blood supply; it then sloughs incompletely
and bleeds irregularly, and sometimes profusely or for a long time.
• When this abnormal process occurs repeatedly, the endometrium can
become hyperplastic sometimes with atypical or cancerous cells.
• Anovulatory menstrual cycles are common at the extremes of
reproductive age, such as early puberty and perimenopause. In such a
case, women do not properly develop and release a mature egg.
• In an anovulatory cycle, the corpus luteum, which is a mound of tissue
that produces progesterone, does not form. As a result, estrogen is
produced continuously, causing an overgrowth of the uterus lining.
• The period is delayed is such cases, and when it occurs menstruation
can be very heavy and prolonged.
• The cause can be psychological stress, weight change, exercise,
endocrinopathy, neoplasm, drugs or it may be otherwise unknown
Causes
Anovulatory cycle
• Estrogen breakthrough bleeding
• Estrogen withdrawal bleeding
• Oral contraceptives, progestin-only preparations, or postmenopausal
steroid replacement therapy
• Adolescent
• Climacteric
Ovulatory cycle
• Defect in endometrial hemostasis
Risk factors
• Adolescence
• Perimenopause
• Obesity
• Polycystic ovarian syndrome
• Cigarette smoking
• Stress
Symptoms
Symptoms of dysfunctional uterine bleeding may include:
• Bleeding or spotting from the vagina between periods
• Periods that occur less than 28 days apart (more common) or more
than 35 days apart
• Time between periods changes each month
• Heavier bleeding (such as passing large clots, needing to change
protection during the night, soaking through a sanitary pad or tampon
every hour for 2 - 3 hours in a row)
• Bleeding lasts for more days than normal or for more than 7 days
Other symptoms caused by changes in hormone levels may include:
• Excessive growth of body hair in a male pattern (hirsutism)
• Hot flashes
• Mood swings
• Tenderness and dryness of the vagina
• A woman may feel tired or have fatigue if she is loses too much blood
over time. This is a symptom of anemia.
Diagnosis
i. History taking
• Rule out the types of bleeding.
• Screening the personal and family history of bleeding disorders.
• The excessive bleeding is assessed by: number of pads used, passage
of clots, duration of bleeding.
• Estimate the hemoglobin percentage.
• Rule out the cause of bleeding, nature of bleeding [cyclic, acyclic], its
relation to puberty, menopause etc.
• Any emotional upset or psychosomatic factors, psychosexual
problems, use of steroidal contraceptive, IUCD insertion etc.
ii. General examination
• Abdominal and pelvic examination should carried out to rule out the
cause of bleeding.
• Bimanual examination including speculum examination should be
done all cases except virgin where rectal examination is done to
exclude palpable pelvic pathology.
• If the vaginal examination is required in virgin it should be carried out
under general anesthesia and along with endometrial curettage.
iii. Special Investigation
• CBC: estimation of hemoglobin, RBC, total and differential count.
• Estimation of bleeding and clotting time.
• Platelet count.
• Liver and thyroid function test in appropriate case.
• Vaginal cyto-hormonal studies to assess the hormonal status and
exclude the possibility of cervical neoplasia.
• Endocervical and endometrial curettage at any age if bleeding is
irregular.
• Pelvic ultrasonography to rule out any uterine or adnexal pathology.
• Hysteroscopy to identify endometrial polyp or carcinoma.
• Hormone test: FSH, LH, androgen, Prolactin, progesterone.
• Ultrasound and color Doppler studies: to find the endometrial
hyperplasia.
Management
The management depends on the age of the patient, severity of the
bleeding and the nature of the abnormality is assessed after examination
and necessary investigations. For the management, the patients may be
divided into three groups; general, medical and surgery.
General management
• Reassurance to the patients.
• Take rest.
• Improved nutritional status by a balanced diet along with iron and
vitamins.
• In the majority of the case, there is spontaneous resolution within six
months.
• If the patient is severely anemic with a hemoglobin count less than
7mg/dl or she has an irregular and continuous bleeding, patient should
be hospitalized and given a blood transfusion.
• Necessary hematological investigation should be carried out to
exclude the other cause of bleeding.
• Emotional stress, hypothyroidism or leukemia should be ruled out.
• If the bleeding is persistent and severe, diagnostic curettage is
necessary.
Non-hormonal management
• Anti-fibrinolytic agents [Traneximix acid]: reduces menstrual blood
loss by 50%. It Prevent conversion of plasminogen into plasmin which
dissolve the fibrin clots occluding the blood vessels. Reduce measured
loss by 40-50%. The effect is dose related. It should be given with the
start of menstruation and continue for 3-4 days.
• Prostaglandin synthetase inhibitors: mefenamic acid is much effective
in women aged more than 35 years and in cases of ovulatory DUB.
The dose is 150 - 600 mg orally in divided doses during the bleeding
phase. NSAIDS can reduce menstrual blood loss by 25 - 40%.
NSAIDs may be used as first line medical treatment.
• Desmopressin acetate (DDAVP)
It has been used to treat abnormal uterine bleeding in patients with
coagulation defects. Transiently elevates factor VIII and von Willebrand
factor. It is given IV (0.3Ug/kg) or intranasal.
Hormonal therapy: Either the following regimen is chosen;
Progestogens: In anovular bleeding medroxyprogesterone acetate 10mg or
Norethisterone acetate 5 mg is used from 5th to 25th day cycle for 3
cycles. Another cyclic therapy is 15th to 25th day course.
• In ovular bleeding where the patients wants pregnancy or in cases of
irregular shedding or irregular ripening endometrium,
medroxyprogesterone 1tab 10 mg daily or twice a day from 15th to 25th
day may cure the state. It is less effective than 5th to 25th day cycle.
• Continuous therapy: Continuous progesterone preparations are widely
used to treat menorrhagia. They counteract the effect of estrogen,
suppress the endometrium and the stroma is decidualised..
Medroxyprogesterone acetate (10 mg/d) or norethindrone acetate (2.5-5
mg/d) is given and treatment usually continued for at least for 90 days.
• Estrogen:
Prolonged uterine bleeding suggests the epithelial lining of the cavity
has become denuded over time. In this setting, a progestin is unlikely to
control bleeding. Estrogen alone will induce return to normal
endometrial growth rapidly. In a situation where the bleeding is acute
and severe, conjugated estrogen 25 mg is given IV. It may be repeated
every four hours till the bleeding is controlled, when oral therapy is
started. Homeostasis is achieved by conjugated equine estrogen 2.5 mg
tablet given 4 times a day. The bleeding usually stops within 2-3 days.
• Oral contraceptives
Oral contraceptive pills (OCPs) suppress endometrial development,
reestablish predictable bleeding patterns, decrease menstrual flow, and
lower the risk of iron deficiency anemia. OCPs can be used effectively
in a cyclic or continuous regimen to control dysfunctional bleeding.
• Danazol: Is an extremely effective drug for treatment of menstrual
problems but its use is limited by its high androgenic side effects. It is
suitable in cases with recurrent symptoms and in patients waiting for
hysterectomy. The dose varies from 200 – 400mg daily in 4 divided
doses continuously for 3 months. A smaller dose tends to minimize the
blood loss and higher dose produces amenorrhea.
• Gestrinone: 2.5 mg orally twice a week for 3 months has been found
useful. It reduces the secretion of FSH and LH. It has a much longer
half life.
• Mifepristone (RU 486): It is an anti-progesterone. It inhibits ovulation
and induces amenorrhea and reduces myoma size.
• Intrauterine progestogen: Levonorgestrel intrauterine system [LNG
IUS] induces endometrial glandular atrophy, stromal decidualisation
and endometrial cell inactivation. It is effective for 5 years. It has
minimal systemic absorption. Reduction of blood loss is up to 97%
after 12 month use. It is considered as a medical hysterectomy. The
levonorgestrel releasing intrauterine device releases LNG at a low
dose of 20ug per day.
• GnRH agonists: Work by reducing concentration of GnRH receptors
in the pituitary via receptor down regulation and induction of post-
receptor effects, which suppress gonadotropin release. Because
prolonged therapy with this form of medical castration is associated
with osteoporosis and other postmenopausal side effects, its use is
often limited in duration and add back therapy with a form of low-
dose hormonal replacement is given. It can be used to achieve short-
term relief from a bleeding problem, particularly in patients with renal
failure or blood dyscrasia.
Surgical Management of DUB
Most cases of DUB can be treated medically. Surgical measures are
reserved for situations when medical therapy has failed or is
contraindicated.
i. Uterine curettage
ii. Endometrial Ablation/resection
iii. Hysterectomy
Uterine curettage:

• It is done predominantly as a diagnostic tool but at times, it has got


hemostatic and therapeutic effect by removing the necrosed and
unhealthy endometrium. The indication is an urgent one, if the
bleeding is acyclic and where endometrial pathology is suspected.
• D&C is an appropriate diagnostic step in a patient who fails to
respond to hormonal management. The addition of hysteroscopy will
aid in the treatment of endometrial polyps or the performance of
directed uterine biopsies. As a rule, apply D&C rarely for therapeutic
use in DUB because it has not been shown to be very efficacious.
Endometrial ablation/resection:
Endometrial ablation is a procedure that destroys (ablates) the uterine
lining, or endometrium. This procedure is used to treat dysfunctional or
abnormal uterine bleeding. Endometrial ablation is an alternative for
those who wish to avoid hysterectomy or who are not candidates for
major surgery.
Endometrial ablation is used to control heavy, prolonged vaginal
bleeding when:
• Bleeding has not responded to other treatments.
• Childbearing is completed.
• Where hysterectomy is risky or the patient is unfit for longer duration
surgery or because of obesity or adhesions
• When patient prefers to preserve her uterus.
Hysterectomy 
It is the surgical removal of the uterus. It may also involve removal of
the cervix, ovaries, fallopian tubes and other surrounding structures.
Usually performed by a gynecologist, hysterectomy may be total
(removing the body, fundus, and cervix of the uterus; often called
"complete") or partial (removal of the uterine body while leaving the
cervix intact; also called "supracervical").
Amenorrhea
Amenorrhea means the absence of menstruation. Amenorrhea is
physiological before puberty, during pregnancy and lactation and
following menopause.
Classification of amenorrhea
According to onset According to hidden or apparent
- Primary amenorrhea - False amenorrhea
- Secondary amenorrhea - True amenorrhea
According to cause
• Physiologic amenorrhea
• Pathologic amenorrhea
Primary amenorrhea
Primary amenorrhea is the failure to start menstruation by the age of 16
in a girl with normal secondary sexual characteristics or by the age of
14 where there is a failure to develop secondary sexual characteristics.
Causes of primary amenorrhea
• Outflow tract obstruction
- Absent of uterus, vagina
- Imperforate hymen
- Transverse vaginal septum
• Developmental defect of genital tract
- Mullerian agenesis
- Absence of uterus
• Turner’s syndrome: Associated with streak ovarian tissue and primary
amenorrhea.
• Androgen insensitivity: A syndrome found in patient with X, Y
chromosome but resistant to androgens (androgen insensitivity). In
this condition there male karyotype (45XY) with female appearance.
Female appearance with normal breast development and external
genitalia.
• Hypothalamic failure (Kallmann’s syndrome): Congenital disorder
characterized by hypogonadism.
• Hypothalamic and pituitary dysfunction
• Emotional stress
• Delayed puberty
Secondary amenorrhea
Secondary amenorrhea is the absence of menstrual periods for 6 months in
a woman who had previously been regular, or for 12 months in a woman
who had irregular periods without any physiological reasons.
Causes of secondary amenorrhea
• Hypothalamic dysfunction:
Hypothalamic dysfunction is one of the most common types of secondary
amenorrhea. Although isolated gonadotropin-releasing hormone (GnRH)
deficiency most commonly presents as primary amenorrhea, it extremely
rarely presents as secondary amenorrhea.
• Systemic illness:
Systemic illness may be associated with menstrual cycle disorders when it
is severe enough to result in a decrease in hypothalamic GnRH secretion
and/or when it is associated with nutritional deficiencies.
• Pregnancy
• Pituitary failure/pituitary tumor: it is usually the acquired type as the
result of trauma treatment of pituitary tumor or infraction after massive
blood loss (Sheehan’s syndrome). Hyperprolactinemia which causes
secondary amenorrhea. Stress, sleep and intercourse can also raise
serum prolactin.
• Pituitary disease: Among pituitary disorders that cause secondary
amenorrhea, lactotrophadenomas are the most common).prolactin
appears to cause amenorrhea by suppressing hypothalamic GnRH
secretion, leading to low gonadotropin and estradiol concentrations.
Unlike the other pituitary hormones, prolactin release is mostly
controlled by inhibition, primarily by hypothalamic dopamine. The
negative regulation by dopamine is so potent that disruption of the
pituitary stalk, by trauma or a large tumor, leads to hyperprolactinemia.
• Other disease of the pituitary: Sheehan syndrome, radiation, infraction,
and infiltrative lesions of the pituitary gland.
• Thyroid disorder/disease and chusing’s disease: Interfere with the
normal functioning of the hypothalamic pituitary- ovarian axis –
present with amenorrhea. High level of thyroxin inhibits FSH release.
• Primary ovarian insufficiency (premature ovarian failure):
The depletion of oocyte before age 40 years is called primary open
insufficiency. Most women experience intermittent follicular
development, estradiol production, LH surges, ovulation, and menstrual
bleeding between months of hypoestrogenic. when POI is complex, lack
of ovarian function leads to estrogen deficiency , endometrial atrophy
and cessation of menstruation. Premature ovarian failure may be due to:
-Chemotherapy and radiotherapy
- Autoimmune disease following viral infection
- Following surgery for condition such as endometriosis
Physiologic amenorrhea
The occurrence of amenorrhea is said to be normal in four states, before
puberty, during pregnancy, during lactating and after menopause

Pathologic amenorrhea
This type of amenorrhea is due to an organic disease; which could be
anatomical or functional.

Cryptomenorrhea
Risk factors
• Hypothalamic tumors and infiltrative lesion
• Systemic illness
• Polycystic ovary syndrome
• Uterine disorders
• Drugs causing hyper prolactinemia
Diagnosis
In primary amenorrhea
• History of the development of secondary sex characteristics, sexual
activity, evidence of psychological dysfunction or emotional stress,
family history of possible genetic anomalies or diabetes, the presence
of galactorrhea, symptoms of a thyroid disorder, weight loss or gain,
hirsutism or menopausal symptoms should be sought.
• If no sexual characteristics are present, there is usually a delay in
puberty due to malnutrition (stunting), chronic childhood illness,
excessive physical activity combined with reduced energy intake or
the delay is constitutional.
History of :
- Infections, especially encephalitis and meningitis
- Abdominal operations. (removal of ovaries)
- Delayed menarche or androgen insensitivity syndrome.
- Chronic childhood disease
- Severe weight loss or gain suggests metabolic disorder that
influences hypothalamic function.
- Cyclic abdominal pain ( suggest underlying malformations)
- Hirsutism
In secondary amenorrhea
Duration of amenorrhea and history of previous cycles. At what age did
menarche start? Did the woman have a regular menstrual cycle (21-35
days) or was it irregular (<21 days or >35 days)? How long has she not
menstruated?
History of :
-contraception
- Subfertility ( around 20% of women with subfertility have
amenorrhea)
- PID and STIs
- Medical history of TB, diabetes, chronic nephritis
- History of severe blood loss or shock after delivery
- Breast feeding
- Galactorrhea
- Endometritis or myometritis or PID
- Fever after miscarriage, deliveries, cesarean section.
- Chronic disease, weight loss, night sweats, fever > 1month,
diarrhea > 1 month. HIV, tuberculosis, cancer, end stage renal
disease etc. can cause a catabolic state with severe wastage.

Physical examination
Investigations
Site of Disorder Diagnosis Investigations
Hypothalamus Hypothalamic- FSH, LH and estradiol low
hypogonadism
Pituitary Pituitary adenoma Prolactin high
Ovary Gonadal dysgenesis FSH and LH High,
Estradiol low
Mullerian tract Absent uterus PCT- negative
Karyotyping- 46XY
Genital tract Imperfotate hymen FSH LH and estradiol-
normal
PCT negative
Management
The overall goals of management in women with amenorrhea include:
• Correcting the underlying pathology
• Helping the women to achieve fertility
• Preventing the complication if the disease process.
Treatment varies depending upon the cause of amenorrhea
• Surgery
- Remove the androgen producing tumor of the ovary
- Remove gonad in testicular feminization
- Imperforated hymen correction
- Creation of functional vagina in absence or hypoplasia of the
vagina
- Hysteroscopic lysis of the intrauterine adhesions
- Surgical resection of the pituitary tumors
- Surgery to correct abnormalities of genital tract
- Incision of membrane in cryptomenorrhea
• Hormonal replacement therapy:
- Testicular feminization
- Turner’s syndrome
- Polycystic ovarian syndrome
- Anovulation
- Primary ovarian insufficiency
- Hyperprolactinemia

• Treatment of thyroid disease with appropriate medical attention


• Counselling in case of eating disorders
• Modification of lifestyle in hypothalamic dysfunction
• Using stress reduction technique
• Cognitive behavioral therapy

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