Amenorrhea - Define, Cause, Sign and Symptoms, Type- Pathological and Physiological Amenorrhea and It's Treatment and management, Cushing Syndrome - Define, Causes, Sign And Symptoms in PPT
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Sep 15, 2020
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About This Presentation
Amenorrhea - Define, Cause, Sign and Symptoms, Type- Pathological and Physiological Amenorrhea and It's Treatment and management, Cushing Syndrome - Define, Causes, Sign And Symptoms in PPT made By Sonal Patel
Classification of amenorrhea:
A) Physiological amenorrhea:
1-Before puberty:due to suppression of the
hypothalamo-pit.-ovarian axis which is
sensitive to the low level of estrogen
2-Pregnancy:due to pregnancy hormones;
estrogen & progesterone which suppress
gonadotropins.
3-Postpartum:Lactation amenorrhea is due to
prolactin which alters LH & FSH secretion or
inhibits GTH release
4-Postmenopausal:due to intrinsic ovarian
failure (depletion of ovarian follicles)
Examination:
General examination:normal 2ry sex characters
Abdominal examination:pelvi-abdominal swelling
may be felt.
Local examination:bulging membrane at the
introitus which appear as a dark blue or purple
color due to retained blood. Gentle pressure on
the swelling will transmit a thrill to the vaginal
membrane.
2-Vaginal septum & atresia with a functioning
uterus:a transverse vaginal septum or
failure of canalization of the entire vagina.
The uterus develops normally
hematometra & hematosalpinx when
menstruation is established.
Treatment:
Excision of vaginal septum.
For vaginal atresia: Laparotomy (open
the uterine cavity) drainage
Artificial vagina is constructed with a
therich skin graft.
3 -Atresia of the cervix with a
functioning uterus:is rare.
b-Acquired causes of
crytomenorrhoea:
1-Adhesive vaginitis:due to senile
vaginitis or due to radiotherapy or
chemical burns
2-Cervical stenosis:due to excessive
cauterization, amputation of the cervix,
trachelorrhaphy and Fothergil
operation
II -True amenorrhea:may be primary or
secondary
Drainage of hematocolpos
*Definitionofprimaryamenorrhea:failureof
menarchetooccurwhenexpectedinrelation
totheonsetofpubertaldevelopment.
Itisdefinedeitherasabsenceofmensesbyage14
yearswiththeabsenceofgrowthordevelopmentof
secondarysexualcharacteristicsorasabsenceof
mensesbyage16yearsregardlessofgrowthand
devlopmentofsecondarysexualcharacteristics.
*Prevalence:about 0.3%
I) Constitutional:no pathology in the endocrine axis.
Constitutional pubertal delay:is caused by
immature pulsatile release of gonadotrophin-
releasing hormone; maturation eventually occurs
spontaneously.
Common cause (20%)
Under stature and delayed bone age (X-ray Wrist
joint)
Positive family history
Diagnosis by exclusion and follow up
No anatomical abnormality and endocrine
investigations show normal results.
Prognosis is good
No drug therapy is required –Reassurance
Constitutional delay
Primary amenorrhea (cont.)
II) Hypothalamic:
1-Frolich's syndrome (Dystrophia adiposo-genitalis):
-Characterized by:
• Adiposity
• Absence of 2ry sex characters
• Atrophy of genital organs (small uterus, acute AVF, pin hole
external os and short vagina)
-Laboratory diagnosis:
• Pituitary gonadotrophins • Estrogen
-Treatment:no specific treatment. Treatment to relieve amenorrhea &
obesity by:
a-Reduction of weight
b-Thyroid hormone
c-Estrogen & progesterone
d-Pituitary gonadotophins FSH & LH
2-Laurance Moon Biedl's syndrome:in addition to findings
in Frolich's syndrome there is: retinitis pigmentosa,
polydactly & mental deficiency
Frolich's syndrome
Primary amenorrhea (cont.)
III) Pituitary causes:
Pituitary infantilism:the failure in the pituitary may be:
a-Selective pituitary failure:
-Specific failure to produce GTH
-Characters:
I.No secondary sex characters.
II.Amenorrhea
III.Genital atrophy
IV.Dwarfism
b-Panhypopituitrism:
-Thyroid failure (Hypotension -hypoglycemia)
-Adrenal failure
-17 ketosteroids
3-Prepubertal polycystic ovarian syndrome
4-Resistant (insensitive ovary syndrome):
1ry amenorrhea with well developed pubic &
axillary hair
Atrophic vaginal & endometrial mucosa
Elevation of FSH & LH with low estrogen
N/E of the ovary resemble prepubertal organ
M/E show numerous primordial follicles not
passed to mature size
Treatment: Ovulation induction by GTH ( high
doses)
V) Uterine causes:
1-Mayer-Rokitansky-Kuster-Hauser
Syndrome(MRKH) (utero-vaginal agenesis):
15% of primary amenorrhea. It is due to
congenital absence of mullerian ducts and is
characterized by:
Normal secondary sexual development &
external female genitalia
Normal female range testosterone level
Absent uterus , fallopian tube, upper vagina
and normal ovaries
Karyotype 46-XX
15-30% renal, skeletal and middle ear
anomalies
DD:Testicular Feminization Syndrome
Treatment:dilatation orvaginoplastyin order to
lead to a normal sexual life
2-Severe degrees of hypoplasia
3-Refractory endometrium
VI) Disorders of adrenal gland:
Adrenogenital syndrome:Late
onset congenital adrenal
hyperplasia (CAH)
Autosomal recessive trait
Most common form is due to 21-
hydroxylase deficiency in adrenal glands,
androgens can not be converted to
corticosteroids Female
pseudohermaphroditism.
Severe forms show signs of severe
androgen excess Defeminization &
masculinization
High 17 α -OH-progesterone blood level
Treatment: cortisolreplacement and may
be corrective surgery for external genitalia
Late onset CAH
Diagnosis of primary amenorrhea:
History:obtaining a thorough history is
essential and comprises:
Childhood growth and development including
height and weight charts and age at thelarche.
Age at menarche of the patient's mother and
sisters
History of chronic illness, trauma, surgery, and
medications
Information regarding exercise, diet and
psychosocial issues.
History of symptoms as cyclic lower abdominal
pain , virilizing changes
Diagnosis of primary amenorrhea (cont.)
Physical examination:special attention should be directed toward
evaluating
General:
Body dimensions ( Height & span) and habitus.
Distribution and extent of body hair
Muscle mass or other signs of virilization.
Extent of breast development by Tanner staging
Look for signs of Turner syndrome
Abdominal:look for pregnancy or pelviabdominal mass e.g ovarian mass
or hematocolpos
Local examination:of external and internal genitalia, with emphasis to
look for
Presence or absence of the uterus
Presence or absence of patent vagina or vaginal pouch
Evidence of exposure to androgens (pubic hair distribution and
clitoromegaly).
Imperforate hymen
Pelvic fullness (pregnancy, ovarian mass, and genital anomalies)
P/R: for virgins, hematocolpos
U/S examination:If a genital examination is
not feasible, an abdominal ultrasound may be
useful to confirm the presence or absence of
the uterus.
Investigations of primary amenorrhea:
Pregnancy test
Physical examination to determine presence of
uterus
FSH
N.B. Some patients will not demonstrate any obvious etiology for their
amenorrhea on history and physical examination. These patients can
be subjected to work up in a logical manner using a stepwise
approach after pregnancy is excluded.
Diagnosis of primary amenorrhea (cont.)
Evaluation of primary amenorrhea
Diagnosis of primary amenorrhea (cont.)
* Treatment of primary amenorrhea:
If possible, the aim of therapy is correction of the cause e.g.
Bromocripitine therapy for prolactinoma
Specific therapy for malnutrition, malabsorption, weight loss,
anorexia nervosa, etc
Imperforate hymen: cruciate incision
Transverse vaginal septum: surgical removal
Hypoplasia or absence of cervix in presence of functioning
uterus hysterectomy is required as repair of the cervix has not
been successful
Absent or short vagina: progressive dilatation or Mcindo’s
split thickness graft
Patients with karyotype XY or mosaic gonads should be
removed
Clomiphene citrate is ineffective in patients with hypogonadism
due to hypoestrogenism but HMG or pulsatile Gn RH can be
effective
Patients with physiologic delay reassurance
Patients with all forms of gonadal failure and hypogonadotropic
hypogonadism cyclic estrogen and progestin therapy
Secondary amenorrhea
Definition:the cessation of menstruation for at least 6 months
or for at least 3 of the previous 3 cycle intervals.
Prevalence:about 3% I- Constitutional Acute infections
Chronic debilitating diseases
Nutritional disorders
Psychological disturbances
Endocrinal (thyroid & pancreas)
II- Hypothalamic Frolich's syndrome
Diseases mid brain
Idiopathic hypothalamic insufficiency
Chiari frommel syndrome
Iatrogenic (drugs & steroids)
III- Pituitary causes Adenomas: . Acidophil .Basophil
. Chromophobe Simmond’s D.
Hypofunction panhypopit. Sheehan’s
( Insufficiency) Levi-lorian syndrome.
d-Anorexia nervosa: disease
of adolescence & is
characterized by
1ry or 2ry amenorrhea is often first
sign
A body mass index (BMI) <17 kg/m²
Hypothalamic suppression
Abnormal body image, emaciation,
intense fear of weight gain, often
strenuous exercise
Sense of wellbeing despite weight
loss
Mean age of onset 13-14 yrs (range
10-21 yrs)
Bulemics less commonly have
amenorrhea due to fluctuations in
body weight.
Anorexia nervosa
II) Hypothalamic amenorrhea:
1-Frolich’s syndrome
2-Diseases of mid brain
3-Idiopathic hypothalamic insufficiency
4-Chiari-Frommel syndrome:representsa prolonged physiological lactational
amenorrhea due to inhibition of PRL inhibiting factor of hypothalamus and is characterized
by: . Atrophy of vaginal & uterine mucosa
. Sustained breast developments
. Low pituitary gonadotropins
N.Bif unassociated with pregnancy, the presence of pituitary tumour should be
considered e.g. Ahumada Del Castello syndrome.
DD of postpartum amenorrhea:
Physiological condition (lactation or pregnancy)
Sheehan's syndrome & anterior pituitary necrosis
History of postpartum D&C causing infection and endometrial scarring
(Asherman's syndrome)
Chiari-Fromel syndrome -Cesarian Hysterctomy
Amenorrhea-galactorrhea
-Phenothiazine derivatives
-Oral contraceptive pills
They hypothalamic releasing
hormone (prolonged effect in some
cases can occur)
5-latrogenic (Drugs & steroids):
Pituitary T. (cont.)
2)Basophiladenoma(Cushing'ssyndrome):
Characters:seediagram
3)Chromophobeadenoma:
-Amenorrheaduetodestructionofpituitary
tissue
-Prolactin producing adenoma are associated
with Forbes Albright syndrome
characterized by persistent lactation in
absence of a previous pregnancy
-Atrophyofuterine&vaginalmucosa
-Smallsizedovaries
-Galactorrhea
Diagnosis:PRLassay
Anylevel>120ng%indicatesinvestigation
forpituitarytumor
Cushing's syndrome
B) Pituitary insufficiency (Scarring):
-Simmond's disease:panhypopituitrism
-Sheehan syndrome:
Etiology:
1-Postpartum hemorrhage
2-Shock
3-Puerperal sepsis
4-Fracture base, meningitis
5-Use of ergot (pituitary thrombosis)
Criteria of Sheehan 's syndrome:
After immediate recovery of pituitary thrombosis
or postpartum collapse, amenorrhea and failure
of lactation occur
IV) Ovarian causes:
1-Premature menopause:
-Caused by disappearance of
oocytes from the ovary
-Etiology: germ cells damage due
to radiation, methotrexate, T.B,
surgical removal and
auto-immune diseases
2-PCOD (Stein Leventhal
Syndrome):see ovulation
disorders
Asherman's syndrome
Secondary amenorrhea (cont.)
V) Uterine causes:destruction of the endometriumdue to T.B or
Asherman'ssyndrome
Diagnosis:
-Endometrial curettage for T.B
-HSG & Hysteroscopy for Asherman'ssyndrome
VI-Disorders of adrenal gland:
1-Adrenogenitalsyndrome: see before
2-Cushing's syndrome:due to excess secretion of corticosteroids by
adrenals
3-Adrenal tumours:
Characters:
1-Marked hypertension due to corticosteroids
2-Early amenorrhea
3-17 ketosteroidsNot suppressed by dexamethasone
4-DHEA-S is specific to adrenal androgens
5-Defeminizationand masculinization
Diagnosis of a case of amenorrhea: (cont.)
2-Examination:
General:for
Heightandweight:calculatebodymassindexif
appropriate.
Signsofexcessandrogens,e.g.hirsutism,acne
Signsofvirilization,e.g.deepvoice,clitoromegalyin
additiontohirsutism,andacne
Signsofthyroiddisease.
Acanthosisnigricans:thishyperpigmentedthickeningof
theskinfoldsoftheaxillaandneckisasignof
profoundinsulinresistance.Itisassociatedwith
polycysticovarysyndrome(PCOS)andobesity.
Breastexaminationforgalactorrhoea.
Fundoscopyandassessmentofvisualfieldsifthereis
suspicionofpituitarytumour.
Diagnosis of a case of amenorrhea
Abdominalexamination:
Pelvi-abdominalmasses:excludepregnancyinevery
caseof2ryamenorrhea.
Pubichair
StriaeasinCushing'ssyndrome.
Localpelvicexamination:
Vulva:developmentisanindexofovarianfunction
Vagina:thin&poorlydevelopedinhypogonadism
Cervix:stenosis
Uterus:masses-size
Treatment of secondary amenorrhea:treat the cause
1-Asherman’s syndrome:
Hysteroscopic resection with scissors or electrocautery
Pediatric Foley catheter placed in uterine cavity for 7-10
days
Systemic B. spectrum antibiotic and 2 month course of
high dose estrogen with monthly progesterone
withdrawal to prevent reformation of adhesions.
2-Ovarian failure:
-Estrogen replacement
-Gonadectomy when y cell line is present
Treatment of secondary amenorrhea:
3-Hypothalamo-pituitary lesions and dysfunctions:
CNStumours:surgicalremoval,radiationtherapyorcombinationofboth
forCNStumoursotherthanprolactinomas
Thyroiddisorders:thyroidhormone,radioactiveiodineorantithyroiddrugs
Hyperprolactinemia:
-Discontinuemedicationsleadingtohyperprolactinemia.
-Bromocriptine -Rarely,surgeryforlargepit.tumours
Panhypopititirism:variousreplacementregimens
-Estrogenreplacementtherapyforlackofgonadotropins
-CorticosteroidreplacementforlackofACTH
-ThyroidhormoneforlackofTSH
-Syntheticvasopressin