Primary Amenorrhea

4,544 views 26 slides Aug 20, 2020
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Primary amenorrhea DR ANITA RAMESH MD,OBGY

DEFINITION Amenorrhea means absence of menstruation TYPES Physiological amenorrhea Pathological amenorrhea Primary amenorrhea Secondary amenorrhea

PRIMARY AMENORRHEA Absence of menses by the age of 14 years with no secondary sexual characters 0R Absence of menses by the age of 16 years irrespective of development of secondary sexual characters

SECONDARY AMENORRHEA Absence of menses for 6 months or absence of atleast 3 of the previous 3 cycles

INCIDENCE OF PATHOLOGICAL AMENORRHEA 3% to 4% About 10% to 20% of those with infertility have amenorrhea

EVENTS CAUSING PUBERTY & MAINTAINENCE OF REGULAR MENSES Normal chromosomal pattern Co ordinated HPO axis Anatomical presence & patency of outflow tract Responsive endometrium Active thyroid & adrenal glands

PHYSIOLOGICAL AMENORRHEA Before puberty Pregnancy Lactation menopause

CRYTOMENORRHEA PATHOPHSIOLOGY

Usg image of haematocolpos

SYMPTOMS OF CRPTOMENORRHEA Amenorrhea Periodic pain in the lower abdomen Abdominal lump Retention of urine

TREATMENT OF CRYPTOMENORRHEA Imperforate hymen:cruciate incision over bulging hymen Cervical stenosis:cervical dilatation Transverse vaginal septum:excision of septum

Primary amenorrhea Hypergonadotropic hypogonadism (43%) Developmental defects of genital tract(30%) Hyogonatotropic hypogonadism (27%) Abnormal chromosomal pattern Dysfunction of thyroid or adrenal gland( adrenogenital syndrome,cretinism ) Metabolic disorders(juvenile diabetes) Systemic illnesses( malnutition,TB,weight loss,anaemia ) Unresponsive endometrium:uterine synchiae due to TB

Hypergonadotropic hypogonadism Primary ovarian failure Resistant ovarian syndrome Galactosemia Enzyme deficiency:17 alpa hydroxylase deficiency

Developmental defects of genital tract Mullerian agenesis or dysgenesis Imperforate hymen Transverse vaginal septum Atresia of upper third of vagina & cervix Complete absence of vagina Absence of uterus(MRKH)

HYPOGONADOTROPIC HYPOGONADISM C onstitutional delay:delayed GnRH pulse activation HPO AXIS DYSFUNCTION:stress,weight loss,excessive exercise,chronic disease(TB),anorexia nervosa Kallmans disease(inadequate GnRH pulse secretion CNS TUMOURS:craniophayngioma (reduced Gnrh RELEASE

Abnormal chromosomal pattern Turner”s syndrome(45X or various mosaics) Pure gonadal dysgenesis (46XX…phenotypically female with streak gonads) Androgen insensitivity syndrome(testicular feministion syndrome…46XY) Partial deletions of X chromosome

Investigations in a pt of primary amenorrhea with n sec sexual characters

Investigations in a pt with absent or poorly dev sec sexual characters

Management in primarary amenorrhea DEVELOPMENTAL ANOMALIES ABSENT VAGINA:Vaginoplasty before marriage CHROMOSOMAL ABNORMALITIES Turner”s syndrome:short course of E+P for breast development XY Gonadal dysgenesis:gonads to be removed for preventing seminoma or dysgerminoma

Androgen insensitivity syndrome:Reared as female only & removal of gonads.HRT with CEE 0.625 mg continued for maintainence of secondary sexual characters HPO Axis defect: Constitutional delay:reassure patient or can be induced using E+P Kallman”s syndrome:periods induced with pulsatile GnRH administration HP tumours like craniopharyngioma needs excision or radiotheraphy

Thyroid & adrenal dysfunction: Cretinism:thyroid replacement theraphy A drenogenital syndrome with enlarged clitoris needs clitoroplasty & corticosteriods for prolonged theraphy 17 alpha hydroxylase deficiency:corticosteroid replacement theraphy Prolactinomas:dopamine agonists

Metabolic & nutritional Diabetes:antidiabetics TB:Anti TB drugs Correction of weight loss Reduction of stress Unresponsive endometrium due to TB Anti TB drugs,adhesiolysis & high dose E+P

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