CAUSES OF PRIMARY AMENORRHOEA THEIR MANAGEMENT AND TREATMENT

 Amenorrhoea

PRIMARY AMENORRHOEA

Summary

1. Causes of amenorrhoea

2. Absent secondary sex characters

3. Normal secondary sex characters

4. Heterosexual development

5. Constitutional delay

6. Management

7. Treatment

The average age of the first menstrual cycle at the onset of puberty (menarche) is thirteen years with the range of 11-15 years. The girls failing to have their menarche by the age of 16, are said to be suffering from primary amenorrhoea.

CAUSES OF PRIMARY AMENORRHOEA

The causes of primary amenorrhoea can be broadly classified into 4 groups depending upon its clinical presentation.

A. About secondary sex character

1.Short stature

i). Hypothalamic pituitary dysfunction

(hypogonadotrophic hypogonadism)

Hydrocephalus

Craniopharyngioma

Panhypopituitarism

( empty sella syndrome)

Damage due to trauma / radiation

ii). Ovarian failure ( hypergonadotrophic hypogonadism )

Turner’s syndrome (45 XO)

Mosaic gonadal dysgenesis

( 45  XO/ 46XY)

2. Normal stature

i). Hypothalamic pituitary dysfunction

( hypogonadotropic hypogonadism )

Isolated GmRH deficiency

Olfactogenital syndrome ( Kallmann’s syndrome )

Hyperprolactinaemia

Excessive exercise

Weight loss/ anorexia nervosa

ii). Ovarian failure (hypogonadotropic hypogonadism )

True gonadal agenesis

Premature menopause

Galactosaemia 

B. Normal secondary sex characters

i). Anatomical abnormalities 

Imperforate hymen

Transverse vaginal septum

Absent vagina and functioning uterus

Absent vagina and non functioning uterus

ii). Androgen insensitivity ( testicular feminizing syndrome )

iii). Resistant ovary syndrome 

iv). Polycystic ovarian disease 

v). prolactinoma

 C. Heterosexual development

i). Congenital adrenal hyperplasia

ii) 5 𝞪 reductase deficiency

iii). Ovarian / adrenal tumours

iv). Absent anti Mullerian factor

v). True hermaphrodite

 D. Constitutional delay

MANAGEMENT

    
The age at which essential amenorrhoea ought to be explored is questionable. Most specialists concur that the persistent ought to be explored on the off chance that she presents after 16 a long time of age. Within the nonattendance of other auxiliary sex characters ( breast , pubic and axillary hair.) it is sensible to commence the examinations at 14 a long time of age. In case no cause is found after intensive assessment, the treatment in such cases may be postponed up to the age of 20 a long time, as the protected delay in menarche can amplify essential amenorrhoea to this age.

Absent secondary sex characters.

    
Initial phase in the administration of essential amenorrhoea is to search for the improvement of other auxiliary sex characters. In the event that the other optional sex characters are additionally missing, measure the stature (< 4' - 10'' ) chromosomal anomaly ought to be suspected. Highlights other than shorts height supporting chromosomal irregularity incorporate expanded conveying point, webbed neck, wide divided areolas, visual weakness and coarctation of aorta. A karyotyping will affirm the sort of chromosomal anomaly. The commonest chromosomal irregularities giving these highlights are Turner disorder (45XO), mosaic Turner ( 45XO/46XX) and blended gonadal dysgenesis (45XO/46XY).

    
Essential amenorrhoea with short height, without previously mentioned related highlights, is bound to be because of one of the intracranial lesions.hydrocephalus would be available since birth, panhypopituitarism has insufficiency of gonadotropins and development chemical as well as thyroid and adrenal organs would likewise be influenced. History of head injury/medical procedure or light ought to be taken. Craniopharyngioma, an intracranial with panhypopituitarism can give migraine. CT output of the noggin is the most suitable examination to separate among these conditions.

At the point when auxiliary sex chracters are missing however the tallness is ordinary than serum FSH and LH levels ought to be estimated. The low levels of these chemicals can be expected to disengaged GnRH insufficiency, olfactogential disorder or hyperprolactinemia. The patients with olfactogential disorder have loss of feeling of smell, while in the event of hyperprolactinemia there might be a background marked by galactorrhoea and prolactin level is raised. Raised LH and FSH levels are because of genuine gonadal agenesis, untimely ovarian disappointment or galactosaemia. Karyotyping should be acted in evident gonadal agenesis, as it tends to be 46XX or 46 XY and balls eliminated in cases with 46 XY in view of expanded danger of gonadal threat.

Normal .secondary sex characters

    
At the point when the other optional characters are all around created and essential amenorrhea is the solitary grievance, the gential plot ought to be altogether assessed for anatomical irregularities. Surge hindrance can undoubtedly be analyzed on clinical assessment. Flawless hymen is viewed as a strained, somewhat blue protruding film at the introitus. If there should be an occurrence of cross over vaginal septum and missing vagina with working uterus, gathered blood over the square is felt as protruding mass on delicate rectal assessment. The finding of different anatomical irregularities related with essential amenorrhoea can be affirmed on ultrasound. The most intriguing condition giving essential amnorrehea within the sight of typical optional sex characters is androgen inhumanity ( testicular treatment ) . The patient with this issue shows up as a typical grown-up female, yet hereditarily he is male ( 46 XY) . The testicles are either intrapelvic , in the crotch or in labia. Upper 66% of the vagina and uterus are missing. Testosterone is ordinarily emitted yet can not tie to the testosterone receptors due to nonappearance of restricting catalysts. Androgen inhumanity is suspected on clinical assessment and affirmed on karyotyping. Subsequent to barring anatomical anomalies and androgen heartlessness , serum FSH, LH and prolactin ought to be estimated FSH and LH are brought up in safe ovary condition, LH/FSH proportion is raised in patients with polycystic ovary and high prolactin level is reminiscent of prolactinoma. Essential amenorrehoea within the sight of ordinary LH, FSH and prolactin level and within the sight of other auxiliary sex characters is expected to consitutional delay.

Heterosexual development

    
At the point when essential amenorrhea is related with highlight of androgen abundance ( skin break out, hirsutism, roughness of voice, virilism ) and raised 17 𝞪 hydroxyprogesterone, it is reminiscent of inherent adrenal hyperplasia, which can be separated from adrenal tumors by dexamethasone concealment test CT examine is the corroborative test for adrenal tumors. Ovarian tumors can be analyzed on pelvic ultrasound . genuine hermaphroditism is analyzed on histology of gonadal examples taken at laparotomy.

TREATMENT

    
When the hidden reason for essential amenorrhea has been recognized, further administration is to treat the reason.

Absent secondary sex characters

Short stature:

In patients with hypothalamic pituitary brokenness, the stature is accomplished human development chemical ( 0.2g/kg/day) first and than for advancement of optional sex n characters low does cstrogen (0.01 mg ethinyl oestradiol ) is given twice day by day for 3 weeks consistently for a while. When the optional sex characters are created, these patients are begun on common joined oral prophylactic pills as chemicals substitution treatment. At the point when the origination is wanted, the ovulation can be incorporated with human menopausal gonadotropin infusions. Fruitfulness possibilities are amazingly poor in patients with Turner condition and blended gonadal dysgenesis. They are made physically develop with low portion estrogen (as above ) and afterward given oral prophylactic pills as chemical substitution treatment. The balls in blended gonadal dysgenesis ought to be eliminated as the danger of threat emerging from that point is 30%.

Normal stature:

    

Separated GnRH insufficiency is dealt with like hypogonadotropic hypogonadism related with short height aside from the human development chemical as tallness in treated with bromocriptine (2.5mg bd) Pituitary adenoma ought to be precluded with CT filter, which may some of the time require careful treatment. Genuine gonadal agenesis is treated as turner disorder and people with XYgenotype would require gonadectomy.

Normal secondary sex characters

Anatomical anomalies would require careful remaking . androgen lack of care notwithstanding vaginoplasty would require gonadectomy (danger hazard 5%) trailed by oral preventative pills as long haul chemical substitution treatment. Fruitfulness is beyond the realm of imagination with androgen inhumanity disorder. Safe ovary condition may react to oral preventative pills followed by ovulation acceptance with clomiphene citrate and richness reestablished wedge resection or laparoscopic ovarian boring are the careful treatment for polycystic ovary followed by ovulation enlistment with clomiphene citrate .

Heterosexual development

Congenital adrenal hyperplasia responds to steroid therapy and fertility restored with clomiphene citrate. ovarian/ adrenal tumors need surgical resection.

Constitutional delay

If no cause is found on detailed evolution and the patient does not want to conceive, the treatment can be delayed upto the age of twenty years, when spontaneous menstruation starts in most of these patients.