Menstruation can be seen as the endpoint of an endocrine cascade that begins with thelarche and ends with menarche. All over the world, the age at menarche is decreasing. This may be attributed to many known and unknown factors. The first period occurs because there has been sufficient endometrial stimulation to result in a withdrawal bleed when there is a temporary fall in the estrogen level. Menstrual irregularities often occur in adolescent girls as cycles tend to be anovulatory. This is a stressful condition for the adolescent girl as well as her parents. As the girls cannot differentiate between normal and abnormal menstrual patterns, medical help is sought either too early or too late. Heavy periods lead to a lot of fear, anxiety and stigma.
Normal and abnormal menstrual pattern during puberty –
Till two years from menarche, most cycles are anovulatory. The length of the cycle varies from 21-40 days, the mean duration is 4-5 days, the average blood loss being 35ml. Whenever the cycle length is less than 21 days, duration is more than 8 days and menstrual blood loss is more than 80ml, the girl requires further evaluation and necessary treatment.
Adolescent with excessive bleeding per vaginum – Differential diagnosis
Menstrual problems are common during adolescence due to slow maturation of the hypothalamic pituitary-ovarian axis and can last 2-5 years after menarche. Menorrhagia (excessive bleeding PV either in duration or quantity or both) at regular intervals and metrorrhagia (cycle irregularity) are commonly seen in adolescent girls. In them, Dysfunctional uterine bleeding (DUB) causes menorrhagia in 74%, primary coagulation disorders are present in 19% and other causes account for 7%. Genital tuberculosis may cause excessive menstrual bleeding in the beginning of the disease process. Cervical polyp and granulosa cell tumour of the ovary may rarely cause menorrhagia. Bleeding disorders like thrombocytopenia, Von Willebrand's disease, factor II, V, VII, X & XI deficiency disorders should be ruled out if menorrhagia starts as soon as menarche sets in.
It is best defined as abnormal bleeding from the uterus in the absence of the organic disease of the genital tract. The bleeding may be abnormal in frequency, amount, duration or any combination of these. This is the commonest cause in adolescent girls suffering from menorrhagia. 40-50% resolve within 2 years and a better prognosis is expected when DUB starts after a period of normal menstruation than who develops it at menarche itself. In most of the girls with puberty menorrhagia, the cycles are anovulatory though in few they may be ovulatory.
Anovulatory DUB – Causes
Ovulatory DUB – Causes
Age at Menarche
Amount, duration and pattern of menstrual bleeding
Symptoms of endocrine / organic disease
Use of contraceptives, if any
Emotional stress / psychiatric disorder
Social and personal background
H/O drug ingestion
General – pallor, thyroid swelling, vital parameters
Treatment of mild DUB –
In cases with mild DUB, reassurance to the girl as well as her parents is absolutely essential. In this way the iatrogenic factors of anxiety and worry are lessened and relieved. Thus explanation and reassurance are the backbone of therapy.
Points to be covered during counselling
Treatment of moderate DUB
Aims - To stop bleeding
To prevent recurrence
To correct anaemia, malnutrition etc.
In these girls, PG synthetase inhibitors like mefenamic acid should be used during period to reduce the amount of bleeding. It acts on cyclo-oxygenase and blocks peripheral PG receptors leading to antiprostaglandin action, dose being 250 to 500 mg 3 times a day. Sometimes ethamsylate can be added. Iron supplementation is a must to treat anaemia. Hormonal therapy in the form of OCS or progestogens is required in cases with irregular cycles. Regular evaluation of hormonal therapy is done as the H-P-O axis matures.
Management of severe DUB
Sometimes, hospitalisation is required. Smith et al (1998) concluded that 63% hospitalised adolescents with menorrhagia required blood transfusion. These girls should be assessed for primary coagulation disorder or any medical problem. Anti fibrinolytic agents like Tranexamic acid which is a potent inhibitor of fibrinolysis should be started in the dose of 500mg three times a day. It also can be given parenterally. Combination of mefenamic acid, tranexamic acid and / or ethamsylate may be used judiciously. Hormonal therapy with oral contraceptive pills or plain progestogens is a must. Progestogens counter balance unopposed estrogen action. Three types of progesterones are available in the market – Dydrogesterone (10mg), Medroxy progesterone acetate (5/10mg) and Norethisterone acetate (5mg). In moderate to severe menorrhagia, Norethisterone acetate or Medroxy progesterone acetate can be given in the dose of 1 BD to 1 TDS for 21 days. This therapy can be given cyclically starting from every 5th day of the period lasting for 21 days for 3 to 4 months. Once the cycles are stabilized, progestogens can be given only in the leuteal phase of the cycle i.e. 1 BD to be started from the 14th day of the cycle for total 10 days. This leuteal phase support can be given for 3-4 months and then the girl is re-evaluated for the necessity of hormones. In cases with mild to moderate menorrhagia, low dose contraceptive pills (OCS with ethinylestradiol 30 ugm) also can be tried in a cyclical manner for 3 to 6 months duration. If the girl is also suffering from polycystic ovarian syndrome (PCOS), acne and hirsuitism then OCS containing desogestrel or cyproterone acetate should be started. These lipid friendly progestogens help in reducing the androgenic effects as well.
What should be avoided
Drugs like danazol, GnRh analogues and daflon which are used in elderly women should be avoided in adolescents. Also, progestogen only pills and LNG system (progestogen containing intrauterine device) are contraindicated. Inj. Depot provera is used rarely. Unlike in women with perimenopausal menorrhagia, diagnostic or therapeutic curettage (D&C) is avoided in adolescent girls in spite of USG finding of thickened endometrium. If endometrial hyperplasia is suspected then she is put on progesterone for a period of 6-12 months, after which she is re-assessed.
Girls requiring hormonal treatment need frequent follow up and evaluation. It may take few years before their cycles get established properly. Till then, they may require to take natural progestogens like dydrogesterone for many months.
Adolescents with Menorrhagia
1 : 5 require hospitalisation
1 : 4 will have Hb of < 10gm%
1 : 3 will require blood transfusion
1 : 2 present at menarche
Girls with menarche menorrhagia fall into a high risk group. They face problems like severe anaemia and blood transfusion. In the long run, they may develop impaired reproductive potential like infertility, spontaneous abortions etc. They are also prone to develop endometrial hyperplasia and rarely carcinoma of the endometrium in later life if neglected. Puberty menorrhagia sometimes can emerge as a therapeutic challenge requiring combined efforts of family physician, gynecologist, paediatrician, endocrinologist and haematologist.
Suggested Reading –
Puberty is the transition period between childhood & adulthood during which secondary sexual characteristics start developing, menstruation begins & psychosexual outlook of the individual changes.
Puberty: is the rendering of the Latin word Pubertasmeaning, "Grown Up". The Oxford Dictionary defines puberty as - the state of being functionally capable of procreation1. Puberty is the stage of physical maturation in which an individual becomes physiologically capable of sexual reproduction and is defined by WHO as the age between 10 and 19 years. Normal puberty starts at 8 to 12 years in Indian girls & it takes 3-4 yrs for the completion of the secondary sexual characteristics. Disorder of pubertal development may occur at any stage of the maturational process leading to either precocious or delayed puberty.
Factors affecting the onset of puberty:
Menstruation usually commences after the maximum growth spurt (6-11 cm per year). There is also a relationship between skeletal maturity and onset of menstruation; it is unusual for menstruation to begin before bone age of 12.5 years or after 14.5 years (Sheil & Turner 1997). Typically the age of menarche is earlier in girls with moderate obesity (upto 30% above ideal body weight), while it is delayed in those with severe malnutrition2. However, the increase in body fat to 23.5% from pre-pubertal 16% is thought to be an important factor influenced by nutrition (Sternleib & Munan 1972). High levels of Leptin, a peptide secreted in adipose tissue that circulates in blood and acts on the CNS regulatory neurons that regulate eating behaviour and energy balance is associated with earlier age of menarche (Matkovic et al, 1997). Evidence also suggests that, pubertal growth acceleration is due to estrogen and concomitant increases in growth hormone production and secondary stimulation of insulin-like growth factor-I levels.
Pubertal Events (Chamberlain 1995, Shah & Turner 1997)
|Physical feature||Age range (yrs)|
|Menarche||11-15 (mean 12.9)|
|Mature sexual hair and breasts||14-15|
If one accepts the mean +/-2.5 standard deviations as encompassing the normal range, then pubertal changes before the age of 8 are regarded as precocious. Increased growth is often the first change in precocious puberty. Occasionally, adrenarche, thelarche, and linear growth occur simultaneously and sometimes menarche may be the first sign. The occurrence of menarche is considered precocious when it occurs prior to 10 years of age.
Precocious puberty can be divided into two classifications:
Precocity occurs 5 times more frequently in girls than boys, and almost three-quarters (80%) of these are idiopathic or constitutional precocity (true sexual precocity), this must be a diagnosis by exclusion with prolonged follow-up in an effort to detect slowly developing lesions of the brain, ovary or adrenal gland. 15% girls and 50% boys with CPP have a lesion of CNS.
I) Gonadotropin- dependent precocious puberty:
a. Idiopathic or constitutional
b. CNS Lesions
II) Gonadotropin-independent precocious puberty
III) Intermediate type of precocious puberty
Gonadotropin-secreting tumors- teratomas, hepatoblastomas, dysgerminomas, ovarian-granulosa cell tumor, arrhenoblastoma, lipid cell tumor, thecoma etc.
A careful history and thorough physical examination is mandatory, along with the required laboratory work-up which will aid in arriving at a proper diagnosis.
History should include the chronology of pubertal events, associated behavioural changes, drug usage or incidental drug ingestion, symptoms suggestive of or past history of intracranial lesions and symptoms of hypothyroidism. The growth velocity should be plotted on appropriate growth charts (cm/yr) to determine growth spurt. A complete systematic examination including palpation for abdominal masses, neurological and endocrinological examination, estimation of visual fields and optic fundi would help in diagnosis of the cause. The clinical stage and type of pubertal development by Tanner's method, the height of the patient, buccal smear (pyknotic nuclei), karyotyping and bone age are the basic investigations. Serum levels of FSH, LH, Oestradiol, Testosterone, TSH, Thyroxine will distinguish complete and incomplete types. High resolution USG of the ovaries and computed tomographic images of the adrenal glands, will be required if peripheral cause is suspected. In CNS disease, MRI of the brain for imaging the hypothalamo-hypophyseal area is indicated.
Recommended Diagnostic approach for Precocious Puberty
Controversy related to age:
Treatment depends on the underlying cause. Objectives for management include:
Gonadal, adrenal, liver or CNS tumors require surgery and /or subsequent chemo/radiotherapy.
For congenital adrenal hyperplasia (CAH), glucocorticoid replacement will suppress production leading to precocity.
Thyroid replacement therapy will suppress precocity by suppressing HPG axis in cases of primary hypothyroidism.
McCune-Albright syndrome is independent of gonadotropins and does not respond to GnRH therapy. The syndrome may be associated with hyperthyroidism or Cushing's syndrome. Treatment includes medical - administration of testolactone (40 mg/kg/day) to suppress conversion of testosterone to estrogen and Orthopaedic management.
Central precocious puberty is gonadotropin dependent and therapy is directed to suppress its secretion. Treatment includes, giving Medroxyprogesterone acetate (Provera) in doses of 100-200 mg i.m. every 2-4 weeks. Alternatively, Cyproterone acetate, a drug with antiandrogenic and antigonadotropic properties is also used and appears superior to MPA in the treartment of precocious puberty. However, nowadays the mainstay of treatment is the use of LHRH analogues. These are administered 0.2-0.3 mg/kg (max 7.5 mg) i.m. every 4 weeks. They down regulate receptors, causes regression of secondary sexual characteristics, cessation of menses, delay short stature by delaying the closure of epiphyses and normalize growth. Dosage given is Triptorelin i.m. every 28 days or Buserelin with cyproterone acetate to improve height.
Indications for GnRH analog treatment in CPP:
Advantages of GnRH analogue Therapy:
Recent Advances & Controversies:
Adolescent and young people stand at the threshold of adult world. People aged 10 to 24 years are called as ‘young people’ This is a period of rapid and sometimes drastic changes taking place in their body, mind and social life. Many are already exposed to challenges from the adult world. We need to focus our attention towards the health needs of this group (a) to reduce mortality and morbidity now, and during their future lives (b) to respect their rights to health care, in general and reproductive health care, in particular (c) to enable them to pass on good health to their own children as they are the future parents.
Young people face dangers more complex than previous generations faced with less support and safety network.
Newer challenges faced by young people
Young people come from different strata and communities – being not only of different ages and sexes but also of different cultures and life experiences. When in trouble, they usually do not know where to go and seek help. They often hesitate to visit any health care facility because they are afraid about the stigma and also wonder whether they will be treated well at a clinic. In addition to general health disorders, adolescent and young people face multiple specific problems.
Health problems faced by young people
Usually it is seen that when young people are looking for urgent treatment for their own problems, public sector health services are often their last resort. There are many barriers in using existing health services.
Adolescent and Youth Friendly health services represent an approach which brings together the qualities that young people demand, with the high standards that have to be achieved in public as well as private services. The characteristics of adolescent and youth friendly health services were discussed during the global consultation process initiated by WHO in 2000, and continued during the discussions by the expert group convened by WHO in Geneva in 2001.
Characteristics of Adolescent Friendly Health Services
Adolescent friendly health services need to be accessible, equitable, acceptable, appropriate, comprehensive, effective and efficient. These characteristics are based on the WHO Global Consultation in 2001 and discussions at a WHO expert advisory group in Geneva in 2002. They require :
Adolescent and Youth friendly health services can be offered at various levels and centres. Making existing services more friendly is one option whereas establishing specific stand alone clinics is yet another option available.
A strategy for planning services –
A variety of services can be provided to young people. Specialised services concentrating on their Reproductive & Sexual health include –
These services can be provided by a team of professionals especially trained in ARSH.
Guidelines for Staff
Staff - Gynecologists, Pediatricians, Physicians, Psychologists, Counsellors, Support Staff, Lab technicians.
Qualities & Skills - Non judgmental & unbiased attitude,Ensure confidentiality, Competence, Warm & Friendly Approach, Optimism & Motivation, Respect for clients irrespective of age, sex, socio-economic & marital status; Adequate time for interaction, Welcome drop-in clients, Skills in Record keeping, Empathy, Sensitivity
Communicating with young people – Good communication skills are very important to reach out to young people. They create trust and develop bonding.
Communicating with young people
Creating awareness and providing specific services catering to adolescent reproductive and sexual health in this manner is the need of the hour. It is essential in transforming today's adolescent boys and girls into tomorrow's responsible citizens, spouses and parents.
Suggested Reading -
Adolescent Friendly Health Services – An Agenda for change, by Peter McInture, Oxford, UK with support from Department of Child & Adolescent Health & Development, WHO, Geneva in 2003.
Hyperprolactinaemia refers to a prolactin level in excess of 20 ng/ml in a non-pregnant woman.
Prolactin is a polypeptide hormone consisting of 198 amino acids. It circulates in three different sizes: the small or monomer form, the big or dimmer form and the big big or polymeric form. A major part (80%) of the hormone is secreted in the small form which is biologically active. The other two forms may be biologically inactive though they are measured by radioimmunoassay. Prolactin is secreted by the chromophobe cells called lactotrophs located in the lateral areas of the pituitary gland and by decidua and endometrium. With a half-life of 20 minutes, Prolactin is both mammogenic (stimulates the growth of mammary tissue) and lactogenic (causes production and secretion of milk).
The secretion of prolactin is controlled mainly by inhibition. Dopamine (prolactin inhibiting factor or PIF) inhibits the synthesis and release of prolactin. On the other hand, the neurotransmitters-serotonin and thyrotropin releasing factor increase the secretion of prolactin. Prolactin is secreted episodically and serum levels fluctuate throughout the day as well as through the menstrual cycle, with peak levels being seen at mid-cycle.
Oestrogen increases the levels of prolactin and, thus, prolactin levels rise in hyperoestrogenic states such as puberty and pregnancy. The rise in prolactin is proportionate to the levels of oestrogen and in the third trimester of pregnancy, prolactin concentration is 200 ng/ml. However, lactation does not occur since oestrogen inhibits the action of prolactin on the breast. After delivery, the levels of oestrogen rapidly decline and lactation is established. Each act of breastfeeding is associated with a rise in prolactin levels.
Pathologic Causes of Hyperprolactinaemia
Hyperprolactinaemia is seen in 15% of all anovulatory women and in 20% of women with indeterminate amenorrhoea. Thirty to eighty per cent of women with hyperprolactinaemia will have galactorrhoea. The incidence of hyperprolactinaemia is higher in women with galactorrhoea and amenorrhoea with low oestrogen levels as compared to women with galactorrhoea and normal or abnormal menses with normal oestrogen levels.
Prolactin levels are physiologically elevated by either nipple or breast stimulation, exercise, sleep, stress and following the noonday meal. The maximum levels are observed during sleep at night.
Pathologic Causes of Hyperprolactinaemia
|1. Central Nervous System Disorders|
|2. Hypothalamic disease||Craniopharyngiomas
Infiltration with sarcoidosis, histiocytosis, leukaemia, carcinoma
|3. Pituitory disorders||Prolactinomas (micro and macro-adenomas)|
|Other tumours that produce acromegaly and Cushing's disease|
|5. Chronic Renal Disease|
|6. Chronic Breast Nerve Stimulation||Thoracic operation scar|
|Herpes zoster, chest trauma|
|Phenothiazine, diazepam,tricyclic antidepressants|
|Propranolol,haloperidol, phentolamine, cyproheptadine|
Anti-hypertensives Oral contraceptive pills Metaclopramide
Of all pituitary adenomas, 80% secrete prolactin and the commonest pituitary tumours are prolatinomas. These tumours have been arbitrarily divided into microadenomas (< 1 cm diameter) and macroadenomas which are larger. In the absence of an adenoma even lactotroph hyperplasia can cause hyperprolactinaemia.
Only 5% of women with hyperprolactinaemia will have a prolactinoma and if the prolactin level exceed 200 ng/ml, there is almost always a prolactinoma.
Prolactinoma should be ruled out in women wih > 100 ng/ml prolactin level. Microadenomas rarely grow in pregnancy. They do not progress to macroadenoma.
Reduced thyroxine (T4) levels in hypothyroidism causes an elevation in thyrotrophin releasing hormone(TRH). This results in increased levels of thyroid stimulating hormone (TSH) and prolactin. Hypothyroidism is found in 3% to 5% of individuals with hyperprolactinaemia. Therefore, a TSH assay is recommended in all cases of hyperprolactinaemia.
In any case of drug-induced galactorrhoea, the drug is discontinued for one month and prolactin measured thereafter. If the medication cannot be discontinued then a prolactin assay is performed as such and CT /MRI is advised if prolactin levels exceed 100 ng/ml.
It is essential to remember that hyperprolactinaemia is in itself simply an abnormal “lab value” that reflects the presence of an underlying pathophysiologic cause and all possible causes must be considered and either identified or ruled out before therapy is initiated (Olive)1. The indications for treatment include -
A patient with idiopathic hyperprolactinaemia or with a microadenoma who has no wish to conceive may be managed expectantly with yearly prolactin levels and CT scan every 2 years. If the woman is hypoestrongenic, oestrogen-progesterone combination therapy or bromocryptine can be used.
Radiation is not the primary mode of therapy. Both external therapy (cobalt, proton beam, heavy particles) and brachy therapy (yttrium 90 rads as pituitary implants) have been used. The results are inconsistent; and delayed. Radation may damage the surrounding normal pituitary tissue causing diabetes insipidus and also damage the optic nerve.
Trans-sphenoidal microsurgical resection of prolactinomas is also performed, but is once again not the treatment of first choice. Surgery should be reserved for those uncommon tumours that do not respond to medical therapy or have a large cystic component or for the occasional patient who cannot tolerate dopamine agonists or who experiences pituitary apoplexy (Biller)2. Though the initial cure rates for micro-and macroadenomas are 65 to 85% and 20 to 40%, respectively, the tumour recurs in 20 to 50% of microadenomas and 20 to 80% of macroadenomas.
Dopamine receptor agonists such as bromoergocryptine, methysergide and metergoline form the main stay of therapy with bromocryptine being the drug of choice. Bromocryptine is a semi-synthetic ergot alkaloid which stimulates dopamine receptors. It is rapidly absorbed orally, peak blood levels being reached in 1 to 3 hours and prolactin levels are depressed for 14 hours, therefore, it is given in a twice daily dosage form. Bromocryptine causes orthostatic hypotension resulting in faintness, dizziness, nausea and vomiting. Other adverse effects include headache, nasal congestion, fatigue, constipation or diarrhoea. Prolactin levels normalize in 90% of patients, fertility is resorted in 80% and galactorrhoea suppressed in 60% of idiopathic hyperprolactinaemia and microadenomas.
Therapy is commenced with a dose of 1.25 mg per day (half tablet) and gradually increased in weekly increments. In 10% of microadenomas, prolactin does not normalize even at a dose of 20 mg/day but many patients conceive and ovulate. After 1 year of bromocryptine therapy, only 10 to 20% of patients permanently normalize. After 2 years of therapy, 75% of microadenomas and 80 to 90% of macroadenomas regress. Bromocryptine is also used in postoperative or postirradiation failure or recurrence of hyperprolactinaemia. With a dose of 5 mg, 95% of patients with no adenomas resume normal menstrual function and galactorrhoea reduces over a period of 4 to 6 weeks and fertility is restored by 8 to 10 weeks duration If an adenoma is present, 50% will normalize menstrual function and galactorrhoea gets corrected by 12 weeks while fertility is restored by 14 to 18 weeks with the same dose. The rest of the patients may require higher doses ( up to 20 mg)m of bromocryptine.
A trial with bromocryptine therapy for up to 6 months is warranted even in macroadenomas. There may be a slow or rapid response, but usually the results are excellent. Of those with macroadenomas who conceive, 20% may have visual disturbances or headache during pregnancy and bromocryptine may be necessary. There is no evidence of teratogenesis, increased rate of abortions or multiple gestation with bromocryptine.
Once a patient with an adenoma conceives, no monitoring is required for microadenomas unless the patient is sympotomatic, while in macroadenomas, monthly visual fields suffice. In the non-pregnant state, macroadenomas should be monitored by 6 monthly visual fields and CT scans.
Cabergoline and quinagolide are two new dopamine agonists that seem to have considerable advantages over bromocriptine (Webster)3. Cabergoline is an ergoline D2 agonist which has a long plasma half-life that enables once or twice weekly administration. Quinagolide, in contrast, is a nonergot D2 agonist with an elimination half-life intermediate between those of bromocriptine and cabergoline. Quinagolide can be administred once daily. Comparative studies here indicated that cabergoline is superior to bromocriptine in efficacy (prolactin suppression, restoration of gonadal function) and in tolerability ( Biller et al)2.. In similar studies, quinagolide appeared to have similar efficacy and superior tolerability to that of bromocriptine. A small crossover study has indicated that cabergoline is better tolerated and has superior activity than quinagolide.
Pregnancy with pituitary adenoma
Usually no monitoring is required for microadenomas unless the patient develops symptoms; where as in macroadenomas monthly evaluation of visual field should be done. Bromocriptine can be used in pregnancy if women complain of headache and visual disturbances. Rarely surgery may be required. Following pregnancy, Prolactin levels decrease in 50% of idiopathic hyperprolactinaemia and 40% with adenomas. Breast feeding should be encouraged.
BASIC PRINCIPLES OF MENSTRUAL FUNCTION
There are five basic factors involved in the onset and continuation of normal menstruation. These are: anatomical patency of the genital tract; normal female chromosomal pattern; co-ordinated hypothalamic-pituitary-ovarian axis; active support by two other endocrine glands, namely thyroid and adrenal cortex and responsive endometrium. Defect in one or more of these factors will therefore, be responsible for amenorrhoea, either primary or secondary(1).
It is defined as absence of menstruation for at least 6 months, not due to pregnancy, in a woman of childbearing age. The usual age limits of menarche and menopause are 16 and 44 years respectively.
Absence of menarche by age 14 in the absence of development of secondary sexual characteristics, or absence of menarche by age 16, regardless of the presence of normal growth, development and presence of secondary sexual characteristics.
Before menarche, after menopause and during pregnancy.
ETIOLOGY OF PRIMARY AMENORRHOEA
Disorders of H-P-O Axis(2)
Primary failures: Dysgenesis (genetic/chromosomal), damage by surgery, radiotherapy, chemotherapy.
Anatomical Causes of Amenorrhoea
Simple Developmental Defects
Developmental Defects of Endocrine Origin
Summary of Primary Investigations(3)
Practical Aspects of Management of Primary Amenorrhoea
The common principles in the management are(5) :
Primary amenorrhoea is a symptom of some underlying disease or disorder. In majority of cases, diagnosis of the specific disorder is possible by its clinical expression. Cytogenetic, biochemical, biophysical and interventional diagnostic procedures are necessary only to corroborate the clinical diagnosis and in few cases where the clinical diagnosis remains disputed. Hence, the indications of special investigations should be oriented on the basis of clinical approach. So far as the results of treatment are concerned, only a few of the disorders are curable, majority are crippling in nature while some may appear life -threatening . Primary amenorrhoea due to polycystic ovarian disease, congenital adrenal hyperplasia or transverse septum of vagina with haematocolpos and haematometra (cryptomenorrhoea) are curable disorders. Chromosomal anomaly leading to primary amenorrhoea is a crippling disease. Intracranial space occupying lesion associated with primary amenorrhoea is a life –threatening condition. Hence, early diagnosis and correct counselling may save these young girls from unnecessary expensive investigations and injudicious treatment. Every possible effort must be done to restore menstrual, coital and reproductive functions in such cases.