Human Reproduction Update, Vol.9, No.5 pp.

493504, 2003

DOI: 10.1093/humupd/dmg038

Menstrual disorders in adolescence: investigation and

management
Martha Hickey1,3 and Adam Balen2
1

Department of Obstetrics and Gynaecology, School of Women's and Infants' Health, University of Western Australia, King Edward
Memorial Hospital, 374 Bagot Road, Subiaco, WA 6008, Australia and 2Clarendon Wing, Leeds General Inrmary, Leeds LS2 9NS,
UK
3

To whom correspondence should be addressed. E-mail: mhickey@obsgyn.uwa.edu.au

Menstrual disorders in adolescence may present diagnostic and management challenges for the gynaecologist. This
review will describe the common and uncommon menstrual disorders that may arise in early reproductive life,
together with guidance on their investigation and management.
Key words: adolescence/amenorrhoea/dysfunctional uterine bleeding/menstruation/PCOS

Introduction
Adolescence is a time of enormous physical and psychological
change for young women. Serious gynaecological pathology is
rare in this age group, but menstrual disturbances are not
uncommon and may add further disruption to this difcult phase
for adolescents and their families.
It is likely that many adolescents with menstrual disturbances
never present to their family doctor or gynaecologist. The
American College of Obstetrics and Gynecology (ACOG, 1996)
takes a proactive stance in adolescent health by recommending an
initial visit to a gynaecologist for health guidance, screening and
the provision of preventative services around the age of 1315
years. This is an opportunity for clinicians to advise the adolescent
on what is `normal' for girls of her age regarding pubertal
development, menarche and menstrual cyclicity and would not
normally include a pelvic examination. This anticipatory guidance
and information to young girls and their parents may help ease the
transition from childhood through puberty and a healthy adolescence (Adams Hillard, 2002). Such guidelines do not exist in the
UK and it is unlikely that an adolescent would encounter a
gynaecologist without the clinical suggestion of pathology.
Effective history-taking from an adolescent requires particular
skills and sensitivities. Adolescents may have difculty raising
issues of menstruation with their doctors (Malus et al., 1987) and
may present with complaints of minor symptoms rather than their
primary concerns (Patton, 1999). Neither the British nor
Australian colleges of Obstetrics and Gynaecology recognize
training in adolescent gynaecology as part of their core curriculum
and there are no UK guidelines for the management and referral of
specialized problems (Balen et al., 2002). Since the formation of
the British Society of Paediatric and Adolescent Gynaecology,
there is now a UK course for the management of adolescent

gynaecological and intersex conditions. In 2003 the UK Royal

College of Obstetricians and Gynaecologists (RCOG) has also
approved a special training module in Paediatric and Adolescent
Gynaecology. The ACOG has recently released a `tool kit'
designed to facilitate the inclusion of adolescents into a general
ob-gyn practice (http://www.acog.org) which may be of use to
practitioners in other countries treating adolescents.
Embarrassment about discussing menstruation, fear of disease
and ignorance about available services are likely to mean that
many problems are not discussed or present following maternal
pressure. Alternatively, presentation with a menstrual disturbance
may disguise other issues, such as those relating to contraception,
pregnancy, sexually transmitted infection (STI) or even sexual
assault, and the gynaecologist should ensure that the young woman
is given the opportunity to raise other concerns. Adolescents do not
access health services in the same manner as adults and effective
services must recognize these patterns and plan accordingly. A
recent survey of 248 obstetric and gynaecology units in the UK
indicated that adolescents constitute up to 5% of new gynaecological referrals and, whilst it was recognized by most that
adolescents had special needs, the majority did not provide
specialized services (Balen et al., 2002).
Condentiality is a primary ethical and professional duty for
doctors, and a crucial issue in adolescent health care. In the USA,
the ACOG, American Academy of Pediatrics and the American
Medical Association have endorsed the principle of condentiality
in adolescent health care. In the UK and Australia the legal
precedence of the Gillick case (Gillick versus West Norfolk and
Wisbech Area Health Authority, UK) allows the practitioner to
determine whether an adolescent has reached sufcient understanding and intelligence to be capable of making up her own mind
on the matter requiring decision. Adolescents are unlikely to
access sexual and reproductive health services unless they can be

Human Reproduction Update Vol. 9 No. 5 European Society of Human Reproduction and Embryology 2003; all rights reserved

493

M.Hickey and A.Balen

assured of condentiality (Rogstad et al., 2002), hence the
gynaecologist should ensure that s/he maintains condentiality
and that the patient is assured of this.

Menarche
The menstrual cycle involves the coordination of many events by
the hypothalamicpituitaryovarian axis and is readily inuenced
by physiological, pathological and psychological changes occurring during the reproductive lifespan. The age of menarche is
determined by general health, genetic, socio-economic and
nutritional factors. The mean age of menarche is typically between
12 and 13 years (Flug et al., 1984; World Health Organization,
1986; Herman-Giddens et al., 1997). The oft-quoted statement that
there has been a decrease in the mean age of menarche in recent
decades has not been substantiated by large population studies,
either in the USA (Chumlea et al., 2003) or in the UK (Whincup
et al., 2001). However, the UK study observed that almost one girl
in eight reaches menarche while still at primary school. There
appears to be a relationship between body weight and the onset of
menarche, supported by earlier menarche seen in obese girls
(World Health Organization, 1986).
Nutrition and body weight play an important role in pubertal
development. Chronic disease, malnutrition, eating disorders and
high levels of physical activity can delay menarche. The mechanism for this relationship has not been conclusively dened.
Insulin has been suggested as a modulator of the tempo of pubertal
development through regulation of insulin-like growth factor
binding protein (IGFBP-1) and sex hormone binding globulin
(SHBG; Ibanez et al., 1997). States of over-nutrition and obesity
are associated with increased serum concentrations of insulin.
Therefore if excessive nutritional intake persists during childhood,
it is possible that hyperinsulinaemia resulting from obesity may
lead to lower levels of IGFBP-1 and reduced SHBG concentrations, thus enhancing IGF-I and sex steroid bioavailability.
Hyperandrogenism associated with PCOS may augment this
situation. The converse would be true in states of malnutrition,
where low levels of insulin would allow for the development of
increased IGFBP-1 and SHBG levels. However, it is still unclear
whether hyperinsulinaemia in childhood is a result of obesity, or if
it is the cause of obesity. The role of genetic factors, which may
determine insulin production and obesity risk in childhood, have
also yet to be clearly explained.
Serum concentrations of leptin relate to body fat mass, and
leptin appears to act on the hypothalamus to control calorie intake,
decrease thermogenesis, increase levels of serum insulin, and
increase pulsatility of GnRH (Mann and Plant, 2002), hence
regulating the onset of puberty (Mantzoros et al., 1997). This may
explain the observed relationships between body fat and ovarian
maturation observed by Frisch and Revelle (1970). Homozygous
mutation of the leptin receptor gene results in early-onset morbid
obesity, and the absence of pubertal development in association
with reduced growth hormone secretion (Clement et al., 1998).
Menstrual bleeding lasts 27 days in 8090% of adolescent girls
(Flug et al., 1984). As a rule of thumb, changing three to six pads
per day without soiling from oversaturated pads suggests a normal
ow (Adams Hillard, 2002). Whilst adolescent menstrual cycles
may initially be variable this does not mean that `anything goes'
and cycles tend to regulate over the rst 23 years following

494

menarche. Most cycles still range from 21 to 45 days, even in the

rst year after menarche, with an upper limit of normal (i.e. 2 SD)
of 4045 days (Southam and Richart, 1966; Flug et al., 1984;
World Health Organization, 1986). By the third year after
menarche, 6080% of cycles are 2134 days long, a pattern
similar to that seen in adults (Widholm and Kantero, 1971; Flug
et al., 1984). Cycles >90 days represent the 95th percentile for
length, even in the rst gynaecological year. Thus consideration
should be given to a gynaecological evaluation in girls whose
cycles are longer than this interval, since amenorrhoea of this
interval or longer may have important implications for long-term
bone and cardiovascular health (Treloar et al., 1967).
Anovulation does not necessarily underlie prolonged or irregular cycles and some cycles are ovulatory with a long follicular
phase (Venturoli et al., 1987). Early menarche is associated with
early onset of ovulatory cycles. When menarche occurs at <12
years, 50% of cycles are ovulatory in the rst year and virtually all
by the fth year. By contrast, it takes 812 years for all cycles to be
ovulatory in girls with later onset of menarche (Vihko and Apter,
1984). This has important clinical implications for advising
adolescents on the `normality' of their menstrual pattern relative
to their age at menarche and likely patterns in the future.
After menarche, pregnancy can occur. It is impossible and
unwise to try to separate menarche from sexual health education in
adolescent girls. Guidance about contraception and STI is
essential, regardless of whether she is currently sexually active.
The teenage birth rate in the UK is second only to that in the USA
and a high percentage of adolescent pregnancies occurs in the
initial months of sexual activity. Early menarche is associated with
earlier age of sexual debut, increased rates of STI and cervical
atypia and is hence an important indicator for continued risk
behaviour regarding reproductive health (Andersson-Ellstrom
et al., 1996). UK teenagers have high rates of STI (Creighton
et al., 2002) and younger age is the strongest risk factor for
Chlamydia trachomatis infection, across a broad range of populations (Burstein et al., 1998). Effective prevention strategies must
include the younger adolescent population, ideally before they
become sexually active.

Disorders of puberty
Precocious puberty

Well-described physical and anatomical changes of puberty,

classied according to Tanner (1962) are evident from 9 to 13
years and follow the increase in adrenal androgens from 6 to 8
years. Precocious puberty has been dened by Marshall and
Tanner (1969) as `Any secondary sex characteristic appearing
in a girl before the age of 8 years (9 years for boys) or the onset
of menstruation prior to 10 years of age'. The denition of
lower limit for the onset of pubertal development in girls is
controversial and pubertal growth may be occurring earlier
than had previously been described, despite the age of
menarche remaining constant. Racial variations are well
established and pubertal development occurs earlier in girls
from an Asian and Afro-Caribbean background than in white
girls (Datani and Brook, 1998; Chumlea, 2003). A US study of
>17 000 girls suggests that pubertal development is seen in
15% of white girls aged under 8 years and 48% of black girls

Menstrual disorders in adolescence

Table I. Causes of precocious puberty
Gonadotrophin dependent (`true' or `central' precocious puberty)
Idiopathic (family history, overweight/obese)
Intracranial lesions (tumours, hydrocephalus, irradiation, trauma)
Gonadotrophin secreting tumours
Hypothyroidism
Variants
Premature thelarche (and thelarche variant)
Adrenarche
Gonadotrophin independent
Congenital adrenal hyperplasia
Sex steroid secreting tumours (adrenal or ovarian)
McCuneAlbright syndrome
Exogenous estrogen ingestion/administration e.g. child ingesting oral
contraceptive pills

(Herman-Giddens et al., 1997), although the average age of

menarche did not differ between these groups. Hence, US
guidelines advise that investigation of breast or pubic hair
development is justied in white girls aged <7 years and black
girls <6 years (Kaplowitz and Oberfeld, 1999). Similar
population gures for the UK are not available, hence the
denition of precocious puberty remains unchanged.
Early signs of pubertal development are likely to cause
alarm in parents and may lead to social and psychological
difculties for the girl aware that her body is becoming
markedly different from that of her peers. In girls, 90% of cases
are constitutional, secondary to premature release of gonadotrophins, without any organic lesion and puberty often
proceeds slowly in these girls (Root, 2000).
In 10% of cases, precocious puberty is secondary to an
intercranial lesion causing premature gonadotrophin release
such as meningitis or cerebral tumour. The rare McCune
Albright syndrome (characterized by brous dysplasia of the
skeletal system, cafe-au-lait spots, and endocrine dysfunction)
is also associated with precocious puberty. In primary
hypothyroidism increased circulating thyrotrophin-stimulating
hormone and consequent increased thyroid-stimulating hormone may also elevate FSH and hence lead to premature
ovarian activity (Duncan et al., 1998). Feminizing estrogensecreting ovarian tumours are rare and the majority are benign.
When precocious puberty presents under the age of 6 years an
underlying disturbance affecting ovarian development is more
likely. When these features occur closer to the time of normal
puberty it is more likely that that problem is one of general
advance of the maturational process (Adams Hillard, 2002).
Rarely, anomalous puberty may arise when there is
inappropriate hormonal secretion with virilization. Causes
include gonadal dysgenesis with a functioning tumour,
congenital adrenal hyperplasia and adrenal tumours.
Precocious adrenarche has been associated with an increased
risk of subsequent insulin resistance and hyperandrogenism/
PCOS (Ibanez et al., 2000; Pathomvanich et al., 2000).
The causes of precocious puberty are summarized in Table I.
In assessing precocious puberty, the child's previous growth

and development as well as timing and sequence of physical

milestones should be considered. This assessment might be
most appropriately performed by a paediatrician. It is important that parents and girls are aware that menarche is likely to
occur in the 2 years following initial pubertal development.
Bone maturation is accelerated in precocious puberty, leading
to premature epiphyseal closure and curtailed stature. Pituitary
suppression with GnRH agonists is frequently indicated to
delay puberty. Therapy with GnRH agonists does not substantially affect adult height in girls who enter puberty between
ages 6 and 8 years (Leger et al., 2000; Root, 2000).
Delayed puberty

Delayed puberty is dened as absence of onset of puberty by >2

SD later than the average age, i.e. >14 years in females.
Delayed puberty may be idiopathic/familial or due to a number
of general conditions resulting in undernutrition. Absence of
puberty may also be due to gonadal failure (elevated
gonadotrophin levels), or due to impairment of gonadotrophin
secretion.
Although some cases of delayed puberty in girls may be due
to constitutional delay and require only reassurance, the patient
requires review within 6 months, at which point investigation is
likely to be indicated. Delayed puberty due to chronic disease
may respond to improved control of the associated condition.
Eating disorders such as anorexia nervosa and bulimia affect
0.51% of young women in developed countries with longterm mortality rates approaching 20% (Tamburrino and
McGinnis, 2002). The classic triad of presenting symptoms is
weight loss >15% of ideal body weight, behavioural changes
and amenorrhoea (secondary or primary). Delayed puberty
associated with low body mass index (BMI) should be
carefully questioned about eating disorders, as amenorrhoea
may precede signicant weight loss. Bulimia is associated with
irregular menstruation and can occur in girls of normal weight.
Intense exercise, such as long-distance running, ballet, rowing,
long-distance cycling and gymnastics, is associated with
delayed menarche in young girls, and with amenorrhoea in
older women (Frisch et al., 1981). These `endurance' sports are
associated with lower bodyweight and percentage fat. The
extent to which menarche is `delayed' is related to the age at
which participation in the sport begins, and to the intensity of
training. Promotion of very thin women as `ideal' role models
through media and fashion has contributed to an increase in
dieting in adolescent girls. In addition, there are future
implications for ovarian function, fertility and sexuality and
psychological support may be required at an early stage.
When chromosomal or structural abnormalities are suspected, or premature ovarian failure, the patient should be
referred directly to a tertiary referral centre offering tailored
services for young women, preferably with the results of
preliminary investigations.
Gonadal failure presenting as delayed puberty requires
specialist assessment and management. Clinicians should be
aware that several studies indicate long delays in both
recognition of premature ovarian failure as abnormal and
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M.Hickey and A.Balen

Table II. Causes of delayed puberty
General
Constitutional delay of growth and puberty
Underweight (due to severe dieting/anorexia nervosa, over-exercise or competitive sports, malabsorption such as coeliac disease or inammatory bowel disease)
Other chronic disease
Gonadal failure (hypergonadotrophic hypogonadism). Ref: http://www.emedicine.com/med/topic117.htm
Prodromal premature ovarian failure: this is a state of ovarian insufciency in which FSH levels are elevated and menses are irregular but not to the degree required
to make a diagnosis of premature ovarian failure. It is also referred to as overt ovarian insufciency.
Karyotypically normal (idiopathic) spontaneous premature ovarian failure
Turner's syndrome
Pure gonadal dysgenesis: The term `pure' here refers to the fact that the syndrome seems to have purely affected the gonad. No associated dysmorphic ndings
exist as are noted in Turner syndrome, which is often referred to as gonadal dysgenesis. Pure gonadal dysgenesis can occur with either a 46,XX or a 46,XY
karyotype
Autoimmune oophoritis
17,20-Desmolase deciency or 17-hydroxylase deciency
Radiation or chemotherapy
Galactosaemia
FSH receptor mutation
Gonadotrophin deciency
Congenital hypogonadotrophic hypogonadism (6 anosmia)
Hypothalamic/pituitary lesions (tumours, post radiotherapy)
Rare inactivating mutations of genes encoding LH, FSH or their receptors

diagnosis by a clinician (Alzubaidi et al., 2002) and that

delayed diagnosis may have serious implications for longerterm health.
In subjects with hypergonadotrophic hypogonadism, puberty
may be induced from any age; however, in Turner's syndrome
delay in induction to ~14 years old possibly permits maximal
response to growth hormone therapy. Low-dose estrogen
therapy (25 mg/day) is given to promote breast development
and cyclical estrogen plus progestogen is then used as
maintenance therapy. There is some evidence that transdermal
therapy may be preferred although it is too early for any
condent recommendation. Ankarberg-Lindgren et al. (2001)
induced puberty in 15 girls with hyper- or hypogonadotrophic
hypogonadism using low doses of transdermal estradiol
patches attached only during the night and compared the
estradiol concentrations obtained with those in healthy girls. A
transdermal matrix patch of 17b-estradiol (25 mg/24 h; Evorel,
Janssen PharmaceuticalsCilag) was cut into pieces corresponding to 3.1, 4.2 or 6.2 mg/24 h initially and attached to the
buttock. After 414 months, the dose was increased gradually.
Serum 17b-estradiol concentrations were measured every 2 h.
This regimen appeared to mimic the spontaneous levels as well
as the diurnal pattern of serum 17b-estradiol in early puberty.
In most of the girls, breast development occurred within 36
months of the start of treatment. The causes of delayed puberty
are summarized in Table I.
Intersex disorders
It is imperative that intersex conditions are managed in centres by
a multi-disciplinary team that includes paediatric surgeons,
urologists (often paediatric and adult), plastic surgeons, endocrinologists, specialist nurses, psychologists and also the gynaecol-

496

ogistwhose role is to help co-ordinate the transition from

childhood through adolescence and then womanhood and help
with issues relating to sexual function and sexual identity,
endocrinology and fertility. It is during the difcult time of
adolescence that the patient usually rst realizes that there are
serious problems and it is often the specialist gynaecologist who
helps her to understand the diagnosis and requirements for
management. The support of a skilled nurse and clinical psychologist is invaluable at this time. The Department of Health in the
UK is currently reviewing the national provision of services for the
management of intersex. Those units that have the skills to manage
intersex conditions usually also have the appropriate facilities to
treat complex anatomical anomalies of the Mullerian tract (for
example cervical and vaginal agenesis).
Disorders of sexual development may result in ambiguous
genitalia or anomalies of the internal genital tract and may be due
to genetic defects, abnormalities of steroidogenesis and dysynchrony during organogenesis. Age of presentation will depend
upon the degree of dysfunction caused. Ambiguous genitalia occur
in ~1:30 000 newborns. Issues concerning genital surgery for
intersex are controversial and data on long-term outcomes are
lacking. However, the effects on sexual function of surgical
removal of parts of the clitoris are largely unknown. A recent UK
study by Minto et al. (2003) of 39 adults with intersex conditions
and ambiguous genitalia and found markedly increased rates of
sexual dysfunction in those who had undergone genital surgery
compared with those who had not had surgery. These risks should
be discussed when counselling parents regarding genital surgery in
infants or children, and provide further evidence that delaying
surgery until individuals can make their own decision is preferable
when possible.
Sensitivity is paramount in dealing with young women found to
have genetic or hormonal abnormalities with profound implications for their future sexual and reproductive heath. The physician

Menstrual disorders in adolescence

should aim to provide access to counselling, further information
about their condition and contact details for support groups
Androgen insensitivity syndrome

Girls who are phenotypically normal but have absent pubic and
axillary hair in the presence of normal breast development are
likely to have complete androgen insensitivity syndrome
(CAIS, previously known as testicular feminization syndrome).
In this condition, the karyotype is 46,XY and, whilst testes are
present, there is an end-organ insensitivity to secreted
androgens because of abnormalities in the androgen receptor.
The incidence is ~1:60 000 genotypic male births and is
inherited as an X-linked trait (the androgen receptor is on the
short arm of the X chromosome). Anti-Mullerian factors
prevent the development of internal Mullerian structures and
the Wolfan structures also fail to develop because of the
insensitivity to testosterone. The external genitalia appear
female. In ~10% the defect is incomplete (PAIS: partial
androgen insensitivity syndrome); the external genitalia may
be ambiguous at birth, with labio-scrotal fusion and virilization
may sometimes occur before puberty. Partial androgen
insensitivity syndrome covers a wide spectrum of undervirilized phenotypes ranging from clitoromegaly at birth to
infertile men.
Diagnosis is conrmed by identication of the androgen
receptor gene mutation. Although patients with complete
androgen insensitivity syndrome are raised as females, patients
with partial androgen insensitivity syndrome should be managed according to age at diagnosis, response to treatment with
exogenous androgens, and the presence of an androgen gene
mutation.
AIS should be distinguished from other forms of male
pseudohermaphroditism such as 17b-hydroxysteroid dehydrogenase type 3 deciency, leydig cell hypoplasia due to
inactivating LH receptor mutations or 5a-reductase type 2
deciency.
AIS may be diagnosed in infancy if a testis is found in either
the labia or an inguinal hernia, in which case both testes should
be removed at this time because of the potential risk of
malignancy. If not diagnosed in infancy, CAIS may only
present at puberty with primary amenorrhoea and removal of
abdominal/inguinal testes should then be performed.
Gonadectomy should be performed whenever there is a Y
chromosome or Y chromosome remnant because of the
theoretical risk of malignancy developing in the dysfunctional
gonad. This is probably best delayed until after puberty
because in the case of AIS the testosterone is converted to
estrogen and can help to initiate some normal breast development. Exogenous estrogen should then be prescribed.
Progestogens are not required because the uterus is absent.
Congenital adrenal hyperplasia

The commonest cause of `female pseudohermaphroditism' is

congenital adrenal hyperplasia. Androgenization of the female
external genitalia may lead not only to clitoral enlargement but
also fusion of the labio-scrotal folds. There may in addition be

a urethral stula which may require careful repair at the time of

an introitoplasty. If clitoral reduction is imperative it should be
undertaken with care to preserve the neurovascular bundle and
sensation. Surgery may be undertaken during the neonatal
period and may be required again during adolescence. As with
all surgery for intersex disorders, the precise timing is open to
debate, as is the degree to which the patientrather than her
parents and physiciansis involved in the decision-making
process. In the rare situation of 17a-hydroxylase deciency,
replacement corticosteroids normalizes the adrenocorticotrophic hormone axis and then estrogen and progestogen
replacement can be prescribed.
Congenital absence of the vagina

Structural disorders of the reproductive tract during embryological development (Mullerian disorders) are usually associated with normal ovarian development and therefore do not
require sex steroid therapy, but for those with an absent vagina
an articial vagina needs to be developed when the young
woman is `appropriately mature' to understand the diagnosis
and the options regarding intervention. Surgical `neo vaginas'
are now rarely advised since vaginal dilators provide excellent
results and are now rst line therapy for vaginal agenesis
(ACOG, 2002). The renal tract should be imaged in those with
Mullerian abnormalities to rule out an associated structural
variation.
Women with MayerRokitanskyKusterHauser syndrome
(MRKH or Rokitansky syndrome) have a 46,XX genotype and
a normal female phenotype with spontaneous development of
secondary sexual characteristics, as ovarian tissue is present
and functions normally. The Mullerian ducts have failed to fuse
and so there is vaginal agenesis. The incidence is ~1:5000
female births and may be associated with renal tract anomalies
(1540%) or anomalies of the skeletal system (1020%). The
external genitalia have a normal appearance, but the vagina is
short and blind-ending, such that either surgery or gradual
dilation is necessary to achieve a capacity appropriate for
normal sexual function. Hormone treatment is not required as
ovarian estrogen output is normal. Indeed ovulation occurs and
ovarian stimulation followed by oocyte retrieval can be
performed in order to achieve a `biological' pregnancy via
surrogacy.
The vaginal dimple can vary in length from just a slight
depression between the labia to up to 56 cm. Vaginal dilators,
made of plastic or glass, are used rst to stretch the vaginal skin
and the patient is encouraged to apply pressure for 15 min
twice daily with successive sizes of dilator. An adequately
sized vagina is usually formed by 6 months but this may take
longer and long-term use of dilators may be required
depending upon the frequency of sexual intercourse.
The diagnosis of Rokitansky syndrome can usually be made
without the need for a laparoscopy. Sometimes, however, an
ultrasound scan will reveal the presence of a uterine remnant
(anlagan) which is usually small and hardly ever of sufcient
size to function normally. If there is active endometrial tissue
within the uterine anlagan the patient may experience cyclical
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M.Hickey and A.Balen

Table III. Causes of primary amenorrhoea
Uterine causes
Mullerian agenesis (e.g. Rokitansky syndrome)
Ovarian causes
Polycystic ovary syndrome
Premature ovarian failure (usually genetic, e.g. Turner's syndrome)
Hypothalamic/pituitary causes (hypogonadotrophic hypogonadism)
Weight loss
Intense exercise (e.g. ballerinas)
Idiopathic
Consitutional delay or secondary
Hyperprolactinaemia
Hypopituitarism
Causes of hypothalamic/pituitary damage (hypogonadism)
Tumours (craniopharyngiomas, gliomas, germinomas, dermoid cysts)
Cranial irradiation, head injuries (rare in young girls)
Systemic causes
Chronic debilitating illness
Weight loss
Endocrine disorders (thyroid disease, Cushing's syndrome etc.)

with two cervices or a partial septum causing a unilateral

obstruction. Excision is required both to prevent retention of
uterine secretions and to permit sexual intercourse.
Transverse fusion abnormalities usually present with primary amenorrhoea and require careful assessment before
surgery. The commonest presentation is of cyclical lower
abdominal pain and a visible haematocolpos with a bulging
purple/blue hymen. The surgery required is a simple incision.
Delayed diagnosis may lead to haematometra and consequent
increased risk of endometriosis (secondary to retrograde
menstruation). A transverse vaginal septum due to failure of
fusion or canalisation between the Mullerian tubercle and sinovaginal bulb may present similarly but is associated with a pink
bulge at the introitus as the septum is thicker. Great care must
be taken during surgery to prevent annular constriction rings
and the procedure should only be performed in dedicated
centres by experienced surgeons.
Primary amenorrhoea

Table IV. Classication of secondary amenorrhoea

Uterine causes
Asherman's syndrome, cervical stenosis
TB endometritis
Ovarian causes
Polycystic ovary syndrome
Premature ovarian failure (genetic, autoimmune, infective,
radio/chemotherapy)
Hypothalamic causes (hypogonadotrophic hypogonadism)
Weight loss, exercise, chronic illness, psychological distress, idiopathic
Pituitary causes
Hyperprolactinaemia
Hypopituitarism
Sheehan's syndrome
Causes of hypothalamic/pituitary damage (hypogonadism)
Tumours (e.g. craniopharyngiomas)
Cranial irradiation
Head injuries
Sarcoidosis
Tuberculosis
Systemic causes
Chronic debilitating illness (such as diabetes mellitus or lupus), weight loss,
endocrine disorders (thyroid disease, Cushing's syndrome etc.)
Drugs causing amenorrhoea
Cocaine and opioids have central effects that may disrupt the menstrual cycle
Psychotrophic drugs
Progestogens
Drugs interfering with the HPO axis such as GnRH analogues

The failure to menstruate by the age of 16 years in the presence of

normal secondary sexual development, or 14 years in the absence
of secondary sexual characteristics, warrants investigation. This
distinction helps to differentiate reproductive tract anomalies from
gonadal quiescence and gonadal failure. Primary amenorrhoea
may be a result of congenital abnormalities in the development of
ovaries, genital tract or external genitalia or a disturbance of the
normal endocrinological events of puberty. Overall it is estimated
that endocrine disorders account for ~40% of the causes of primary
amenorrhoea, the remaining 60% having developmental abnormalities. For an excellent review of this topic see http://
www.emedicine.com/med/topic117.htm and for a summary see
Table III.

Secondary amenorrhoea
Secondary amenorrhoea is the absence of menstruation, which
may be temporary or permanent and of >6 months duration,
although 6 months is largely an arbitrary denition and
amenorrhoea should be considered within its clinical context.
Any cause of secondary amenorrhoea may also cause primary
amenorrhoea. PCOS and pregnancy may both present with
secondary amenorrhoea in early reproductive life. A prolonged
hypoestrogenic state carries the risk of bone demineralization,
osteoporosis and fracture. Therefore, secondary amenorrhoea
associated with low estrogen levels requires estrogen supplementation to maintain bone density. The causes of secondary
amenorrhoea are summarized in Table IV.

HPO = hypothalamicpituitaryovarian; TB = tuberculosis.

Examination and investigation of amenorrhoea in

adolescence
pain and the anlagan should be excised (usually laparoscopically).
Fusion abnormalities of the vagina

Longitudinal fusion abnormalities may lead to a complete

septum that may be associated with two complete uterine horns
498

Taking a gynaecological history from an adolescent should include

questions about sexual activity and risk-taking behaviour.
However, pregnancy should be ruled out with laboratory testing
in cases of oligoamenorrhoea or amenorrhoea even if sexual
activity is denied, since adolescence may feel unable to conde in
a clinician about this (Adams Hillard, 2002).

Menstrual disorders in adolescence

Table V. The investigation of amenorrhoea
Method
History
Physical examination
Note body mass index, pubertal development,
Stigmata of PCOS and other endocrine disease
Acne and hirsutism
Endocrine assessment
Pregnancy test if suspected
FSH, LH, prolactin, thyroid function tests
Testosterone (if stigmata of PCOS)
Further endocrinology only if above do not provide diagnosis
Ultrasound
Morphology of ovaries and endometrial thickness (for estrogenization)
Pelvic imaging
MRI if suggestion of complex anatomical problem
Pituitary/hypothalamic imaging
As indicated clinically
Bone mineral densitometry
If hypoestrogenic
Karyotype
If premature ovarian failure
PCOS = polycystic ovary syndrome.

The need for a bimanual examination in a young woman who

has never been sexually active should be carefully considered. The
majority of adolescents can be assessed using an examination of
the external genitalia and imaging studies such as ultrasound if
needed. However, a cooperative adolescent, even if never sexually
active, can often be evaluated with a single nger bimanual
examination, allowing her mother to be present if the patient
wishes. Examination may identify a blind ending or absent vagina
or haematocolpos, and the visualization of the cervix.
Transabdominal ultrasound may indicate whether the uterus and
ovaries are present. In the USA, magnetic resonance imaging is the
primary investigation of preference and can provide information
additional to ultrasound. Examination under anaesthesia (EUA) is
the nal step in diagnosis which might rarely need to be combined
with laparoscopy, for example in complicated Mullerian
abnormalities (Letterie et al., 1995).
A transabdominal ultrasound examination of the pelvis is an
excellent non-invasive method of obtaining valuable information
in these patients. An examination under anaesthetic is sometimes
indicated for cases of intersex with primary amenorrhoea; it is
rarely required in cases of secondary amenorrhoea.
The investigative techniques for amenorrhoea are listed in
Table V. Signs of hyperandrogenism (acne, hirsutism, alopecia)
are suggestive of the polycystic ovary syndrome (PCOS, see
below). Hyperandrogenism must be distinguished from virilization, where high circulating androgen levels are associated with
deepening of the voice, increase in muscle bulk and cliteromegaly.
A baseline assessment of the endocrine status should include
measurement of serum prolactin and gonadotrophin concentrations and an assessment of thyroid function. Elevated gonadotrophin (FSH and LH) concentrations indicate ovarian failure
whilst suppressed levels suggest hypogonadotrophic hypogonadism. Hyperprolactinaemia due to prolactinoma may present with

secondary amenorrhoea in adolescence and is associated with

osteopenia or osteoporosis. Colao et al. (2000) observed that
although prolactin levels were normalized by dopamine antagonist
therapy, bone density did not return to normal, emphasizing the
importance of early diagnosis and longer-term follow-up of these
patients. A moderate and transient elevation in prolactin may
occur in response to `stress' or even a recent breast examination. A
more permanent but still moderate elevation (>700 mIU/l) is
associated with hypothyroidism and is also a common nding in
women with PCOS.
Amenorrhoea in women with PCOS is secondary to acyclical
ovarian activity and continuous estrogen production. A positive
response to a progestogen challenge test, which induces a
withdrawal bleed, may distinguish patients with PCOS-related
hyperprolactinaemia from those with polycystic ovaries and
unrelated hyperprolactinaemia, because the latter causes estrogen
deciency and therefore failure to respond to the progestogen
challenge. However, a positive withdrawal bleed may also be
associated with weight gain in a previously underweight subject.
Amenorrhoea may also have long-term metabolic and physical
consequences. In women with PCOS and prolonged amenorrhoea,
there is a risk of endometrial hyperplasia and adenocarcinoma. For
this reason, endometrial shedding should be encouraged at least
every 3 months, either via therapy with a low-dose contraceptive
pill or cyclical progestogens, or via intermittent progestogens.

Premature ovarian failure

Premature ovarian failure may present as primary or secondary
amenorrhoea in adolescents. Karyotyping is indicated to exclude
Turner's syndrome (45X, or 46XX/45X mosaic) or other sex
chromosome mosaicisms. Thyroid-stimulating hormone, fasting
glucose and adrenal antibodies should be checked and repeated
annually, due to the association with autoimmune disease in those
with a normal karyotype. Idiopathic familial POF is also well
recognized and may be associated with a mutation of the inhibin a
gene (Marozzi et al., 2002). Ovarian antibodies are rarely useful.
Measurement of bone mineral density is indicated in amenorrhoeic
women who are estrogen decient. A total of 8% of bone mass is
lost in the rst year after menopause and thereafter between 1 and
2% (Luisetto et al., 1993). The vertebral bone is more sensitive to
estrogen deciency and vertebral fractures tend to occur in a
younger age group (5060 years) than fractures at the femoral neck
(>70 years). For review see Bakalov and Nelson (2001). Dualenergy X-ray absorptiometry (DEXA) is valuable in predicting
patients at risk of fracture. However, DEXA scans of adolescents
require normal values for this population that may not be routinely
included with the scanner software (http://www-stat-class.stanford.edu/pediatric-bones/).

Oligomenorrhoea
Oligomenorrhoea may be dened as menses occurring less
frequently than every 35 days. The commonest cause of
oligomenorrhoea is PCOS and other causes include either
temporary disturbances of menstrual cycle control, body weight
(obesity or underweight) and hyperprolactinaemia as well as
developing causes of secondary amenorrhoea.

499

M.Hickey and A.Balen

PCOS in adolescence

PCOS appears to underlie irregular menses in up to one-third of

girls (Venturoli et al., 1986). Menarche is not usually delayed,
but bleeding is then persistently irregular. In adolescents,
PCOS can present with primary or secondary amenorrhoea,
acne, hirsutism or merely irregular periods, as set out in
Table IV.
Ovarian dysfunction leads to the main signs and symptoms
of the PCOS and the ovary is inuenced by external factors, in
particular the gonadotrophins and insulin, which are themselves dependent upon both genetic and environmental inuences. Approximately 2033% of women of reproductive age
will have polycystic ovaries on ultrasound scan (Polson et al.,
1988; Michelmore et al., 1999); while perhaps 7580% of
these will have symptoms consistent with the diagnosis of
PCOS. Menstrual disturbance is likely to be the main issue for
adolescents with PCOS but the established long-term risks of
obesity, subfertility and diabetes as well as the possible risks of
endometrial hyperplasia and carcinoma (Hardiman et al., 2003)
and cardiovascular disease (recently reviewed by Rajkowha
et al., 2000) and breast cancer (Balen, 2001) require consideration. Obesity also compounds the clinical manifestations of
PCOS and weight loss may lead to symptom improvement.
PCOS may rst manifest in adolescence but its origins are
likely to be much earlier. PCOS is associated with increased
weight gain during puberty (Balen and Dunger, 1995). Genetic
study of PCOS has identied links with insulin secretion and
action as well as increased ovarian androgen secretion).
Association has been reported with common allelic variation
at the variable number of tandem repeat locus (VNTR) in the
promoter region of the insulin gene (Waterworth et al., 1997).
This locus has been variably associated with the risk of obesity,
insulin resistance and type 2 diabetes. The identied association has been with the class III/III genotype, particularly in
women who have anovulatory cycles and are hyperinsulinaemic (Waterworth et al., 1997; Michelmore et al., 2001).
Management of PCOS in adolescence

The clinical management of young women with PCOS should

be focused on her individual problems. Obesity worsens both
symptomatology and the endocrine prole and so obese girls
(BMI >25 kg/m2) should be encouraged to lose weight, and
they should also have a test of fasting glucose tolerance (e.g. 2
h GTT) and fasting insulin to assess the glucose:insulin ratio.
Impaired glucose tolerance has been seen in up to one-third of
adolescents with PCOS (Palmert et al., 2002).
Hyperandrogenism and hirsutism are distressing symptoms
for young women and the clinician should be aware that
cosmetic measures may disguise the extent of the problem.
Optimally treatment combines cosmetic and medical therapies.
Medical regimens stop further progression of hirsuitism and
slow the rate of hair growth. However, drug therapies may take
69 months or longer before any benet is perceived and so
laser, electrolysis, waxing and bleaching may be helpful in the
interim. Medical therapy is aimed at slowing the rate of hair
growth whilst cosmetic treatments attempt to remove existing
500

hair. Laser therapy works best in women with dark hair on fair
skin, but is expensive and not permanent (despite claims to the
contrary). Electrolysis is the only permanent method of hair
removal and should be delayed until androgens are effectively
suppressed.
Hyperandrogenism can be treated by a combination of an
estrogen (such as ethinylestradiol, or a combined contraceptive
pill), and the anti-androgen cyproterone acetate (CPA, 50100
mg). Estrogens lower circulating androgens by a combination
of a slight inhibition of gonadotrophin secretion and gonadotrophin-sensitive ovarian steroid production and by an increase
in hepatic production of sex hormone-binding globulin resulting in lower free testosterone. The CPA is taken for the rst 10
days of a cycle (the `reversed sequential' method) and the
estrogen for the rst 21 days. After a gap of exactly 7 days,
during which menstruation usually occurs, the regimen is
repeated. As an alternative, the preparation Dianette
(Schering, UK) contains ethinylestradiol in combination with
CPA, although at a lower dose (2 mg). CPA acts as a
competitive inhibitor at the androgen receptor. CPA can rarely
cause liver damage and liver function should be checked after 6
months and then annually. Combined oral contraceptive pills
(COCP) have the added advantage of providing effective
contraception for adolescents, given the high rates of
unintended pregnancy in this group. If contraception is needed,
counselling should include the information that COCP will not
protect against STI and additional barrier contraception is
advised. However, the COCP has the disadvantage of increasing insulin resistance (Morin-Papunen et al., 2000). However,
this effect on insulin sensitivity has not been observed with the
norgestimate-containing OCP in non-obese PCOS subjects
(Cibula et al., 2002).
Spironolactone, a potassium sparing diuretic, has antiandrogenic properties and is useful in women for whom the
oral contraceptive pill is contra-indicated (e.g. because of
hypertension). Spironolactone, at a dose of 50200 mg daily,
may result in erratic menstrual bleeding and should be
combined with reliable contraception. A new COCP,
Yasmin (Shering UK), contains the progestogen, drospirenone, which is a derivative of spironolactone, with potential
anti-androgenic properties and benets for women with PCOS.
Although diet is the rst line treatment for improving insulin
sensitivity in overweight adolescents with PCOS, insulinsensitizing agents, such as metformin, are becoming increasingly popular in the management of PCOS as they act directly
on insulin resistance and help correct both metabolic and
endocrine problems. Although initial studies suggest an
improvement in menstrual regularity in PCOS, the role of
insulin-sensitizing medications in adolescents with PCOS is
unclear. Primary prevention of diabetes mellitus and cardiovascular disease by lifestyle modication, including regular
exercise and a balanced diet, is particularly important in
adolescents, who have the opportunity to establish healthy
habits before entering adulthood. The ndings of diabetes
prevention trials suggest that these interventions may be more
efcacious than pharmacological therapy (Legro, 2002;

Menstrual disorders in adolescence

Table VI. The spectrum of clinical manifestations of polycystic ovary syndrome
Signs and symptoms
(% patients affected)

Endocrine disturbances

Possible late sequelae

Obesity (38%)

Insulin
Sex hormone-binding globulin

Dyslipidaemia
LDL, HDL, triglycerides

androgens (testosterone and

androstenedione)

Diabetes mellitus

Menstrual disturbance (66%)

Oligomenorrhoea 47%
Amenorrhoea 19%
Regular cycle 30%
Hyperandrogenism (48%)
Infertility (PCOS underlies 73% of anovulatory infertility)
Asymptomatic: 20% of those with polycystic ovaries

LH
prolactin

Kaufman et al., 2002; Kiess et al., 2003). However, the

implications of PCOS for long-term health remain unclear,
and, although mortality rates have not conclusively been shown
to be elevated in PCOS patients, concerns exist that women
with this syndrome cluster risk factors for premature morbidity
and mortality (Wild, 2002).
Management of amenorrhoea
The general principle of treatment is to replace estrogen when
hypoestrogenaemia is demonstrated in order to prevent the
consequences of long-term estrogen deciency. Even short-term
estrogen deciency leads to bone loss, increasing the risk of
osteoporosis. More prolonged deciency may also increase
cardiovascular risk. When a uterus is present, progestogens should
also be given to avoid endometrial hyperplasia. Longitudinal
studies are needed to determine the optimum replacement needed
for young hypoestrogenic women such as those with Turner's
syndrome. In these patients, growth hormone supplements may
also be used to increase stature.

Management of dysfunctional uterine bleeding in

adolescence
The term `dysfunctional uterine bleeding' (DUB) is taken to mean
`excessively heavy, prolonged or frequent bleeding of uterine
origin, which is not due to pregnancy or to recognizable pelvic or
systemic disease'(Fraser, 1985). Ovarian cycles associated with
bleeding may be ovulatory or anovulatory, and the condition may
be acute or chronic. In later reproductive life, DUB is regular and
ovulatory in ~80% of cases, but in adolescence and early
reproductive life, anovulatory DUB with irregular cycles is
relatively common, up to 50% of cycles in the rst year of
menarche.
Young women presenting with ovulatory DUB are occasionally
found to have underlying coagulopathies. If there is a family
history of coagulopathy or a history of heavy bleeding at surgery or
tooth extraction, it is worth discussing with a haematologist which
special investigations may be indicated and how the blood should

Cardiovascular disease
Hypertension
Endometrial carcinoma

be collected. In the majority of cases, no signicant underlying

pathology will be found and reassurance of the patient and her
parents is the most appropriate management.
General physical examination should search for evidence of
pelvic and systemic pathology that may account for heavy and/or
irregular bleeding. Investigations may include abdominal ultrasound
scan and, if necessary, examination under anaesthesia. An estimation of circulatory haemoglobin levels should be performed on all
females complaining of menorrhagia. Immune thrombocytopaenia
purpura (ITP) may also present with menorrhagia in the adolescent
(associated with bruising, petechiae and mucosal bleeding) requiring specialist haematological assessment (Bevan et al., 2001). The
incidence of these disorders in the UK has not been reviewed
recently, but a 9 year audit of paediatric cases in the USA from 1971
to 1980 revealed that a primary coagulation disorder was found in
almost 20% of 59 patients admitted to a children's hospital for acute
menorrhagia, where genital tract pathology had been excluded. Onequarter of those with severe menorrhagia (haemoglobin <10 g/100
ml), one-third of those requiring transfusion, and one-half of those
presenting at menarche had such an underlying disorder (Claessens
et al., 1981). Red blood cell indices and serum ferritin levels may be
indicated if there is iron deciency anaemia. In anovulatory DUB,
serum LH and FSH changes may indicate PCOS or a premature perimenopausal state. Transabdominal ultrasound and serum androgens
will provide additional information in the diagnosis of PCOS. If
there is irregular and acute bleeding, complications of pregnancy
should be excluded.

Management of DUB
Management of DUB in the adolescent is medical in almost all
circumstances. Modern low-dose oral contraceptive pills can be
safely prescribed to most young women, provided that they are not
known to have contraindicating factors. The combined oral
contraceptive pill has been shown by objective measurement to
reduce menstrual blood loss in ovulatory and anovulatory DUB
(Nilsson et al., 1971).
In the treatment of anovulatory DUB, progestogens may be
suitable for women who are unable or unwilling to take estrogen-

501

M.Hickey and A.Balen

Table VII. Differential diagnosis of dysmenorrhoea and pelvic pain in adolescence (data derived from Quint, 2002)
Cyclical, or associated with bowel and bladder
symptoms, dyspareunia if sexually active

Uterus and ovaries

Related to bowel motions, associated with bloating

Dysuria

Gastrointestinal tract
Genitourinary system

Associated with back pain

Musculoskeletal system

Variable pattern

Psychosocial

Endometriosis
Ovarian pathology
Adhesions
Infection
Mullerian abnormalities
IBS, ulcerative colitis, Crohn's disease
Recurrent cystourethritis, renal abnormality such
as pelvic kidney, interstitial cystitis
Skeletal abnormality such as scoliosis, kyphosis,
spondylosis, spondylolithesis, myofascial syndrome
Consider family stress, sexual and physical abuse

IBS = irritable bowel syndrome.

containing compounds. Oral progestogens are only effective if

given for >21 out of 28 days and low-dose luteal phase
progestogens are not an effective treatment for menorrhagia
(Lethaby et al., 2000).
In the adolescent with anovulatory DUB, cyclical oral
progestogens may be required until spontaneous regular ovulation
occurs.
The disadvantages of oral progestogen regimens for DUB
include the need for long-term oral medication and the possibility
of unwanted `pre-menstrual symptoms' including: bloating,
edema, headache, depression and reduced libido; androgenic
effects (depending on the progestogen used), such as acne and
hirsutism; irregular breakthrough bleeding and a change in
carbohydrate tolerance and lipid balance. The contraceptive
depot preparation of medroxyprogesterone acetate (such as
Depo-Provera) will induce amenorrhoea in 50% of users at 1
year. For those who can accept the 1520% rate of irregular or
prolonged breakthrough bleeding, this may provide a safe and
effective treatment regimen. The Mirena intrauterine implant
system (IUS) releasing 20 mg of levonorgestrel/day (LNG-IUS 20)
is a highly effective long-term treatment for both ovulatory and
anovulatory DUB (Anderson and Rybo, 1990). The system also
provides reversible contraception. The Mirena has a place in the
management of refractory DUB in young women but the physician
should consider insertion of the device under general anaesthetic.
Hysteroscopy at the same time may also be indicated to exclude
(rare) intrauterine pathology if a general anaesthetic is planned.
Irregular `breakthrough' bleeding is the main unwanted effect of
all progestogen preparations and the main reason why women
choose to discontinue these preparations, despite their other
advantages.
Menstrual disturbances in PCOS

In obese girls with PCOS, weight loss may lead to resumption

of ovulation (Kiddy et al., 1992). Cyclical progestogen
treatment for 10 days every 6 weeks will generally lead to
withdrawal bleeding and prevent hyperplasia. The COCP is an
alternative method of producing a regular withdrawal bleed,
but concerns about effects on insulin resistance must be
considered (see above).
502

Endometriosis and pelvic pain

Dysmenorrhoea is a common gynaecological complaint among

adolescent girls. Traditional teaching has distinguished primary (or spasmodic) and secondary dysmenorrhoea, but there
is little objective evidence that these are distinct conditions
with differing pathologies. It is likely that many cases of
endometriosis in young women have been put down to
`spasmodic' dysmenorrhoea and persistent, disruptive and
severe dysmenorrhoea unresponsive to non-steroidal antiinammatory drugs (NSAID) and COCP warrants investigation and treatment. Endometriosis may also present with noncyclic pain, bowel and urinary symptoms.
The differential diagnosis of chronic pelvic pain in the
adolescent (>6 months) is extensive (see Table VII). History
should include questioning about bowel symptoms suggestive
of irritable bowel syndrome or inammatory bowel disease as
well as a social and sexual history.
Pelvic examination may be helpful if the young woman is
sexually active, and may reveal uterosacral tenderness or
nodularity suggestive of endometriosis, or cervical excitation
suggestive of infection. Pelvic ultrasound is more sensitive for
ovarian pathology. It is reasonable to offer an empirical 3
month trial of COCP, taken continuously for dysmenorrhoea.
Failure to respond to this therapy and persistent gynaecological
symptoms is an indication for laparoscopy. The patient's
consent should be obtained before laparoscopy to excise visible
endometriosis.
Menstrual disorders in adolescents with intellectual disability

Historically there has been a tendency to aim for amenorrhoea

in institutionalized women with intellectual disability, using
continuous progestogens or by surgery. This has arisen from
concerns about the ability of a young intellectually disabled
woman to cope with menstruation and the risks of sexual abuse
and unplanned pregnancy. Common menstrual disorders in this
group include dysmenorrhoea (or behavioural changes thought
to reect pain), amenorrhoea associated with delayed puberty
or secondary to low weight, menorrhagia and cyclic exacerbation of conditions such as epilepsy (Grover, 2002).

Menstrual disorders in adolescence

Endometrial ablation has been reported as an option for
managing heavy and irregular bleeding in this population
(Wingeld et al., 1994) but little is known about the medium to
long-term efcacy of this treatment in young women. In
addition, since endometrial ablation does not guarantee
contraception and subsequent pregnancy is associated with
very poor fetal outcome, tubal ligation is usually recommended
at the same time. In a case series of >100 adolescents with
signicant intellectual disability and moderate to high support
needs requiring specialist advice for menstrual disorders,
Grover (2002) encountered only two young women requiring
hysterectomy or endometrial ablation for menstrual disorders.
Alternative management included providing information about
menstrual hygiene training, and improvement of family
support services including respite care. The capacity of
women with disabilities, even those with high support needs,
to gain additional skills in their personal menstrual management may often be underestimated (Grifn et al., 1994).
Effective medical managements for menstrual disorder in
intellectually disabled girls and women were essentially the
same as those for non-disabled women and included NSAID
for dysmenorrhoea and COCP or DMPA for menorrhagia.
Surgery was only performed as a `last resort'. Since this study
the Mirena (Schering, UK) intrauterine system has become
widely available. It is likely that Mirena will have a major
impact on menstrual management in this population, and may
obviate the need for surgery with its attendant risks and loss of
reproductive potential in these vulnerable women. Mirena has
demonstrated an 86% reduction in menstrual blood loss at 3
months and a 97% reduction at 12 months in women with
ovulatory dysfunctional uterine bleeding (Anderson and Rybo,
1990), which continues over a 5 year period. Nearly 50% of
Mirena users experience amenorrhoea during 3 years of use
(Baldaszti et al., 2003). Menstrual blood loss is also reduced in
anovulatory bleeding. The system also acts as a highly
effective and reversible contraceptive. Insertion under general
anaesthetic may be preferable in women with intellectual
disability.
Summary
Adolescent menstrual disorders are relatively common and it
is unclear who these young women present to and what
information is provided to them. The issues of when to talk,
how to talk and what to say to young girls and their
families regarding the processes of early maturation and
menstruation are unresolved. These discussions will inevitably
stray into issues of self-condence, body image and sexuality
and it is possible that many gynaecologists may not feel
sufciently comfortable or experienced in these areas. Those
adolescents who do attend the gynaecologist will often attend
with a mother or friend and sensitivity in dealing with even minor
disorders is paramount. Referral to specialized centres for rarer
but serious endocrine or structural abnormalities is necessary.
Liaison with paediatricians or psychosexual counsellors may also
be required.

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