Puberty is a dynamic continuum with the first signs in girls appearing at around the age of 9 years (see Chapter 1). Pubertal development that occurs before the age of 8 years is considered to be precocious. Precocious puberty occurs twelve times more commonly in girls than in boys. It may be central, in which there is premature activation of the hypothalamic—pituitary—ovarian axis or it may occur independent of the axis, in which case it is referred to as pseudopuberty or pseudosexual precocity.

Central precocious puberty

Central precocious puberty is also more common in females than in males by a factor of 23. In girls, most causes are idiopathic. Causes identified include brain abnormalities, such as hydrocephalus, tumours (particularly hamartomas), congenital abnormalities, trauma and infections. Hamartomas may cause precocious puberty at an extremely early age, even as early as the neonatal period. Precocious puberty is also associated with a wide variety of diverse conditions including neurofibromatosis, tuberous sclerosis and hypothyroidism. Hypothyroidism is particularly likely to be the diagnosis if the girl's pubertal development occurs without the accompanying growth spurt.

Because of the association with underlying brain pathology, it is recommended that all girls with precocious puberty have brain imaging performed with either computed tomography or, preferably, magnetic resonance imaging, even in the absence of neurological symptoms or signs, as the abnormality may be small.

Girls with central precocious puberty undergo a normal sequence of pubertal development.

Menarche

The menarche is an important event in a girl's life and one that has special significance in many cultures. There has been a gradual reduction in the age of menarche in the UK over the 20th century, with a decrease of about 3–4 months per decade, which has been thought to be due to improvements in health and nutrition. The average age of the menarche in 1840 was 16.5 years and is presently 12.8 years (with a normal range of 10.0–16.5 years). There has been a recent upturn in the age of menarche in the UK for reasons that are not clear, although it has been suggested that an increase in exercise and the desire to be slim may be factors. This reversal in the age of menarche has also occurred in other countries, including Iceland, Poland, Italy and Sweden but not Germany.

FACTORS DETERMINING THE AGE OF MENARCHE

Genetic factors

Nutritional factors

Socio-economic status

Geographic location

General health

Exercise

The age of menarche is determined by several factors. The importance of genetic factors is underlined by studies showing mother-daughter pairs and in twins, with identical twins having a closer relationship in age of menarche than non-identical twins. The role of body weight and the percentage of body fat in the age of menarche has been debated, with the consensus of opinion being that weight is the more important factor.

With changes in lifestyle and improvements in the standard of living, young people are reaching physical maturity much earlier than in previous generations. As a result of this, adolescents are reaching sexual maturity earlier. Emotional and psychological maturity tends to lag behind, although this is not recognised by the teenager who strives for more independence from the family unit. The rate of teenage sexual activity has increased steadily and consistently over the second half of the 20th century. Young people now start sexual activity at an earlier age and have more sexual partners in their life. Premarital sex no longer carries a stigma and a culture of serial monogamy is now considered by many to be the norm.

Unfortunately, the use of contraception lags behind the increase in sexual activity, with the UK having the highest teenage conception rate in Europe. This prompted the implementation of a Teenage Pregnancy Strategy by the UK Government, combining provision of family planning services with education. This has contributed a steady decline in teenage pregnancy rates from 46.6/1000 in 1998 to 41.3/1000 in 2005.

Sex education

As part of the Teenage Pregnancy Strategy, local authorities were told to prioritise sex and relationship education in schools and charged with developing a comprehensive programme of sex and relationship education in all schools.

Sex education is not based entirely around anatomy and the act of sexual intercourse.

Problems with heavy or painful menstruation are the most common reasons for seeing a teenager at a gynaecological clinic. It is extremely important when assessing an adolescent with a complaint of abnormal menstruation that a careful history is taken and that some time is spent trying to make an objective assessment of the degree of the problem. It is well appreciated among gynaecologists how difficult it is to do this in adults and it can be more difficult in adolescents, who have fewer criteria against which to measure the degree of their menstrual loss. Failure to do this and to opt for treatment of what is essentially a normal cycle will confirm the girl in her belief that her menstruation is abnormal, with resultant problems in later years. One study, which is rather old now (1966), found a high proportion of girls with adolescent menstrual problems having hysterectomy performed in their early 20s. The mother's fears and expectations also have to be dealt with. The scenario ‘I had problems with my periods when I was her age and I ended up having a hysterectomy before I was 30 and my daughter is going the same way’ is a well-recognised one which requires sensitive handling. It is important for the girl to understand that she is not necessarily going to have the same problems as her mother.

Gynaecological tumours are rare in childhood and adolescence. Malignant tumours are fortunately particularly rare. Their rarity, however, may lead to problems, as the diagnosis may not be considered and individual clinicians may have insufficient experience to ensure appropriate treatment.

Childhood cancers differ from those found in adult life in their classification. Those found in the neonatal period are often embryonic tumours, while those in childhood are often sarcomas, as opposed to the carcinomas more frequently found in adult women. This chapter gives an overview of gynaecological tumours in childhood and adolescence only, as their management is extremely specialised and details are not appropriate at this level.

Tumours of the vulva

Tumours of the vulva are particularly rare in this age group. Tumours such as squamous cell carcinoma, malignant melanoma and sarcoma botryoides occur rarely and the only one that even specialists may see is haemangioma.

HAEMANGIOMA

Haemangiomas used to be classified as being either capillary or cavernous but this classification has now been abandoned. They are not usually present at birth but appear within a few weeks, undergoing a phase of rapid growth before undergoing spontaneous involution, although the time period for this is usually rather long.

Haemangiomas are composed, in the early stages, of proliferating masses of endothelial cells with occasional lumina, later, as they resolve, developing into large endothelial lined spaces. Haemangioma of the vulva, as elsewhere on the body, may be superficial or deep.

Vaginal discharge is the only gynaecological condition that could be considered common in the prepubertal child and it is certainly the most common paediatric gynaecological problem presenting to the paediatric gynaecologist. Other gynaecological problems seen in this age group include vulval irritation without discharge, labial adhesions and, occasionally, vaginal bleeding. Ambiguous genitalia, tumours and precocious puberty, although less common, warrant individual attention and are covered in the relevant chapters.

Vaginal discharge

The newborn female often has a clear or white odourless vaginal discharge, which is produced as a result of circulating maternal estrogen. Occasionally, in the neonatal period, the discharge may be bloodstained, owing to the breakdown of the endometrium, which has been stimulated by maternal estrogen levels.

As the child gets older, the most common cause of vaginal discharge is bacterial infection, commonly known as vulvovaginitis. Specific infections can occur in association with another focus of infection, such as a sore throat or a viral illness. The child transmits the infection from one part of the body to another digitally. This type of vulval infection causes few concerns, as it tends to resolve with the resolution of the primary infection. Recurrent non-specific infections, however, are difficult to manage. In the majority of cases, bacterial culture shows no growth or organisms of low virulence. Symptoms recur frequently, causing significant distress to both the child and parent, while proving difficult for the doctor to manage. This condition is known as recurrent bacterial vulvovaginitis.

Amenorrhoea is not as common a problem as heavy and painful periods but is often poorly understood and managed. Amenorrhoea is a symptom that requires investigation and not a diagnosis in itself.

AMENORRHOEA

Primary: girl has never experienced a menstrual period

Secondary: periods have been absent for 6 months or more.

Causes of primary amenorrhoea

The causes of primary amenorrhoea are considered differently, depending on whether or not secondary sexual characteristics are present (Figure 6.1). In girls with no secondary sexual characteristics, the cause is usually hormonal, whereas in girls with normal pubertal development the cause is usually anatomical.

As a general rule, girls who have primary amenorrhoea with no secondary sexual characteristics should be investigated by the age of 14 years. Common causes include:

1. • constitutional delay

2. • chronic systemic disease

3. • absence of ovarian function

4. • hypothalamic pituitary dysfunction.

CONSTITUTIONAL DELAY

These girls have delayed maturation of the hypothalamic—pituitary—ovarian axis, with resultant delay in the whole process of puberty. There is frequently a family history of similar symptoms in the mother or older siblings. Examination shows a normal relationship between skeletal growth and sexual maturity. Hormone profile shows low levels of FSH, LH and estradiol. Estimation of bone age, if performed, will show delayed skeletal maturation with the bone age being behind the chronological age. Ultrasound examination of the ovaries may be helpful, as the finding of follicles in the ovaries is reassuring and confirms the presence of gonadotrophin activity.

Female genital mutilation is a complex area that raises issues of competing cultural backgrounds, autonomy, health, education and sexuality. Only the medical aspects of female genital mutilation as they relate to teenagers will be considered in this chapter.

Background

Female genital mutilation is practised in many cultures, most frequently in a belt of 28 African countries, with some occurring in the Middle and Far East. With prevalence rates as high as 98% in some countries (Sudan, Somalia), it is estimated that 200 million women are affected. With an increase in migration, female genital mutilation is now encountered in Europe, USA and Australia. While it is most widely associated with the Muslim culture, there is no reference to the practice of circumcision in the Koran. Incidences of the practice in non-Muslim cultures, such as Ethiopian Jews, have been reported. World Health Organization figures suggest that two million women and children have the operation performed every year.

The age at which female genital mutilation is performed varies from culture to culture. In some areas it is carried out at birth. In some parts of Nigeria, it is the custom for the procedure to be performed during the woman's first pregnancy. Most commonly, however, it is carried out in girls before puberty. Immigration figures show that the number of women from communities that traditionally practice female genital mutilation is rising in the UK.

Child abuse has existed for centuries but society has been slow to acknowledge it. It can constitute physical, emotional and sexual abuse as well as neglect. Up until the 17th century, children were considered to be the possessions of their parents and it was accepted that they might harm them. It was only in the 1970s that non-accidental injury of children was accepted as a common occurrence, following the description of battered child syndrome in 1962. Corporal punishment existed in schools in Britain until 30 years ago and was thought to be a necessity. Now there is debate regarding the smacking of children.

The first child protection agency was formed in Liverpool in 1883, followed by the National Society for the Prevention of Cruelty to Children (NSPCC) in 1890. The start of the education system in 1870 allowed for children to be observed at school every day for the first time.

Child sexual abuse was less talked about. Incest was a crime according to the Christian Church from the 1700s but was only made a criminal offence in the UK in 1908. Papers in the literature relating to child sexual abuse start mainly from the 1980s. There have been many high-profile cases reported in the press over the years, such as Maria Colwell in 1974, the Cleveland Inquiry in 1987 and, more recently, Victoria Climbie, who died in 2000 with 108 injuries on her body. All have highlighted loopholes in the child protection system.

Paediatric and adolescent gynaecology adds an interesting dimension to the spectrum of work for gynaecologists but it can be intimidating, especially when one feels inadequately prepared. This concise book lays out the fundamentals of both investigation and management of the child and thereby enhancing confidence.

The text was extremely popular when the first edition was published and I have no doubt that this new edition will be equally successful. Professor Anne Garden is a recognised authority in this field and she has collaborated with colleagues to bring everything into contemporary focus. The new edition includes an important chapter on child sexual abuse – something a gynaecologist may encounter and has the obligation to do the right thing for the sake of the child.

The book is easy to read and makes a handy reference source for the MRCOG candidate and is also likely to be kept close at hand to refresh the memory of the established practitioner who intermittently encounters the younger patient.

Many people believe that the management of children born with disorders of sexual differentiation is the major management problem in paediatric gynaecology. In fact, it forms only a small part of the practice of a paediatric gynaecologist, occurring in about one in 4500 births. The paediatric gynaecologist is but one member of the multidisciplinary team involved in the care of such children and their parents that includes a paediatric endocrinologist, neonatologist, paediatric surgeon, paediatric urologist, clinical geneticist, clinical psychologist and, if available, social work, nursing and medical ethicist.

The first question asked by parents following delivery of their child is ‘What sex is it?’ Not to be able to answer that question is extremely distressing for all concerned and requires sensitive and informed care. Initial management should include giving the parents as full an explanation as possible but they should be warned that it may take some time before a complete answer can be given, although the information required to assign the sex of rearing is usually available within 48–72 hours.

Embryological development

A degree of knowledge of the development of the internal and external genitalia is required to understand the clinical appearance of the child at birth. Chromosomal sex is determined at fertilisation and depends on whether the ovum is fertilised by a sperm bearing an X or a Y chromosome. Until about 6–7 weeks of gestation, the embryo develops in the same manner, irrespective of gender, and both sexes have both wolffian and müllerian ducts.