Paediatric and adolescent gynaecological disorders

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Chapter 15 Paediatric and adolescent gynaecological disorders

Gynaecological problems encountered in children and adolescents are unique to these age groups and involve sensitivity and skills differing from those utilised for adults.

Paediatric disorders

• normal genital anatomy in the prepubertal child

• maternal oestrogenisation wanes by 6 months

• hypooestrogenisation causes labia to be atrophic, and hymen to appear erythematous

• hymen may be crescentic, annular (circumferential), microperforate or cribriform

• cervix is often flush with the vaginal vault or protrudes slightly

• uterus may be so small that it may be difficult to identify on ultrasound in inexperienced hands

Examination of a prepubertal child

• examine only with parental consent, and once the child and parent are comfortable with you

• start with a general examination

• appropriate explanation to child and parent is important: it will not involve internal examinations, it should not hurt, there is no use of force, listen to the child if she wants you to stop

• with appropriate rapport and explanation, these examinations are usually tolerated very well; if very resistant, consider deferring examination or performing an examination under anaesthesia (EUA) depending on urgency

• frog-leg position on mother’s lap if more comfortable

• genital inspection then lateral and outward traction on labia majora will enable hymen to open; ask child to cough while inspecting lower vagina

Vulvovaginal abnormalities

Vulvovaginitis

• symptoms: pruritis, dysuria, soreness of the vulval area, vaginal discharge, occasionally vaginal bleeding

• usually non-specific inflammation with no identifiable pathogen (80% of cases)

• specific pathogens: group A beta-haemolytic streptococci and Haemophilus influenzae, from the upper respiratory tract, are the most common

Vaginal bleeding

• pathological and warrants further investigation

• consider the following:

Vaginitis

• commonest cause

• if suspected, may treat with antibiotics alone initially, but if recurrent bleeding do EUA to exclude another cause

Foreign body

• commonly toilet paper

• EUA and removal of the object

Sarcoma botryoides (embryonal rhabdomyosarcoma)

• presents with lower abdominal mass and vaginal bleeding

• may sometimes see grape-like lesions at introitus

Genital trauma

• Straddle injuries may cause haematomas and genital lacerations (usually involving labia or fourchette) and associated with bruising of labia or thigh. A consistent history is important.

• Labial fat pads generally protect the hymen and vagina. Hymenal, vaginal or perianal lacerations, without labial or thigh bruising, is suspicious for non-accidental penetrating injury. Note that most children who have been sexually abused have normal clinical findings (<5% have abnormal findings). Any concerns regarding sexual abuse should be immediately referred to a skilled professional in the field so that appropriate investigation, documentation and follow-up can be organised.

Management

• ensure voiding is possible, sitz baths

• EUA if ongoing bleeding expanding haematoma, concerns about anatomical integrity

Urethral prolapse

• precipitants: hypo-oestrogenisation, chronic straining

• treat with oestrogen cream nocte and sitz baths; regression seen over days to weeks

• if persists and symptomatic, may require surgical excision

Other anatomical causes

• vascular lesions

• Crohn’s disease

Precocious puberty

• bone age, follicle-stimulating hormone (FSH), luteinising hormone (LH), oestradiol, pelvic ultrasound to look for ovarian activity

Lichen sclerosus

• commonest age of presentation in premenarcheal girls is 5–7 years

• symptoms: pruritus, papules, fissures, erosions, bleeding

• 75% improvement in symptoms after menarche

• 0.05% betamethasone dipropionate for 6–12 weeks until resolution, then maintenance with low potency steroid for 3 months

• recurrences common

Genital ulcers

• may raise suspicion for sexual abuse, but often associated with non-specific viral illness

• differential diagnosis of solitary ulcers syphilis, chancroid (Hemophilus ducreyi), Crohn’s disease, pyoderma gangrenosum, ulcerative vulvitis (bacterial pathogen or trauma) and malignancy

• multiple ulcers (more common): herpes, chicken pox or shingles (varicella zoster virus (VZV)), mononucleosis (Epstein-Barr virus (EBV))

• secondary syphilis, candidiasis, scabies, Behçet’s syndrome, aphthous ulcers due to non-specific viral illness (very common), and fixed drug reactions

• investigation: viral, bacterial fungal swabs, serology (syphilis, EBV, VZV); consider autoantibody screen, biopsy

Genital warts

• Genital warts may raise suspicion for sexual abuse, particularly in children over 3 years; however, they may be transmitted in other ways (e.g. vertical and latent infection may possibly reactivate later in life).

• Warts are found in up to 0.2%–3% of children who have been sexually abused.

• The presence of warts alone without supporting clinical or social information is not diagnostic of abuse.

• Human papillomavirus (HPV) typing is not recommended. The viral subtype alone cannot determine the mode of transmission, as the virus doesn’t display 100% site specificity in children.

• DNA fingerprinting to test for clonality is not useful. HPV6 and HPV11 are stable viruses, and do not vary markedly between hosts. Viral variants between the child and an alleged perpetrator are likely to be identical, as they represent circulating type for that area, and would not indicate the mode of transmission or the source.

• Get advice if concerned.

Precocious puberty

Definition. The onset of pubertal development prior to 8 years of age in females may represent precocious puberty (some healthy children begin development before this age). Normal pubertal development often occurs in sequence: growth acceleration, thelarche, adrenarche, menarche. The time between onset of breast development to menarche is around 2 years. The mean age of menarche in Australian girls is 13 years (range 12.2–14.2 years).

Aetiology

Treatment of precocious puberty

Centrally mediated precocious puberty

• GnRH analogues: a full 3 months are needed to suppress the hypothalamic–pituitary–gonadal axis. Depot preparations are preferable.

• Medroxyprogesterone acetate and cyproterone acetate are no longer commonly used.

Gonadotrophin-independent

• McCune-Albright syndrome is the commonest cause, and treatment with GnRH analogues is ineffective. Suppress gonadal steroidogenesis directly with aromatase inhibitors (which block conversion of testosterone to oestradiol). Other options are ketoconazole and tamoxifen.

Adolescent gynaecology

Adolescent health principles

• accessible, non-judgmental, confidential, adolescent-friendly care

• only some exceptions to the duty of confidentiality, including the risk of self-harm or harm to others, or abuse

Consent to treatment

A young woman under 18 years can give consent to treatment if judged a ‘mature minor’, if she is assessed to be mature and competent and where she fully comprehends the nature of the specific condition for which treatment is sought, the purpose, methods, risks, potential benefits of treatment and alternatives to treatment. Assessment of maturity and competence include general maturity of speech, level of schooling, ability to understand the nature and rationale of treatment, and to be able to explain it in words.

Adolescent menorrhagia

• The commonest cause is anovulatory dysfunctional uterine bleeding.

Adolescent metrostaxis (acute heavy loss)

• The commonest cause is still dysfunctional uterine bleeding, but consider pregnancy complication, genital tract trauma, coagulopathy, arterio-venous malformation and malignancy (all rare).

• In the acute phase, vital signs may remain stable despite significant blood loss, and then drop very quickly. Aggressive medical management is required.

• Resuscitate with intravenous fluids, transfuse and get haematological opinion.

• If hypo-oestrogenic (perimenarchal, slim, poor breast development), consider oestrogen first, then may try OCP four times a day over a few days, and then wean slowly to one daily.

• If clinically normo-oestrogenic, then try provera 10 mg every 2 hours until bleeding stops, and then wean slowly over a few weeks.

• Maintenance hormonal treatment with OCP daily thereafter may be beneficial while under investigation for bleeding disorder over ensuing months.

• Surgical investigation is rarely required.

Adolescent dysmenorrhoea

• Prostaglandin-mediated primary dysmenorrhoea is the commonest cause.

• Endometriosis is being diagnosed more commonly with increasing laparoscopy rates (47%–67% prevalence in adolescents with refractory symptoms). The disease tends to be in the cul de sac, and atypical in appearance, and usually early stage. It is unclear if surgical intervention changes the natural history of disease in adolescents or improves reproductive potential. Current standard of care at Department of Gynaecology Royal Children’s Hospital, Melbourne, is to manage with medical therapy and resort to diagnostic laparoscopy in refractory cases.

• Uterine anomaly: always consider in severe refractory primary dysmenorrhoea and pain in an adolescent.

Uterine anomaly

Imperforate hymen

• amenorrhea, cyclic pain, urinary retention, bluish introital bulge with abdominal pressure, treat with cruciate incision

• consider microperforate hymen in someone with scant vaginal loss and similar symptoms

Transverse vaginal septum

• incidence: 1 in 70,000

• amenorrhea, bulge at introitus absent with abdominal pressure, yet haematocolpos seen on ultrasound

• correct diagnosis is crucial, as requires Z-plasty, and possibly use of vaginal dilators preoperatively and vaginal mould postoperatively (can only be done when the young woman is psychologically ready)

• menstrual suppression with the OCP prior to this to manage pain is successful

Obstructed longitudinal vaginal septum, uterine didelphys, renal agenesis

• incidence: 1 in 35,000

• menstruating from normal side, unilateral haematocolpos

• menstrual suppression for pain management until septum resected vaginally

• mould not required

Rudimentary uterine horn

• most are diagnosed in the third decade

• non-communicating horns even with little endometrium may carry a pregnancy and rupture in the second trimester causing fetal loss and significant maternal morbidity

• recommended that rudimentary non-communicating horns be removed prior to childbearing

Vaginal agenesis

• incidence: 1 in 5000

• associated with renal spinal cardiac anomalies and hearing problems

• 80% success with use of dilators to create neovagina (when the young woman is ready)

• modified Sheares vaginoplasty may be used in other cases with use of mould and dilators postoperatively (timing driven by the young woman)

• important not to misdiagnosis late presentation of complete androgen insensitivity syndrome, by performing karyotype, and asking/checking for presence or absence of axillary or pubic hair, inguinal hernias

Adolescent polycystic ovarian syndrome (PCOS)

Incidence is 3% in the adolescent population. While current definitions of PCOS do not vary between adult and adolescent, some feel that the Rotterdam criteria will overdiagnose PCOS in adolescence. Adolescents typically have relative androgenaemia, insulin resistance, cystic ovaries and anovulatory cycles. Special considerations regarding PCOS in adolescents are:

• Primary amenorrhoea may be a presenting feature of severe PCOS.

• Clues to the presence of PCOS in adolescent women are persistence of menstrual irregularity 2 years postmenarche, premature pubarche and adrenarche, insulin resistance as determined by acanthosis nigracans and a fasting insulin:glucose ratio <7 (as opposed to 4.5 in an adult).

• A follicular phase morning 17-alpha-hydroxyprogesterone is useful to exclude non-classic congenital adrenal hyperplasia.

• Small studies with the use of metformin have shown a favourable effect on ovulation, resumption of menses and reduction of central adiposity, but treatment is generally reserved for those who have failed other lifestyle measures.

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