Adolescent Gynaecology

Question 1

Puberty

Answer 1

• Usual onset between 8-14 years
• Secondary sexual characteristics <8 years = Precocious puberty or Menarche <9 years
• Nil secondary sexual characteristics > 14 years =delayed puberty
• Primary amenorrhoea = nil menarche by age 16 years despite normal Secondary sexual characteristics

Question 2

Progression of puberty

Answer 2

Telarche (Breast development) - Adrenarche (Hair development: Independant of HPO axis and usually follows telearche by few months) - Menarche ( Onset of menstruation, Usually 2-3 years post telarche)

Breast and Hair development assessed using Tanner staging system from stage 1 (Nil) to stage v ( adult pattern)

Question 3

Delayed Puberty

Answer 3

• Hypergonadotrophic Hypogonadism: gonadal failure
  Normal Karyotype: POF, Chemo, radiation, autoimmune, galactosaemia
  Abnormal Karyotype: Turners 45X0 or Swyers 46XY with abnormality SRY gene
• Hypogonadotrophic Hypogonadism
  Reversible: Familial/constitutional, Intracranial tumour (Pituitary adenoma, craniopharyngioma), weight loss/anorexia/excessive exercise, Primary Hypothyroidism
  Irreversible:
  Chronic illness
• Diabetes
• Renal impairment
• Cystic fibrosis
• Crohn’s disease
• Coeliac disease
  Kallman’s syndrome
• 1:7500
• X-linked mutation of Kal-1 gene
• Dysgenesis of olfactory bulbs and GnRH neurones
• Causes delayed puberty associated with anosmia
  Sheehan’s syndrome
  Panhypopituitarism

Question 4

Management Delayed Puberty

Answer 4

Hypo/Hypo:
- Treat underlying cause if known/possible
-Pulsatile gonadotrophins via subcutaneous pump or puberty induction as described below
- Consider ceasing treatment intermittently if cause unknown to see if spontaneous menstruation occurs
- Fertility – can have ovulation induction
Hyper/Hypo:
Puberty induction
Low-dose oral oestrogen (ie 2mcg EE)
Increase dose every 6 months
Once breakthrough bleeding occurs and breast development complete add progesterone
* Cyclical HRT or cOCP
o Fertility
Requires oocyte donation for fertility

Question 5

Precocious puberty

Answer 5

• Central precocious puberty (gonadotrophin dependent) 80%
  o ‘True’ precocious puberty, usually idiopathic
  o Rarely due to brain tumours/CNS malformations
  o Elevated FSH/LH
• Peripheral precocious puberty (gonadotrophin independent)
  o Pseudopuberty, always pathological
  o Hormone-producing ovarian tumours – ie granulosa cell tumour
  o Exogenous oestrogen exposure
  o Congenital adrenal hyperplasia
  o McCune-Albright Syndrome
• Genetic mutation
• Polyostotic fibrous dysplasia and café-au-lait spots
  o Hypothyroidism (elevated TSH stimulating FSH receptors)

Question 6

Management Precocious puberty

Answer 6

• Treat cause if known
• Aim: Slow growth to achieve maximal adult height, attenuate and diminish established precocious characteristics, achieve maximal adult health, facilitate avoidance of abuse
• Central causes
  o Continuous GnRH agonist administration
  o Downregulates pituitary GnRH receptors and suppresses LH/FSH production
• Peripheral causes
  o Treat underlying cause
  Minimal treatment options available for McCune Albright