Adolescent Gynaecology Flashcards
Puberty
- 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
Progression of puberty
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)
Delayed Puberty
- 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
Management Delayed Puberty
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
Precocious puberty
- 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)
Management Precocious puberty
- 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