adolescent gynae Flashcards
what is the average age of puberty in
a) girls
b) boys
a) girls 10.5 years (secondary sex characteristics range from 9-13 years)
b) boys 11.5 years
what do the following terms mean:
Consonant puberty
Disconsonant puberty
Isosexual
contrasexual
constant: follows normal sequence of pubertal development (i.e. tanner stages)
Disconsonant puberty: abnormal sequence of pubertal changes
Isosexual - secondary sex characteristics match karoytpe
contrasexual - secondary sex characteristics opposite to karotype.
at onset of puberty what happens to GnRH release
changes from continuous to pulsatile release at night
what hormone does the pulsatile GnRH release trigger and is vital for the start of puberty?
LH
pre-pubertal what is the FSH: LH ratio
FSH>LH
pubertal what is the FSH: LH ratio
LH> FSH
what age defines precocious puberty in
a) girls
b) boys
a) girls < 8 years
b) boys <9 years
what is the first pubertal sign in females
a) Menarche
B) thelarche
c) pubarche
d) adrenarche
b - thelarche (avg age 9-13 years, commonly age 10)
what is the name of the staging system used in pubertal development
tanner stages (5 stages); stage 1 pre-pubertal stage
what happens in adrenarche?
adrenal glands produce androgens which are converted by the liver and ovaries to oestrogen. Responsible for:
- pubic hair growth
-acne/ oily skin
-body odour
-sweating
what is the second sign of pubertal development in girls
a) Menarche
B) thelarche
c) pubarche
d) adrenarche
c) pubarche (pubic hair growth)
what is the third and final stage of pubertal development in girls?
a) Menarche
B) thelarche
c) pubarche
d) adrenarche
a) menarche (avg age 12.8 years)
what is the average age of menarche?
12.8 years
what age would you classify primary amenorrhoea with no secondary sex characteristics
age 13
what age would you classify primary amenorrhoea when secondary sex characteristics are present
age 15
what tanner stage does menarche coincide with
tanner stage 3 (only 5cm of growth left at onset of menarche)
what gonadotrophins are responsible for male puberty and development of male secondary sex characteristics
testosterone and DHT (metabolite of T)
what is the average age of spermarchy in boys and what tanner stage does this correspond to?
13.4 years - tanner stage 3 -4
how can we categorise causes of precocious puberty
central (gonadotrophin hpg axis dependent) or peripheral
what is the usual order of male pubertal development
testicular enlargement
scrotum thickens and develops
spermarche - avg age 13.4 years
growth spurt –> GH driven
what are the two physiological processes that must take place in order for pubertal development
- adrenarche = increased production of androgens by adrenal cortex –> responsible for pubic hair, oily skin, acne, body odour, sweating etc (this can happen age 6-8 years in girls!)
- gonardarche - activation of gonads due to pulsatile release of GnRH – triggers LH release
When is precocious puberty more of a worrying sign - in females or males?
males worrying feature,
usually benign in females
can you name some of the causes of central precocious puberty
80% females and 40% males no cause is found
HPG dependent cause
- CNS lesions - tumours( gliomas/ hcg secreting tumours) , hypothalamic harmatoma
- CNS trauma
- iatrogenic - radiotherapy to CNS
- infective - meningitis/ encephalitis
-congenital CNS lesions e.g. hydrocephalus, arachnoid cysts
central precocious puberty what is the gold standard diagnostic test:
- ACTH suppression test
- early morning cortisol
- FSH and LH
- GnRH stimulation test
- MRI/CT head
- estradiol
- GnRH stimulation test
note - estradiol levels not reliable
If diagnosing central precocious puberty which of the following results would confirm the diagnosis: (SBA)
- GnRH stimulation test - no response, LH and FSH low
- GnRH stimulation test - LH and FSH very high, FSH > LH
- GnRH stimulation test - LH and FSH very high, LH > FSH, ratio > 8
- GnRH stimulation test - LH and FSH very high, LH > FSH, ratio > 5
- GnRH stimulation test - LH and FS very high i.e. good response, LH> FSH ratio > 8
what effect does raised prolactin e.g. due to a pituitary adenoma would you expect to see in terms of pubertal development
prolactinaemia usually causes delayed puberty rather than precocious
what is the main medical treatment method of central precocious puberty
a) HRT
b) estrogen
c) GnRH analogues
d) hydrocortisone
Treat the underlying cause e.g. surgery
c) GnRH analogues (agonist) e.g. leuprorelin, goserelin - these suppress the hpg axis
aim is to halt/ regress progress of secondary sex characteristics,
prevent early menarche
postpone bone maturation and improve final height
the earlier you start the GnRH analogue the better the outcome of improving final height (, 6 years old)
what effect do GnRH analogues when used in the treatment of central precocious puberty have on BMD in children
no effect
how can you monitor the effect of GnRH analogues when used in the treatment of central precocious puberty
GnRH stimulation test –> LH suppression indicates effective treatment
At what point when treating CPP do you consider stopping GnRH analogues and how should this be managed
MDT discussion
aim to stop at average time of puberty
takes approx 18 months from stopping for menarche to begin
describe the pathophysiology that occurs in PPP (peripheral precocious puberty)
secretion of sex steroids independent to the hpg axis (independent of hypothalamus and pituitary)
name some causes of PPP
- sex steroid secreting tumours .e.g granulosa cell tumour (oestrogen secreting), leydig cell tumour (Testosterone secreting)
congenital causes
- CAH (21 hydroxyls deficiency most common –> raised androgens, decreased mineral and glucocorticoids - low BP, adrenal crisis)
- McCune Albright
3.. Silver russel syndrome - Severe hypothyroidism
- Testotoxicosis
- rare hcg secreting tumours
what results following a GnRH stimulation test would be suggestive of PPP
no response to GnRH stimulation test - low fsh and lh
T & E levels are raised
what type of precocious puberty is the following description typical of
‘Breast development in girls aged <3 years, can spontaneously regress. Caused by maternal oestrogens. True puberty occurs at the normal time’
isolated premature thelarche
(is a benign variant)
what type of precocious puberty is the following description typical of
‘Early pubic hair development (with or without axillary hair) without other features of puberty’
isolated premature pubarche
what type of precocious puberty is the following description typical of
’ Isolated early vaginal bleeding in the absence of other causes or features.’
Isolated premature menarche
You suspect precocious puberty in an adolescent what would your approach to history and examination
are present
age, family history
CNS symptoms (central CNS causes e.g headaches, visual symptoms)
establish what secondary sex characteristics
growth chart
examine to look for signs of secondary sex characteristics - e.g. pubic hair, axillary hair, breast develop, testicular size, acne, facial hair in boys
height and weight - plot on growth chart
CNS exam + fundoscopy
testicular/pelvic exam
what investigations would you do investigate precocious puberty
- Sex steroids - early morning Testosterone (raised in early puberty), estradiol levels less reliable but if significantly raised suggestive of oestrogen secreting tumour
- Gonadotrophins - LH more useful than FSH
- raised LH>FSH suggestive of central cause ratio > 8
-normal levels with raised sex steroids suggestive of peripheral cause - Gonadotrophin stimulation test
(poor response/no response peripheral causes, raised LH and FSH in central cause) - TFTs - severe hypothyroidism cause of peripheral precocious puberty
- adrenal sex steroid precursor (if suspected CAH)
HCG if considering hcg secreting tumours
- Imaging
pelvic USS - look for ovarian cysts/ tumours
wrist x-ray- bone age
consider CT/MRI head dependent upon symptoms
what condition is caused by GNAS1 gene mutations and presents with cafe au lait spots, abnormal bone cysts and premature menarche?
McCune Albright syndrome (affects bones, skin and endocrine tissues)
sporadic mutation occurs post fertilisation not inherited from either parent
describe vulva pre-pubertal (pH, oestrogen state etc)
hypoestrogenic -thin, atrophic and underdeveloped labia
pH neutral due to lack of lactobacilli
urethra and vagina in close proximity with rectum increases risk of contamination
Daisy is 6 years old and mum has bought her to the GP due to complaining of soreness ‘down below’. On examination the there is erythema and inflammation in a nappy rash distribution. Daisy says it gets itchy at times.
What do you think is the diagnosis?
vulvovaginitis
describe the aetiology of vulvo-vaginitis which are group are most commonly affected
vulvovaginitis - peak age is 3-7 year incidence
very common;
80% of cases non-infective cause
often due to poor hygiene
how would you manage a child with vulvovaginitis
non infective management - strict hygiene
- wiping front to back
- avoid irritants e..g bubble baths/bath soaps, shampoos etc
- barrier cream
- avoid tight clothing and sleeping in pants at night
- daily soak in warm water for 10-15 mins
can lichen sclerosis present in paediatric population and how would you manage?
yes - 7-15% cases in paeds
manage as you would adults - potent steroids reducing regime e.g clobetasol propionate 0.05% dermovate OD for 4 weeks, alternate days for 4 weeks then twice weekly for four weeks before stopping
in combination with an emollient
what is the distribution of LS
bimodal - peaks in prepubertal females and post menopausal age groups
You examine Erin, she is aged 2 and mum has bought her to clinic as she is worried about the appearance of her labia. On examination there is a fine membrane extending from the posterior fourcette to the urethra. What do you think the diagnosis is and how could you treat if causing symptoms? what would you tell Mum?
labial adhesions
very common in age 3 months - 3 years due to hypoestrogenic state
often asymptomatic and will resolve spontaneously by puberty.
however is symptomatic (irritation/ urinary symptoms) can use topical oestrogen on end of a fine cotton bud - e.g. overton 1% or estradiol 0.01%) applied twice daily down the centre of the fusion with gentle pressure for 6 weeks
what diagnosis can be confused for labial adhesions and should be considered and ruled out
fgm
what causes vaginal hymen abnormalities
due to malformations during embryological development in the urogenital sinus
how might hymenal variants present clinically
difficulty inserting tampons
unable to have penetrative sex
dependent on the degree of hymen abnormalities can present with menstrual irregularities and malodous discharge (collection of vaginal secretions)
on TV USS the sonographer notices that the vagina is dilated with blood. What is the name of this radiological finding and what condition is most commonly associated with?
haematocolpos - due to imperforate hymen
- describes the dilation and filling of the vagina with blood often presents with primary amenorrhoea and cyclical pelvic pain
if haematocolpos is seen on TV USS and there is also distention of the uterus what is the term used to describe these findings
haematometrocolpos (‘metro’ addition)
Sophie is 15 years old and has developed normally secondary sex characteristics (breasts, axillary and pubic hair). She has come to your GUM clinic as she is worried she can’t insert tampons. She hasn’t yet started her periods but her GP said this was normal. She isn’t yet sexually active.
On PV examination you notice a blue bulging membrane when you apply pressure to the abdomen - what do you think is the diagnosis and what sign are you eliciting
Imperforate hymen (develops due to failure to obliterate the hymen during neonatal development, don’t know why it happens).
Crede manovre (pressure to lower abdomen causing bulging of blue thin membrane vaginally)- this is due to hamatocolpos (build up of blood vaginally)
can present with primary amenorrhoea and cyclical abdominal pain
what symptoms can someone with imperforate hymen present with
primary amenorrhoea, cyclical pelvic pain due to haematocolpos
urinary retention
constipation
pelvic mass - haematometra (build up of blood in the uterus)
Management of imperforate hymen
simple surgical procedure = hymenectomy
how can you differential vaginal septum and imperforate hymen
both can present with primary amenorrhoea, cylical pelvic pain but don’t get bulging membrane in vaginal septum like you do with imperforate hymen
what is the most common type of vaginal septum
high up in the vagina - transverse vaginal septum i.e. this blocks the passage fo menstrual blood so can present with cyclical pelvic pain, primary amenorrhoea etc..
can vaginal septum be transverse or longitudinal
yes - transervse due to failure vaginal plate and mullein ducts to fuse.
longitudinal often a/s with mullein abnormalities e.g. bicornate uterus
whilst investigating vaginal septum - what medication might you start
GnRH analogues to suppress symptoms whilst a/w MRI
surgical correction for Mx
what is the peak age for incidence of benign ovarian cysts in children?
1st year of life and around menarche - 12.8 avg
are the majority of ovarian cysts in paeds benign or malignant
majority are benign and will resolve, 10% malignant
what approach is taken in terms of management of simple cysts in adolescents <7cm
conservative approach, follow up with 3 monthly USS –> most resolve
(use this approach if < 7cm)
what is most common benign ovarian cysts in children?
A) haemorrhagic cyst
b) granulosa cell
c) dermoid cyst
d) functional cyst
d) functional cysts = 60% of all cysts
functional cysts occur if no egg is released from the follicle the fluid remains in the follicle and builds up to form a cyst
how might a haemorrhagic cyst present
PV bleeding mid cycle
what is the most common type of complex benign ovarian cyst in adolescence and how might it present?
A) haemorrhagic cyst
b) granulosa cell
c) dermoid cyst
d) functional cyst
c) dermoid cyst (type of germ cell tumour) can present with torsion (15% torsion, less than 1% malignant)
rare, germ cell tumour, gonadal dysgenesis in presence of a Y chromosome. Benign, but can evolve to malignant
A) haemorrhagic cyst
b) granulosa cell
c) dermoid cyst
d) functional cyst
e) gonadoblastoma
e) gonadoblastoma
what is the most common type of malignant ovarian cyst in paeds?
A) haemorrhagic cyst
b) granulosa cell
c) dermoid cyst
d) functional cyst
b) juvenile granulosa cell tumour
how might a juvenile granulosa cell present
peripheral precocious puberty (independent of HPO axis) due to oestrogen secretion from granulosa cell tumour- a/s with PV bleeding, darkening areolar, pubic hair growth, breast development i.e. secondary sex characteristics
what is another name for a dermoid cyst
mature cystic teratoma (contain, teeth, hair, sebum, blood, cartilage, bone etc)
if a paeds patient has an ovarian cyst that is < 7cm how would you manage
no need for bloods
simple cyst - f/u up in PAG (paeds adolescent gynaecology) with 3 monthly TA USS (or transrectal could be offered, avoid TV if not SA) - to ensure resolved cyst/ reducing in size or not growing… if growing may need to consider further Ix
Sally is 14 years old and is under PAG USS surveillance for a simple ovarian cyst which is measuring 8cm. Unfortunately it doesn’t appear to be resolving what should she be offered
either repeat TV USS in months or laparoscopic cystectomy with aim to preserve ovary.
(this applies to any simple cyst >7cm)
what investigations should be done in ovarian complex cysts presenting in paeds?
tumour markers
AFP - raised in yolk sac tumours ( endodermal sinus tumours another name)
HCG (raised in choriocarcinomas)
LDH
Ca-125
mri pelvis
when should surgery be considered on what size dermoid cyst and why
consider if >5cm due to risk of torsion
if suspecting haemorrhagic cyst how should you manage
repeat USS scan in 6-8 weeks to see if resolved, as should resolve over 2-3 cycles
if complex cyst and tumour markers negative, paeds patient symptomatic at what size do you consider operating?
> 5cm, laprascopic cystectomy preferred as reduced risk recurrence.
