Paeds And Adolescent Gynae

Question 1

ASRM Classification

Answer 1

Segmental Hypoplasia or agenesis: Type I
Unification defect:
- type II- unicornuate
- type III- didelphus
- type IV- bicornuate
Canalisation defect:
- type V- septate
- type VI- arcuate
DES- exposure in utero: type VII

Question 2

Reproductive implications of unification defects of female genital tract

Answer 2

No effect on fertility
Increased PTB
Fetal malpresentation

Increased first trimester miscarriage with unicornuate and bicornuate uterus only

Question 3

Reproductive implications of septate uterus

Answer 3

The worst reproductive outcomes out of all genital tract anomalies

Reduced conception rate
Increased first trimester miscarriage
PTB
Fetal malpresentation

Question 4

Management of septate uterus

Answer 4

NICE guideline recommend offer women with history of recurrent miscarriages hysteroscopic metroplasty.

Pre- op GnRH agonist for endometrial suppression may improve visualisation but insufficient evidence, therefore just aim to perform procedure in early follicular phase

Question 5

Causes of Disorders of Sex Development

Answer 5

CAH

Partial and Complete Androgen insensitivity (PAIS and CAIS)

5- alpha reductase deficiency- enzyme that converts testosterone to dihydrotestosterone which is required for fetal genital virilization

Question 6

What is puberty

Answer 6

The maturation of secondary sexual characteristics and the onset of menstruation in females.

It is triggered by changes in the HPO axis and by the effect that GH has on insulin production.

The rise in insulin causes a drop in SHBG with higher levels of free sex steroids in the blood

Question 7

Stages of Pubertal Development

Answer 7

Thelarche
Adrenarche
Growth spurt
Menarche

Question 8

What is precocious puberty

Answer 8

Menstruation before 9 years of pubertal changes before 8 years

Question 9

Definition of true precocious puberty

Answer 9

Also known as central or gonadotrophin- dependent

80% of cases

Early activation of the HPO axis

Causes:
- brain tumour
- CNS malformations
- idiopathic

Not associated with premature menopause.

Most serious effect is adult short stature due to early epiphyseal fusion

Question 10

Definition of pseudopuberty

Answer 10

Also known as peripheral or gonadotrophin- independent precocious puberty

20% of cases

Due to extra- pituitary secretion of oestrogen/ androgens independent of HPO stimulation.

Causes:
- CAH
- chronic hypothyroidism- high TSH acts as FSH
- hormone- producing ovarian tumours
- exogenous administration of oestrogen
- McCune- Albright Syndrome

These tumours can also secrete HCG in addition to oestrogen and androgens

Question 11

What is a GnRH stimulation test

Answer 11

Gold standard to diagnose true precocious puberty.

GnRH is administered and LH levels measured.
High LH (>5)= true precocious puberty

Question 12

Management of true precocious puberty

Answer 12

Manage distress caused by bodily changes
Main aim is to slow growth velocity and avoid early skeletal maturation
Give GnRH analogues (Zoladex)
- suppresses LH and FSH, stopping and reversing the physical and psychosocial effects of puberty
- continue treatment until more appropriate age (10 or 11). Once withdrawn, puberty will progress normally until menarche within 12-18 months

Monitoring of patients:
- 3-6 monthly growth assessment + pubertal staging
- annual bone age x- Ray (left wrist)
- measure GnRH levels 1h after injection

Question 13

Management of peripheral precocious puberty

Answer 13

Surgical resection of tumours if present

McCune- Albright syndrome:
- block oestrogen biosynthesis or block oestrogen action
- letrozole (aromatase inhibitor)

CAH- treat with glucocorticoids

Follow-up:
- 3-6 monthly clinical assessment of height, weight, pubertal stage
- annual bone age x- ray
- stop treatment at normal age of puberty, menarche will resume in 12-18 months