Paediatric Gynaecology

Paediatric Gynaecology Re-activation of hypothalamic-pituitary-ovarian axis (previously suppressed by low levels of gonadal steroids) Sleep related pulsatile release of gonadotrophins à all day pulsatile release à production of ovarian oestrogen à physical changes of puberty à adolescent growth spurt (early sign) à Thelarche - subareolar breast bud appears (Tanners stages) due to adrenal oestrogens à Pubarche - public and axillary hair due to adrenal maturation à Menarche average 12.8 ys (80% reach menarche by bone age of 13-14yrs) often FH Body Weight 48kg is “critical body weight” – problems / delay linked to • malnutrition (inc anorexia & bullemia) • slow growth before and after birth • twins (compared to singletons of same population) • athletic training (à increased muscle, decreased fat) Delayed Puberty Delayed Puberty - Absence of physical manifestations of puberty by age 13 Primary Amenorrhoea – no menstruation by 14 yrs + failure of 2ndry sexual characteristics Or no menstruation by 16 yrs with normal 2ndry sexual characteristics Constitutional delay – short stature (although appropriate for bone age), prepubertal levels of gonadotrophins, low gonadal steroids, normal karyotype. Will enter puberty on reaching bone age 1113 Hypogonadotrophic Hypogonadism – defect in GnRH à no FSH & LH. Linked with conditions affecting body weight (malnutrition, anorexia), CNS tumours (eg craniopharygioma), Kallmans syndrome Primary gonadal failure (Hypergonadotrophic Hypogonadism) – eg turners syndrome Investigations – plasma FSH, LH, oestrodiol, prolactin, TFTs, karyotype, X-ray for bone age, cranial CT/MRI Treatment – replacement of relevant hormones (may require pulsatile release via infusion pump) Precocious Puberty Precocious Puberty - Appearance of signs of sexual maturation prior to 8yrs or menache <10yrs Constitutional – 80% of cases, changes occur in normal sequence. Investigate before accepting blood loss as menses (check foreign body, neoplasm, trauma etc) Intracranial lesions – from encephalitis, meningitis, hydrocephaly or space occupying lesion Feminising tumours – of ovary or adrenal à vaginal bleeding without signs of pubertal development Investigations - plasma FSH, LH, oestrodiol, prolactin, TFTs, karyotype, X-ray for bone age & of long bones (McCune Albright syndrome), cranial CT/MRI, US of abdomen & pelvis Treatment – aim to arrest / induce regression of signs of puberty inc menstruation & to avert rapid advance in bone age (to prevent premature fusion of epiphyses). Include GnRH agonists to suppress gonadotrophin secretion. Abnormal genital tract development Horizontal vaginal septae – cyclical pain & haematocolpos. Requires surgical correction Vertical vaginal septae – dyspareunia, infertility or problems in labour Vaginal atresia – with absent or rudimentary uterus (Rokitanksy syndrome). Presents with amenorrhoea, with normal 2ndry sexual characteristics Unicornate uterus – higher miscarriage risk of preterm labour Bicornate uterus – can be corrected by “strassman procedure”