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DR TAN THIAM CHYE Consultant Dept of Obstetrics and Gynaecology Assistant Professor Duke-NUS Graduate Medical School YLL School of Medicine, NUS
PRIMARY AMENORRHOEA
Definition Never experienced menstrual period by age 16
Primary amenorrhoea
Constitutional delay Similar history in mother or sisters
Primary ovarian failure Could be due to chemotherapy or radiotherapy Chromosomal disorders Developmental disorders
Swyer syndrome 46 XY karyotype Pure gonadal dysgenesis No androgens or Mullerian inhibitory factors Allows Mullerian duct to develop into fallopian tubes, uterus and upper vagina
Complete androgen insensitivity syndrome 46 XY karyotype Phenotypically female Tall with good breast development Normal female external genitalia Sparse pubic and axillary hair No uterus and upper vagina
Rokitansky-Kuster Hauser syndrome Failure of Mullerian duct development Congenital absence of uterus and upper vagina Normal 46 XX karyotype
Management
Refer OBGYN for investigations and management of primary amenorrhoea For constitutional delay, no treatment is needed except reassurance For chromosomal disorders and primary ovarian failure, small dose of ethinyl oestradiol 1g daily can be started for 6 months, increasing to 2, 5, 10 and eventually 20 g with increments at six monthly intervals. This is then followed by combined oral contraceptive pills. For vaginal and mullerian agenesis, vaginal reconstruction is necessary. This could be achieved by vaginal dilators or surgical procedures like Williams vulvo-vaginoplasty, McIndoes procedure or skin graft. For the XY female, counseling with the parents is important to discuss on psychological issues of gender of rearing and gender identity. Management includes gonadectomy as the dysgenetic testes have a high lifetime risk of malignancy (30%).
SECONDARY AMENORRHOEA
DEFINITION
Cessation of menses for 6 months
Hypothalamic dysfunction
Polycystic ovarian syndrome (PCOS) Menopause Premature ovarian failure < 40 years old
Evaluation: History
Weight loss or gain, Psychological dysfunction/ emotional stress Presence of galactorrhoea Symptoms of thyroid disorder Hirsutism, change of voice, Menopausal symptoms Family history of possible genetic anomalies History of uterine/cervical surgery/ medication Sexual/contraceptive practice
Investigations:
1. Urine pregnancy test to exclude pregnancy 2. Follicular stimulating hormone (FSH) on Day 2-3 of menses
Suggests ovarian failure if > 30 IU/L Low level suggests hypothalamic/ pituitary dysfunction Reversal of LH/FSH ratio > 3:1 suggests PCOS
INVESTIGATIONS
Perform urine pregnancy test to exclude pregnancy related problems (threatened miscarriage, inevitable miscarriage or ectopic pregnancy) If there are bleeding tendencies, exclude blood dyscrasias for adolescents (13-18 years old) If there are symptoms of thyroid disorder or galactorrhoea, check thyroid function test or prolactin level Perform pelvic ultrasound scan to exclude pelvic pathology (fibroids/ adenomyosis) Perform endometrial assessment (if 40 years old or failed conventional medical treatment) to exclude endometrial hyperplasia or carcinoma
Goals of R x
Alleviate acute bleeding. Give IM progesterone 100 mg stat Prevent future episodes of noncyclic bleeding aim to give a bleed which is predictable in timing and amount Decrease risk of long term complications (eg development of endometrial cancer)
Common medical treatments Cyclic progestogen (at least 10-14 days per cycle) NSAIDs (eg mefenamic acid 500mg tds)
Norethisterone 5-10mg bd Provera 10mg bd
Re r OBGYN for endometrial assessm and further treatment if failed fe ent conventional initial treatm or age 40 and above ent
OOther hormonal medical treatments MIRENA/ Levonorgestrel (LNG)-releasing Intrauterine System Releases 20ug of LNG daily which affects the endometrium locally Lasts 5 years Low local hormonal effect in the endometrium which provides shorter and lighter menses and reduces dysmenorrhea 20% amenorrhoea after 1 year 20% intermittent per-vaginal spotting in 1st 6 months Lower risks of pelvic inflammatory disease and ectopic pregnancy compared with copper-IUCD
PROGYLUTON
Cyclical sequential combined hormonal replacement therapy with 2mg estradiol valerate for first 11 days and 2mg estradiol valerate with 0.5mg norgestrel for next 10 days Regulates menstrual cycle and does not affect endogenous hormone production Does not interfere with ovulation Can be used by pre- and peri- menopausal patients
Depot Provera
Intramuscular Depot Provera 150mg every 3 monthly Induce endometrial atrophy and amenorrhoea Irregular bleeding in first 3 months Side-effects: abdominal bloating, breast tenderness, weight gain, depression, water retention
Danazol
Induce amenorrhoea in majority if taken in moderate/ high dose (> 400 mg daily) If taken at low dose (200 - 400 mg daily) it will induce amenorrhoea in some while others may experience light but often unpredictable bleed Masculinising side-effects such as hirsutism, acne, voice change (irreversible)
Surgical Management
Endometrial ablation
Hysterectomy
Positive
Negativee
Positive
Negative
Bleeding 6 months
Case 1
LMP: Cannot remember Clinically stable parameters UPT positive POC seen at cervical os
Case 1
Diagnosis: Complete miscarriage Conservative management Oral augmentin x 1 week
Case 2
27 years old. 1 X LSCS for CPD (2002). 1 X m/c (2003) evacuation done. LMP 31/12/05 & p/v bleeding since then. Was treated as DUB on 23/01/06 in OPD. NE & Folic acid was given. Fybogel & enema x1. Presented to 24 hrs clinic on 24 Jan2006 at 1.10am.
Presenting Complaints
Prolonged PV bleeding
Since 31/12/05. 2 pads / day No signs of anemia.
UPT +
Initial Assessment
In 24 hour Clinic
1. 2. HR 97 bpm; BP 95/63, Pain score 5/10 Abdomen soft but guarded in LIF; With lower abdominal fullness. No rebound. 3. VE left adnexal tenderness; No cervical excitation.
Urgent DI Scan
Mass at left adnexa, adjacent to left ovary (visualised) ring like, vascular. ? Ectopic Pregnancy Hemoperitoneum Empty uterus except for blood clots
In the Ward
Hb 13.4g/dl; BHCG 387.8IU/L. Patient and husband counseled on the high possibility of an ectopic pregnancy Agreeable for Laparoscopic salpingectomy KIV cystectomy KIV evacuation of uterus/ KIV laparotomy. Risks of op explained.
Operative findings
Haemoperitoneum 200 mls Ruptured left tubal ectopic pregnancy 4cm MIS Left salpingectomy done Left ovary normal,right tube normal