Paediatric gynaecology and pelvic pain Flashcards
Discuss ambiguous genitalia
-Incidence
-Causes - 3 groups
- Incidence: 1:4000. 50% secondary to CAH
- Causes
Virilization of female karyotype (46XX)
-CAH most common
-Exposure to maternal androgens
-Placental aromatase deficiency
Under masculinisation of male karyotype 46XY
-Partial gonad dysgenesis - Swyers
-Complete or partial androgen insensitivity syndrome
-Defect in testosterone biosynthesis
-5-alpha reductase deficiency -converts testosterone to DHT (Important for peripheral virilization)
Mosacism of XX and XY - true hermaphrodism - 20% of DSD
What investigations should be done to investigate disorders of sex development (ambiguous genitalia)
-4 groups
- FISH and Karyotype
- Bloods
-Serum 17 hydroxyprogesterone levels (CAH)
-Urea and electrolytes (CAH with salt wasting)
-Synacthen test
-Basal GnRH FSH and LH
-Androgen levels - basal and HCG stimulated (Functioning sertoli cells produce testosterone with HCG stimulation)
-AMH - low in dysgenic gonads - USS - check for uterus and ovaries
- EUA to assess morphology of urogenital sinus
How should disorders of sex development (Ambiguous genitalia) be managed?
-Who should be involved
-What are the most important first steps
-What surgery should be considered
- Do not guess genitalia at birth
- MDM approach should be taken lead by paeds endocrinologist/ urologist/psychologist
- 50% of DSD caused by CAH. Needs to assess and correct salt wasting
- Do full work up for causes and then discuss with parents
- Consider gonadectomy when
-non-virilized 46XY assigned to female. PAIS - 30% chance of malignancy - do straight away. CAIS 3% chance can wait. - Consider feminising surgery with child input
Discuss Congenital Adrenal Hyperplasia
-Incidence
-Pathophysiology
-Presentation
-Management
- Incidence
-Most common DSD 50%
-1:10,000 - Pathophysiology
-Caused by 21 hydroxylase deficiency in 90% of cases
-Caused by shunting steroid production towards testosterone resulting in reduced costisol and aldosterone - Presentation
-Diagnosis is high levels of 17-OH hydroxyprogesterone. If equivocal then synachten test can be done
-Causes varying degrees on virilisation due to androgen excess
-May only be picked up at puberty. Hard to distinguish from PCOS - Management
-Hydrocortisone
-Manage salt wasting early
Discuss androgen insensitivity
-Types
-Incidence
-Pathophysiology
-Presentation
-Management
- Incidence - 1:40-60,000 CAIS>PAIS
- Complete androgen insensitivity and partial androgen insensitivity
- Pathophysiology
-x-linked inheritance
-Normal testicular function but abnormal androgen receptors
-Testes produce AMH causing regression of Mullarian structures so absent fallopian tubes, uterus, and upper 2/3rds of vagina. - Presentation
CAIS - normal female appearance but short vagina. Picked up with primary amenorrhoea usually
PAIS - Ambiguous genitalia with varying virilisation
Dx with rise in testosterone after HCG stimulation test - Management
-Disclose 46XY karyotype and implications for reproduction and menstruation (can’t)
-CAIS - 3% chance of gonads causing malignancy - consider removing after puberty.
-Estrogen replacement, vaginal expansion or dilators
-PAIS - 30% chance of malignancy - remove immediately
Discuss 5 alpha reductase deficiency
-Incidence
-Pathophysiology
-Presentation
-Management
- Rare
- Caused by lack of enzyme which converts testosterone to DHT (dihydrotestosterone).
DHT important for peripheral virlisation - Presentation
Ambiguous or female genitalia. Virlisation seen at puberty
Dx: Normal testosterone, decreased DHT - Gondectomy at puberty if raised female to avoid virilisation
Discuss the embryology of the genital tract
- The mullerian ducts for the female genitalia
- Production of testosterone + AMH from XY fetus suppresses mullerian duct formation
- Where testosterone + AMH is absent the female genitalia develop
- The mullerian ducts form the fallopian tubes uterus and upper vagina
- The external genitalia develop from the genital tubercule -clitoris, labioscrotal swelling - labia majora and urogenital folds - lower vagina
What are the Fraser Guidelines for competency of minors
-5 guidelines
- That she understands the advice
- That the health care professional cannot persuade her to inform her parents or are not allowed to inform them
- That she is already having sex or will start and do so without contraception if not provided
- That her physical +/- mental health is likely to suffer without contraception
- That it is in the best interests of the girl to have contraception without parental consent
Discuss congenital anomalies of the genital tract
-Incidence (4)
-Pathophysiology
-Classification
- Incidence
-5% in unselected population
-8% in infertile women
-13% in women with miscarriage
-25% in women with infertility and miscarriage - Defect in Mullerian duct embryology
- Classification
-ASRM classification system
I - Hypoplasia/Agenesis
II-IV - Unification defects
V-VI - Canalisation defects
VII - DES defects
Describe the ASRM classification of uterine congenital abnormalities
-7 types
-Definitions
- Bicorunate - external fundal indentation>1cm
- Didelphys - different levels of fusion.
- Septate - septum >1.5cm and <90 degrees (T shaped)
- Arcuate - septum <1cm and angle of indentation>90 degrees, big scoop
How does congenital genital abnormalities present (7)
- Primary amenorrhoea
- +/- cyclical abdo pain
-Seen with imperforate hymen, transvaginal septum, cervical agenesis, uterine agenesis - Dysmenorrhoea
-Seen with non-communicating or obstructed horn - Dysparenunia
-Seen with transverse septum - Infertility
- Miscarriage
- Obstetric complications
- High rates of endometriosis - increased rate of retrograde menstruation
How should congenital genital tract abnormalities be investigated (5)
-Screening
-Dx
- USS or HSG for screening
- 3D TVUSS - diagnosis
- MRI - diagnosis
- Laparoscopy / hysteroscopy - traditional dx method
- Image kidneys as 50% in Type I+II have renal abnormalities
What are the reproductive implications for congenital genital anomalies and mechanisms?
-Antenatal (4)
-Intrapartum (2)
-Postpartum (1)
- Antenatal
-Infertility, miscarriage, IUGR, PET/BP
-Due to abnormal endometrium / placental insertion site / poor/disordered blood supply - Intrapartum
-Labour dystocia
-Increased risk for CS - Postpartum
-PPH - due to incordinate uterine contractions + retained placenta
Discuss reproductive implications for congenital uterine anomalies by type
-Canalisation defects (5)
-Unification defects -Bicornuate and unicornuate (4)
-Unification defects - didelphys (3)
- Canalisation defects - septate and subseptate
-Worst reproductive outcomes
-Reduced conception rate OR 0.86
-Increased first T miscarriage OR 2.9
-Preterm birth - OR 2.14
-Fetal malpresentation - OR 6.14 - Unification defects - bicornuate and unicornuate
-No effect on fertility
-Increased first T miscarriage OR 3.4
-Increased preterm birth - OR 2.55
-Fetal malpresentation - 5.89 - Unification defects - didelphys
-No effect on fertility
-Increased preterm birth - OR 3.58
-Fetal malpresentation - OR 3.7
Discuss the management of congenital genital tract anomalies
-Imperforate hymen
-Vaginal septum
-Cervical agenesis
-Non-communicating horn with functional endometrium
-Bicornuate or didelphic uterus
-Septate or subseptate uterus
- Surgical resection of hymen
- Surgical resection of septum
- Anastamosis of uterus to vagina laparoscopically
- Excision of horn (open or laparoscopically)
- Strassman procedure - abdominal metroplasy - doesn’t improve reproductive outcomes
- Hysteroscopic resection of septum
- unclear if improves reproductive outcomes
-Do in early follicular phase for visualisation
Discuss Mayer-Rokitansky-Kuster-Hauser syndrome
-What it is
-Incidence
-Pathophysiology
-Presentation (4)
-Management (5)
- Absent or rudimentary uterus or bilateral rudimentary horns on either pelvic side wall, absence of upper 2/3rd of vagina.
- Incidence: 1: 4-6,000
- Agenesis or hypoplasia or Mullerian ducts. Multifactorial
- Presentation
-Primary amenorrhoea 70%
-Short vagina
-Normal other secondary sexual characteristics
-Associated with renal, auditory, cardiac and skeletal anomalies - Management
-Pyschological - sterile
-Surrogacy for fertility
-Enlarge vagina with dilators alone or post surgery
-Enlarge vagina with surgery
-McIndoe-Reed - vagina from buttock grafts
-Bowel Vaginoplasy - made from sigmoid
-Davydov - Lined with pelvic peritoneum
-Vecchitti - synthetic olive placed on tension
-Investigation and management of associated anomalies
Discuss precocious puberty
-Definition
-Incidence
- Definition
-Onset of pubertal development before age 8
-Onset of menarche before age 9 - Incidence
-0.2-2%
-Incidence is ethnicity dependent
-Female to male ration 5:1
-80% central causes; 20% peripheral causes
Discuss the causes of precocious puberty
1. Central causes
2. Peripheral causes
- Central causes - true precocious puberty - 80%
-Gonadotrophic dependant
-80% idiopathic
-Brain tumours / cysts / trauma / congenital / hydrocephalus / radiation / Infiltrative
-Specific gene mutations - Peripheral causes
-Exposure to exogenous sex steroids
-Chronic primary hypothyroidism
-Ovarian tumour - 2-5% (Mostly Granulosa cell tumours)
-Feminising adrenal tumours
-McCune Albright syndrome
-G protein mutation leading to continual activation despite absence of hormonal stimulation.
-Associated with cafe au lait spots and fibrous dysplasia
Discuss puberty
-Definition
-Timing of onset
-Order of anatomical changes
-Pathophysiology
- Definition
-Time between childhood and adulthood when sexual and reproductive maturation occurs - Average age of onset ethnicity dependent
-12.3 Blacks, 12.8 Whites
-Normal 8-12 yrs - Boobs, pubes, spirt, squirt
-Thelarche - 8-13
-Adrenarche - 3 months after Thelarche
-Growth - 9.5-14.5 yrs
-Menarche 4th by 2-3 yrs post thelarche - Pathophysiology
-GnRH, FSH and LH low and HPO axis quiescent
-Increase in pulsitile GnRH in frequnecy and amplitude.
-Initially noctunal pulsations only
-Increase GnRH leads to increase FSH and LH
-Increase LH and FSH leads to steroidogenesis and folliculogenesis leads to increased estrogen
-Increased estrogen leads to thelarche
-GnRH pulsations during the day allow for ovulation and menarche
Discuss the management for precocious puberty
1. Who should manage
2. Central precocious puberty
3. Peripheral precocious puberty
- Treatment should be by paediatric endocrinologist
- Central precocious puberty
-GnRH analogues - zoladex / Lucrin
-Stops or reverses puberty
-Continue until 10 or 11.
-Give psychological support
-Monitor yearly, growth, bine maturation - Peripheral causes
-Surgical tumour resection
-McCune Albright syndrome
-block action or biosynthesis of estrogen (Letrozole)
-Remove exposure to exogenous sex steroids
-Monitor growth and bone age annually
-Stop treatment at normal age for puberty
Discuss delayed puberty
-Definition
-Incidence
-Causes - 2 groups
- Definition
-Absence of secondary sexual characteristics by 13yrs
-No menstruation by 16yrs or 3 yrs after breast development - Incidence - 2.5% of population
- Causes:
Hypogonadotrophic hypogonadism - No GnRH to stimulate FSH and LH
-Constitutional delay (Esp if fam Hx)
-Chronic illness
-Functional - excess exercise, anorexia, stress
-Kallmans Syndrome
-Hydrocephalus, CNS tumour, infiltrative disease, pituitary adenoma, panhypo
Hypergonadotrophic hypogonadism - Non functional gonads
-POI
-Turners syndrome
-Swyers syndrome
-Premature ovarian failure
-Chemotherapy / radiotherapy
-Autoimmune / infection / Toxins
-Gonadal agenesis
Discuss the management of delayed puberty (8)
- Administer small doses of oestrogen with increasing amount until breast development complete / break through bleeding. Small doses doesn’t imped growth
- Add in progesterone (cyclical) - can use COCP
- If Hypogonadotrophic hypogonadism can use pulsatile GnRH
- In those with gonadal failure need estrogen and progesterone for bone and heart protection
- If patient has Y chromosome consider gonadectomy given malignancy risk
- Fertility requires oocyte donation
- Psychological support
- Improve nutritional intake and mineral supplementation
Discuss Kallman’s syndrome
1. Incidence
2. Pathophysiology
3. Presentation
- 1: 7500 females 7:1 ratio F:M
- Pathophysiology
-Dysgensis of olfactory bulbs and GnRH neurons
-X - linked
-Mutation in Kal-1 gene - Presentation
-Anosmia and delayed puberty
Discuss Turners Syndrome
-Incidence
-Pathophysiology
-Presentation
-Management
- 1:2500
- Pathophysiology
-45 XO female phenotype - Presentation
-Antenatal - cystic hygroma, IUGR, non0immune hydrops
-Short stature, neck fold, low hair line, wide space nipples
-Aortic coarctation, renal abnormalities, rudimentary ovaries
-Delayed puberty (Menopausal level at puberty), insulin resistance, hypothyroid - Management
-Growth hormone for height
-Induction of puberty with estradiol (Peripheral cause)
-Long term HRT for heart and bone health
-Fertility with egg donation
-Breast implants if concerned
Discuss Swyers syndrome
-Incidence
-Pathophysiology
-Presentation
-Management
- Rare
- Pathophysiology
-46XY with genetic mutation in SRY gene = no AMH or testosterone = female genitalia but gonadal dysgenesis
-Uterus and cervix small as no estrogen from gonads - Presentation:
-Primary amenorrhoea
-Small undeveloped breasts and sparce pubic hair (some androgens from adrenals) - Management:
-Induction of puberty
-Gonadectomy as risk of malignancy 30%
-Long term HRT
-Child bearing with egg donation
Discuss genital tract tumours in adolescence
-Presentation (5)
-Risk factor (1)
- Presentation:
-Vaginal discharge
-Vaginal bleeding (esp malignant)
-Abdominal mass (pelvis not developed)
-Urinary symptoms
-Premature sexual development if hormone secreting - Risk factors:
-DES exposure in utero for clear cell of CX and vagina
Discuss the types of genital tract tumours seen in adolescence
1. Ovary
2. Uterus
3. Lower genital tract
- Ovary - 1% of all childhood tumour. 80% benign
Germ cell tumours - most common
-Teratoma benign and immature - malignant
- Dysgerminoma
- Endodermal sinus tumour
- Embryonal carcinoma
- Choriocarcinoma
Epithelial
-Clear cell - Uterus - no tumours recorded
- Lower genital tract
Benign
-Gartners cyst (mesonephric duct cyst
-Teratomas
-Haemangiomas
-Hymenal cysts
-Benign granulomas of perineum
-Condylomata acuminata
Malignant
-Botryoid sarcoma - most common if <3yrs
-Endodermal carcinoma
-Mesonephric carcinoma
-Clear cell carcinoma
Discuss management of tumours of the genital tract in adolescence
-Benign lower genital tract
-Benign ovarian
-Malignant ovarian
-Malignant vulval
- Benign lower genital tract
-If Asx treatment not required - Benign ovarian - aim for fertility sparing
-cystectomy.
-Do frozen section if unsure of dx - Malignant ovarian
-Germ cell - USO +/- Chemo
-Epithelial - usually need TH + BSO - Malignant vulva
-Surgical resection if possible
-Chemo
Discuss vulvovaginitis in prepubertal girls
-Incidence
-Causes (4)
-Risk factors (4)
-Presentation (5)
-Management (4)
- Most common gynae problem in young girls
- Causes:
-Infection - Most common mixed flora then GAS or haemophilis influenza. Thread worm possible. Candidiasis rare.
-Forgein bodies
-Dermatological conditions - contact dermatitis, lichen sclerosis, eczema
-Non-specific irritants - bubble bath etc - Increased risk in prepubesent girls because:
-Thin vaginal mucosa
-Alkaline pH
-Smaller vulval fat pads and no pubic hair
-Poor hygiene and proximity of vagina to anus - Presentation
-90% vaginal discharge
-Itch
-Discomfort - 80%
-Dysuria
-Bleeding = rare - think trauma/ cancer/ precocious puberty - Management
-Antibiotics if swab +
-Hygiene advice
-Avoid irritants
-Anti-helminths if suspected
Discuss labial adhesions
-Definition
-Incidence
-Pathophysiology
-Risk factors (2)
-Presentation (5)
-Management (3)
- Adhesions between labia minora
- Incidence - 1-3% of pre pubertal girls. Peak incidence 1-2yrs
- Pathophysiology unknown but likely raw thin labia agglutinate
- Risk factors: Nappies, poor hygiene
- Presentation
-ASx if minor
-If labia fused - dysuria, postmicturition dribble, recurrent UTI, retention - Management
-If minor and asx no treatment required. Spont resolution at puberty
-Topical oestrogen cream for max 6/52
-Surgical separation if recalcitrant to estrogen. Often reform
Discuss adolescent sexual assault
-Legal requirements
-How to approach disclosure of sexual assault
-Hx
-Exam
-Ix
-Management
-FU
- No legal requirement for mandatory reporting
- Approach
-Acknowledge and affirm disclosure
-Act on any injury that requires immediate attention - PVB/Trauma
-Maintain confidentiality/ Gain consent to proceed/ Assess competence
-Offer support person
-Offer MEDSAC - if unsure they can do forensic exam and decide later
-If they decide yes for MEDSAC then:
-NBM, Don’t pee/shower/keep same clothes.
-If they decide no to MEDSAC then do Hx/Ex/Ix - History:
-Event info
-What they have done since, brushed teeth, shower etc
-Risk of pregnancy (menstrual hx, contraception)
-STI
-Social supports and safety
-General Hx as per usual - Exam:
-Top to toe
-Speculum not required unless PVB / pain of if cx specimen required - Investigations
-STI screen swabs and bloods
-Blood and urine toxicology - Management
-Manage physical injuries
-Treat STI’s
-Given HIV prophylaxis if high risk
-ECP if required
-ACC sensitive claims - FU
-Call in a week with results +/- treatment
-3 weeks in person check in, examine injuries, PT, counselling
-3 months - HIV and Hep B repeat
Discuss primary amenorrhoea
-Definition
-When to investigate (3)
-Incidence
- Def: Absence of menstruation
- When to investigate:
-No secondary sexual characteristics by age 13
-Secondary sexual characteristics but no menstruation at 15yrs
-No menstruation 3yrs after breast development - Incidence 0.1-0.3% (Much less common than secondary amenorrhoea)
What are the causes of primary amenorrhoea with % makeup (5)
- Pituitary driven (Hypogonadotrophic hypogonadism) - 35%
-Constitutional delay, chronic disease, Kallmans, Infection, Infiltration, Rad/Chemo, Tumour, Functional - Ovarian driven (Hypergonadotrophic hypogonadism) - 40%
-POI, Turners, Swyers, Pure gonadal dysgenesis, Galactosaemia, Fragile X premutation, CAH, Aromatase deficiency, Autoimmune, Chemo/Rad/Surg, Infection - Reproductive tract - 20%
-Absent uterus - MRKH, CAIS
-Obstructive - imperforate hymen, vaginal septum, cervical agenesis - Thyroid
-Hyper / hypo thyroid - Hyperandrogenism
-PCOS
-HAIR-AN (Hyperandrogenism, insulin resistance, acanthosis niagracans)
-Ovarian tumour
Discuss dysfunctional uterine bleeding in adolescence
-Definition
-Aetiology
-Management
- Definition: Heavy and irregular bleeding
- Aetiology
-85% DUB is due to anovulation cycles from an immature HPO axis
-Infection
-Endocrine abnormalities - Thyroid, prolactin
-Stress
-Bleeding disorders
-Rarely structural abnormalities - polyps, fibroids etc - Management
-Correct anaemia
-NSAIDs, TXA, COCP
Discuss primary dysmenorrhoea
1. Definition
2. Incidence
3. Presentation
4. Pathophysiology
5. Management
- Definition: Pain during menstruation
- Incidence: Up to 60%
- Presentation
-Pain typically begins 6-12 months post menarche when ovulatory cycles begin
-Pain typically starts before or during menstruation and lasts up to 72hrs. - Pathophysiology: Decrease in progesterone ->Increased prostaglandins -> Increased uterine contractility ->Myometrial ischemia and pain.
- Management
-NSAIDS to interrupt COX mediated prostaglandin production
-Start 2 days prior to menses and for 2-3 days during. Decreases blood flow by 30%
-COCP to inhibit ovulation
-If no response to therapy consider secondary dysmenorrhoea work up
Discuss endometriosis in adolescents
-When to suspect
-Incidence
-Diagnosis / imaging
-When to treat surgically
-Treatment - medically
-Appearance
- Suspect if chronic pelvic pain not responsive to medical management, Family Hx, Hx of congenital genital tract abnormalities, cyclical absenteeism
- 66% of adolescence undergoing laparoscopy for chronic pelvic pain will have endometriosis
- Diagnosis
-TVUSS if appropriate and if not TAUSS or MRI - Consider risk and benefits of surgery as a shared decision
Consider surgery if no improvement with medical treatment - Treatment medically
-NSAIDS
-Hormones - consider bone density effect
-GnRH agonists up to 1 yr with add back if Lap confirmed endo - Adolescent endometriosis = clear or red not powder lesions. Tend to be more metabolically active with increased prostaglandins and inflammation.
Discuss adenomyosis
-Histology
-Definition
-Subtypes
-Pathophysiology of symptoms
-Co-existing pathology
-Incidence
- Endometrial glands and stroma surrounded by hypertrophic and hyperplastic myometrium. lack of submucosa endometrial/myometrial interface
-Estrogen dependant. Not seen in PM - > 2.5mm of glandular extension below the endometrial/myometrial interface
- Subtypes:
-Focal - adenomyoma
-Diffuse - more common - Pathophysiology of symptoms
-HMB (60%) from increased vascularity of uterus, increased endometrial surface area and impaired myometrial contractility
-Pain (25%) from trapped endometrial cells in myometrium during menstruation leading to myometrial hypertrophy - Fibroids, Endometriosis 30%
- Incidence
-20-35% of women
What imaging should be used for adenomyosis and what are the USS findings (7)
- Imagine
-USS or MRI - Similar specificity / sensitivity (USS 87% and 81%)
-MRI not first line. - USS findings
-No set definitions
-Asymetrical wall thickening
-Venitian blinds
-Myometrial cysts
-Disrupted myometrial/endometrial junction
-Subendometrial lines or thickening
-Focal hyperechoic lesions
-Increased vascularity on doppler
How should adenomyosis be managed
-Medical (3)
-Surgical (4)
- Medical management
-Limited evidence. Hormonal seem to work (None superior)
-GnRH decreases uterine size
-Mirena effective for HMB - Surgical intervention
-Surgical resection if focal lesion - fertility sparing (needs CS)
-Endometrial ablation if disease is superficial - No evidence
-Hysterectomy - no evidence that it will improve pain but will impact HMB
-High intensity focused USS. Non-invasive and fertility sparing
-Image guided thermal ablation - targeted ablation of adenomyosis tissue
Discus ovarian torsion
1. Epidemiology
2. Pathophysiology
3. Risk factors(5)
4. USS findings (6)
5. Management principles
- Epidemiology
-Mostly occurs in women of reproductive age
-25% occurs in children
-Dermoids most common type of cyst (10% tort) - Pathophysiology
-Rotation around the IP and utero-ovarian ligament
-Initially impacts venous drainage
-Ovary becomes oedematous increasing vascular compression
-Follicles are pushed to periphery secondary to oedema - Risk factors
-High risk of R ovary torsion
-Pelvic mass (85% of torsions have a mass)
-Mass >5cm
-Pregnancy
-PCOS or ovulation induction - USS findings
-Enlarged ovary
-Adnexal mass
-Peripheral multiple follicles - string of pearls
-Ovary anterior to uterus
-Decreased or absent blood flow (not always)
-Whirl pool sign (twisting of thickened vascular pedicle - Management principles
-Leave ovary even if dusky unless
-Obviously necrotic tissue
-Concern for malignancy
- Post menopausal - stop recurrency
-Prompt surgical intervention in pre-menopausal
-Prevention of recurrence - ovulation suppression, oopherpexy (no high qual evidence)
Discuss the complications of ovarian cysts (4)
- Torsion
- Rupture
-usually functional
-Occurs day 20-26 of cycle usually - Haemorrhage
-Usually CL or theca interna as vascular
-Usually occurs day 20-26 of cycle - Infection
-1% of dermoids can become infected - coliforms mostly
Discuss the management of benign cysts in premenopausal women
-Asymptomatic simple cysts
-Symptomatic or large cysts
- Simple asx cysts
- <5cm no follow up
- 5-7cm annual FU with USS, If persistent or increasing in size consider surgical management
- >7cm consider surgical intervention or further imaging - MRI - Symptomatic cysts, ?dermoid / borderline / malignancy, >7cm cysts should be considered for surgery
-Laparoscopic approach gold standard
-Avoid aspiration - spillage and high recurrence rates - 54-84%
Discuss ovarian cysts in pregnancy
1. Epidemiology
2. Complications
3. Tumour markers
4. Surgical principles (7)
5. FU
- Epidemiology
- 1:6 masses in pregnancy malignant
-0.05 - 2.5%
- <5% of ovarian cysts in pregnancy require intervention
-50% dermoid
-Functional cysts - typically resolve by 16 weeks - Complications
-Torsion esp in first trimester. Occurs in 5% - Ca125, AFP and HCG all raised in pregnancy so not helpful
- Surgical principles
-Ideally between 16-20 weeks
-Laparoscopy with Hassan entry (Avoid veres)
-Left lateral if >20 weeks
-Max pressure 13mmHg
-Fetal heart monitoring
-Consider steroids
-VTE prophylaxis - If cyst >5cm or complex components FU with USS 1/12 PP
Discuss the different O-RADS scores in terms of findings and risk of malignancy
O-RADS 1 = Simple cyst or CL.
= Normal and benign
O-RADS 2 = Haemorrhagic cysts, dermoids, endometriomas (<10cm) = Risk of malignancy <1%
O-RADS 3 = Risk of malignancy 1-10%
O-RADS 4 = Risk of malignancy - 10-50%
O-RADS 5 = Risk of malignancy >50%
When an ovarian mass is identified in premenopausal women when should tumour markers be done?
- If cyst looks simple then no need to do a Ca125
- If women less than 40 and cyst looks complex then tumour markers should be done and include: LDH, AFP, HCG
- If serum Ca125 is done and less than 200 then consider other causes (Increased in fibroids, endo/adeno, chronic illness, Infection)
- If Ca125 >200 discuss with GONC
How should ovarian cysts be worked up in post menopausal women
- Ca125 should be done in all women. No other tumour markers are necessary
- TVUSS is first line for investigating suspected ovarian cysts
- RMI should be calculated and if >200:
-Refer to GONC
-CT abdo pelvis.
How should ovarian cysts be managed in post menopausal women
Ovarian cyst >1cm for post menopausal women
1. Ca125 + TVUSS (MRI if USS inconclusive)
2. Calculate RMI
3. RMI <200 and cyst = aSx, unilocular, unilateral, <5cm
->Conservative management. Repeat evaluation at 6 and 12 months. If no change discharge
4. RMI <200 and cyst has any of the following features: Sx, >5cm, multilocular or bilateral
->Offer surgery -laparoscopy BSO
5. If RMI >200 then CT abdo pelvis and ref to GONC MDM
What is the definition of the following:
-Allodynia
-Hyperalgesia
-Persistent pelvic pain
- The experience of pain triggered by a stimuli which is not normally painful
- Increased pain response to stimuli which are normally painful
- Non-cyclical pelvic pain lasting 6 months or more, can be intermittent or constant. Sufficient to cause functional disability
Discuss persistent pelvic pain
-Epidemiology (2)
-Risk factors (5)
-Pathophysiology (4)
- Epidemiology
-1 in 6 women
-Prevalence 15-27% - Risk factors
-Previous abuse
-PTSD
-Unclear if depression and anxiety has role
-Female
-Poor sleep - Pathophysiology
-Prolonged noxious stimulation
-Local factors like alpha tumour necrosis factor and chemokines may change peripheral nerve function
-Altered excitability of dorsal horn neutrons leading to pain from stimulation of injured area and surrounding uninjured area
-This enhancement of response is called sensitisation
Descending information from the CNS (previous experiences, emotion, fear) may modify pain perception
Describe the causes of persistent pelvic pain by system
-Gynaecological
-Gastrointestinal
-Urological
-Neurological
-MSK
-Psychological
- Gynaecological
-Endo/adenomyosis
-Pelvic congestion
-Adhesions (only if dense vascular adhesion does adhesiolysis have an impact)
-Residual ovarian syndrome or trapped ovarian syndrome
-Infection
-Fibroids - GIT
-IBS (present in 50% of those with PPP)
-Assess with Rome III criteria - Urological
-Interstitial cystitis - (Present in 34-84% of those with PPP) - Neurological
-Nerve entrapment. (Present in 3.7% post pfannestiel) - MSK
-Referred lower back pain
-Pelvic floor spasm - Psychological
-Increased in women who have experienced abuse
How should chronic pelvic pain be managed
-Assessment (3 points relevant to Hx)
-Therapy (5 points)
- Assessment
-Time to listen to their story
-Consider woman’s theory of pain
-Explore multifactorial approach to pain - Therapy
-Multifactorial approach done simultaneously best approach
-If pain cyclical trial hormonal treatment as first line. Dx lap as second line (50% of women with PPP have a neg lap)
-If IBS trial FODMAP and antispamodics
-Consider analgesia but avoid opiates. If no improvement involve pain team
-If interstitial cystitis - amytriptyline first line
-If MSK component consider PT
-For centralised pain
-CBT
-Pain education and health literacy
-Hyperanalgesia retraining
-Physical therapy - exercise
-Improve sleep
-Manage mental health
Discuss endometriosis
-Incidence (4)
-Definition
-Frequency of symptoms (5)
- Incidence
-2-10% of women of reproductive age (11%)
-40% of women with genital tract anomalies
-50% of women with infertility
-70% of women with pelvic pain - Definition
-Presence of endometrial like tissue outside uterine cavity inducing a chronic inflammatory reaction. - Symptoms
-Chronic pelvic pain - 70%
- Dysmenorrhoea - 62%
-Dyspareunia - 55%
- Cyclical Dyschezia - 48%
-Subfertility - 40%
-Cyclical dysuria
Describe the theories of endometriosis pathogenesis (6)
- Samson theory - retrograde menstruation
- Haematological / Lymphatic dissemination of cells
- Coelomic metaplasia - prolonged irritation and oestrogen exposure may induce metaplasia in these cells.
- Genetics
- Altered immunity - not recognising abnormal cells leading to implantation
- Iatrogenic dissemination with Lap surgery, hysteroscopy, CS
Describe the ASRM staging for endometriosis
-Basis
-Stages
-Advantages (3), Disadvantages (3)
- Based on a points system depending on size, depth, location and associated adhesions
- Stages
Stage 1 = minimal (1-5 points)
Stage 2 = mild (6-15 points)
Stage 3 = moderate (16-40 points)
Stage 4 = Severe (>40 points)
- Complete obliteration of POD - 40 points
- >3cm bilateral infiltration of ovary (20 points each side)
- Dense adhesions around tube or ovary (16 points each) - Advantages:
-Accepted globally
-Useful for research
-Can be helpful for patients
Disadvantages:
-Poor correlation with patient symptom severity and fertility
-Lots of inter-observer bias
-Doesn’t classify DIE in retroperitoneal structures
Discuss endometrial classification system
-ENZIAN (3)
-EFI (4)
- ENZIAN
-Designed to completement ASRM for DIE
-Designed according to location and depth
-Better correlation with symptom severity - EFI (Endometriosis fertility Index)
-Designed to predict fertility
-Does not correlate with pain
-Reflects pregnancy rates better then ASRM
-Reflects IVF success rates better than ASRM
Discuss management of endometriosis
-Conservative
-Medical (6)
-Types (3)
-Points (4)
Treat symptoms not the disease stage
1. Conservative management
-Simple analgesia (NSAIDS +/- Paracetamol)
-Neuromodulators (pregabalin/gabapentin - no evidence for or against)
-Psychological support
-No evidence for alternative therapy
-Refer to pain specialist
-Encourage sleep hygiene and exercise
2. Medical
Hormonal therapy
-Doesn’t decrease lesion size. Will not result in remission. Pain returns once stopped
-No hormonal treatment has been found to be superior
-Good post surgery for delaying recurrence
-Don’t use if seeking fertility
-COCP - continuous better than cyclical
-Progesterone only
-GnRH agonists - second line. Give add back
-GnRH anatogonists - second line Give add back
-Aromatase inhibitors - third line
-Danazol, anti-progesterones, uterosacral nerve ablation presacral neurectomy no longer recommended
3. Points
-Medical same as surgical
-All medicines equivalent
-3/12 GnRH pre-op for DIE recommended to reduce operative complications
-Don’t treat asx endometriosis
Discuss surgical management of endometriosis
-Recurrence post op
-Treatment recommendations pre/post op
-Management of peritoneal disease
-Management of DIE
-Management of endometrioma
-Evidence for hysterectomy
-Prognostic markers to aid in selection of patients
- Recurrence post-op
-20% undergo repeat surgery in 2 yrs. 30% within 5 years - Post op hormonal management can prolong time to recurrence and improve pain
Pre-op hormonal not recommended
->stops dysmenorrhoea but not pelvic pain or dyspareunia - Management of peritoneal disease
->Ablation or excision. Excision allows for histology confirmation
->Excision has better outcomes for pain - Management of DIE
->Surgical removal for stages 3-4 doesn’t improve fertility
->Surgical removal effective for pain and QoL
->Consider MDT input - Management of endometrioma
->Aim for cystectomy not drainage. reduces recurrence.
->Post op use hormonal meds to stop recurrence (GnRH or COC)
->No evidence that surgical removal before ART improves outcome but does drop AMH
->Excision increases the rate of spont pregnancy - There is no evidence for effectiveness of hysterectomy for management of endometriosis
All endometriotic lesions should be excised at the time of hysterectomy
Total hyst is recommended if undertaken - Prognostic markers
-There are no factors which help to indicate which patients will benefit from surgery
What are the risk factors for endometriosis
- Risk factors
-Low BMI
-Family hx
-Congeital genital tract anomalies
-Nulliparity
-Prolonged estrogen exposure
-HMB
Discuss endometriosis
-Histological findings
-Pathophysiology of pelvic distortion
-Cancer risk
- Histological findings:
-endometrial glands and stroma, histocyctes and hemosiderin laden macrophages. Tissue is like but no the same as endometrium - Pathophysiology
-Increased fibrosis in lesions cause nodularity
-Inflammation and fibroisis cause shortening and thickening of uterosacral ligaments and ovarian ligaments
-Ovaries pulled medially (Kissing ovaries)
-Inflammation and fibrosis causes obliteration of POD - Endometriomas ass with increased risk of endometroid and clear cells ovarian cancer.
-Risk 2 extra cases per 10,000
-Risk of malignancy 1% in premenopausal and 2.5% in post menopausal
-No role for surveillance
Discuss consent
-Code of rights
-Requirements for informed consent (4)
-If unable to given informed consent
- Consent is outlined in right 7 of code of rights
- Infomred consent requires
-Information that can be understood by the patient
-Information needs to be specific to the intervention
-Consent needs to be voluntarily given
-Patient needs to have capacity - If patient unconscious or life threatening situation
-Seek next of kin to assess patient’s preference
-If no next of kin act in patient’s best interest (Duty of care)
Discuss the diagnosis of endometriosis (6)
- Examination is important
- Normal examination or imagining doesn’t rule out endometriosis
- USS should be considered
- MRI and CT are not first line investigations.
-Consider MRI for DIE if USS negative but symptoms suggestive - Don’t do ca125
- In Laparoscopy Bx for diagnosis is recommended (Gold standard and highly specific)
Discuss endometriosis in postmenopausal women
-Occurrence (1)
-Treatment (2)
-Management of menopausal sx in women with Hx of endo
- Occurrence
-Can still be active in post menopausal women - Treatment
-Consider surgical management
-Aromatase inhibitors - Menopausal sx
-Avoid estrogen only treatment - leads to malignant transformation
-Treat surgical menopause with combined MHT until natural menopause
How does adenomyosis impact pregnancy
-Pathophysiology of subfertility (5)
-Impact to pregnancy (7)
- Pathophysiology of subfertility
-Uterine dysperistalsis
-Impaired sperm transport
-Impaired endometrial receptivity for implantation
-Increased endometrial inflammatory factors
-Impaired endometrial / myometrial interface - Impact to pregnancy - poor implantation
-Miscarriage esp in IVF
-PET
-IUGR
-APH and PPH
-PTB
-Increased CS rate
-Increased malpresentation
What are the investigations for precocious puberty
-Bloods (5)
-Imaging (3)
-Stimulation (2)
- Bloods:
-LH and FSH - high in central, low in peripheral
-Oestradiol (E2)
-DHEAs, 17-OH progesterone (High androgens), testosterone
-TFT
-IGF-1
-Prolactin - Imaging
-Pelvic, abdo USS - ovarian, adrenal tumour
-MRI brain - central tumour
-Wrist XR - bone maturation (good measure for PP) - Stimulation test
-GnRH stimulation test - gold standard for central precocious puberty.
Give GnRH and measure LH. If LH is high then suggests prematurely mature HPO axis
-ACTH stimulation test to unmask mild CAH with increased levels of 17-OH progester
