Gynecology 1.8
LECTURER: Dr. Capitulo DATE: Nov. 27, 2014
PEDIATRIC GYNECOLOGY
OUTLINE I. II. III. IV.
REFERENCES PPT Recording Book
NOTE
Thelarche o Breast development Starts at 9 to 11 years old o Adrenarche Development of pubic and axillary hair o o Occurs a few months to a year after start of breast development Growth Spurt Period of maximum growth comes earlier in girls than boys o o Around 11 to 12 years old Menarche o Average age = 12.9 years in Filipino girls
GYNECOLOGICAL EXAMINATION PUBERTAL DEVELOPMENT SEXUAL MATURITY RATING COMMON GYNECOLOGICAL DISORDERS a. VULVOVAGINITIS b. LABIAL ADHESION c. PRIMARY AMENORRHEA d. PRECOCIOUS PUBERTY
Underlined statementsemphasized in the lecture and may come out in the exams (12 items)
Average ages for different changes in puberty (no need to memorize) o B2: 10.8 + 1.10 years Breast Budding (B2) to full breast development takes 2 to 6yrs o PH2: 11.0 + 1.21 years Menarche: 12.9 + 1.2 years o B2 to peak height velocity: 1.0 + 0.77 years o B2 to PH5: 3.1 + 1.04 years o B2 to menarche: 2.3 + 1.03 yrs (1.5 – (1.5 – 5 5 yrs) o o B2 to B5: 4.5 + 2.04 years
GYNECOLOGICAL EXAMINATION PELVIC EXAMINATION 2 positions that positions that pediatric patient can assume when doi ng pelvic examination: Supine Frog Leg position and Prone Knee-Chest positio n Supine Frog-Leg Position Can be done on a patient as young as 2 years old o Still at the risk of being kicked by the patient After assuming the position, Separate the labia o o Can visualize the lower 1/3 of the vagina Useful when the child puts in a foreign body
SEXUAL MATURITY RATING (TANNER STAGING) MEMORIZE! BREAST DEVELOPMENT
FIGURE 4. Tanner Staging of breast development B1
FIGURE 1. The Supine Frog-Leg Position
Prone Knee-Chest Position For patients 5 to 6 years old and onwards o o Patient’s chest and knees are against the examining table, table, then separate the labia Entire vaginal canal up to the cervix may be seen when the o patient’s labia are separated Can be done in place of a speculum exam o
B2 B3 B4
B5
Prepubertal No breast development, flat chest Breast budding “Marble underneath the nipple/areola” Enlargement of the breast due to increase in glandular tissue No separation of breast contour Secondary mound formed by the areola Separation of breast contour Small breast formed by the the areola and nipple, on top of the main breast mound Transient, thus, rarely observed in PE Normal adult breast Single contour of breast and areola Nipple is slightly pointed forward PUBIC HAIR DEVELOPMENT
FIGURE 2. The Prone Knee-Chest Position PUBERTAL DEVELOPMENT
Thelarche
Adrenarche
MaxGrowth
Menarche
10.8+/-1.1
11+/-1.21
11.8+/-0.77
12.9+/-1.2
FIGURE 3. The stages of pubertal development in females
FIGURE 5. Tanner Staging of female pubic hair development
GYNECOLOGY 1.8 PH1 PH2 PH3 PH4 PH5
Prepubertal No hair Hair on the labia Hair on the labia and mons pubis Spread of hair upwards Hair on the labia, mons pubis, and the sides of the labia Spread of hair laterally Normal adult “inverted triangle” configuration Hair on the labia, mons pubis, sides of the labia, and the medial thighs COMMON GYNECOLOGIC DISORDERS
CASE 1 5-year old girl with yellowish, slightly foul-smelling vaginal discharge associated with a sore perineum She is in kindergarten at a local preschool PE showed erythematous vulva with yellow discharge, intact annular hymen GS of discharge showed some gram (+) cocci and gram (-) rods
VULVOVAGINITIS The most common gynecologic condition in girls (40-85% of gynecologic visits) Inflammation of the vulva or vagina in response to any of a variety of stimuli
FIGURE 6. Vulvovaginitis
Mode of transmission is not always due to sexual means, it may also be due to poor hygiene. Sexually transmitted pathogen are isolated only in 5% of the cases
Compared to acidic pH in adults, where Lactobacillus sp. predominates, normal flora in children include other microorganism, which may be opportunistic in nature Inadequate front-to-back wiping Inattention to perineal hygiene Use of bubble baths and scented deodorant soaps Prepubertal children are easily irritated by bubble baths Sand box play Confining clothing Due to moisture build-up especially in chubby kids, ballerinas Sexual abuse Only in 5% of cases SIGNS AND SYMPTOMS Vaginal discharge (53-88%) – most common Erythema – 33% Pruritus – 32% o Itching upon drying of discharge in the skin May be seen in fungal infection, which is rare in children because Candida albicans prefers acidic pH Dysuria – 12-15% Pus/discharge can irritate urethral opening differential diagnosis: UTI Vaginal bleeding – 1-9% check for foreign objects Odor – 8% Pain/soreness – 1-4% DIAGNOSIS Microbiotic investigation is indicated if there is visible discharge on examination reflecting profuse discharge or moderate to severe inflammation Gram stain may suffice, but special cultures should be used when there is suspicion of sexual abuse (i. e. gonorrhea) Diagnosis: vaginoscopy o Scope is around 5mm (hymenal opening = 7mm) So as not to injure hymen indicated in recurrent vulvovaginitis or if without an identifiable cause
80% are non-specific (i.e. poor hygiene) 20% are due to specific organisms Respiratory – inoculated the pathogen to the vulvar area Gastrointestinal – i.e. inoculation from infectious diarrhea, Enterobiasis Sexually transmitted pathogens
PREDISPOSING FACTORS (OF FEMALE CHILDREN) Anatomic and physiologic peculiarities o Shorter perineal body Perineal Body- space between the introital opening and the anal opening Easily contaminated by fecal material No pubic hair, no labial fat o Hair and fat assume protection from irritation No estrogen o thin mucosa, makes vagina prone to irritants pH of the vagina and vulva is closer to neutral (6.5-7)
FIGURE 7. Vaginoscopy
TREATMENT Behavioral intervention FIRST LINE! Because 80% is non-specific. It is done to eliminate risk factors such as proper hygiene, washing with mild unscented soap, avoiding adult feminine hygiene products since it doesn’t have the same pH as prepubertal’s vaginal pH, wearing cotton underwear, proper hygiene, avoiding sand.
GYNECOLOGY 1.8 Antibiotics for specific pathogens Topical corticosteroids Used when no specific pathogen is identified Topical estrogen Used when symptoms are recurrent and when no specific pathogen is identified To thicken mucosa
CASE 2 2-year old girl with “ambiguous genitalia” No other associated symptoms PE showed a minute valvular opening just below the clitoris with midline raphe Normal female karyotype
(+) secondary sexual characteristics and (-) menarche by age 16.5 (16 years and 6 months) OR (-) secondary sexual characteristics and (-) menarche by age 14 Absence of menses in a woman who has never menstruated by the age of 16.5 years OR girls who have menstruated within 5 years of breast development (breast development, thelarche, should occur by age 13 or otherwise requires evaluation. Common reason for consult among teenagers
LABIAL ADHESION
FIGURE 8. Labial Adhesion. Note the thin line of demarcation between labia minora.
Partial to complete fusion of the labia majora Due to thin labia minora, if f uses and obliterates that vaginal canal, leaving small opening for urethra aka labial fusion, Labial agglutination and Adhesive vulvitis 2ND most common gynecologic condition in pediatric female The fused portion is usually identified by a thin line of demarcation or raphe, differentiating it from other conditions Etiology has been postulated as estrogen deficie ncy and inflammation with thinning of the superficial mucosal layers Diagnosis is clinical, no other laboratory or imaging studies are necessary Differential diagnosis: o Imperforate hymen – rare condition, no demarcation line Ambiguous genitalia is a rare condition due to persistent cloaca o
HISTORY/PE Patient History: o Menstrual History – no menses Illness, Drug Use – some drugs may induce amenorrhea o o CNC Chemotherapy or Radiation – may have affected the pituitary gland o Pelvic Radiation – may have damaged the ovaries o Psychosocial Stressors – young athletes undergoing rigorous training sometimes have primary amenorrhea Sexual Activity – rule out pregnancy o Breast Changes o o Anosmia Since the olfactory nerve is near the pituitary gland Because GnRH secretion occurs after migration of these specific cells from the olfactory lobe to the hypophysis during embryogenesis, anosmia may also occur in some patients with gonadotropin deficiency.[3] Abdominal Pain o o Vasomotor Changes Family History: Genetic Defects o Pubic Hair Pattern o o Infertility Menarche and Menstrual History of Mother and Sisters o o Pubertal History Physical Examination: Anthropometric Striae, buffalo hump, o o Measurements central obesity, etc. BMI Tanner Staging o o Dysmorphic Features Thyroid Examination o o o Rudimentary or Absent o Vaginal Septum or Uterus Imperforate Hymen o o Pubic Hair Pattern Signs of Virilization EVALUATION OF PRIMARY AMENORRHEA MEMORIZE! After making the diagnosis of primary amenorrhea look for the presence of secondary sexual characteristics (breast development, pubic hair, growth spurt).
FIGURE 9. A: Imperforate hymen. B: Ambiguous genitalia
TREATMENT Spontaneous resolution in 6 months Topical estrogen (estriol) – Ovestin Applied twice daily o Separation of labia minora may be seen as early as 2 weeks Surgical separation o Done if it does not resolved with topical estrogen after 6 months Done under anesthesia o
PRIMARY AMENORRHEA
FIGURE 10. Algorithm of evaluation of primary amenorrhea
GYNECOLOGY 1.8 EVALUATION OF PRIMARY AMENORRHEA WITHOUT SECONDARY SEXUAL CHARACTERISTICS If no secondary sexual characteristic and no menses is observed by age 14y/o, it is called hypogonadism, FSH and LH should be measured Failure of gonadal development is the most common cause of primary amenorrhea
FIGURE 11. Algorithm of evaluation of primary amenorrhea without secondary sexual characteristics
REVIEW: Hypothalamic-Pituitary-Ovarian Axis (HPA) Hypothalamus will send Gonadotropin Releasing Hormone (GnRH) to the pituitary gland. Pituitary gland will release FSH and LH. FSH and LH will act on the ovaries and will stimulate the follicles to mature. The granulosa cells surrounding the follicles wil l start producing estrogen. Estrogen which will cause ovulation and stimulation of the appearance of the secondary sexual characteristics and growth spurt o Stimulate pubic hair and axillary hair because estrogen i s converted to testosterone at the peripheral tiss ues. o Stimulate the endometrium to thicken. If thick enough, the pituitary gland will produce increased amounts of LH (LH surge) which will cause the patient to ovulate HYPOGONADOTROPIC HYPOGONADISM Hypogonadotropic: low FSH and LH Hypogonadism: Gonads are not functioning Etiology Hypothalamic failure o The hypothalamus will not be able to produce GnRH to further stimulate the pituitary gland t o make FSH and LH In inadequate GnRH synthesis or release, or both Can be due to a tumor, infection, or radiation o Pituitary gland failure The pituitary gland will not be able to produce FSH and LH Gonads are not functioning since there is no stimulation coming from the brain. The ovaries can be normal but there is no signal telling t he gonads what to do.
HYPERGONADOTROPIC HYPOGONADISM Hypergonadotropic: high FSH and LH Hypogonadism: Gonads are not functioning Etiology: ovarian problem The hypothalamus will send GnRH to the pituitary, then the pituitary will send levels of FSH and LH to the o varies but the ovaries will not respond. When ovarian follicles are absent, synthesis of ovarian steroids and inhibin does not occur Breast development does not occur because of the very low circulating E2 levels. Absent negative Feedback estrogen and inhibin to HPA, thus, gonadotropin levels are markedly elevated, with FSH levels being higher than LH Estrogen is not necessary for mullerian duct development or wolffian duct regression, so the internal and external genitalia are phenotypically normal female. Karyotype should be requested. o normal female karyotype (46,XX), Premature Ovarian Follicle (POF) or there is death of the gonads even before menarche may be due to viral infections such as mumps – oophoritis o 45, XO Turner Syndrome
EVALUATION OF PRIMARY AMENORRHEA WITH SECONDARY SEXUAL CHARACTERISTICS If secondary sexual characteristics (breast, pubic hair, growth spurt) are observed, but no menses at the age of 16.5y/o, do UTZ of uterus
FIGURE 12. Algorithm of evaluation of primary amenorrhea with secondary sexual characteristics AIS-Androgen Insensitivity Syndrome UTERUS (-) ABNORMAL UTERUS If there is no uterus or there is an abnormal uterus, request for a karyotype
MULLERIAN AGENESIS Absent or abnormal uterus with normal female karyotype (46,XX) The Mullerian duct was lost during embryological development, where the fallopian tubes, uterus and upper 2/3rd of the vagina stem from. Hence, vagina is shallow. This is called the Mayer-RokitanskyKüster-Hauser Syndrome (MRKH)
GYNECOLOGY 1.8
ANDROGEN INSENSITIVITY SYNDROME Absent or abnormal uterus with male karyotype (46,XY) The patient is a male! Formerly called testicular feminization syndrome Absent receptor for androgen, unable to recognize the androgen Recall, that in the absence of testosterone (or in this case inability to detect it) will by default produce female characteristics-externally Absent uterus, ovaries and fallopian tube. Vagina is shallow since it is not fully developed Since there is androgen production, it can be converted in the periphery to estrogen, developing the breast, axillary hair and pubic hair.
UTERUS (+) OR NORMAL If the uterus is normal on ultrasound but there is no menstrual flow, note if there is an outflow obstruction If there is an outflow obstruction, possible cases include imperforate hymen or transverse vaginal septum. The menstrual blood was stored inside and is accumulated o Treated by removing the obstruction by opening/stretching the o hymen to allow a bigger opening fo r the blood to come out. o What can be done is excise the septum in some cases just to allow the blood to flow.
Note: Other causes are not included in this lecture
OUTFLOW TRACT OBSTRUCTION
GOALS OF TREATMENT GENERAL Progression of normal pubertal development Preservation of fertility Prevention of complications of hypoestrogenic state Prevent osteoporosis, dyslipidemia, etc.. o
WITH NO SECONDARY SEXUAL CHARACTERISTICS Progression of normal pubertal development – HRT o To develop the breast HRT-Hormone Replacement Therapy, containing estrogen and o progesterone Preservation of fertility – HMG or pulsatile GnRH To induce ovulation o HMG-Human Menopausal Gonadotropin o Prevention of complications of hypoestrogenic state – HRT, calcium, vitamin D Remove gonads if with Y chromosome since it is prone to develop malignancy
ANDROGEN INSENSITIVITY SYNDROME Progression of normal pubertal development- remove gonads after full breast development and epiphyseal closure Preservation of fertility- none Prevention of complications of hypoestrogenic state- estrogen replacement therapy, no need to give preparations with progesterone since these individuals have no uterus MULLERIAN AGENESIS They will develop normal puberty since they only lack uterus, fallopian tube and upper 2/3rd of the vagina, ovaries are functioning well Preservation of fertility-none Surrogacy (but this is illegal in the Philippines) o Surgical reconstruction of absent vagina- vaginal canal is extended
Hymenectomy Resection of vaginal septum PRECOCIOUS PUBERTY Appearance of any signs of secondary sexual maturation at an early specific age more than 2.5 standard deviations below the mean Initiation of secondary sexual characteristics before age 8, menarche before age 9, thelarche at the age of 8 Concerns with this disorder: Social stigma of child being physically different from her peers o Diminished ultimate height o Shorter than normal adult height due to: Limited duration of rapid growth spurt Accelerated bone maturation Premature closure of the distal epiphyseal growth centers Types: Central, peripheral, incomplete CENTRAL PRECOCIOUS PUBERTY GnRH-dependent precocious puberty Physiologically normal pubertal development that is chronologically early Meaning, individual follows the normal stages of development: thelarche, adrenarche, growth spurt, menarche Results from GnRH-stimulated episodic gonadotropin secretion GnRH, LH, FSH, sex steroids levels are similar to those in normal puberty Idiopathic in 70% of cases CNS dysfunction in 30 % of cases part of the work-up: head CT scan and MRI Congenital defects (septooptic dysplasia) o o Destruction from tumors (craniopharyngioma) Destruction from other lesions (arachnoid cysts) o o Excessive exposure to sex steroids (McCune Albright) o Excessive pressure (hydrocephalus) Infection or inflammation (brain abscess, meningitis) o o Injury (head trauma, irradiation) GnRH secreting tumors (hypothalamichamartomas) o PERIPHERAL PRECOCIOUS PUBERTY GnRH-independent precocious puberty or pseudoprecocious puberty They go into puberty but bypassing some stages, Pubertal development resulting from stimulation with a hormo ne other than hypothalamic GnRH May or may not follow the chronology of normal pubertal development Etiology Estrogen-secreting ovarian tumor ( 60% of cases) o GRANULOSA CELL TUMOR – most common, most notoriuos Mixed germ cell tumor Cystadenomas Gonadoblastoma o Exogenous sex steroids or gonadotrophin Example: A 4-y/o, patient, had developed breasts and started to menstruate because she had mistaken her mother’s oral contraceptive pills (OCPs) for candies. All the work-up findings were normal. Upon discontinuation of the pill, all the findings in the child regressed. Chronic primary hypothyroidism o Feminizing adrenal tumors o
GYNECOLOGY 1.8
INCOMPLETE PRECOCIOUS PUBERTY 2 types: Premature Thelarche, Premature Adrenarche/Pubarche PREMATURE THELARCHE Isolated unilateral or bilateral breast development Not accompanied by other signals of pubertal development Usually occurs at age 1 to 4 years Benign self-limiting condition that does not require treatment. Management: observe the patient o Premature Thelarche resolves within 2 years If It does not resolve, it might be a case of central precocious o puberty, which has a different management PREMATURE PUBARCHE OR ADRENARCHE Early isolated development of pubic or axillary hair without other signs of secondary sexual maturation Self-limited but must be followed up carefully If it progresses to growth spurt or menarche, treat for Central Precocious Puberty
EVALUATION OF PRECOCIOUS PUBERTY Meticulous history and PE o Height Tanner stage o o Neurologic exam Serum estradiol, FSH, LH, androgens and thyroid function tests BONE AGE (hand-wrist films) – repeated every 6 months; very important; films of the patient are compared to films of children of same age-group Imaging studies like ultrasound, skull x-ray, cranial CT scan and MRI – to rule out any CNS condition leading to precocious puberty
MANAGEMENT OF PRECOCIOUS PUBERTY Depends on the cause, extent and progression of precocious signs CENTRAL PRECOCIOUS PUBERTY treat the patient if with: Progressive thelarche and pubarche o o Bone age >2 years for age because the epiphysis might close already Goals of Treatment for Central Precocious Puberty: o Reduce GnRH secretion and peripheral actions of sex steroids Decrease growth rate to normal o Slow skeletal maturation to allow development of maximal adult o height GnRH Analogues Drug of Choice o GnRH ANALOGUES NOT GnRH ANTAGONIST MOA: Down regulation of the receptors o When all the receptors are filled up with GnRH there will be no receptors for GnRH that patient is p roducing, so there will be cessation of symptoms Most effective in 4 to 6-year olds o Decreases gonadotrophin and sex steroids to Prepubertal levels o o Causes regression of precocious signs Continue treatment until progress of puberty is ageo appropriate and consistent with emotional maturity, current height and height potential Example: When the diagnosis is made at 6, whereas normal breast development occurs as 9, treat the patient for 3 years until it paralleled to the normal time for pubertal changes. PERIPHERAL PRECOCIOUS PUBERTY Remove the source of the hormone o If it is caused by adrenal tumor →excise the tumor If it is caused by an ovarian tumor → excise the ovarian tumor o o If it is caused by an exogenous intake of hormone →stop intake INCOMPLETE PRECOCIOUS PUBERTY Observe for resolution or progression Edited by: aia
