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Pediatric gynecology


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Pediatric gynecology

  2. 2. Gynecological care begins in the delivery room as part of the newborn examination with palpation of the breast buds and examination of the external genitalia
  3. 3. Newborn Anatomy <ul><li>1 st few weeks of life, residual maternal sex hormones may have physiologic effects on newborn </li></ul><ul><li>Breast budding in majority of term female infants and there may be marked breast enlargement or even nipple discharge but no treatment is required </li></ul><ul><li>Labia majora are bulbous </li></ul><ul><li>Labia minora are thick & protruding </li></ul><ul><li>Clitoris is relatively large (normal is 0.6cm 2 or less) </li></ul><ul><li>Vaginal epithelium dull pink initially and then as maternal residual estrogen levels decline, genitalia take on their juvenile appearance with small labial fat pads and the vaginal epithelium (1-3 cells thick) becomes bright red </li></ul>
  4. 4. Newborn Anatomy <ul><li>Vaginal discharge is common, usually composed of cervical mucus & exfoliated vaginal cells </li></ul><ul><li>Vaginal bleeding is also common as the estrogen levels fall and the stimulated endometrial lining sheds. Generally stops in 7-10 days. </li></ul><ul><li>Hymen has varied configuration & size of opening. Initially turgid </li></ul><ul><li>Vagina is approximately 4 cm long </li></ul><ul><li>Uterus is enlarged (4 cm) with no axial flexion </li></ul><ul><li>Ratio b/w Cx & corpus is 3:1 </li></ul><ul><li>Columnar epithelium protrudes through os </li></ul><ul><li>Ovaries are abdominal organs into early childhood </li></ul>
  5. 5. Early Chilhood Anatomy <ul><li>Little estrogen stimulation </li></ul><ul><li>Labia majora flatten, labia minora and hymen become thin </li></ul><ul><li>Vagina has atrophic mucosa and is very susceptible to trauma & infection </li></ul><ul><li>Cx is flush with vaginal vault as vaginal fornices do not develop until puberty </li></ul><ul><li>Uterus regresses in size until about age 6 </li></ul><ul><li>Ovaries begin to enlarge & descend </li></ul><ul><li>By age 7-10 years, genitalia start to show signs of estrogen stimulation with thickening of the mons pubis, labia majora, hymen, & vaginal mucosa. </li></ul><ul><li>The labia minora become rounded & the vagina elongates to 8cm </li></ul><ul><li>Uterus starts to grow and rapid endometrial proliferation occurs as menarche becomes imminent </li></ul><ul><li># ovarian follicles increases </li></ul>
  6. 6. History & Physical Exam <ul><li>Give child an opportunity to speak with you alone when appropriate </li></ul><ul><li>Give child as much control as possible over situation & get them involved in the exam if possible </li></ul><ul><li>Be mindful of abuse and be aware of appropriate steps in suspicious cases </li></ul><ul><li>Never restrain a child (general anesthetic may be required) </li></ul><ul><li>Have parents sit on table with child </li></ul><ul><li>Use frog leg and knee/chest positions in younger children </li></ul><ul><li>Inspect hymen carefully for signs of breaks or trauma as minor external injuries may hide serious vaginal lesions </li></ul><ul><li>Inspect anal region but do vaginal/rectal exams only when needed (imaging often better option) </li></ul>
  7. 7. Instruments <ul><li>May need instruments to visualize the upper 1/3- 1/2 of the vagina </li></ul><ul><li>Office vaginoscope can be tried (0.5 cm in infancy/childhood & 0.8 cm in premenarcheal girls) </li></ul><ul><li>Water cystoscope allows some distention of vagina & cleans debris </li></ul><ul><li>Can use urethroscope/laparoscope </li></ul><ul><li>Topical lidocaine to anesthetize vulva </li></ul><ul><li>General Anesthesia if exam not easy </li></ul><ul><li>Huffman-Graves & Pedersen specula should be used for adolescents </li></ul><ul><li>Saline soaked swabs are used for vaginal samples in children because this is adequate given immature lining </li></ul><ul><li>Speculum exam with Cx cultures may be necessary in adolescent </li></ul>
  8. 8. Genital Ambiguity <ul><li>1/14 000 newborns </li></ul><ul><li>Needs immediate identification and response as life threatening salt wasting congenital adrenal hyperplasia may be cause </li></ul><ul><li>Ambiguity can result from masculinization of a female child, exogenous hormone ingestion, maternal or fetal overproduction of androgens, incomplete virilization of a male infant, hormonal insensitivity, gonadal dysgenesis, or chromosomal abnormalities </li></ul><ul><li>Assessment should include lytes, 17-hydroxyprogesterone, cortisol, karyotype, and any other relevant blood work </li></ul><ul><li>Careful genital exam and pelvic u/s to detail internal anatomy </li></ul><ul><li>Multidisciplinary approach essential </li></ul>
  9. 9. Clitoral Abnormalities <ul><li>Normal clitoris 1-1.5 cm long and 0.5 cm wide </li></ul><ul><li>Abnormalities uncommon </li></ul><ul><li>Clitoromegally usually associated with  androgen exposure (often assoc with labial fusion) </li></ul><ul><li>Clitoral splitting is rare and caused by a midline fusion defect </li></ul><ul><li>Bifid clitoris usually assoc with bladder extrophy </li></ul><ul><li>Extrophy rare (1/30 000 births) </li></ul><ul><li>> 40% assoc with some genital tract abn </li></ul>
  10. 10. Imperforate Hymen <ul><li>Represents a persistent portion of the urogenital membrane </li></ul><ul><li>One of most common obstructive lesions in the female genital tract </li></ul><ul><li>Incidence 1/1000 live born ♀ </li></ul><ul><li>Generally sporadic anomaly </li></ul><ul><li>Often not diagnosed until puberty with c/o cyclical abd pain & 1 O amenorrhea </li></ul><ul><li>Classic is bluish bulge at introitus </li></ul><ul><li>Mucocolpos or hematocolpos may cause pain, difficulty voiding/defecating </li></ul><ul><li>Variations include imperforate, microperforate, septate, and cribriform hymens </li></ul><ul><li>Requires surgical resection if clinical significance evident </li></ul>
  11. 11. Transverse Vaginal Septum <ul><li>Results from faulty fusion or defective canalization of the urogenital sinus & mullerian ducts </li></ul><ul><li>1/75,000 women </li></ul><ul><li>46% upper, 40% mid, & 14% lower vagina </li></ul><ul><li>Septa in the upper vagina more likely to be patent </li></ul><ul><li>Complete septum has similar signs & Sx as an imperforate hymen except without the bulge at the introitus </li></ul><ul><li>Membrane excised with surrounding ring of subepithelial tissue & may then require an end to end reanastomosis of upper and lower vaginal mucosa (depending on the thickness of the septum) </li></ul>
  12. 12. Longitudinal Vaginal Septum <ul><li>Duplication of vagina very rare & often associated with duplication of vulva, bladder, & uterus </li></ul><ul><li>More commonly, longitudinal septa form when the distal ends of the mullerian ducts fail to fuse properly </li></ul><ul><li>Surgical excision not required unless symptomatic or worries re: SVD </li></ul>
  13. 13. Vaginal Agenesis <ul><li>Incidence 1/5000 </li></ul><ul><li>Most common cause is Mayer-Rokitansky-Kuster-Hauser Syndrome (46XX) </li></ul><ul><li>Not inherited but is an accident of development </li></ul><ul><li>External genitalia normal with variable levels of uterine development, although often cervical & uterine agenesis are present </li></ul><ul><li>May be urinary tract, spinal, middle ear, & other mesodermal structural abnormalities </li></ul><ul><li>75% with MRKH have complete vaginal agenesis and 25% have a short vaginal pouch </li></ul>
  14. 14. Vaginal Agenesis <ul><li>Typically normal female karyotypes with normal ovaries and ovarian function </li></ul><ul><li>Normal secondary sexual characteristics </li></ul><ul><li>Often present with 1 O amenorrhea </li></ul><ul><li>Creation of a vagina should be delayed until pt wishes to be sexually active </li></ul><ul><li>Vaginal dilators or surgical creation </li></ul><ul><li>Complete androgen insensitivity may also present as vaginal agenesis and must be correctly identified 2 O risk of gonadoblastoma (4-5% risk) </li></ul><ul><li>Chromosomal analysis is definitive </li></ul>
  15. 15. Uterine Abnormalities <ul><li>Result from agenesis of the mullerian duct or a defect in fusion or canalization </li></ul><ul><li>Most are asymptomatic & are only picked up incidentally or when they interfere with reproduction </li></ul><ul><li>Bicornuate uterus (37%), arcuate uterus (15%), incomplete septum (13%), uterine didelphys (11%), complete septum (9%), & unicornuate uterus (4%) </li></ul><ul><li>Mullerian anomalies occur in 1-3% ♀ </li></ul>
  16. 16. Unicornuate Uterus <ul><li>Single horned uterus with corresponding fallopian tube & round ligament </li></ul><ul><li>Results from agenesis of 1 mullerian duct with absence of structures on 1 side </li></ul><ul><li>If other hemiuterus present, a small rudimentary horn is created </li></ul><ul><li>If this horn does not communicate with other cavity or vagina, may develop dysmenorrhea and hematometra </li></ul><ul><li>Higher risk preterm labor, infertility, endometriosis, & malpresentation </li></ul>
  17. 17. Uterine Didelphys <ul><li>Failure of fusion of the mullerian duct may result in 2 separate uterine bodies </li></ul><ul><li>Generally good reproductive outcomes </li></ul><ul><li>Vaginal septae may require resection if causing difficulty with intercourse, vaginal delivery, or pain from obstructed menstruation </li></ul>
  18. 18. Bicornuate Uterus <ul><li>Results from partial fusion of the mullerian ducts which leads to varying degrees of separation of the uterine horns </li></ul><ul><li>Reproductive function is generally good </li></ul>
  19. 19. Septate Uterus <ul><li>Results from failure of canalization or resoption of the midline septa between the 2 mullerian ducts </li></ul><ul><li>Higher risk of miscarriage with increasing length of septa </li></ul><ul><li>Hysteroscopic resection may need to be considered </li></ul>
  20. 20. Vulvovaginitis <ul><li>Most common gyne complaint of children </li></ul><ul><li>Children are susceptible to pruritus & vaginal discharge from irritation/infection as the vulva is thin without labial fat pads and pubic hair, closer to the anus, unestrogenized vagina is atrophic, pH is excellent for bacterial growth, & perineal hygiene is suboptimal </li></ul><ul><li>Itch/scratch cycle and subsequent inflammation & bleeding </li></ul>
  21. 21. Vulvovaginitis <ul><li>Sand boxes, wet clothes, etc contribute </li></ul><ul><li>May have large extension onto thighs </li></ul><ul><li>Note of other derm conditions/lesions and whether there are signs of abuse </li></ul><ul><li>Wet mount may show numerous leuks </li></ul><ul><li>Cultures & evaluation of vaginal secretions as appropriate </li></ul><ul><li>Most cases resolve with better hygiene & avoidance of irritants </li></ul>
  22. 22.
  23. 23. Foreign Bodies <ul><li>Very common in children </li></ul><ul><li>Often present with vulvovaginitis, pain, foul smelling purulent/bloody d/c </li></ul><ul><li>Often fragments of toilet paper but may be toys etc which child may not remember or admit to </li></ul><ul><li>Foreign bodies in the lower 1/3 vagina can often be flushed out with warm saline irrigation </li></ul><ul><li>Vaginoscopy in many cases appropriate </li></ul>
  24. 24. Labial Agglutination <ul><li>Common in prepubertal children </li></ul><ul><li>Etiology unknown but likely secondary to low estrogen levels </li></ul><ul><li>Skin covering labia is thin and local irritation may denude the labia causing adherence in the midline and reepithelialization </li></ul><ul><li>Must distinguish from vaginal atresia </li></ul><ul><li>Most children are asymptomatic but may have urinary Sx & recurrent infections </li></ul><ul><li>Tx if symptomatic is estrogen cream bid for 7-10 days </li></ul><ul><li>Surgical separation may be necessary in some cases </li></ul><ul><li>Recurrence common </li></ul>
  25. 25. Trauma <ul><li>Straddle injuries most common cause of genitalia trauma in young girls </li></ul><ul><li>Seasonal peak in spring with bikes </li></ul><ul><li>Contusions generally require no tx </li></ul><ul><li>Hematomas are generally controlled with pressure & an ice pack although an enlarging hematoma may need incision & ligation of bleeders </li></ul><ul><li>May need to pack vagina </li></ul><ul><li>Catheter if hematoma blocking urethra </li></ul><ul><li>Pelvic X-ray & Abx as appropriate </li></ul><ul><li>Must rule out more severe injury (eg above hymenal ring) </li></ul>
  26. 26. Abuse <ul><li>May victims are not seen immediately </li></ul><ul><li>Suspect </li></ul><ul><li>Know who to call & be sure of evidence collection </li></ul><ul><li>Tx all injuries, good perineal care, screen for STD’s & tx as needed </li></ul><ul><li>Pregnancy test if appropriate </li></ul><ul><li>Counselling & support </li></ul>
  27. 27. Neoplasms <ul><li>Uncommon but about 50% genital tumors found in children are premalignant or malignant </li></ul><ul><li>Benign tumors of the vulva/vagina include teratomas, hemangiomas, simple cysts of the hymen, granulomas, & condylomata acuminata, </li></ul><ul><li>Only large, suspicious, or symptomatic lesions require surgical removal </li></ul><ul><li>Embryonal vaginal carcinomas most commonly seen < 3y </li></ul><ul><li>Tumors arise in the submucosal tissues & spread rapidly beneath an intact vaginal epithelium so that the mucosa bulges into a series of polypoid growths (botryoid sarcoma) </li></ul>
  28. 28. Neoplasms <ul><li>Ovarian tumors most common genital tumor (1% total) </li></ul><ul><li>Most common neoplasm in girls is the dermoid </li></ul><ul><li>70 % germ cell </li></ul><ul><li>Abdominal pain & mass most common sx </li></ul><ul><li>Tumors present abdominally </li></ul>