By Dr. Nadia Saddam AL.Assady C.A.B.O.G

1. Genital tract malformation By Dr. Nadia Saddam AL.Assady C.A.B.O.G

2. Uterine anomalies: • a-absence of the uterus: • in this condition the uterus either absence or rudimentary development so incapable for any kind of function it is called " • Rokitansky syndrome" , it is usually found with absent vagina & clinically presented with primary amenorrhea, they have (46XX) karyotype & normal ovarian function. Cases of absent uterus & development of the lower part of the vagina ending blindly & absence or scanty appearance of pubic hair raise the suspicion of androgen insensitivity syndrome. No treatment is available to absent uterus apart from psychological support is very important.

3. b-fusion anomilies: 1-bicornuate uterus: the cornul parts of the uterus remain separated giving the organ a heart shaped appearance, no evidence to give signs & symptoms. 2-septate or sub septate uterus: the presence of septum extending over some or all of the uterine cavity, it present with clinical feature in form of recurrent spontaneous abortion or mal presentation ( a persistence transverse lie of the fetus in late pregnancy suggest uterine anomalies).

4. 3-uterus didelphus: complete duplication of the uterus & cervix, it may be associated with failure to descent the head in late pregnancy or obstruction of lobar in late pregnancy. 4-rudimantory horn: it give rise to serious problem if the pregnancy is implanted in this part lead to rupture of the horn with profound bleeding may occur as the pregnancy increase in size. • It resemble that of rupture ectopic pregnancy but here the amenorrhea is in months not in weeks as in ectopic pregnancy & shock may be profound , it may also give to dysmenorrhea & pelvic pain & it treated by surgical correction.

5. Vaginal anomalies: Absence of vagina: it is associated with absence of the uterus or rudimentary uterus called Rokitansky syndrome, rarely uterus is present & the vagina or large part of it is absent. They present at ( 16 years of age) with primary amenorrhea, the secondary sexual characters are normal because the ovary is normally function, so combination of both normal secondary sexual development & primary amenorrhea suggest anatomical causes.

6. . Abdominal examination is required to exclude any blood collection in the upper genital tracts. Vulval development is normal & there is absence vagina, if short blind vagina so it arising from androgen insensitivity syndrome & karyotype should be done. The renal system should examine because (40%) of patient with lower genital tract abnormalities have renal abnormalities so U/S & IVU may be required. Treatment: counseling & psychological support & graduated glass dilators & vaginoplasty.

7. hematocolpos: imperforated membrane exist at the lower end of the vagina, this recognized at puberty when retention of menstrual flow give rise to hematocolpos, rarely present in newborn as hydrocolpos. Clinically present with abdominal pain, primary amenorrhea & rarely interference with micturition, the patient is usually between (13-15 years) old & give history of regular cyclical lower abdominal pain for previous several months, may present as acute emergency if

8. urinary obstruction develops. On examination there is lower abdominal swelling & large bulging mass in the vagina may be seen, vulval inspection may reveal imperforated membrane. Treatment is by simple excision of the membrane & release of retained blood resolve the problems. Gynecological disorders of childhood : Vulvo-vaginities: its common & the etiology is based on opportunistic bacteria colonizing the lower vagina & inducing inflammatory response because at birth the vulva & vagina are well estrogenized due to intra uterine exposure of the fetus to placental estrogen this cause

9. thickening of vaginal epithelium which is entirely protective against any bacterial invasion, but within ( 2-3 weeks) of delivery the resultant hypo estrogenic state leads to change in the vulval skin which become thinner & also vaginal epithelium become thinner. The vulval fat pad disappears & easily traumatized by injury, irritation, infection or allergic reaction. The close apposition of the anus means that the vulva & lower vagina are exposed to fecal bacterial contamination & the hypo estrogenic state in the vagina mean there is no lacto bacilli, so the PH is 7 making it ideal culture medium for low virulent organism.

10. Causes: 1-bacterial: non- specific ( common) , specific ( rare). 2-fungal: candida of the vulva & its rare condition it usually associated with diabetes & immune compromise. 3-virual: rare e.g herpes simplex & condylomata accuminta, so should alert the clinician to possibility of sexual abuse. 4-dermatities: it is not uncommon specially atopic & contact dermatitis specially those who have eczema so refer to dermatologist.

11. 5-lichen sclerosis: cause persistent vulva itching & skin undergoes atrophy & fissuring & very susceptible to secondary infection. 6-sexual abuse present with vaginal discharge so any child who have recurrent attacks of vaginal discharge should alert to this condition. 7-enuresis: many girls suffer from urinary incontinence specially at night this lead to moist vulva & allows secondary bacterial infection. 8- foreign body.

12. Diagnostic procedure: The first is inspection of the vulva & vagina with good illumination, if there is history of foreign body so we examine the vagina by vaginoscope & the second is taken bacteriological specimen by using pipette rather than cotton swab, if the diagnosis of pin worms to be excluded then a piece of sticky tape over the anus early in morning before the child gets out of bed reveal the presence of eggs on microscope. The result of microbiology is difficult to interpret & the vast majority of children not have pathological microorganism.

13. treatment: advise about perineal hygiene because all parents of child with chronic vaginal disease are worried that this may cause long term affect in fear of sexual dysfunction or subsequent infertility, but this not occur so parents should reassure this a local problem only so proper care of perineum, so child should taught to clean her vulva specially after defecation from front to back as this avoids the transfer of entero bacteria to vulva area, after micturition the vulva should clean & not leave the skin wet as this worm environment is ideal culture for bacteria. During acute attacks of non- specific recurrent vulvo- vaginitis children complain of burning during micturition due to passage of urine across the inflamed vulva, the use of barrier creams in these circumstances may be very useful

14. labial adhesion: it is trivial problem, but it's important because misdiagnosed as congenital absence of vagina, in post-delivery hypo estrogenic state the labial minora stick together in the midline leaving small opening anteriorly through which urine is passed, clinically no symptoms but older children may complain from spraying when they pass urine. Treatment: no treatment require just reassurance, in those children in whom there are some clinical problem local estrogen cream can applied for about 2 weeks & resolve the problem, if the adhesion is still present so gentle separation may be undertaken using a probe then application barrier cream to prevent further adhesion.

15. Gynecological disorder of adolescent: Those patients usually present with one of these disorder: first: problems associated with menstrual cycle & menstrual dysfunction like dysmenorrhea & premenstrual syndrome. Second: primary amenorrhea. Third: problem of teen age hirsutism. Menstrual problems: The management of these cases usually without active treatment but by support & understanding of the condition

16. Heavy menstruation: We should take accurate history from the child & it is difficult if the mother is present, normal menstrual loss not more than 80 ml during the periods, although in (5%) of cases its heavier but cause no trouble so clinician should assess the child if has truly menstrual loss because this is a serious medical condition & this done by measurement of Hb: 1-if the Hb > 12 gm% so explanation to the mother & child about normal physiology of menstrual establishment & require no active treatment & 6 months interval follow up to see establishment of menstruation & reassurance very important.

17. 2- if the Hb (10-12 gm%) so explanation required & giving iron treatment to correct mild iron deficiency anemia, menstrual loss need to be reduce & this achieved by using either ( progesterone cyclically for 21 days or the use COCP) & follow up annually is required & reassessment again, there after the child require no further treatment & reassurance is essential. 3-if the Hb< 10 gm%so serious anemia is result from menstrual blood loss so require explanation but urgent attention to treatment is needed progesterone is less likely to be effective in this group so COCP is the treatment of choice giving continuously for short period of time so that anemia can be corrected using oral iron & then the pills may be used in normal way so menstrual loss occur monthly. Any girls have continuous menstrual loss & uncontrolled by this management need U/S to exclude uterine pathology.

18. Primary dysmenorrhea: It is define as pain which begins in association with menstrual bleeding so treatment by both use of NSAIDs & the COCP but again failure of the treatment require U/S to exclude any uterine pathology. Premenstrual syndrome: It is difficult problem in adolescence as the psychological changes that occurring during this time of women life are complex & stressful, the PMS is stress related disorder, therefore teenager girls may need help of psychiatrist.

19. Hirsutism: Androgen affect some areas of human body & increase hair growth rate & also thickness of terminal hairs. Androgens are involve in sebum production & may cause this to be excessive, in some women excessive hair growth may occur on the arms, legs, abdomen, breast & back such that it constitute the problem of hirsutism & also associated with acne which may occur not only on the face but on chest & back.

20. D.DX: 1-androgenic causes include: a- congenital adrenal hyperplasia: ether classical or late onset. b-androgen secreting tumor. 2-PCOS it is difficult to diagnose at this age but this is a big problem. 3-XY gonadaldysgensis. 4-idiopathic. Some girls have constitutional basis for their hirsutism & familial body hair pattern should be borne in mind when considering whether or not a young patient does in fact have hirsutism.

21. Treatment: as in adult , in adolescence the main stay of androgen excess treatment has been the COCP, as the majority of these girls have some ovarian dysfunction , if this insufficient to gain control of hair growth then the use of cyproterone acetate & spironolactone may be consider other measures include hair removal by shaving, waxing & electrolysis & the use of bleaching to change hair color there by gaining cosmetic benefit.

22. Thank you