Transcript Pediatrics-And-Adolesence-Gynecology-(GroupA)

PEDIATRIC AND
ADOLESCENCE
GYNECOLOGY
SUPERVISOR : PROF. AYMAN SHAAMASH
DONE BY : GROUP A
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Group A
INTRODUCTION
▪ An awareness of the problems that are unique to pediatric and
adolescent gynecology is invaluable for proper management
of the young patient .
Particular care is essential in addressing gynecologic concerns in
emotional trauma this age group because both physical and
may be inadvertently inflicted .
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Group A
A. Normal findings in a pediatric patient
. A mucoid vaginal discharge
. An introitus that is located more anteriorly than normal and a
clitoris that is more prominent than normal ( 1 to 2 cm )
. A redundant hymen that may protrude on straining and that
remains essentially the same size until 10 years of age
. A vaginal epithelium that is uncornified and erythematous with
an alkaline pH
. A small uterus ( 2.5 to 3 cm ) in length
. A cervical os that is covered with glandular epithelium and
normally appears red ( ectropion )
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Group A
B. Normal findings in an adolescent patient
. Intact hymen in those not sexually active
. Postpubertal gynecologic examination in
an adolescent is similar to an adult
female .
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C. Visualization of the vagina
. Instruments for visualizing the vagina include the vaginoscope ,
the urethroscope , and the pediatric speculum . To determine
the presence or absence of internal genitalia , ultrasound and
MRI are helpful .
D. Rectal examination
. Rectal examination is often more informative than a vaginal
examination .
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Vulvovaginal Lesions
A. Lichen sclerosus et atrophicus :
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Group A
A. Lichen sclerosus et atrophicus
. Clinical picture : vulvar itching . A white , papular lesion
resembling leukoplakia may cover the vulva and perianal
regions
. Etiology : Causes are unknown .
. Diagnosis : Biopsy
. Management : This condition is benign and can be
Self-limiting . Improved hygiene is the first line of therapy .
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B. Trauma
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B. Trauma
. Clinical picture
Tears , abrasions , ecchymoses , and hematomas are common in
preadolescent girls . The incidence is highest in children
between 4 and 12 years of age .
The most common mechanism of injury are sexual abuse,
straddle injuries , accidental penetration , sudden abduction
of the extremities , and pelvic fractures .
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Group A
Management
. A vaginoscope is used to visualize the vagina to locate sources
of bleeding . A large vaginal laceration may result in an
expanding hematoma in the retroperitoneal space . Superficial
abrasions and lacerations of the vulva , if not actively bleeding
, can be cleaned and left alone .
. Conservative therapy .
. In sexual abuse , antibiotic therapy is advised as prophylaxis
against sexually transmitted diseases .
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C. Labial agglutination
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C. Labial agglutination
• Clinical picture Adhesion of the labia minora in the midline is the
usual presentation. This vertical line of fusion distinguishes labial
agglutination from imperforated hymen or vaginal atresia. The
agglutination encourages retention of urine and vaginal secretions and
can lead to vulvovaginitis or urinary tract infection.
• Management
• If a symptomatic, improved hygiene may be all that is necessary.
• Treatment is indicated if there is a chronic vulvovaginitis or
difficulty urinating.
• Lubrication of the labia with a bland ointment.
• Topical estrogen.
• Surgical separation is rarely necessary.
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Group A
Prolapsed Urethra
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• Clinical picture A small, hemorrhagic, friable mass
surrounding the urethra is the most common
presentation.
• Management
• If voiding is uninhibited, local therapy.
• Topical estrogen and sitz bath are the
mainstays of therapy. The prolapse usually
resolves after 4 weeks of therapy.
• If urinary tract retention or necrosis is present,
surgical repair and catheterization are
necessary.
Group A
E. Vaginal Discharge
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Group A
Clinical Picture
• A mucoid discharge is common in infants for up 2 weeks
after birth; it result from maternal estrogen. It is also a
common finding in prepubertal girls, who experience
increased estrogen production by maturing ovaries.
• Pathologic discharge may result from any of the following
conditions:
– Infections with organisms, such as E.coli, Proteus,
Pseudomonas.
– Hemolytic streptococcal vaginitis.
– Monial vaginitis.
– A foreign body.
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Managment
• Conservative management is advisable, as follows:
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Culture to identify causative organisms.
Urinanalysis to rule out cystitis.
Review proper hygiene.
Perianal examination with transparent tape to test for
pinworms.
• In cases of persistent discharge, examination under
anesthesia is indicated to rule out foreign body.
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III. Neoplasms
A. Tumors of the vagina
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Group A
Clinical Picture
• It appears as an edematous, grape-like mass
that bleeds readily on touch. It is usually
multi-centric and extension is usually local,
with rare instances of distant metastases.
• Management A combination of surgery and
chemotherapy is most commonly used.
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Group A
B. Ovarian Tumors
• Clinical Picture:
• Non-germ cell origin
• Lipoid cell tumors( estrogen producing)
• Granules theca cell tumors(estrogen producing)
• Germ cell origin
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Benign cystic teratomas.
Benign cysts.
Arrhenoblastomas (androgen producing).
Dysgerminomas and gonadoblastomas ( tumors of
dysgenetic gonads).
• Endodermal carcinomas ( human chorionic gonadotropin
[hcg]- secreting tumors).
• Immature teratomas.
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Managment
• Treatment is surgical, alone or in combination
with chemotherapy, depending on the tumor.
Radiation is sometimes used to treat
dysgerminomas.
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Congenital
Anomalies in the
Pediatric Patient
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A. Mullerian agenesis (Mayer-von RokitanskyKauster-Hauser [MRKH] syndrome)
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Clinical pictures
Amenorrhea
Ovarian development is normal
The uterus is usually absent
Vaginal agenesis(normal 46xx karyotype)
Management
Surgical creation of neovagina
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B-Ectopic ureter with vaginal
terminus
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Clinical pictures
• Ectopic ureter,the most common cause of vaginal cysts in
infants, presents as uretrocele,which appears as cystic mass
protruding from the vagina.
• Irritation
• Vaginitis may be presenting signs
• Hydroureter
• Hydronephrosis
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Diagnosis
• Intravenous pyelography, which allows visualization of the
entire urinary tract .
Management
• Resect the lowest portion of the ureter and implant
it into the bladder .
• Remove the ureter and associated portion of the kidney
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C-vaginal ectopic anus
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Clinical picture
• Imperforate anus associated with rectovaginal communication
Management
• Surgical correction is indicated
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V-Developmental defects of the External
Genetalia (Ambiguous Genitalia)
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• Ambiguous genitalia results when hormones
with androgenic activity are present during
development of female fetus,or androgens or
androgen activity are absent during
development of male fetus.
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A-Congenital adrenal hyperplasia (CAH)
• Cortisol and aldosterone pathways are impaired .
• The 21-hydroxylase defect is the most common cuse of
distinct virilization of the female newborn.
• Its incidence is 1 in 5000 births,and it accounts for 95% of all
cases of congenital adrenal hyperplasia, which is inherited as
an autosomal recessive trait.
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Clinical picture
• The chromosomes,gonads,and internal genitalia are
female,but the external genitalia are virilized to varying
degrees.
• Urogenital orifice closed .
• Clitoral enlargement and accentuation of labial folds are
characteristic .
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Diagnosis
• Serum17-hydroxyprogestrone and dehydroepiandrosterone
(DHEA) obtained after 24 hours of life are both elevated.
• A blood karyotype
• Serum electrolytes should be followed because salt wasting.
Management
• Hydrocortisone.
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B-Adrenal tumors
• These tumors,which may cause virilization of the external
genitalia after infancy, should be suspected in children with
high levels of dehydroepiandrosterone sulfate (DHEAS).
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C-maternal ingestion of androgenic
substances
• This condition can result in masculinization of the female
fetus. Causal agents identified include androgens,danazol,any
synthetic progestins(in doses much higher than in oral
contraceptive pills).
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Clinical picture
• The clitoris is enlarged and the labia may be fused, but the
vagina, tubes, and uterus are normal .
• Growth and development are normal, and progressive
viritization does not occur.
Diagnosis
• The condition can be diagnosed based on positive history and
on exclusion.
Management
• Clitoral reduction and surgical correction of the fused labia
maybe necessary.
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D-childhood ingestion of androgens
• This condition usually involves preparations that have
androgenic activity.
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Group A
Clinical picture
• Clinical manifestations are the same as those resulting from
maternal ingestion of androgenic substances (i.e.,
masculinization).
Management
• Therapy involves clitoral reduction and surgical correction of
the fused labia ,if necessary.
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E-Androgen insensitivity
syndrome
(testicular feminization)
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Clinical picture
• A 46xx genotype is present, but a female phenotype develops.
• Androgens are produced by the testes (which develop in the
presence of y chromosome) and by adrenal glands.
• External genitalia are feminized
• No uterine,cervical,upper vaginal, or fallopian tube
development.
• A short vagina .
• Labia,may contain testes.
• An incomplete form (reifenstein syndrome) occurs in which
external genitalia appear virilized.
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Diagnosis
• Primary amenorrhea.
• A blind or absent vagina.
• Lack of pubic and axillary hair development.Breast
development does occur because estrogen concentration is
high due to conversion of testosterone produced after
puberty.
• A karyotype .
• This syndrome is distinguished from MRKH by absent axillary
and pubic hair development and high testosterone level.
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Management
• The gonads should be removed because of an incresed risk of
malignancy (3% to 4% before 25 years of age )
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F. True hermaphroditism
Clinical picture
• The genotype is 46xx
• The external genitalia may appear male,female,or ambiguous.
• Both male and female internal genitalia may be present.
Management
• The genitlia that are inconsistent with sex assignment should
be surgically removed or modified.
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G-maternal virilizing tumor during
pregnancy(luteoma of pregnancy)
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Group A
• This condition may result in masculinization of the female
fetus. The clinical picture and therapy are similar to those for
the maternal ingestion of androgenic substances.
• Psychological development and mental capacity are consistent
with choronologic age.Reproductive potential is not adversely
affected,and the patient can become pregnant.
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Normal Pubertal Development
• Increased production of lutenizing hormone and folliclestimulating hormone,as other factors(such as leptin), is
responsible for the irritiation of the pubertal process.
• Growth spurt.begins before the onset of other signs of the
puberty. Usually 1 year before menarch.
• Thelarche. The onset of breast development usually begins
between 9 and 11 years of age . It is a sign of ovarian estrogen
production. Tanner stages describe the normal changes in the
transition from the pubertal to the mature breast contour.
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• Adrenarche and pubarche. Adrenarche refers to the
production of androgens from the adrenal gland,and
pubarche is the development of axillary and pubic hair that
results from the adrenal and gonadal androgens. Pubarche
usually follows thelarche in the pubertal .
• Menarche. In menarche,vaginal bleeding occurs in response
to hormonal changes ,specifically production of estrogen by
the ovary, for the first time. The average age of the first
menses is 12 to 13 years.
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Abnormal pubertal development
• Delayed puberty: is characterized by the
absence of breast development by age 13
years or the absence of menses by age 16
years
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• Hypergonadotropic hypogonadism : this layed
condition affects almost 50 % of all patients
with delayed puberty. It includes conditions in
which the ovaries or gon ads are not
functioning and are unable to respond to
gonadotropins as a result gonadotropin levels
are high .
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• Hypogonadotropic hypogonadism :
This condition accounts for 10% to 15% of
patients with pubertal delay . The ovary is
normal ,however, there is a lack of production
of
hormonal
stimulation
from
the
hypothalamus .
hypogonadotropic
hypogonadism
includes
Kallmann syndrom (isolated GnRH deficiency )
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• Eugonadotropic: constitutional delay accounts
for 10% to 20% of cases .
These patients have normal progression of the
stages of puberty
The initiation of the process is simply delayed
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Managment
• Treatment is based on the etiology of the
delay . In cases of gonadal dysgenesis when a
Y chromosome or fragment of a Y
chromosome is present , the gonads should be
removed at diagnosis because of the risk of
neoplastic degeneration . Otherwise ,
hormone replacement with estrogen and
subsequently both estrogen and progesterone
, is required to promote sexual development
and menarache.
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Special problems of the adolescent
• A. Dysmenorrhea :
Dysmenorrhea is defined as cramp –like pain in
the lower abdomen associated with menstrual
flow .
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Etiology
• Primary dysmenorrhea accounts for most cases
and is attributed to increased prostaglandin
production with menses in the presence of
normal anatomy .
• Secondary dysmenorrhea results from conditions
such as endometriosis and anomalies with
obstruction in a portion of the outflow tract
which leads to pain in the obstructed segment
but menstrual flow is not affected .
• Examples include obstructed hemiuterus and
uterus didelphys with obstructed hemivagina
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Clinical picture
• Sever , cyclic , cramp-like pain located in the
lower abdomen and pelvis is associated with
menses
• Pain may radiate to the thighs and back and
be accompanied by nausea , vomiting , and
diarrhea
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Managment
• First-line therapy is prostaglandin inhibitors
(nonsteroidal anti-inflammatory drugs [NSAIDs]
• If the symptoms are not adequately : start oral
contraceptives or obtain a pelvic ultrasound to
evaluate for abnormal anatomy depending on the
loction,progression,andseverityofthesymptoms
• Laparoscopyisrecommended if medical treatment
fails to control the symptoms .
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B. Dysfunctional uterine bleeding
• DUB is defined as excessive , prolonged , or
irregular bleeding not associated with an
anatomic lesion.
• Most adolescent girls have anovulatory
menstrual periods for the first 2 to 3 years
following menarche .
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Etiology
• The cause of DUBin 75% of cases is an
immature hypothalamic –pitutary axis
resulting in anovulation
• Other cases include psychogenic factors
,juvenile hypothyrodism and coagulation
disorders (von willebrand disease )
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Clinical picture
• Menometrorrhagia ( irregular , heavy
bleeding) is the most characteristic symptom .
• Bleeding can be prolonged and heavy in some
cases leadng to sever anemia .
• The condition is usually self – limited
• Management
Cyclic hormonal manipulation with progestins or
comined oral contrceptive pills .
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Group A
Thank You
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