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PEDIATRIC & ADOLESCENT

GYNECOLOGY

FRANCISCO, BIANCA DENISE D.


OLFU CLINICAL CLERK
BULACAN MEDICAL CENTER DEPARTMENT OF OBSTETRICS AND GYNECOLOGY
OCTOBER 2019 ROTATOR
OBJECTIVES:

• To discuss the most commonly encountered


pediatric gynecology cases
• To provide a guideline for physical examination,
diagnosis and usual management of pediatric
gynecologic patients
GYNECOLOGIC VISIT & EXAMINATION OF A
CHILD
•The most common gynecologic condition of
children is vulvovaginitis.
• Other commonly seen diagnoses at a
pediatric gynecology visit include labial
adhesions, vulvar lesions, suspicion of sexual
abuse, and genital trauma.
SUCCESSFUL GYNECOLOGIC EXAMINATION
OF A CHILD
• pace that conveys both gentleness and patience with the time
spent and not seem to be hurried or rushed
• physician should sit, not stand, during the initial encounter
• interruptions should be avoided.
• speculums and instruments that might frighten a child or parent
should be within drawers or cabinets and out of sight.
PERFORMANCE OF THE
GYNECOLOGIC EXAM IN A CHILD
• child should be reassured that the examination will not hurt.
• give the child a sense that she will be in control of the examination
process.
• emphasize that the most important part of the examination is just
“looking” and there will be conversation during the entire process.
• one needs the cooperation of the patient and a medical assistant.
• assure the adult that has accompanied the child that speculums are
not part of the examination.
• defer the genital examination until a second visit
PERFORMANCE OF THE
GYNECOLOGIC EXAM IN A CHILD
• A child should never be restrained for a gynecologic examination.
• Often reassurance and sometimes delay until another day are the
best approaches.
• most important technique to ensure cooperation is to involve the child
as a partner.
• Draping for the gynecologic examination may produce more anxiety
than it relieves and is unnecessary in the preadolescent child.
• handheld mirror may help in some instances when
• discussing specifics of genital anatomy.
PERFORMANCE OF THE
GYNECOLOGIC EXAM IN A CHILD
• FIRST ASPECT of the pelvic examination is
evaluation of the external genitalia
• An infant may be examined on her mother’s
lap.
• Young children may be examined in the frog
leg position
• children as young as 2 to 3 years of age may
be examined in lithotomy with use of stirrups.
• Lithotomy is generally used for girls 4 to 5
years of age and older.
•SECOND PHASE of the examination involves evaluation of the vagina.
•can be accomplished without the use of any insertion of instruments
•One method is to utilize the knee chest position. Oto/ophthalmoscope is
used as a magnifying instrument and light source but is not inserted into
the vagina.
NORMAL FINDINGS: HYMEN AND VAGINA OF
PREPUBERTAL CHILD

Figure 12.3 Types of hymens. A, Crescentic. B, Annular. C, Redundant.


• A mounding of hymeneal tissue is often called a bump.
• Bumps are usually a normal variant and are often
attached to longitudinal ridges within the vagina.
D, Microperforate. E, Septated. Hymens in newborns are estrogenized, resulting in a
pink thick elastic redundancy.
• Older un-estrogenized girls will have thin non-elastic
hymens with significant signs of vascularity.
Not every variant of hymen is normal and transections
between 3 and 9 o’clock should raise a suspicion for
abuse, as these are likely acquired.

F, Imperforate. G. Hymeneal tags.


NORMAL FINDINGS: HYMEN AND VAGINA OF PREPUBERTAL CHILD
• The vaginal epithelium appears redder and thinner
• The vagina is 4 to 6 cm long
• secretions have a neutral or slightly alkaline pH; is narrower, thinner, and lacks the distensibility of
the vagina
• Recurrent vulvovaginitis, persistent bleeding, suspicion of a foreign body or neoplasm, and
congenital anomalies may be indications to perform vaginoscopy and examine the inside of the
vagina.
• Vaginoscopy most often requires sedation with a brief inhalation or intravenous anesthetic, but it
can also be performed in the office with older, cooperative children in select circumstances.
• The ideal pediatric endoscope is a cystoscope or hysteroscope because the accessory channel
facilitates the retrieval of foreign bodies as well as vaginal lavage.
• Local anesthesia of the vestibule may be obtained with 2% topical viscous lidocaine (Xylocaine) or
longer- acting products such as lidocaine/prilocaine.
• The LAST STEP in the pelvic examination may be a rectal examination.
• Common reasons to perform a rectal examination include genital tract
bleeding, pelvic pain, and suspicion of a foreign body or pelvic mass
• The child should be warned that the rectal examination will feel similar to
the pressure of a bowel movement
• The normal pre pubertal uterus and ovaries are non- palpable on rectal
examination
• The relative size ratio of cervix to uterus is 2 to 1 in a child, in contrast to
the opposite ratio in an adult.
THE OFFICE VISIT AND
EXAMINATION OF THE
• Before puberty, the girl’s reproductive organs are in a
ADOLESCENT FEMALE resting, dormant state.
• Pubertal changes are frequently a cause of concern for
adolescent females and their parents, the gynecologist
must offer the adolescent female an empathetic, kind,
knowledgeable, and gentle approach.
• The critical factors surrounding the pelvic examination of
a female adolescent are different from those of
examinations of children 2 to 8 years old.
• Many female adolescents do not want their mother,
guardian, or other observers in the examining room.
In many adolescent gynecology visits, a full pelvic exam is
unnecessary (Lara-Torre, 2008).
• Patient in early adolescence (12 to 14 years of age) may behave and need
similar support as those in the prepubertal stages.
• They may ask for their mothers to be there, be fearful of the examination
concept, and need more than one visit to achieve the goals of the visit.
• Those in middle or late adolescence (15 to 19 years of age) may be more
acceptable to the idea of an exam and more likely to cooperate with the
proper counseling and in the appropriate setting (ACOG, 2011).
• Teens should be assured that although the exam may include mild
discomfort, it is not painful
• Allowing them to see and touch the instruments also may assist in
demystifying the exam and allow for it to flow more smoothly.
• Use of the “extinction phenomenon” may be helpful in this setting. The
examiner provides pressure lateral to the introitus on the perineum prior
to insertion of the speculum.
PROBLEMS IN
PREPUBERTAL CHILDREN
VULVOVAGINITIS

• most common gynecologic problem in the prepubertal female.


• Classic symptoms of vulvovaginitis: introital irritation (discomfort/pruritus) or
discharge (Farrington, 1997).
•The prepubertal vagina is neutral or slightly alkaline.
•With puberty the prepubertal vagina becomes acidic under the influence
of bacilli dependent on a glycogenated estrogen-dependent vagina.
•Breast budding is a reliable sign that the vaginal pH is shifting to an acidic
environment.
PATHOPHYSIOLOGY:
• primary irritation of the vulva, which may be accompanied by
secondary involvement of the lower one third of the vagina.
• Most cases involve an irritation of the vulvar epithelium by normal
rectal flora or chemical irritants
NONSPECIFIC VULVOVAGINITIS PREDISPOSING FACTORS:
• use of perfumed soaps
• the pressure from tight seams of jeans or tights which create
denudation, allowing the rectal flora to easily infect the irritated
epithelium.
OTHER CAUSES:
 group A or group B β-hemolytic streptococci, Haemophilus influenzae, and Shigella boydii
 Neisseria gonorrhoeae, Trichomonas vaginalis, and Chlamydia trachomatis may also be responsible
in cases associated with abuse but are significantly less common.
 PINWORMS are another cause of vulvovaginitis in prepubertal children. (Enterobius vermicularis)
 Classic symptom of pinworms is nocturnal vulvar and perianal itching.
 At night the milk-white, pin-sized adult worms migrate from the rectum to the skin of the vulva
to deposit eggs.
 They may be discovered by means of a flashlight or by dabbing of the vulvar skin with clear
cellophane adhesive tape ideally before the child has arisen in the morning.
 The tape is subsequently examined under the microscope.
 Mycotic vaginal infections may be seen in immunosuppressed prepubertal girls such as HIV
patients, diabetic children, or patients on chronic steroid therapy. It can also present in
patients using diapers as a chronic colonization (diaper rash). Systemic diseases, chicken
pox, and herpes simplex infection.
PHYSIOLOGICAL CAUSES BEHAVIORAL CAUSES
• vulva and vagina are exposed to bacterial contamination from the
rectum
•child lacks the labial fat pads and pubic hair of the adult, when a • major factor in childhood vulvovaginitis is poor perineal hygiene
child squats, the lower one third of the vagina is unprotected and •youngsters wipe their anus from posterior to anterior and thus
open inoculate the vulvar skin with intestinal flora
•no significant geographic barrier between the vagina and anus •minor vulvar irritation may result in a scratch- itch cycle, with the
•vulvar and vaginal epithelium lack the protective effects of estrogen possibility of secondary seeding because children wash their hands
and thus are sensitive to irritation or infection infrequently
•labia minora are thin and the vulvar skin is red because the •children’s clothing is often tight fitting and non- absorbent, which
abundant capillary network is easily visualized in the thin skin keeps the vulvar skin irritated, warm, moist, and prone to
•vaginal epithelium of a prepubertal child has a neutral or slightly vulvovaginitis.
alkaline pH, which provides an excellent medium for bacterial •nonspecific vulvovaginitis may be caused by carrying viral
growth. infections from coughing into the hands directly to the abraded
•vagina of a child lacks glycogen, lactobacilli, and a sufficient level of vulvar epithelium
antibodies to help resist infection. •a child with an upper respiratory tract infection may auto-inoculate
•normal vagina of a prepubertal child is colonized by an average of her vulva
nine different species of bacteria: 4 aerobic and facultative anaerobic
species and 5 obligatory anaerobic species.
Signs & Symptoms:
• first awareness comes when the mother notices staining of the
child’s underwear
• child complains of itching or burning
• quantity of discharge can be minimal to copious, color ranges
from white or gray to yellow or green
• bloody and purulent discharge is likely not from vulvovaginitis
but from a foreign body
• patients infected with some pathogens, particularly Shigella
boydii, often present with a bloody or blood-tinged discharge
• vulvar erythema, edema, and excoriation – non diagnostic signs
Differential diagnosis of persistent or recurrent vulvovaginitis
not responsive to treatment:
• foreign body
• primary vulvar skin disease (allergic or contact dermatitis)
• ectopic ureter
• child abuse
• If the predominant symptom is pruritus, then pinworms or an
irritant/nonspecific vulvitis is the most likely diagnosis.
• The vulvar skin of children may also be affected by systemic skin diseases,
including lichen sclerosus, seborrheic dermatitis, psoriasis, and atopic
dermatitis.
• The classic perianal “figure-8” or “hourglass” rash is indicative of lichens
scleroses with white patches and in some cases local trauma.
• An ectopic ureter emptying into the vagina may only intermittently
release a small amount of urine; thus this rare congenital anomaly
should be considered in the differential diagnosis in young children.
TREATMENT OF VULVOVAGINITIS
 The child should be instructed to void with her knees spread wide apart (even while facing
the toilet to improve urine draining) and taught to wipe from front to back after defecation
 Loose-fitting cotton undergarments should be worn.
 Chemicals that may be allergens or irritants, such as bubble bath, must be discontinued
 Harsh soaps and chemicals should be avoided
 use nonmedicated, non-scented wipes rather than toilet paper may prevent the self-
inoculation of the vagina with small pieces that can initiate a chronic discharge
 majority of symptoms improve with hygienic changes and sitz baths (warm water, no soaps
or chemicals)
 Utilizing this approach for a 2-week period should resolve most symptoms in patients with
nonspecific vulvovaginitis.
SPECIMEN COLLECTION:
• very slim urethral Dacron swab moistened with non-
• When intervention fails, the suspicion for
bacteriostatic saline (used for collection of male
bacterial colonization is greater and a
reasonable approach is the use of broad- urethral cultures).
spectrum oral antibiotics such as • catheter within a catheter, uses a No. 12 red rubber
AMOXICILLIN or TRIMETHOPRIM bladder catheter for the outer catheter and the hub end
SULFAMETHOXAZOLE given for 10 to 14 days of an intravenous butterfly catheter for the inner
• Without continuation of the hygiene catheter .The outer catheter serves as an insulator, and
measures, then broad-spectrum antibiotics the inner catheter is used to instill a small amount of
will only offer temporary relief, and the saline and aspirate into the vaginal fluid.
problem is likely to recur (Bercaw-Pratt, • The results of the vaginal culture may demonstrate a
2014)
single organism that is a respiratory, intestinal, or
• Collect a culture of the vulvovaginal
discharge prior to initiation of the antibiotics. sexually transmitted disease pathogen
If unable to collect a specimen an empiric • The presence of sexually transmitted organisms in a
treatment may be started child is usually a strong indication that sexual abuse
may have taken place and appropriate referral and
follow-up is necessary
OTHER PUBERTAL
GYNECOLOGIC PROBLEMS
LABIAL • literally mean the labia minora have adhered or agglutinated together at the
midline.
ADHESIO • Another term sometimes used is adhesive vulvitis.
NS • Denuded epithelium of adjacent labia minora agglutinates and fuses the two labia
together, creating a “flat appearance” of the vulvar surface. A telltale somewhat
translucent vertical midline line is visible on physical exam at the site
agglutination.
•Thin, narrow line in a vertical direction is pathognomonic for labial adhesions
•Labial adhesions are often partial and only involve the upper or lower aspects of the
labia
•Small adhesions are common in preschool children, and perhaps as many as 20%
will have some degree of labial adhesions on routine examination (Bacon, 2015)
•Inexperienced examiners may confuse labial adhesions for an imperforate hymen or
vaginal agenesis
•Physical exam findings are significantly different, all of these conditions may
occlude the visualization of the vaginal introitus.
•In the patient with an imperforate hymen, the labia minora normally appear like an upside
down V, and no hymeneal fringe is visible at the introitus.
•In vaginal agenesis, the hymeneal fringe is typically normal, but the vaginal canal ends blindly
behind the hymeneal fringe.
•Labial adhesions are most common in girls between 2 and 6 years of age, with up to 90% of
cases occurring before age 6.
•Estrogen reaches a nadir during this time, predisposing the non-estrogenized labia to
denudation.
•In the most advanced cases, there is fusion over both the urethral and the vaginal orifices.
• It is extremely rare for this fusion to be complete, and most children urinate through openings
at the top of the adhesions, even when the urethra cannot be visualized (pinpoint opening).
•partially fused labia may form a pouch in which urine is caught and later dribbled, presenting
as incontinence.
•Most patients will be asymptomatic or present with intermittent dysuria
•Some children will present with symptoms and may include voiding difficulties,
dysuria, frequent urinary infections, urine dribbling after voiding, recurrent
vulvovaginitis, discomfort from the labia pulling at the line of adhesions, and in rare
cases bleeding from the line of adhesion pulling apart.
•It is very painful, and the raw edges are likely to adhere again as the child will be
reticent to allow application of medication after being subjected to this degree of
pain.
•Even with local anesthesia, such as lidocaine ointments
or creams, the potential pain and traumatic experience for the child should deter
from this intervention, except in the well-motivated, mature child
the combination of labial adhesions and scarring of the posterior fourchette,
especially in children with new- onset labial adhesions after age 6, should prompt
the clinician to consider sexual abuse in the differential diagnosis.
RECURRENCES ARE COMMON
TREATMENT:
 In asymptomatic patients is observation (Bacon, 2015)
 Most of the time treatment requests are driven by the parental concern of a closed vagina and their
interpretation that this may lead to an inability to have children in the future or engage in intercourse
 If spontaneous separation does not occur at puberty, and manual separation is required, the presence of
a better estrogenized skin will decrease the chances of recurrence which in children can range from
25% to 65%
 Attempts to separate the adhesions apart in the office by pulling briskly on the labia minora should not
be done most commonly utilized treatment of this condition is topical estrogen cream applied onto the
labia two times per day at the site of fusion. This will usually result in spontaneous separation, usually in
approximately 2 to 8 weeks
 when resolution takes longer than several weeks, the clinician can reexamine the patient
 If increased pigmentation is noted lateral to the midline line of agglutination, the caregiver should be
reinstructed to apply the cream to the line, as the lateral pigmentation indicates the estrogen is being
applied lateral to the actual adhesion.
The action of estrogen as well as the application over the adhesion line itself
makes the treatment more effective for more than 6 to 8 weeks
Prolonged use of topical estrogen has been associated with breast budding
and in some less common cases vaginal bleeding from the peripheral effects
of the absorption of estrogen.
Failure of separation within the normal time frame should trigger alternate
treatment.
When patients fail estrogen therapy, and symptoms persist, the use of topical
CORTICOSTEROIDS TWICE A DAY FOR 6 TO 8 WEEKS has also shown
adequate results and can be consider as a first or secondary line of treatment
Once the condition has been resolved,recurrence can often be prevented by
applying a BLAND OINTMENT (SUCH AS ZINC OXIDE CREAM OR PETROLEUM
JELLY) to the raw epithelial edges for at least 1 month or even longer.
Physiologic discharge of puberty
• gray-white coloration, although it may appear slightly yellow but is not
purulent
• represents desquamation of the vaginal epithelium.
• The estrogenic environment allows acid-producing bacilli to become part of
the normal vaginal ecosystem
• The acids the bacilli produce cause a desquamation of the prepubertal
vaginal epithelium.
• Under the microscope, sheets of vaginal epithelial cells are identified.
• Little symptomatology is associated with this discharge.
• Occasionally the thickness of the discharge causes the vulva to be “pasted”
to undergarments and causes some symptoms of irritation and erythema
• Usually the only treatment necessary is reassurance of both mother and
child that this is a normal physiologic process that will subside with time.
• Symptomatic children may be treated with sitz baths
and frequent changing of underwear.
URETHRAL PROLAPSE
• most common presentation is not urinary symptomatology but
prepubertal bleeding
• Often a sharp increase in abdominal pressure, such as coughing,
precedes the urethral prolapse
• On examination, the distal aspect of urethral mucosa may be
prolapsed along the entire 360 degrees of the urethra
• This forms a red donut-like structure. The prolapse may be partial
or incomplete, presenting as a ridge of erythematous tissue.
• It is critical to distinguish this from grapelike masses of sarcoma
botryoides that originate from the vagina. Occasionally the
prolapse becomes necrotic and blue- black in color.

Treatment:
• conservative and nonsurgical
• topical estrogen has been found effective
• surgery is seldom necessary, except in rare
cases where necrosis is obviously present.
LICHEN SCLEROSUS
• Lichen sclerosus (LS), or lichen sclerosus
atrophicus, is a skin dystrophy most commonly
seen in postmenopausal women and pre-
pubertal children.
• cause is unclear, it may be associated with
autoimmune phenomena.
• Histologically, there is thinning of the vulvar
epithelium with loss of the rete pegs
• most common symptoms are pruritus and
vulvar discomfort
• Other presentations may include prepubertal
bleeding from trauma, constipation, and
dysuria (Bercaw-Pratt, 2014)
• The appearance of LS varies, but lesions are
always limited by the labia majora.
"
TREATMENT:
• always start with avoiding irritation or trauma to the
genital epithelium.
• Children should be encouraged to avoid straddle
activities such as bicycle or tricycle riding when
symptomatic
• Patients should clean the labia by soaking in sitz
baths.
• Parents sometimes may assume lack of cleanliness is
contributing to the disorder and scrub the area with
soap, which may actually exacerbate the disease.
• Tight clothing such as jeans or tights may also
abrade and irritate the vulva
• The North American Society of Pediatric and
Adolescent Gynecology (NASPAG) recommends
the use of high-potency steroids such as
CLOBETASOL as the initial step in treatment of this
condition.
• Tapering the steroid level should be considered as soon as a
response is seen or within a 4- to 6-week interval
• The tapering can be achieved by following the initial treatment with a
2- to 3-week of midpotency steroid such as BETAMETHASONE and
conclude with 1% HYDROCORTISONE for another 2 weeks The use
of ointments is preferred over creams given there is less irritation
compared with creams and the petroleum base of ointments appear
to help it stay in place longer.
• The parents should apply the drug sparingly but consistently,
avoiding application to nonaffected areas to prevent systemic effects
of the drug such as adrenal suppression.
• Recurrence or “flares” of the condition are common and continue for
a significant period of time in most patients.
PREPUBERTAL BLEEDING WITHOUT SECONDARY SIGNS
OF PUBERTY
•Approximately one third of the African- American girls had thelarche or adrenarche at age
7 and almost 50% by age 8.
•Approximately 15% of white girls had initiated puberty by age 8 and almost 40% by age 9.
•Mean ages for thelarche and adrenarche were 8.9 and 8.8 for African-American girls and 10
and 10.5 years for white girls, respectively.
•The mean age of menarche was almost 12.2 years for the African-American girls compared
with 12.8 years in the white girls.
•In girls younger than 8 with CNS or behavioral issues, a pathologic etiology of development
should be entertained. A common clinical problem that is sometimes mistaken for
precocious puberty is prepubertal bleeding in children without any other signs of puberty
such as breast development
VAGINAL BLEEDING
• In children with prepubertal bleeding without breast budding, there is almost never an endocrinologic cause, with the
exception being a rare presentation of McCune- Albright syndrome (polyostotic fibrous dysplasia) or the uncommon
presentation of isolated premature menarche
• DIFFERENTIAL DIAGNOSIS of vaginal bleeding without pubertal development includes foreign body, vulvar
excoriation, lichen sclerosus, shigella vaginitis, separation of labial adhesions, trauma (abuse and accidental), urethral
prolapse, and friable genital warts
• Often also include accidental estrogen exposure (for example, from ingestion of a mother’s birth control pills or
prolonged used of estrogen topical therapy)
• Rare causes include malignant tumors (sarcoma botryoides and endodermal sinus tumors of the vagina) and an
unusual presentation of McCune-Albright syndrome
• Neonates may develop a white mucoid vaginal discharge or a small amount of vaginal spotting because of the
withdrawal of maternal estrogens
• The discharge and vaginal spotting are self-limited
• It should be remembered that although the differential diagnosis of prepubertal bleeding includes sexual abuse, most
sexually abused children do not have prepubertal bleeding
• In some settings, such as emergency departments, it is more likely that prepubertal bleeding is due to sexual abuse
than in primary pediatric office or a tertiary referral practice.
FOREIGN BODIES
• majority of foreign bodies are found in girls between 3 and 9 years of
age; history is usually not helpful because an adult has not witnessed,
nor does the child remember
• most common are small wads of toilet paper
• Other common foreign objects include small, hard objects such as
hairpins, parts of a toy, tips of plastic markers, crayons, and sand or
gravel
• Some of these objects are not radiopaque
• When small swabs are used to perform vaginal cultures, the examiner
may note an odd sensation of touching something other than vaginal
mucosa
• Objects such as coins and plastic toys are often easily visible on vaginal
examination, especially in the knee- chest position
• Children may insert foreign bodies because the genital area is pruritic
or when naturally curious children are exploring their bodies.
•CLASSIC SYMPTOM is a foul, bloody vaginal discharge. Discharge is often purulent
and without blood
•The natural history probably reflects the object initially causing irritation,
creating a purulent discharge, and then as the object imbeds itself into the vaginal
epithelium, bleeding and spotting may occur
• The foreign body may become partially “buried” or imbedded within the vaginal
wall. It is difficult to remove without discomfort and may require sedation.
•Unexplained vaginal bleeding is an indication for a vaginoscopy. Especially in
children younger than 6 years of age, without of signs of an endocrinopathy
(breast budding, estrogenization of the hymen), this should be done expeditiously
to rule out malignant vaginal tumors
•Children who insert foreign objects often have recurrences. This may be
secondary to persistent pain or pruritus in the genital area that was not addressed
at the initial encounter, and the child uses the object (solid or toilet paper) to rub
or scratch the genital area.
MANAGEMENT:
 If an object is seen on exam, the clinician may be able, in a
cooperative child, to either grasp the object with a forceps or
wash the object out by irrigation.
 Catheter technique may be utilized.
 Use of a pediatric feeding tube with room
temperature or warmed saline can also be used to
“flush” the vagina.
 Care should be taken to minimize contact with the
hymen, as it is a sensitive area at this age and the sensation can be
enough for the child to stop cooperating with the procedure.
 In many instances this is not possible because the child
cannot cooperate or because a solid object is imbedded into the
vaginal wall. In these cases the object can be removed at
vaginoscopy.
 If the foreign object is toilet paper, then having the child use
wipes instead of toilet paper may reduce recurrences.
SHIGELLA VAGINITIS
•Approximately half of all cases of Shigella vaginitis present with prepubertal
bleeding.
•There is generally no concurrent gastrointestinal symptomatology.
•Cultures for Shigella should be strongly considered in any child with no
obvious cause for prepubertal bleeding.
•Rarely, vaginitis caused by other organisms can also present with prepubertal
bleeding.
RARE CAUSES: VAGINAL TUMORS AND MCCUNE-
ALBRIGHT SYNDROME
MCCUNE-ALBRIGHT SYNDROME
" rare somatic mutation that occurs during embryogenesis in neural crest cells.
" The mutation does not occur in the germline, it is not inherited.
" The mutation affects G protein receptors and has a variable expression, depending on how many
early cells are affected (an example of mosaicism).
" GNAS1 gene is the affected area.
atients with the syndrome may manifest the classic triad of café-au-lait spots, abnormal bone lesions, and
precocious puberty.
" Most McCune-Albright patients present with prepubertal bleeding along with thelarche.
" Rarely a child may present with bleeding and no breast budding.
" Examination of the child with prepubertal bleeding should include examination of the skin for café-au-lait
spots, and the historical intake should include queries about frequent bone fractures.
" In cases of unexplained prepubertal bleeding, the possibility of McCune-Albright should be considered,
and serial breast examinations may reveal breast budding.
SARCOMA BOTRYOIDES AND ENDODERMAL
SINUS TUMORS OF THE VAGINA
• " Almost all cases of sarcoma botryoides of the vagina in
• prepubertal children occur prior to age 6 (although cases up until age 8 have been
reported), and endodermal sinus tumors occur prior to age 2.
• " Althoughthesetumorsareextremelyrarecausesof prepubertal bleeding, they must
be considered in every young child.
• " Both are aggressive malignancies, and prompt diagnosis is critical.
" In young children with no evident cause of prepubertal bleeding, a vaginoscopy should be
done to rule out these malignancies. VAGINOSCOPY FOR PREPUBERTAL BLEEDING
WITHOUT SIGNS OF PUBERTY
" Even though many vaginoscopies are negative, it is
especially important for clinicians to perform them promptly in young prepubertal
bleeders to exclude rare but aggressive vaginal malignancies.
ACCIDENTAL GENITAL
TRAUMA
• The usual cause of accidental genital trauma
during childhood is a fall
• 75% percent of accidental trauma to the vulva
and vagina involves straddle injuries
• Sexual abuse is an important consideration in
the differential diagnosis (Bond, 1995).
VULVAR TRAUMA: LACERATIONS AND STRADDLE IN

One of the most common causes of genital trauma in a child is a straddle injury.
Common straddle injuries in children occur on playground climbing structures, such as
a monkey bar, or fence rails and around the edges of pools.
straddle injury generally results in unilateral and superficial injury and rarely involves
the hymen. In cases of hymeneal transection with a history of straddle injury, sexual
abuse should be strongly considered.
If hymeneal transection has occurred, the examiner must confirm that the object has
not penetrated into the vaginal wall, which could result in a dangerous hematoma,
perforation into the cul-de-sac, or perforation of the abdominal cavity with potential
visceral damage.
A vaginoscopy or laparoscopy (or both) is generally required to rule out these
possibilities.
•Perforations into the abdomen may not result in significant vaginal bleeding.
•" In children presenting with trauma and genital bleeding, the examiner must first ascertain the site,
extent, and amount of bleeding.
•" Viscous lidocaine or a longer-acting topical agent such as lidocaine/prilocaine can be applied and
allowed appropriate time to provide anesthesia.
•" Then the area can be gently washed by irrigating with sterile warmed water onto the labial area.
•" Typical lacerations may involve denudation around the urethra or labia.
•" The posterior fourchette is less commonly involved.
•" In children with vulvar trauma, considerations should be
•given to giving a booster injection of tetanus toxoid if the last immunization was more than 5 years
before the trauma.
•" Lacerations that are superficial (equivalent to first- degree obstetric lacerations) generally do not
require repair in contrast to deeper lacerations.
•" Often, superficial lacerations can be adequately treated by applying oxidized cellulose or similar
products to stop the bleeding.
•"
•Slightly deeper lacerations can be repaired with small Steri-Strips.
•" In some deeper lacerations, one well-placed suture will stop substantial bleeding. This
scenario is typical of lacerations on the inferior aspect of the labia minora. Placement of
the suture may be aided by injection of lidocaine in cooperative children or by conscious
sedation in the emergency department.
•" General anesthesia is usually required for diagnosis and treatment of extensive
lacerations and deep lacerations or in children who are unable to tolerate repair in the
office or emergency department.
•" In patients in whom the extent of the laceration cannot be visualized an exam under
anesthesia should be performed to prevent missing deeper lacerations than what is
visualized in the emergency room. While anesthetized, the laceration should be irrigated
and débrided, the vessels ligated, and the injuries repaired.
•" Occasionally it is necessary to perform laparoscopy or an exploratory laparotomy for a
suspected retroperitoneal hematoma or intraabdominal injury.
•" Appropriate consultation with urology or pediatric surgery is recommended when the
extent of the laceration is beyond the vulvar-vaginal areas.
VULVAR HEMATOMAS
• If the vulva strikes a blunt object, a hematoma usually results. The lack of the
mature reproductive woman’s fat pad in the vulvar area predisposes a young child
to bleeding from trauma.
• If the object is sharp, such as a fence post or skating blade, the injury may be a
laceration with the potential for penetration of the perineum and injury to internal
pelvic organs.
• Other common causes of vulvar and vaginal trauma include sexual abuse,
automobile and bicycle accidents, kicks sustained in a fight, and self-inflicted
wounds. The size of vulvar and vaginal hematomas varies widely.
• Initially there is bleeding into the loose connective tissue. When the pressure
from the expanding hematoma exceeds the venous pressure, in most cases the
hematoma will stop growing.
• The treatment of nonexpanding vulvar hematomas is observation by serial
examinations and the use of an ice pack or cool sitz bath and pain medications.
"
SEXUAL ABUSE
HISTORY IN SEXUAL ABUSE
There are two situations in which health care providers need to garner information
regarding potential sexual abuse.
 child or family that presents with potential sexual abuse as the chief complaint.
 child is seen for another complaint, such as a purulent discharge, but the provider
considers the possibility of sexual abuse based on historical information or physical
examination.
 Urgent evaluation is necessary if the abuse has occurred within 72 hours (for
forensic evidence), if the child is currently in a danger of repeated abuse or self-
harm, or for obvious injuries such as lacerations require treatment. If none of these
criteria are encountered, the child and her family can be evaluated on a nonurgent
basis.
 Interview should be performed prior to a genital examination, unless not if there are
compelling medical reasons.
 latency age children may not be able to separate the exam from touching involved
in abuse, making the history more difficult to obtain. in the majority of abused children
the exam is completely normal.
• exam of a potentially sexually abused child should include a general exam.
•" Attention should be directed at evaluating skin for bruising, lacerations, or trauma.
•" Parents or concerned adults should be counseled that a genital exam in children who have been
abused is usually normal.
•" In situations in which abuse has occurred within 72 hours, careful collection of forensic
evidence is important.
•" Collection of all clothing and undergarments is critical. Approximately two thirds of forensic
evidence is obtained from linens and clothing.
•" Motile sperm will be present in the prepubertal vagina for approximately 8 hours, and
nonmotile sperm for approximately 24 hours.
•"
• Because prepubertal children do not have cervical mucous, sperm do not exist for the longer durations
seen in reproductive females within the cervical canal. “Rape” kits will also often include testing for a
protein specific to the prostate.
•" Vaginal specimens may be obtained by using small swabs within the vagina, similar to the method
described for obtaining vaginal cultures.
•" approximately 5% of abused children acquire a sexually transmitted infection (STI), providers must
decide when STI testing is indicated.
•" Both gonorrhea and chlamydia cause a vaginitis, not a cervicitis, in prepubertal children, so a vaginal
culture should be done.
•" If a child was abused in an isolated incident, an STI may not be found on testing immediately after the
abuse. However, a purulent discharge would prompt testing and be a red flag for possible ongoing abuse
rather than an isolated incident.
HYMEN IN THE EVALUATION OF SEXUAL
ABUSE
Complete transections of the hymen, and clefts that extend to the junction of the
hymen between 3 o’clock and 9 o’clock, are not congenital, but if present they could
be from abuse or a child inserting an object. Controversies exist as to the significance
of incomplete transections
GENITAL WARTS
• Human papillomavirus (HPV)
• The incubation interval from transmission to the presence of visible genital warts has not been
defined in children; however, it appears likely that most warts appearing prior to 3 years of age are
from maternal- child transmission.
• If the child is 3 years of age or older, serious consideration should be given to the possibility of
sexual transmission.
• Approximately half of lesions will regress over 5 years.
• Treatment in children is difficult. Caustic treatments such as trichloroacetic acid are painful
even if children are pretreated with local anesthesia.
• Topical imiquimod cream is labeled for use in children 12 years and older and can cause
significant vulvar irritation, but has been used successfully in younger children.
• If the child accidentally carries imiquimod cream to the cornea, it could cause damage to the
eye.
• Laser and/or ultrasonic treatment is an option for significant wart tissue but must be performed
under anesthesia and can be associated with significant postoperative pain.
THE OVARY & ADNEXA IN
PEDIATRIC & ADOLESCENT
GYNECOLOGY
CYSTS, TUMORS & TORSION
•Cysts of follicular development will be clear without significant solid components and almost always are less than 7
to 10 cm in size in reproductive adolescents.
•Management of adolescent functional cysts is essentially the same as the management in reproductive females.
•Cysts in neonates can generally be observed until resolution.
•Neonates and children can be observed for any signs of torsion and advised to seek immediate medical attention,
unless they have exceptionally large cysts.
•Torsion can certainly occur and is not rare.
•Many neonatal cysts were initially identified on
•antenatal ultrasound.
•Cysts during the preschool and early grade school years are unusual, reflecting that gonadotrophins are low.
•Corpus luteum cysts are often more complex than other follicular cysts.
•Consideration should be given to dermoids and the possibility of germ cell tumors if a mass has both solid
•and cystic components.
• In rare cases of intersex, such as mixed gonadal dysgenesis, suspicion of malignancy should be high.
•A rare presentation of hypothyroidism is pediatric ovarian cysts.
NEONATAL OVARIAN CYSTS
Simple cystic ovarian masses in newborns and neonates are common and can be followed expectantly.
Parents should be given ovarian torsion warnings, and if the infant presents with acute vomiting or
abdominal pain, she should be immediately evaluated for ovarian torsion. Repeat serial
ultrasonography should be performed every 4-6 weeks until the cyst resolves.
Almost all will resolve if they do not undergo torsion.
Malignancy is not a consideration in newborns when deciding therapy.
Aspiration is an option for large cysts.
•The management of cystic ovarian structures in children and adolescents should also be expectant
unless they are extremely large (>10 cm), in which case the possibility of functional cysts becomes
less likely.
•Many times, physiologic and functional cysts are discovered on an abdominal ultrasound
performed for complaints such as abdominal pain.
• Often the presence of a cyst is incidental and unrelated to the complaint.
• In patients with pain, the possibility of ovarian torsion should be entertained.
•Pain from ovarian cysts generally stems from three sources:
• expansion of the ovarian cortex (which is typical during the growth phase of follicles and
lasts less than 72 hours)
• peritoneal bleeding from rupture (particularly com- mon in bleeding disorders and patients
on anticoagulation) ovarian torsion
• These causes of pain do not typically present as chronic pelvic/abdominal pain.
• Recurrent functional ovarian cysts may be prevented by the use of anovulatory agents,
such as combined oral contraceptives in adolescents, but these agents do not assist in
the resolution of cysts that are actively present.

OVARIAN TUMORS IN CHILDREN AND
ADOLESCENTS
• Germ cell tumors are the most common gynecologic neoplasm in this age group, and
fortunately, most are benign ovarian teratomas.
• The most common malignant germ cell tumor
1. dysgerminoma
2. endodermal sinus tumors
3. immature teratomas.
• Bilateral tumors are seen in 10% to 15% of dysgerminomas, but this condition is rare in all
of the other germ cell tumors of the ovary except for immature teratomas.
• Sex cord tumors,:
1. granulosa
2, thecal cell tumors, can also be seen in this age group and often produce steroids
(estrogen and testosterone respectively).
• Rare tumors:
gonadoblastomas, a germ cell and sex cord tumor, are seen in patients with intersex
disorders such as mixed gonadal dysgenesis.
• Recurrent abdominal pain is a frequent complaint of school- age children, and this common
symptom is often a presenting symptom in patients with ovarian
neoplasms.
• A young child may not be able to differentiate lower
abdominal pain from pelvic pain because of the small size of the preadolescent female
pelvis, making the ovaries essentially abdominal organs
• Increasing abdominal girth is a frequent finding associated with ovarian enlargement.
• The most common clinical manifestation of an ovarian tumor is lower abdominal pain or
the presence of a mass.
• Some ovarian tumors in children produce only vague discomfort, such as abdominal
fullness or bloating
• Adnexal masses in children are more frequently associated with acute complications—
such as torsion, hemorrhage, and rupture—than are similar tumors in adults.
DIAGNOSTIC TOOLS:
Ultrasound, magnetic resonance imaging(MRI),or PREOPERATIVE WORKUP
abdominal computed tomography (CT) may be May be screened for elevated tumor
utilized in the evaluation of a suspected pelvic mass markers
or abdominal pain of uncertain origin in children. • more markers such as α-fetoprotein,
 Abdominal ultrasonography may be used to both alpha and beta human chorionic
establish that the origin of the mass is in the pelvis, gonadotropin (HCG), inhibin (A and
whether the mass is cystic or solid, and the presence B), lactate dehydrogenase, estradiol,
of ascites (Anthony, 2012) and should be considered and testosterone
as the initial imaging modality. • tumor markers that are associated
 Calcifications in an ovarian mass may appear with other neoplasms can be
toothlike, indicating a likely diagnosis of an ovarian detected in girls.
teratoma. • HCG may be positive for either the α
or the β subunit, so a pregnancy test
that only tests for the β subunit is
inadequate.
Ovarian tumors
• in preadolescent females, both benign and malignant, are usually unilateral.
It is imperative to be as conservative as possible in managing the opposite ovary in order to protect
potential future fertility.
• During surgery the opposite ovary should be carefully inspected and palpated if possible.
• It is generally unnecessary and potentially harmful to perform a biopsy on a normal-appearing
contralateral ovary in a pre- adolescent female.
• This is especially true in patients with dermoids.
• It was common practice to perform a “wedge biopsy” of the contralateral ovary, but that practice was
not evidence based and was abandoned as it clearly increased the possibility of scarring and infertility.
• Children with suspected ovarian cancer should be referred to specialists
• The role of adjuvant therapy should be individualized for each patient.
• The use of tumor markers to help differentiate patients with benign teratoma from malignancies is
helpful in triaging appropriate referrals, regardless of what the makers show, referral is prudent.
•benign, and approximately 15% to 25% are malignant neoplasms.
•The risk is less in young children.
•In a review of ovarian masses in children, Brown and
•coworkers reported that the risk of malignancy was only 3% uptoage8.
• Even though ovarian neoplasms are rare in children, diagnosis should be considered in a young girl
with abdominal pain and a palpable mass.

GOALS OF SURGICAL THERAPY


 appropriate surgical procedure including selective evaluation of lymph nodes and appropriate
staging procedures
 preservation of future fertility, as hysterectomy is usually not necessary, even in rare cases of
bilateral childhood or adolescent ovarian malignancy.
 The uterus should be retained to keep the patient’s options for future fertility intact.
 Even in the absence of ovaries, fertility may be possible with artificial reproductive technology and
the use of donor eggs.
Ovarian Torsion
•Torsion in prepubertal females may be secondary to a pelvic mass or
due to mechanical factors that occur in the peripubertal interval.
•In early puberty, the ovaries drop from their prepubertal position at
the pelvic brim into the pelvis.
•This drop occurs under the influence of gonadotropins that surge at
puberty.
•Some young women may have longer supportive ligaments,
predisposing them to twisting.
•Approximately two thirds of the time, ovarian torsion occurs on the
right side, increasing the likelihood of the process being confused with
appendicitis.
• The sigmoid colon in the left lower quadrant helps
prevent the left ovary from twisting.
• Both appendicitis and torsion can present with acute pain
and rebound:
• the gradual progression = appendicitis
• Acute severe pain + Nausea and emesis = torsion
• The young girl with an acute onset of pain and
simultaneous emesis likely has ovarian torsion
rather than appendicitis.
• Even in children without an ovarian mass, after
torsion the ovary will become swollen and enlarged
as the lymphatic flow is blocked.
• Radiologic evaluation to rule out appendicitis may
reveal a pelvic mass.
• The presence of vascular flow in the ovary does not rule
out torsion.
• It is important to consider the patient’s future fertility.
REFERENCE:

Lobo, R., Gershenson, D., Lentz, G. and Valea, F.


(n.d.). Comprehensive gynecology.

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