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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_32_библиотеки_им_акад_М_И_Перельмана
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25
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Pediatric and Adolescent Gynecology
VERONICA I ALANIZ and PATRICIA HUGUELET
Topics covered
Prepubertal lichen sclerosus
Vulvovaginitis
Labial adhesions
PREPUBERTAL LICHEN
SCLEROSUS
Introduction
Lichen sclerosus is a chronic inammatory condition that aects the anogenital skin and typically
develops during times of low estrogen production.
Although the majority of cases are diagnosed in
postmenopausal women, approximately 10–15%
of cases occur during childhood. e estimated
incidence in prepubertal girls is approximately 1
in 900. e exact pathogenesis of lichen sclerosus is unknown and is likely to be multifactorial.
Lichen sclerosus has been associated with conditions such as vitiligo, alopecia, and thyroiditis,
suggesting an autoimmune mechanism contributing to the disease.
Presentation
Prepubertal lichen sclerosus typically presents
with intense and unrelenting genital itching. Other
Labial hypertrophy
Hymenal and vaginal anomalies
Ovarian masses
presenting symptoms may include vulvar discomfort or soreness, genital bleeding, dysuria, or pain
with defecation. On examination of the externa l genitalia, the classic appearance is hypopigmentation in
a gure of eight pattern surrounding the vestibule
and anus. e vulvar skin is typically thin and akin
to “parchment paper”. Other examination ndings
include ssures or supercial breaks in the skin and
subepithelial hemorrhages or “blood blisters”. For
those who are unfamiliar with prepubertal lichen
sclerosus the appearances of genital ecchymosis and
bleeding can sometimes raise concerns regarding
genital trauma (see Figure 25.1). Resorption of labia
minora, adhesions of the prepuce resulting in a buried clitoris, and introital narrowing can occur in late
stage and untreated cases of lichen sclerosus.
Investigations
Prepubertal lichen sclerosus can almost invariably be diagnosed on the basis of the clinical history and examination of the external genitalia
alone. Vulvar biopsy should not be routinely performed and is rarely indicated.
333
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Figure 25.1 Prepubertal lichen sclerosus. (Boms, S.,
Gambichler, T., Freitag, M., Altmeyer, P., & Kreuter, A.
(2004). Pimecrolimus 1% cream for anogenital lichen
sclerosus in childhood. BMC Dermatol, 4(1), 14.
doi:10.1186/1471-5945-4-14.)
Treatment
associated with thinning of the vulvar skin and
superimposed infections. For refractory cases,
topical immune modulators, such as pimecrolimus or tacrolimus can also be used, though
published data regarding the use of these agents
in children is very limited. Recurrent symptoms
occur with an average frequency of 1–2 ares
(exacerbations) per year. Acute exacerbations of
symptoms are treated with further courses of
topical steroids which can be modied depending on their severity. Maintenance steroids can be
considered for girls who are experiencing more
frequent episodes. Surgery is reserved for symptomatic adhesions or scarring.
Prognosis
Prepubertal lichen sclerosus was previously
thought to resolve during puberty with the onset
of endogenous estrogen production. However,
several reports have shown that many patients
will experience persistent signs or symptoms in
adolescence. Although post-menopausal women
with lichen sclerosus are known to be at increased
risk of vulvar squamous cell carcinoma, the risk
of malignancy associated with prepubertal lichen
sclerosus has not been established. Girls with
lichen sclerosus should be seen for follow up every
6–12 months.
e goals of treatment are to relieve symptoms
and prevent physical changes of the vulva. Girls
with acute symptoms are treated with a course of
topical steroids over 2–3 months, starting with
a high potency steroid ointment, then titrating
down to a lower potency steroid (see Table 25.1).
Patients treated with topical steroids should
be monitored as prolonged treatment can be
Table 25.1 Example of lichen sclerosus treatment
Topical steroid Frequency Period
Clobetasol propionate 0.5% Twice daily 2 weeks
Clobetasol propionate 0.5% Once daily 2 weeks
Triamcinolone acetonide 0.1% Twice daily 2 weeks
Triamcinolone acetonide 0.1%
Triamcinolone acetonide 0.1%
VULVOVAGINITIS
Introduction
Inammation of the vulva, vestibule, and
vagina occurring in prepubertal girls is of
Once daily
1–2 times weekly
2 weeks
Maintenance
Labial Adhesions / Presentation 335
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non-specic etiology in the majority of cases.
Anatomical factors which predispose to vulvovaginitis in this age group include; close proximity of the vagina to the anus, lack of labial
fat pads, and thin vaginal mucosa with an alkaline pH. Behavioral factors such as poor hand
washing, incorrect wiping, chronic constipation, and exposure to irritants also contribute
to the development of vulvo vaginitis. An infectious etiology is identied up to 25% of cases
and when bacteria are isolated they are usually
enteric or respiratory in origin. Unless other
risk factors are present yeast infection is rarely
the cause of vulvovaginitis in toilet trained prepubertal girls because the unestrogenized and
alkaline environment of the prepubertal vagina
does not permit the growth of candida.
Presentation
The most common symptoms are vulvar redness and soreness. Vaginal discharge, genital
itching, and bleeding from irritation or breakdown of mucosal surfaces can also occur. The
presenting symptoms are generally chronic in
nature, with waxing and waning in their severity. On examination of the external genitalia
the findings are varied and may appear normal or erythematous. Bacterial vulvovaginitis
is more likely to be accompanied by significant
vaginal discharge, severe inflammation, and
offensive odor.
Table 25.2 Vulvar and vaginal hygiene
recommendations
•
Avoid irritants including bubble bath or
scented soaps.
•
Wear plain white, cotton underwear.
•
Use unscented detergent.
•
Wear a nightgown or loose clothing for
sleeping. Try sleeping without underwear.
•
Change quickly out of wet swimsuits and
exercise clothes.
•
Take a bath every day and use water only
to clean the genitalia.
•
Always wipe from front to back after bowel
movements.
•
Apply a non-medicated barrier cream once
to twice daily.
Treatment
e primary treatment for non-specic vulvovaginitis is health education and measures to
improve vulvar hygiene (see Table 25.2). Patients
with persistent symptoms or suspected bacterial
vulvovaginitis can be treated with a course of oral
antibiotics, such as amoxicillin.
LABIAL ADHESIONS
Introduction
Investigations
Vulvovaginitis is diagnosed on the basis of the
clinical history and examination of the external
genitalia. An aerobic culture can be collected
when bacterial vulvovaginitis is suspected or discharge is visible at the vestibule. If an intravaginal specimen is needed, this should be obtained
without touching the hymen. A small moistened
swab can be inserted gently past the hymen to
collect a specimen. Patients with foul smelling,
blood tinged, or recurrent vaginal discharge
which does not respond to typical treatments
may need vaginoscopy to look for a possible vaginal foreign body.
Agglutination (adhesion) of the labia minora is
most common between 3 months and 3 years of
age and occurs in response to vulvar irritation
and low estrogen levels in this age group. e
mucosal surfaces of the labia minora become
inamed and raw from irritation and then adhere
together in the midline.
Presentation
For many girls, labial adhesions are asymptomatic and are discovered incidentally during the
course of a routine “well child” examination.
Symptomatic presentations include; genital discomfort, abnormal urine stream, post void dribbling, and in rare cases, urinary tract infections.
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Expectant management is reasonable in patients
who are asymptomatic. When the adhesions are
extensive or causing symptoms, medical management is indicated. First line treatment consists of
the application of a topical estrogen twice daily for
2 weeks. Caregivers should be advised to apply a
small (pea-sized) amount of cream to the adhesion
using a digit or Q tip. Generally, topical estrogen is
well tolerated with minimal systemic eects due to
absorption of estrogen. Side eects with prolonged
use include vulvar hyperpigmentation or breast
budding. An alternative to topical estrogen is a
topical steroid, such as betamethasone 0.05% ointment applied twice daily for 4–6 weeks. Division
of adhesions can be performed aer application
of local anesthetic cream in clinic. Manual separation or sharp dissection in the operating room
is only indicated for symptomatic cases which are
refractory to medical management.
Prognosis
Figure 25.2 Labial adhesion. (Al Jurayyan
NAM (2012) Labial agglutination (adhesion) in
pre-pubertal girls. What a primary care physician should know? Primary Health Care 2:128.
doi:10.4172/2167-1079.1000128.)
When gentle lateral traction is applied to the
labia majora, a midline area of clear or grey
brotic tissue is seen covering the vaginal opening. Labial adhesions cause varying degrees of
occlusion of the introitus and in severe cases
only a pinpoint residual opening may be present
(Figure 25.2).
Investigations
Labial adhesions are diagnosed on the basis of the
clinical history and examination of the external
genitalia.
Treatment
●
Nothing
●
Topical estrogen
●
Topical steroids
●
Rarely, surgery
Adhesions may resolve spontaneously and almost
always separate during puberty with onset of
endogenous estrogen production. Medical management with topical estrogen is eective in 90%
of cases, though recurrence is quite common. To
prevent recurrence, non-medicated barrier cream
and vulvar hygiene is recommended.
LABIAL HYPERTROPHY
Introduction
Labial hypertrophy is poorly dened. Although
variability in the appearance of external genitalia is expected, few studies have attempted to
dene the normal size of labia minora. e width,
or protrusion, of the labia minora in adolescent
females can range from a few millimeters to several centimeters. In general, a width of greater
than 5–6 cm is considered hypertrophied.
Presentation
Enlargement of the labia minora can be unilateral or bilateral. Patients may be asymptomatic
Hymenal and vaginal anomalies / Hymenal Anomalies 337
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and present primarily because of dissatisfaction
with the appearance of their genitalia. Functional
symptoms also occur and may include irritation,
discomfort or pulling with movement, interference with intercourse, and diculty with menstrual hygiene.
Investigations
e width of the labia minora is measured from
the vestibule to the distal/free end of the labia
minora with and without stretch (Figure 25.3).
Treatment
Clinicians should rst and foremost educate and
reassure patients and parents about the normal
variation in the appearances of the female genitalia. e use of online resources such as e Labia
Library (www.labialibrary.org.au) and Great Wall
of Vagina (www.greatwallofvagina.co.uk) can
be helpful in demonstrating the dierences in
normal anatomy. Measures to alleviate discomfort should be discussed – including supportive
undergarments, avoidance of irritants, use of a
barrier cream for minor irritation, and arranging
or tucking the labia prior to physical activity. In
the United States and many European countries
female genital surgery in children is legally prohibited unless there are valid medical indications.
Figure 25.4 Labiaplasty incisions.
Surgical intervention (labiaplasty) should therefore not be performed for cosmetic reasons
in patients under 18 years of age. Labiaplasty
should only be considered for patients with labial
enlargement which is outside the typical range
and whose functional symptoms are not relieved
with supportive measures. ere are several surgical techniques for labiaplasty, of which wedge
resection and free edge amputation are the most
commonly performed (Figure 25.4). e surgical
risks associated with labiaplasty are pain, bleeding, hematoma, infection, would dehiscence,
need for reoperation, and dissatisfaction with
appearance.
Figure 25.3 Measurement of labial hypertrophy.
HYMENAL AND VAGINAL
ANOMALIES
Introduction
Hymenal and vaginal anomalies are congenital
abnormalities of the lower genital tract and can be
obstructing or non-obstructing (see Table 25.3).
Hymenal Anomalies
During adolescence, an imperforate hymen
typically presents with primary amenorrhea
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Table 25.3 Hymenal and vaginal anomalies
Obstructing Non-obstructing
Hymenal Hymenal
Imperforate hymen Septate hymen
Vaginal Microperforate hymen
Obstructed hemi-vagina Cribiform hymen
Transverse vaginal septum Vaginal
Distal or partial vaginal agenesis Perforated transverse vaginal septum
Longitudinal vaginal septum
Mayer Rokitansky Kuster Hauser (MRKH) Syndrome
and cyclic abdominal pain. If signicant hematometrocolpos is present, patients may have
an abdominal mass and a bulging hymen at
the perineum. Patients with partially occlusive hymenal anomalies (septate, cribiform, or
microperforate hymen) have normal menstruation but may have diculty using tampons for
menstrual hygiene. e most common hymenal
anomaly is a septate hymen (Figure 25.5). Girls
with this anomaly may specically report diculty removing a tampon, which remains stuck
behind a hymenal band. Hymenal anomalies
diagnosed in childhood on routine examination
Figure 25.5 Septate hymen.
do not require intervention and surgery should
be deferred to adolescence. Surgical management
consists of hymenectomy in which the obstructing hymenal tissue is excised and the cut edges
reapproximated with interrupted absorbable
sutures such as 4–0 Vicryl. e risk of genital
scarring and recurrent obstruction is minimal
aer hymenectomy, and therefore, post-operative dilation is not required.
Transverse Vaginal Septum
Transverse vaginal septa are rare anomalies that
result from failed fusion of the vaginal plate and
Müllerian ducts. A transverse vaginal septum
can be located anywhere in the vagina, with or
without a perforation. A complete transverse
vaginal septum presents in a similar fashion to
an imperforate hymen with primary amenorrhea and worsening pelvic pain in early adolescence. However, no bulging hymen or vagina
is visible on the perineum. In contrast, patients
with a perforated transverse septum have normal
menstruation and are more likely to present in
later adolescence or early adulthood with dysmenorrhea, dyspareunia, diculty using tampons or infertility. It is especially important to
determine the thickness of the septum with pelvic examination and magnetic resonance imaging (MRI) when planning surgery. e goals for
management of a transverse vaginal septum are
to relieve pain and other symptoms associated
with obstruction and to restore anatomy so that
the vagina will permit normal menstruation and
sexual intercourse. e management options
consist of either primary resection of the septum
Hymenal and vaginal anomalies / Mayer Rokitansky Kuster Hauser (MRKH) Syndrome 339
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or, alternatively, menstrual suppression (with or
without drainage of hematometrocolpos) followed by delayed septum resection. If drainage is
required to relieve pain this should be performed
percutaneously because of the high risk of infection with a vaginal drain. Surgical management
consists of resection of the entire vaginal septum,
with re-approximation of the vaginal mucosa
with interrupted stitches. Mobilization of the
upper vagina may be required if there is a thick
septum or partial vaginal agenesis and on rare
occasions the use of a gra material may also be
also be necessary. Post-operative dilation is recommended aer treatment of a transverse vaginal septum because of the higher risks of stenosis
and recurrent obstruction. For this reason, the
age and maturity of the patient should be considered before denitive surgical management.
Obstructed Hemi Vagina
A didelphys uterus can be associated with
obstructed hemi-vagina and ipsilateral renal
anomalies (OHVIRA). Because only half the
vagina is obstructed, patients will typically present with normal menarche, but worsening dysmenorrhea. On examination a lateral bulge is
palpated in the vagina. Pelvic imaging with MRI
conrms the diagnosis. Denitive treatment
consists of surgical resection of the obstructing
septum. Stenosis and re-obstruction is unlikely
unless the septum is high or incompletely
excised. In these cases, a hemi-hysterectomy may
be indicated.
Longitudinal Vaginal Septum
A longitudinal vaginal septum is a non-obstructing anomaly of the vagina which results from
failed fusion of the distal Müllerian ducts. is
vaginal anomaly is typically associated with
didelphys or septate uterus. Patients may present with inability to use tampons, leaking of
menstrual blood despite the use of tampons, dyspareunia, and post coital hemorrhage. On examination, a septum is identied which divides the
vagina into two halves (Figure 25.6). e length
of the septum may be partially or completely
Figure 25.6 Longitudinal vaginal septum.
dividing the vagina. Septum resection should be
oered to patients who are symptomatic.
Mayer Rokitansky Kuster Hauser
(MRKH) Syndrome
Müllerian agenesis (MRKH) occurs in approximately 1 in 4,500 female births and results from
the failure of normal development of the Müllerian
ducts. Girls with MRKH have normal pubertal
development and usually present with primary
amenorrhea. Most girls with MRKH do not experience associated pain, though cyclic pain can occur
during ovulation or as a result of an obstructed
uterine remnant or endometriosis. On examination, the external genitalia including the hymen
are normal but the vagina is underdeveloped. e
diagnosis is conrmed with pelvic imaging (usually MRI) in combination with chromosomal
analysis and/or measurement of plasma testosterone to rule out complete androgen insensitivity
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syndrome (CAIS). Associated renal and skeletal
anomalies are common and can be screened for
with a renal ultrasound scan and spinal X-ray. e
loss of fertility is particularly challenging for young
patients with MRKH and emotional support is
perhaps the most important aspect of their care.
All patients should be put in contact with support
groups and oered counseling. Surgery to create a
neo vagina should only be considered when this
is desired by the patient. e rst line treatment
of vaginal agenesis consists of vaginal dilation –
which is successful in up to 90% of those patients
who are motivated and committed to achieving
a successful outcome. Patients are provided with
a set of serial vaginal dilators (Figure 25.7) and
instructed to insert the dilator into the vagina
and apply pressure to it for 20–30 minutes once to
twice daily. e successful creation of a functional
vagina usually requires dilation over 6–12 months.
Coital dilation can augment the use of serial dilators. Surgical vaginoplasty can be considered in
patients in whom vaginal dilation is unsuccessful. A range of dierent procedures have been
described. e Vecchietti procedure is a laparoscopically assisted vaginal dilation. Alternatively,
a neo-vagina can be created with bowel, buccal
mucosa, skin, or peritoneum.
OVARIAN MASSES
Simple Ovarian Cysts
Unilocular and anechoic ovarian cysts are almost
always benign. ey can represent a functional
ovarian cyst, serous cystadenoma, mucinous
cystadenoma, or para tubal cyst. Simple ovarian
cysts may be asymptomatic or may present with
symptoms such as acute or chronic pelvic pain,
increasing abdominal girth, nausea and vomiting.
Surgical management, consisting of ovarian cystectomy is indicated for symptomatic cysts and for
those which are persistent and enlarging beyond
4–6 cm in diameter. Asymptomatic cysts which are
less than 10 cm in diameter can safely be observed
and followed with serial pelvic ultrasound scans.
Complex Ovarian Masses
A complex mass with both solid and cystic components may represent a hemorrhagic functional
cyst, a benign neoplasm such as a mucinous cystadenoma or mature teratoma, a borderline tumor
or, rarely a malignant tumor. Complex masses
that do not have classic features of a mature teratoma should be evaluated by measurement of
tumor makers which are oen elevated in the
most common ovarian malignancies occurring
in adolescence (see Table 25.4).
Figure 25.7 Syracuse vaginal dilators.
Table 25.4 Evaluation of complex ovarian
masses
Tumor type Tumor markers
Germ cell tumors Alpha-fetoprotein (AFP)
Lactate dehydrogenase
(LDH)
Human chorionic
gonadotropin (HCG)
Sex cord tumors
Epithelial tumors
Inhibin A&B
Estradiol
Testosterone
Cancer antigen 125
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Functional Ovarian Cysts
ese cysts arise during normal and abnormal
ovulation. A follicular cyst is a simple thin walled
cyst which either develops prior to ovulation or
persists and enlarges following failed ovulation.
Typically these cysts are 2–3 cm in size, though
they can increase in size when due to failed ovulation. A corpus luteum cyst develops aer ovulation and has more complex appearances on
ultrasound with internal echoes related to hemorrhage. Functional ovarian cysts can be asymptomatic and found incidentally on imaging.
Alternatively, they may present with acute pelvic
pain secondary to cyst rupture or hemorrhage.
Functional ovarian cysts resolve spontaneously
within 2–8 weeks and do not require surgical
intervention. Hormonal contraception to suppress ovulation can be used to prevent recurrence
of physiologic ovarian cysts.
Endometrioma
Endometriomas are rare benign cysts that result
from ectopic growth of endometrial tissue within
the ovary in adolescents. ey may present with
an enlarging mass, dysmenorrhea, or generalized
pelvic pain. On ultrasound they appear as complex cystic lesions with homongenous low level
echoes and may be unilocular or multilocular.
Endometriomas do not resolve with hormonal
management and should be removed laparoscopically if they are symptomatic or if the cyst is
greater than 5 cm.
Mature Teratoma (Dermoid Cysts)
Benign dermoid cysts are the most common
germ cell tumor occurring in this age group
and are bilateral in in 10–15% of cases. Patients
with dermoid cysts may be asymptomatic or
may present with an enlarging mass, pelvic pain,
or ovarian torsion. e cysts are composed of
mature tissue elements and have characteristic
appearances on ultrasound due to the presence
of sebaceous uid, hair, and calcications (see
Figure 25.8). Surgical intervention, preferably
ovarian cystectomy, is indicated for large and/
or symptomatic dermoid cysts. If cyst contents
are spilled intraoperatively the abdominal cavity
and pelvis should be copiously irrigated to prevent chemical peritonitis.
Figure 25.8 Ultrasound appearance of dermoid cyst.
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