Objectives: to learn how to diagnose and to prescribe special
therapy for patient with endometriosis.
Professional motivation:
Basic level:
1. Etiology of endometriosis.
2. What specialist consults women with endometriosis?
3. How often can medical conditions complicate the course of
endometriosis?
STUDENTS' INDEPENDENT STUDY PROGRAM
I. Objectives for Students' Independent Studies
You should prepare for the practical class using the available
textbooks and lectures. Special attention should be paid to the
following:
1 - Etiology of endometriosis.
2- Classification of endometriosis.
3- Examination and urgency aid for women with endometriosis.
4. Treatment and prevention of endometriosis.
Key words and phrases: women with endometriosis.
Summary.
Endometriosis : Etiology, Pathology, Diagnosis, Management
KEY TERMS AND DEFINITIONS
An isolated area of endometrial glands and
stroma in the uterine musculature that can be
Adenomyoma
identified grossly.
Adenomyosis
The growth of endometrial glands and stroma
into the uterine myometrium to a depth of at
least 2.5 mm from the basalis layer of the
endometrium.
Chocolate Cyst
A cystic area of endometriosis in the ovary.
Coelomic
Metaplasia
The potential ability of coelomic epithelium to
develop into several different histologic cell
types.
Danazol
A synthetic steroid, an attenuated androgen,
that is active when taken orally.
Dyschezia
Difficult or painful evacuation of feces from the
rectum.
Endometrioma
An area of endometriosis that can be identified
macroscopically, usually in the ovary.
Endometriosis
The presence and growth of glands and stroma
identical to the lining of the uterus in an
aberrant location.
GnRH Agonists
A group of synthetic hormones that suppresses
gonadotrophin secretion, causing secondary
diminution of ovarian steroidogenesis.
Retrograde
Menstruation
The flow of menstrual blood, endometrial cells,
and debris via the fallopian tubes into the
peritoneal cavity.
KEY POINTS
•
Endometriosis is a benign, usually progressive, and sometimes recurrent disease that invades locally and
disseminates widely.
•
Endometriosis is present in 5% to 15% of celiotomies
performed on women of reproductive age.
•
The incidence of endometriosis is 30% to 45% in women
with infertility.
•
Approximately 5% of women with endometriosis are
diagnosed following menopause. Postmenopausal
endometriosis is usually secondary to the use of exogenous
estrogen.
•
Possible causal factors of endometriosis include retrograde
menstruation, coelomic metaplasia, vascular metastasis,
immunologic changes, iatrogenic dissemination, and a
genetic predisposition.
•
Some postulate that there is a complex interplay between a
dose-response curve of the amount of retrograde
menstruation and an individual woman's immunologic
response.
•
One of 10 women with severe endometriosis will have a
sister or mother with clinical manifestations of
endometriosis.
•
The ovaries are the most common site, being involved in
two of three women with endometriosis. The pelvic
peritoneum over the uterus; the anterior and posterior culde-sac; and the uterosacral, round, and broad ligaments are
also common sites where endometriosis develops. Pelvic
lymph nodes are involved in 30% of cases.
•
Grossly, endometriosis appears in many forms, including
red, brown, black, white, yellow, pink, or clear vesicles
and lesions. Biopsy is important in establishing the
diagnosis.
•
Red, blood-filled lesions have been shown, by histologic
and biochemical studies, to be the most active phase of
endometriosis.
•
Three cardinal features of microscopic endometriosis are
ectopic endometrial glands, ectopic endometrial stroma,
and hemorrhage into the adjacent tissue.
•
Viable endometrial glands and stroma cannot be identified
on pathologic examination in approximately 25% of cases
of endometriosis.
•
Classic symptoms of endometriosis are cyclic pelvic pain
and infertility. However, approximately one third of
patients with endometriosis are asymptomatic.
•
Endometriosis is discovered in approximately one of three
women whose primary symptom is chronic pelvic pain.
•
Secondary dysmenorrhea usually begins 36 to 48 hours
prior to the onset of menses. Unlike primary
dysmenorrhea, the pain may last for many days, including
several days before and after the menstrual flow.
•
The most prominent pelvic sign of endometriosis is a
fixed, retroverted uterus with scarring and tenderness
posterior to the uterus. The characteristic nodularity of the
uterosacral ligaments and cul-de-sac of Douglas may be
palpated on rectovaginal examination.
•
Serum CA-125 levels have a low sensitivity for the
diagnosis of early or minimal endometriosis.
•
Approximately 10% of teenagers who develop
endometriosis have associated congenital outflow
obstruction.
•
The two primary short-term goals in treating endometriosis
are the relief of pain and promotion of fertility. The
primary long-term goal in the management of a woman
with endometriosis is attempting to prevent progression or
recurrence of the disease process.
•
Recurrent bleeding in the ectopic implants is one of the
most important pathophysiologic processes to interrupt.
•
Optimal regression secondary to medical treatment is
observed in small endometriomas that are less than 1 to 2
cm in diameter.
•
The choice of medical therapy for the individual patient
depends on the clinician's evaluation of adverse effects,
side effects, cost of therapy, and expected patient
compliance. The recurrence rate following medical therapy
is 5% to 15% in the first year and increases to 40% to 50%
in 5 years.
•
The side effects of danazol are related to its androgenic
and anabolic properties, as well as to the
“pseudomenopause” produced by the drug.
•
Approximately three of four women note significant improvement in symptoms following danazol therapy.
Unfortunately, 15% to 30% of women will have a
recurrence of symptoms within 2 years following
completion of medical therapy.
•
With chronic administration of GnRH agonists, specific
suppression of gonadotropin secretion occurs, with
secondary diminution of ovarian steroidogenesis. The
GnRH agonists bind with receptors for a prolonged time
and induce protracted periods of down-regulation.
•
GnRH agonists produce a “medical oophorectomy”
without the side effects of danazol on steroid-sensitive
target organs.
•
The side effects associated with GnRH agonist therapy are
primarily those associated with estrogen deprivation,
similar to menopause. The three most common symptoms
are hot flushes, vaginal dryness, and insomnia. A decrease
in bone mineral content of trabecular bone has been
demonstrated in the cortical bone on the lumbar spine by
quantitative computed tomography.
•
It has been established that the decrease in bone density
associated with 6 months of therapy with GnRH agonist
completely recovers between 12 and 24 months after
discontinuing therapy.
•
Initial clinical response to GnRH agonist therapy depends
on when the therapy is begun in relation to the menstrual
cycle.
•
Many clinicians “add-back” very low doses of estrogen,
low doses of progestins, or both in combination with
chronic GnRH agonist therapy.
•
Laparoscopy is employed frequently for both diagnostic
and therapeutic reasons. The major advantage of treating
endometriosis with the laparoscope, using surgical
instruments, laser, or electrocautery, is that patients may
be treated at the time of diagnosis.
•
The goals of conservative surgery for endometriosis
include removal of macroscopic endometriosis, lysis of
adhesions, and restoration of normal anatomy.
•
Classic symptoms of endometriosis of the large bowel
include cyclic pelvic cramping and lower abdominal pain
and rectal pain with defecation, especially during the
menstrual period.
•
The pathogenesis of endometriosis of the bladder is
controversial. Interestingly, approximately 50% of women
with endometriosis of the urinary tract have a history of
previous pelvic surgery.
•
Endometriosis of the bladder is discovered most often in
the region of the trigone or the anterior wall of the bladder.
Bladder endometriosis produces midline, lower abdominal,
and suprapubic pain, dysuria, and, occasionally, cyclic
hematuria.
•
Surgical therapy is preferred for ureteral obstruction
secondary to endometriosis.
•
Adenomyosis is frequently asymptomatic. If multiple
serial sections of the uterus are obtained, the incidence
may exceed 60% in women 40 to 50 years of age.
•
Symptomatic adenomyosis primarily occurs in parous
women older than age 35. The classic symptoms are
secondary dysmenorrhea and menorrhagia. The most
common physical sign is a diffusely enlarged uterus,
usually two to three times normal size.
•
The severity of pelvic symptoms associated with
adenomyosis increases proportionally to the depth of
penetration and the total volume of disease in the
myometrium.
•
Adenomyosis rarely causes uterine enlargement greater
than a size at 14 weeks' gestation unless there is
concomitant uterine pathology.
ENDOMETRIOSIS
Endometriosis is a benign, but in many women, a progressive
disease. The wide spectrum of clinical problems that occur with
endometriosis has frustrated gynecologists, fascinated pathologists,
and burdened patients for years. Although endometriosis was first
described in 1860, the classic studies of Sampson in the 1920s were
the first to emphasize the clinical and pathologic correlations of
endometriosis. Even today, many aspects of the disease remain
enigmatic.
By definition, endometriosis is the presence and growth of
the glands and stroma of the lining of the uterus in an aberrant or
heterotopic location. Adenomyosis is the growth of endometrial
glands and stroma into the uterine myometrium to a depth of at least
2.5 mm from the basalis layer of the endometrium. Adenomyosis is
sometimes termed internal endometriosis; however, this is a
semantic misnomer because most likely they are separate diseases.
It is usually stated that the incidence of endometriosis has
been increasing over the past 30 years. This “opinion” is secondary
to an enlightened awareness of mild endometriosis as diagnosed by
the increasing use of laparoscopy. During the past 10 years,
diagnostic delay, the average time to the first diagnosis of the
disease, has decreased dramatically. However, it has been estimated
to take an average time of 11.7 years in the United States and 8
years in the United Kingdom. Evers has advanced a provocative
hypothesis that endometrial implants in the peritoneal cavity are a
physiologic finding secondary to retrograde menstruation, and their
presence does not confirm a disease process. The prevalence of
pelvic endometriosis in the general female population has been
suggested to be 6% to 10%. The age-specific incidence or
prevalence of endometriosis is not known and has only been
estimated. Many patients are diagnosed incidentally during surgery
performed for a variety of other indications. Conservative estimates
find that endometriosis is present in 5% to 15% of laparotomies
performed on reproductive-age females. The prevalence of active
endometriosis is approximately 33% in women with chronic pelvic
pain. The incidence of endometriosis is 30% to 45% in women with
infertility. It must be emphasized that all studies of the prevalence
of endometriosis are subject to selection bias and are dependent on
the definition of “active disease.”
The cause of endometriosis is uncertain and may involve
retrograde menstruation, vascular dissemination, metaplasia,
genetic predisposition, immunologic changes, and hormonal
influences, as discussed later on. In addition, there is increasing
evidence that environmental factors may also play a role, including
exposure to dioxin and other endocrine disruptors. Clinically, it is
most difficult to predict the natural course of endometriosis in any
one individual. For example, the clinician is uncertain as to which
woman with mild disease in her 20s will progress to severe disease
at a later age.
The typical patient with endometriosis is in her mid-30s, is
nulliparous and involuntarily infertile, and has symptoms of
secondary dysmenorrhea and pelvic pain. The classic symptom of
endometriosis is pelvic pain. However, in clinical practice the
majority of cases are not “classic.” Aberrant endometrial tissue
grows under the cyclic influence of ovarian hormones and is
particularly estrogen dependent; therefore the disease is most
commonly found during the reproductive years. However, 5% of
women with endometriosis are diagnosed following meno-pause.
Postmenopausal endometriosis is usually stimulated by exogenous
estrogen. Endometriosis in teenagers should be investigated for
obstructive reproductive tract abnormalities that increase the
amount of retrograde menstruation.
Endometriosis is a disease not only of great individual
variability but also of contrasting pathophysiologic processes (
Table 1 ). It is a benign disease, yet it has the characteristics of a
malignancy—that is, it is locally infiltrative, invasive, and widely
disseminating. Although the growth of ectopic endometrium is
stimulated by physiologic levels of estrogen use of contraceptive
steroids of various doses are usually beneficial for treatment.
Another contrast often noted is the inverse relationship between the
extent of pelvic endometriosis and the severity of pelvic pain.
Women with extensive endometriosis may be asymptomatic,
whereas other patients with minimal implants may have
incapacitating chronic pelvic pain. However, as would be expected,
women with deep infiltrating endometriosis, especially in
retroperitoneal spaces, often experience severe episodes of pain.
Finally, there is speculation as to the underly-ing pathophysiology
that produces infertility in women with endometriosis.
Table 1 -- Endometriosis: A Disease of Clinical Contrasts
Characteristics
Contrasts
Benign disease
Locally invasive
Widespread disseminated foci
Proliferates in pelvic lymph nodes
Minimal disease
Severe pain
Many large
endometriomas
Asymptomatic patient
Cyclic hormones cause
growth
Continuous hormones reverse the
growth pattern
Etiology
There are several theories to explain the pathogenesis of
endometriosis. However, no single theory adequately explains all
the manifestations of the disease. Most important, there is only
speculation as to why some women develop endometriosis, and
others do not. One popular theory is that there is a complex
interplay between a dose-response curve of the amount of
retrograde menstruation and an individual woman's immunologic
response.
Retrograde Menstruation
The most popular theory is that endometriosis results from
retrograde menstruation. Sampson suggested that pelvic
endometriosis was secondary to implantation of endometrial cells
shed during menstruation. These cells attach to the pelvic
peritoneum and under hormonal influence grow as homologous
grafts. Indeed, reflux of menstrual blood and viable endometrial
cells in the pelvis of ovulating women has been documented.
Endometriosis is discovered most frequently in areas immediately
adjacent to the tubal ostia or in the dependent areas of the pelvis.
Metaplasia
In contrast to the theory of seeding from retrograde
menstruation is the theory that endometriosis arises from metaplasia
of the coelomic epithelium or proliferation of embryonic rests. The
müllerian ducts and nearby mesenchymal tissue form the majority
of the female reproductive tract. The müllerian duct is derived from
the coelomic epithelium during fetal develop-ment. The metaplasia
hypothesis postulates that the coelomic epithelium retains the
ability for multipotential development. The decidual reaction of
isolated areas of peritoneum during pregnancy is an example of this
process. It is well known that the surface epithelium of the ovary
can differentiate into several different histologic cell types.
Endometriosis has been discovered in prepubertal girls, women
with congenital absence of the uterus, and very rarely in men. These
examples support the coelomic metaplasia theory.
Metaplasia occurs after an “induction phenomenon” has
stimulated the multipotential cell. The induction substance may be a
combination of menstrual debris and the influence of estrogen and
progesterone. Batt and Smith have hypothesized that the
histogenesis of endometriosis in peritoneal pockets of the posterior
pelvis results from a congenital anomaly involving rudimentary
duplication of the müllerian system. The peritoneal pockets that
they describe are found in the posterior pelvis, the posterior aspects
of the broad ligament, and the cul-de-sac of Douglas. Similarly,
Nisolle and Donnez postulate that metaplasia of the coelomic
epithelium that invaginates into the ovarian cortex is the
pathogenesis for the development of ovarian endometriosis.
Lymphatic and Vascular Metastasis
The theory of endometrium being transplanted via lymphatic
channels and the vascular system helps to explain rare and remote
sites of endometriosis, such as the spinal column and nose.
Endometriosis has been observed in the pelvic lymph nodes of
approximately 30% of women with the disease. Hematogenous
dissemination of endometrium is the best theory to explain
endometriosis of the forearm and thigh, as well as multiple lesions
in the lung.
Iatrogenic Dissemination
Endometriosis of the anterior abdominal wall is sometimes
discovered in women after a cesarean delivery. The hypothesis is
that endometrial glands and stroma are implanted during the
procedure. The aberrant tissue is found subcutaneously at the
abdominal incision. Rarely, iatrogenic endometriosis may be
discovered in an episiotomy scar.
Immunologic Changes
One of the most perplexing, unanswered questions
concerning the pathophysiology of endometriosis is that some
women with retrograde menstruation develop endometriosis, but the
majority do not. Multiple investigations have suggested that
changes in the immune system, especially altered function of
immune-related cells, are directly related to the pathogenesis of
endometriosis. Whether endometriosis is an autoimmune disease
has been intensely debated for many years. Studies have
demonstrated abnormalities in cell-mediated and humoral
components of the immune system in both peripheral blood and
peritoneal fluid. Depicted in Table 19-2 are various cytokines and
growth factors that have been implicated in the pathogenesis of
endometriosis.
Another attractive theory is the recent finding of a protein
similar to haptoglobin in endometriosis epithelial cells called Endo
1. This chemoattractant protein-enhanced local production of
interleukin-6 (IL-6) self perpetuates lesion/cytokine interactions.
Further compounding the proliferative activity of endometriosis
lesions are angiogenic factors that are increased in lesions. Here the
expression of basic fibroblast factor, IL-6, IL-8, platelet-derived
growth factor (PDGF), and vascular endothelial growth factor
(VEGF) are all increased.
Genetic Predisposition
Several studies have documented a familial predisposition to
endometriosis with grouping of cases of endometriosis in mothers
and their daughters. An investigation by Simpson and coworkers
demonstrated a sevenfold increase in the incidence of endometriosis
in relatives of women with the disease compared with controls. One
of 10 women with severe endometriosis will have a sister or mother
with clinical manifestations of the disease. Women who have a
family history of endometriosis are likely to develop the disease
earlier in life and to have more advanced disease than women
whose first-degree relatives are free of the disease. Recent studies
have identified deletions of genes, most specifically increased
heterogenicity of chromosome 17 and aneuploidy, in women with
endometriosis compared with controls. The expression of this
genetic liability most likely depends on an interaction with
environmental factors. Preliminary data suggest some bilateral
ovarian endometrial cysts may arise independently from different
clones.
The majority of endometrial implants are located in the
dependent portions of the female pelvis. The ovaries are the most
common site, being involved in two of three women with
endometriosis. In most of these women the involvement is bilateral.
The pelvic peritoneum over the uterus; the anterior and posterior
cul-de-sac; and the uterosacral, round, and broad ligaments are also
common sites where endometriosis develops. Pelvic lymph nodes
are involved in 30% of cases ( Fig. 19-7 ). The cervix, vagina, and
vulva are other possible pelvic locations. Brosens and Brosens have
emphasized the importance of distinguishing between superficial
and deep lesions of endometriosis. Deep lesions, penetrations of
greater than 5 mm, represent a more progressive form of the
disease. Distinguishing superficial implant lesions on peritoneal
surfaces, including the ovary, from deep endometriotic ovarian
cysts and cul-de-sac modules is important for therapy (discussed
later on) in that these latter abnormalities may suggest different
causes of the disease (e.g., metaplastia), which require a surgical
approach. .
Clinical Diagnosis
Symptoms
It is important to reemphasize that endometriosis has many
different clinical presentations, with one in three women being
asymptomatic. Most importantly, the disease has an extremely
unpredictable course. The classic symptoms of endometriosis are
cyclic pelvic pain and infertility. The chronic pelvic pain usually
presents as secondary dysmenorrhea or dyspareunia (or both).
Secondary dysmenorrhea usually begins 36 to 48 hours prior to the
onset of menses. However, approximately one third of patients with
endometriosis are asymptomatic, with the disease being discovered
incidentally during an abdominal operation or visualized at
laparoscopy for an unrelated problem. Conversely, endometriosis is
discovered in approximately one of three women whose primary
symptom is chronic pelvic pain.
Clinicians have appreciated the paradox that the extent of
pelvic pain is often inversely related to the amount of endometriosis
in the female pelvis. Women with large, fixed adnexal masses
sometimes have minor symptoms, whereas other patients with only
a few small foci with deep infiltration may experience moderate to
severe chronic pain. Fedele and colleagues could find no correlation
between the anatomic stage of the disease and the patient's
perception of severity of pelvic pain. In their study, 124 women
were staged according to the revised scoring system of the
American Society of Reproductive Medicine. No relationship was
found between stage of the disease and frequency or severity of
pain symptoms.
The cyclic pelvic pain is related to the sequential swelling
and the extravasation of blood and menstrual debris into the
surrounding tissue. The chemical mediators of this intense sterile
inflammation and pain are believed to be prostaglandins and
cytokines. Infiltrative endometriosis, which involves extenive areas
of the retroperitoneal space, often is associated with moderate to
severe pelvic pain. Recently, studies of pain mapping by
laparoscopy under minimal sedation have found that the pelvic pain
arises from areas of normal peritoneum adjacent to areas of
endometriosis.
Secondary dysmenorrhea is a common component of pain
which varies from a dull ache to severe pelvic pain. It may be
unilateral or bilateral and may radiate to the lower back, legs, and
groin. Patients often complain of pelvic heaviness or a perception of
their internal organs being swollen. Unlike primary dysmenorrhea,
the pain may last for many days, including several days before and
after the menstrual flow.
The dyspareunia associated with endometriosis is described
as pain deep in the pelvis. The cause of this symptom seems to be
immobility of the pelvic organs during coital activity or direct
pressure on areas of endometriosis in the uterosacral ligaments or
the cul-de-sac. Sometimes patients describe areas of point
tenderness. The acute pain, experienced during deep penetration,
may continue for several hours following intercourse.
Abnormal bleeding is a symptom noted by 15% to 20% of
women with endometriosis. The most frequent complaints are
premenstrual spotting and menorrhagia. Usually this abnormal
bleeding is not associated with an anovulatory pattern. On the other
hand, patients with endometriosis frequently have ovulatory
dysfunction. Approximately 15% of women with endometriosis
have coincidental anovulation.
An increased incidence of first-trimester abortion in women
with untreated endometriosis has been reported. However, recent
epidemiologic studies question a true increased incidence and, if
there is, raise serious doubt as to a cause-and-effect relationship.
Less common yet troublesome are the symptoms resulting
from endometriosis influencing the gastrointestinal and urinary
tracts. Cyclic abdominal pain, intermittent constipation, diarrhea,
dyschezia, urinary frequency, dysuria, and hematuria are all
possible symptoms. Bowel obstruction and hydronephrosis may
occur. One rare clinical manifestation of endometriosis is
catamenial hemothorax, bloody pleural fluid occurring during
menses. Massive ascites is a rare symptom of endometriosis, but it
is important because the disease process initially masquerades as
ovarian carcinoma.
Signs
The classic pelvic finding of endometriosis is a fixed
retroverted uterus, with scarring and tenderness posterior to the
uterus. The characteristic nodularity of the uterosacral ligaments
and cul-de-sac may be palpated on rectovaginal examination in
approximately one third of women with the disease. Advanced
cases have extensive scarring and narrowing of the posterior
vaginal fornix. The ovaries may be enlarged and tender and are
often fixed to the broad ligament or lateral pelvic sidewall. The
adnexal enlargement is rarely symmetrical, as one might expect in
some benign pelvic conditions. In one study of 561 women with
ovarian endometriomas, bilateral cysts were observed in 158 (28%).
In women with unilateral endometriomas, 63% were found in the
left ovary.
Endometriosis is a disease that produces tenderness of the
pelvic structures and scarring that restricts movement of the pelvic
organs. Occasionally the physician discovers endo-metriosis in an
old surgical incision or a site of a previous amniocentesis.
Speculum examination may demonstrate small areas of
endometriosis on the cervix or upper vagina. Lateral displacement
or deviation of the cervix is visualized or palpated by digital exam
of the vagina and cervix in approximately 15% of women with
moderate or severe endometriosis. The diag-nosis is straightforward
if a patient presents with secondary dysmenorrhea, deep
dyspareunia, and infertility and if during a pelvic examination the
physician discovers a fixed posterior uterus, bilateral adnexal
tenderness, and beading of the uterosacral ligaments. An
experienced clinician may instruct the patient to return for a pelvic
examination during the first or second day of her menstrual flow
when the diagnosis of endometriosis is in doubt. This is the time of
maximum swelling and tenderness in the areas of endometriosis.
The diagnosis can be confirmed in most cases by direct
laparoscopic visualization of endometriosis with its associated
scarring and adhesion formation. In many patients it is discovered
for the first time during an infertility investigation. Biopsy of
selected implants gives confirmation of the diagnosis. However,
sometimes the pathologist may be unable to find glandular elements
and endometrial stroma in the biopsy specimens.
Ultrasound examination shows no specific pattern to screen
for pelvic endometriosis but may be helpful in differentiating solid
from cystic lesions and may help distinguish an endometrioma from
other adnexal abnormalities. Since the lesions are vascular,
increased Doppler flow may be demonstrated in endometriosis.
Magnetic resonance imaging (MRI) provides the best
diagnostic tool for endometriosis but is not always a practical
modality for its diagnosis. With a detection ratio and specificity of
around 78% for implants, MRI for endometriosis has a reported
sensitivity and specificity of approximately 91% to 95%. There is a
characteristic hyperintensity on T1-weighted images and a
hypointensity on T2-weighted images.
Although a benign disease, endometriosis exhibits
characteristics of both malignancy and sterile inflammation.
Therefore the common considerations in the differential diagnosis
include chronic pelvic inflammatory disease, ovarian malignancy,
degeneration of myomas, hemorrhage or torsion of ovarian cysts,
adenomyosis, primary dysmenorrhea, and functional bowel disease.
Occasionally a large endometrioma of the ovary may rupture
into the peritoneal cavity. This results in an acute surgical abdomen
and brings into the differential diagnosis conditions such as ectopic
pregnancy, appendicitis, diverticulitis, and a bleeding corpus luteum
cyst. Studies have not demonstrated any temporal relationship
between the timing of an endometrioma's acute rupture and the day
of the menstrual cycle.
Natural History
Endometriosis is a chronic and sometimes progressive
disease. The disease is usually first diagnosed in a woman during
her mid to late 20s. The rate of progression of the disease varies
widely from one patient to another. Serial pelvic examinations are a
poor indicator of progression of the disease. Therefore the natural
history of the disease is largely speculation. In some centers,
second-look or reassessment laparoscopy is performed routinely.
These limited studies have given insight into the success of therapy
but have not been shown to be a benefit in women trying to
conceive. Serum levels of cancer antigen-125 (CA-125) have been
used as a marker for endometriosis. CA-125 levels are elevated in
most patients with endometriosis and increases incrementally with
advanced stages. However, assays for serum levels of CA-125 have
a low specificity because they also increase with other pelvic
conditions such as myomas, acute pelvic inflammatory disease, and
the first trimester of pregnancy. Similarly, serum CA-125 levels
have a low sensitivity for the diagnosis of early or minimal
endometriosis. It is also useful for tracking the course of the disease
with treatment.
Glycodelin, previously known as placental protein 14, has
been shown to be elevated in endometriosis and is produced in
endometriotic lesions. Levels also fall with removal of disease.
However, because of great variability in levels, glycodelin has not
proved to be useful clinically.
It would be optimal to identify women who are going to
develop endometriosis. All we have currently is family history.
However, the optimal preventive therapy for a young teenager not
desirous of pregnancy until her late 20s is unknown. Clinical
options include no treatment, continuous use of oral contraceptives,
or cyclic oral contraceptive therapy. Controlled prospective studies
are needed to answer the difficult clinical question of the best
method to inhibit progression of the disease. Approximately 10% of
teenagers who develop endometriosis have associated congenital
outflow obstruction. Therefore teenagers with pelvic pain should be
examined for this possibility.
At one time there was a general belief that pregnancy
improved endometriosis. A careful study by McArthur and Ulfelder
of external endometriosis, which could be observed throughout the
pregnancy, discovered that this generalization was not invariably
true. In some cases endometriomas rapidly increase in size during
the first few weeks of pregnancy. In general, during the third
trimester, symptoms are less severe and the size of the external
lesions decreases. Clearly endometriosis and large endometriomas
have been found at the time of cesarean section, although this is an
unusual occurrence.
In the past few years, there has been the realization that
endometriosis may be associated with ovarian cancer. Not only are
lesions found at the time of diagnosis of ovarian cancer, but the risk
of developing ovarian cancer may be increased fourfold in women
with endometriosis. Loss of heterozygosity and mutations in
suppressor genes, for example, p53, may explain this commonality.
These findings warrant caution in the long-term follow-up of
women who have extensive disease and ovarian endometriomas.
The association of other cancers with endometriosis, although
suggested, has not been substantiated. However, cervical
endometriosis is a particular condition that can produce
abnormalities in cervical cytology.
Endometriosis is dependent on ovarian hormones to stimulate
growth. With natural menopause, there is a gradual relief of
symptoms. Following surgical menopause, areas of endometriosis
rapidly disappear. However, it is important to note that 5% of
symptomatic cases of endometriosis present after menopause. The
vast majority of cases in women in their late 50s or early 60s are
related to the use of exogenous estrogen.
Management
The two primary short-term goals in treating endometriosis
are the relief of pain and promotion of fertility. The primary longterm goal in the management of endometriosis is attempting to
prevent progression or recurrence of the disease process. Presently,
there is a paucity of definitive, evidence-based literature on which
to select the most appropriate method of treatment. The appropriate
treatment for endometriosis varies widely because of the vast
differences in the spectrum of clinical symptoms and in the extent
of the disease from one woman to another. Therefore the treatment
plan must be individualized. Choice of therapy, for women whose
primary symptom is pelvic pain, depends on multiple variables,
including the patient's age, her future reproductive plans, the
location and extent of her disease, the severity of her symptoms,
and associated pelvic pathology. Most patients should undergo a
diagnostic evaluation, which may necessitate a diagnostic
laparoscopy to establish the nature and extent of endometriosis
before embarking on prolonged therapy. However, if other
gynecologic conditions such as chronic pelvic inflammatory disease
or neoplasia have been ruled out, empiric medical therapy for 3
months with a gonadotropin-releasing hormone (GnRH) agonist is a
reasonable option. Although pelvic pain may decrease, such therapy
has not been thought to help diagnostically.
Treatment of endometriosis can be medical, surgical, or a
combination of both. Most of the sex steroids, alone or in
combination, have been tried in clinical studies to suppress the
growth of endometriosis. Optimal regression secondary to medical
treatment is observed in small endometriomas that are less than 1 to
2 cm in diameter. Response in larger areas of endometriosis may be
minimal with medical therapy. A poor therapeutic result may be
governed by the reduction of blood supply to the mass caused by
surrounding scar tissue.
Surgical therapy is divided into conservative and definitive
operations. Conservative surgery involves the resection or
destruction of endometrial implants, lysis of adhesions, and
attempts to restore normal pelvic anatomy. Definitive surgery
involves the removal of both ovaries, the uterus, and all visible
ectopic foci of endometriosis.
Medical Therapy
The primary goal of the hormonal treatment of endometriosis
is induction of amenorrhea. Recurrent bleeding in the ectopic
implants is one of the most important pathophysiologic processes to
interrupt. Brosens has advanced the hypothesis that endometriosis is
a physiologic process unless recurrent bleeding in the ectopic
implants produces progressive disease and symptoms. Therefore, he
postulates that effective medical therapy can be established by
amenorrhea without the induction of hypoestrogenism. Recent
clinical studies confirm that effective medical treatment can be
achieved without the induction of severe hypoestrogenism. It is
hoped that hormonal treatment will create an environment that will
inhibit growth and promote regression of the disease. Medical
therapy is very effective in relieving pain while the patient is taking
medication. However, symptoms often recur several months after
discontinuing therapy. Both clinical symptomatology and findings
on second-look laparoscopy have demonstrated that clinical
improvement correlates directly with establishment of amenorrhea.
The choice of medical therapy for the individual patient depends on
the clinician's evaluation of adverse effects, side effects, cost of
therapy, and expected patient compliance. The clinical
effectiveness, as measured by relief of symptoms and recurrence
rates of current medical therapies, are similar. The recurrence rate
following medical therapy is 5% to 15% in the first year and
increases to 40% to 50% in 5 years. Obviously the chance of
recurrence is directly related to the extent of initial disease. In
summary, medical therapy usually suppresses symptomatology and
prevents progression of endometriosis, but it does not provide a
long-lasting cure of the disease. The recurrence rate in women who
initially had minimal disease is approximately 35% while in those
women whose initial disease was severe the rate is approximately
75%. The only two approved therapies for endometriosis are
danazol
and
GnRH
agonists.
Surgical Therapy
The choice between medical treatment to suppress
endometriosis and surgical therapy to remove lesions depends on
the patient's age, symptomatology, and reproductive desires.
Surgical therapy often occurs concurrently when a laparoscopy is
performed to establish the diagnosis of the disease. Obviously,
surgical therapy is the only option for failed medical therapy.
Because endometriosis is a puzzling disease, with great individual
variation in its natural course, many therapeutic regimens exist, and
individualized treatment is necessary.
Surgery has been the foundation of treatment for women with
moderate or severe endometriosis especially those with adhesions
and when the disease involves nonreproductive organs. A surgical
approach is mandatory in cases involving acute rupture of large
endometriomas, ureteral obstruction, compromise in intestinal
function or for large ovarian endometriomas.
Laparoscopy is employed frequently for both diagnostic and
therapeutic reasons. The major advantage of treating endometriosis
with the laparoscope, using surgical instruments, the laser, or
electrocautery, is that patients may be treated at the time of
diagnosis. Depending on the operative technique chosen,
endometriosis is coagulated, vaporized, or resected. Surgical
treatment for endometriosis should mainly be carried out via laparoscopy rather than by laparotomy because of a shorter recovery
period and reduction in the extent of subsequent adhesions.
Pediatric and Adolescent Gynecology : Gynecologic
Examination, Infections, Trauma, Pelvic Mass, Precocious
Puberty
KEY TERMS AND DEFINITIONS
A self-limited condition in which denuded
epithelium of the adjacent labia minora
Adhesive Vulvitis.
agglutinates and fuses the two labia
together.
Adolescence.
A transitional period of life during which an
individual matures physiologically and
occasionally psychologically from a child
into an adult.
Labial adhesions.
Agglutination of the adjacent labia minora
from a denuded epithelium that fuses the two
labia together.
Lichen Sclerosus
Atrophicus.
A skin dystrophy involving the labia seen
typically in prepubertal children and
postmenopausal women.
A rare syndrome of café-au-lait spots,
fibrous dysplasia, and lesions in the skull
McCune-Albright
and long bones, accompanied by precocious
Syndrome
(Polyostotic Fibrous puberty. The disorder is caused by a somatic
mutation (thus noninherited) in neural crest
Dysplasia).
cells with a mutation in the G protein system.
Puberty.
The process of biologic and physical
development after which sexual reproduction
first becomes possible.
Urethral prolapse.
Prolapse of the distal urethral mucosa seen
typically in prepubertal children.
Vulvovaginitis.
Inflammatory process involving the vulva
and vagina.
Gynecologic diseases are uncommon in children, especially
compared with the incidence and prevalence of diseases in women
of reproductive age. This chapter considers gynecologic diseases of
children from infancy through adolescence. Congenital anomalies,
precocious development, and amenorrhea are covered in more
detail in other chapters.
The evaluation of children's gynecologic problems involves
considerations of physiology, psychology, and developmental
issues that are different from those of adult gynecology. The
evaluation of young females is age-dependent. For example, the
physical presence of the mother often may facilitate examining a 4year-old girl but may inhibit the cooperation of a 14-year-old
adolescent. Thus, the gynecologic physical examination is
performed differently in a prepubertal child than in an adolescent of
reproductive age or a mature reproductive woman.
An outpatient visit by a prepubertal child to a gynecologist
should be structured differently from a gynecologic visit by a
woman of reproductive age. Considerable effort should be devoted
to gaining the child's confidence and establishing rapport. If the
interaction is poor during the first visit, the negative experience will
detract from future physician–patient interactions. The pediatric
gynecologic visit may be unique to both the child and the parent.
Most pediatric visits are preventive in nature. However, the
pediatric gynecologic visit is problem-oriented. This may create
considerable and understandable anxiety in the child and parent.
The vast majority of children's gynecologic problems are treated by
medical rather than surgical means.
The most frequent gynecologic disease of children is
vulvovaginitis. Vulvitis is generally the primary presenting
problem, with vaginitis of secondary importance and
symptomatology. Other common reasons for a pediatric gynecology
visit include labial adhesions, vulvar lesions, suspicion of sexual
abuse, and genital trauma.
Adolescence is the period of life during which an individual
matures physiologically and begins to transition psychologically
from a child into an adult. This period of transition involves
important physical and emotional changes. Before puberty, the girl's
reproductive organs are in a resting, dormant state. Puberty
produces dramatic alterations in both the external and the internal
female genitalia. Because the pubertal changes are frequently a
cause of concern for adolescent females and their parents, the
gynecologist must offer the adolescent female a kind,
knowledgeable, and gentle approach. These interactions between
the physician and the adolescent female will allow the physician an
opportunity to educate the pubertal teenager about pelvic anatomy
and reproduction.
KEY POINTS
•
In the field of pediatric gynecology, most diagnostic errors
result from errors of omission during the examination
rather than errors of commission.
•
It is important to 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 that there will be conversation during the entire
process.
•
Many gynecologic conditions in children can be diagnosed
by inspection alone.
•
The vaginal epithelium of the prepubertal child appears
redder and thinner than the vaginal epithelium of a woman
in her reproductive years. The prepubertal vagina is also
narrower, thinner, and lacking in the distensibility of the
vagina of a reproductively mature woman. The vagina of a
child is 4 to 5 cm long and has a neutral pH.
•
During the physical examination and rectal examination of
the prepubertal child, no pelvic masses except the cervix
should be palpable. The normal prepubertal uterus and
ovaries are nonpalpable. The relative size ratio of cervix to
uterus is 2 to 1 in a child.
•
Many female adolescents do not want other observers in
the examining room.
•
It is estimated that 80% to 90% of outpatient visits of
children to gynecologists involve the classic symptoms of
vulvovaginitis: introital irritation and discharge.
•
Positive identification of Trichomonas infection,
gonorrhea, or chlamydia in a child with premenarcheal
vulvovaginitis often indicates sexual molestation.
However, many infants are infected with Chlamydia
trichomatis during birth and remain infected for several
years in the absence of specific antibiotic therapy.
•
The major factor in childhood vulvovaginitis is poor
perineal hygiene.
•
A vaginal discharge that is both bloody and foul-smelling
strongly suggests the presence of a foreign body.
•
In the period from 6 to 12 months before menarche,
children often develop a physiologic discharge secondary
to the increase in circulating estrogen levels.
•
The foundation of treating childhood vulvovaginitis is the
improvement of local perineal hygiene.
•
The vast majority of cases of persistent or recurrent
nonspecific vulvovaginitis respond to improved hygiene
and treatment of irritation due to trauma or irritating
substances.
•
The classic symptom of pinworms (Enterobius
vermicularis) is nocturnal vulvar and perianal itching, the
treatment for which is the antihelminthic agent
mebendazole (Vermox).
•
The most common vaginal foreign body in preadolescent
females is a wad of toilet tissue.
•
Persistent vaginal bleeding is an extremely rare symptom
in a preadolescent female. However, it is important to do a
thorough workup because of the serious sequelae of some
of the causes of vaginal bleeding.
•
Labial adhesions do not require treatment unless they are
symptomatic or voiding is compromised. If necessary,
small amounts of daily topical estrogen to the labia may be
used for treatment.
•
The usual cause of genital trauma during childhood is an
accidental fall. The majority of such trauma involves
straddle injuries.
•
Accidental genital trauma often produces extreme pain and
overwhelming anxiety for the child and her parents.
Because of compassion and empathy, the gynecologist
may underestimate the extent of the anatomic injuries.
Thus, if in doubt, examine the child under general
anesthesia.
•
Small follicular cysts are common in preadolescent
females and are usually self-limiting.
•
Ovarian tumors constitute approximately 1% of all
neoplasms in premenarcheal children. In preadolescent
females, both benign and malignant ovarian tumors are
usually unilateral. Biopsy of the contralateral ovary should
be avoided. Possible exceptions to this rule are
dysgerminomas and immature teratomas.
•
Approximately 75% to 85% of ovarian neoplasms
necessitating surgery are benign, with cystic teratomas
being the most common.
•
The most common malignancy in preadolescent females is
a germ cell tumor.
•
Even though ovarian neoplasia is rare in children, this
diagnosis must be considered in a young girl with
abdominal pain and a palpable mass. The surgical therapy
should have two goals: removal of the neoplasm and
preservation of future fertility.
GYNECOLOGIC EXAMINATION OF A CHILD
General Approach
A successful gynecologic examination of a child demands
that the physician adapt an exam pace that conveys both gentleness
and patience with the time spent and not seem to be hurried or
rushed. One excellent technique is for the physician to sit, not stand,
during the initial encounter. This conveys an unhurried approach.
The ambiance of the examining room may decrease the anxiety of
the child if familiar and friendly objects such as children's posters
are present. Interruptions should be avoided. Speculums and
instruments that might frighten a child or parent should be within
drawers or cabinets and out of sight during the evaluation. If a child
is scheduled to be seen in the middle of a busy clinic, the staff
needs to be alerted that the pace and general routine will be
different during her visit.
Performance of the Gynecologic Exam in a Child
The components of a complete pediatric examination include
a history; inspection with visualization of the vulva, vagina, and
cervix; and, if necessary, a rectal examination.
Obtaining a history from a child is not an easy process.
Children are not skilled historians and will often ramble,
introducing many unrelated facts. Much of the history must be
obtained from the parents. However, young children can help define
their exact symptomatology on direct questioning. While obtaining
a history an opportunity exists to educate the child on vocabulary to
describe the genital area. One way to describe genital area and
breasts is to call them “private areas”—areas that are covered by
your bathing suit. The exam also allows a period of opportunity to
counsel children about potential sexual abuse. During the history
and most of the general physical examination, the child should sit
on the edge of the examination table.
After the history has been obtained, the parents and the child
should be reassured that the examination will not hurt. It is
important to give the child a sense that she will be in control of the
examination process. A helpful technique is to place the child's
hand on top of the physician's hand as the abdominal examination is
being performed. This will give the child a sense of control as well
as divert the child's attention if she is ticklish or is squirming.
Emphasize that the most important part of the examination is just
“looking” and there will be conversation during the entire process.
To successfully examine a child, one needs the cooperation of the
patient and a medical assistant such as a nurse.
A child's reaction will depend on her age, emotional maturity,
and previous experience with health care providers. She should be
allowed to visualize and handle any instruments that will be used.
Many young children's primary contact with providers involves
immunizations; children should be counseled and assured that this
visit does not involve any “shots.” It is also helpful to assure the
adult that has accompanied the child that adult speculums are not
part of the examination.
Occasionally it is best to defer the pelvic examination until a
second visit. This is a difficult decision and is based on the extent of
the child's anxiety in relation to the severity of the clinical
symptoms. A physician may elect to treat the primary symptoms of
vulvovaginitis for 2 to 3 weeks before searching for a foreign body.
However, in the field of pediatric gynecology many errors are
errors of omission rather than of commission.
A child should never be restrained for a gynecologic
examination. Often reassurance and sometimes delay until another
day are the best approach. Sometimes after performing the other
elements of the general exam enough rapport has been established
that the child will feel safe enough to allow a gynecologic
examination. In rare circumstances it may be necessary to use
continuous intravenous conscious sedation or general anesthesia to
complete an essential examination. The most important technique to
ensure cooperation is to involve the child as a partner. Children
should ideally feel they are part of the exam rather than having an
“exam done to them.”
Draping for the gynecologic examination produces more
anxiety than it relieves and is unnecessary in the preadolescent
child. A handheld mirror may help in some instances when
discussing specifics of genital anatomy. It is critical to have all
tools, culture tubes, and equipments within easy reach during a
pediatric genital examination. Children often cannot hold still for
long intervals while instruments are being searched for.
The 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, and
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.
The second phase of the examination involves evaluation of
the vagina. This can be accomplished without use of any insertion
of instruments. One method is to utilize the knee chest position. The
child lies prone and places her buttocks in the air with legs wide
apart. The vagina will then fill with air, aiding the evaluation. The
child is told to have her abdomen sag into the table. An assistant
pulls upward and outward on the labia majora on one side while the
examiner does the same with the nondominant hand on the
contralateral labia. Then an otoophthalmoscope is used as a
magnifying instrument and light source in the examiner's dominant
hand. The otoophthalmoscope is not inserted into the vagina. A
bright light helps to illuminate the upper vagina and cervix. The
light is shone into the vagina as the examiner evaluates the vaginal
walls through the otoophthalmoscope. The cervix appears as a
transverse ridge or pleat that is redder than the vagina. This
technique is generally successful in cooperative children unless
there is a very high crescent-shaped hymen, in which case it is too
difficult to shine the light into the small aperture at the vaginal
introitus. Foreign object and the cervix may be visualized using this
technique. Following inspection of the vagina and cervix, vaginal
secretions may be obtained for microscopic examination and
culture.
Normal Findings: Hymen and Vagina of a Prepubertal Child
The hymen of a prepubertal child exhibits a diverse range of
normal variations and configurations ( Fig. 13-5 ). Hymens are
often crescent-shaped but may be annular or ringlike in
configuration. There are no reported cases of congenital absence of
the hymen. 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. Hymens in newborns are
estrognenized, resulting in a thick elastic redundancy. Older
unestrogenized girls will have thin nonelastic hymens. Prospective
studies of hymens in children has demonstrated that complete
transections of the hymeneal tissue between 3 o'clock and 9 o'clock
are not congenital but likely acquired. Noncongenital “bumps” may
be present near hymeneal transections. The subject of hymens in
relation to sexual abuse is covered later in this chapter.
The vaginal epithelium of the prepubertal child appears
redder and thinner than the vagina of a woman in her repro-ductive
years. The vagina is 4 to 6 cm long, and the secretions in a
prepubertal child have a neutral or slightly alkaline pH. Recurrent
vulvovaginitis, persistent bleeding, suspicion of a foreign body or
neoplasm, and congenital anomalies may be indications for
vaginoscopy. Introduction of any instrument into the vagina of a
young child takes skillful patience. The prepubertal vagina is
narrower, thinner, and lacking in the distensibility of the vagina of a
woman in her reproductive years.
Vaginoscopy often requires a brief inhalation anesthesia but
can be preformed in the office in very cooperative children in some
circumstances. There are many narrow-diameter endoscopes that
will suffice, including the Kelly air cystoscope, contact
hysteroscopes, pediatric cystoscopes, small-diameter laparoscopes,
plastic vaginoscopes, and special virginal speculums designed by
Huffman and Pederson. The ideal pediatric endoscope is a
cystoscope or hysteroscope because the accessory channel
facilitates lavage of the vagina. A nasal speculum or otoscope is
usually too short. Local anesthesia of the vestibule may be obtained
with 2% topical viscous lidocaine (Xylocaine). The physician can
divert the child's attention from the endoscope in the vagina by
simultaneously gently compressing one of the patient's buttocks, an
extinction technique. Vaginal evaluation should never be performed
under duress or by force.
The last step in the pelvic examination is a rectal
examination. This most distressing aspect of the examination may
sometimes be omitted, depending on the child's symptoms.
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
prepubertal uterus and ovaries are nonpalpable 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. Except for the
cervix, any mass discovered on rectal examination in a prepubertal
exam should be considered abnormal.
Examination of the Adolescent Female
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
other observers in the examining room. In one study at a university
hospital clinic, 24% of inner-city youths did not want a chaperon
present. In many adolescent gynecology visits a full pelvic exam is
unnecessary.
Adolescents often come for examinations with preconceived
ideas that it will be very painful. Slang terminology for speculums
among teens includes the threatening label “the clamp.” Teens
should be assured that although the exam may include mild
discomfort it is not painful. Providers can counsel patients that they
will inform them of each step in the process and then ask the teen if
she is ready before performing each step. This places the teen in
control of the tempo and allows her to anticipate the next element of
the examination. Use of the “extinction phenomena” may be
helpful. The examiner provides pressure lateral to the introitus on
the perineum prior to insertion of the speculum.
Vulvovaginitis
Vulvovaginitis is the most common gynecologic problem in
the prepubertal female. It is estimated that 80% to 90% of
outpatient visits of children to gynecologists involve the classic
symptoms of vulvovaginitis: introital irritation and discharge.
The prepubertal vagina is neutral or slightly alkaline. With
puberty the prepubertal vagina becomes acidic under the influence
of bacilli dependant on a glycogenated estrogen-dependant vagina.
Breast budding is a reliable sign that the vaginal pH is shifting to an
acidic environment.
The severity of vulvovaginitis symptoms varies widely from
child to child. The pathophysiology of the majority of instances of
vulvovaginitis in children involves a primary irritation of the vulva
with secondary involvement of the lower one third of the vagina.
Most cases involve an irritation of the vulvar epithe-lium by normal
rectal flora. This is referred to as nonspecific vulvovaginitis There
may be a predisposing vulvar irritation secondary to a topical
allergy, a vulvar irritant such as perfumed soaps or the tight seams
of blue jeans, which creates denudation, allowing the rectal flora to
easily infect the irritated epithe-lium. Cultures from the vagina
return as normal rectal flora or Escherichia coli. In a primary care
setting nonspecific vulvovaginitis accounts for the vast majority of
vulvovaginitis cases.
There are both physiologic and behavioral reasons why a
child is susceptible to vulvar infection. Physiologically, the child's
vulva and vagina are exposed to bacterial contamination from the
rectum more frequently than are the adult's. Because the child lacks
the labial fat pads and pubic hair of the adult, when a child squats,
the lower one third of the vagina is unprotected and open. There is
no geographic barrier between the vagina and anus. The vulvar and
vaginal epithelium lack the protective effects of estrogen and thus
are sensitive to irritation or infection. The labia minora are thin and
the vulvar skin is red because the abundant capillary network is
easily visualized in the thin skin. The vaginal epithelium of a
prepubertal child has a neutral or slightly alkaline pH, which
provides an excellent medium for bacterial growth. The vagina of a
child lacks glycogen, lactobacilli, and a sufficient level of
antibodies to help resist infection. The normal vagina of a
prepubertal child is colonized by an average of nine different
species of bacteria: four aerobic and facultative anaerobic species
and five obligatory anaerobic species.
A major factor in childhood vulvovaginitis is poor perineal
hygiene. This results from the anatomic proximity of the rectum
and vagina coupled with the fact that following toilet training, most
youngsters are unsupervised when they defecate. Many youngsters
wipe their anus from posterior to anterior and thus inoculate the
vulvar skin with intestinal flora. A minor vulvar irritation may
result in a scratch–itch cycle, with the possibility of secondary
seeding because children wash their hands infrequently. Children's
clothing is often tight-fitting and nonabsorbent, which keeps the
vulvar skin irritated, warm and moist, and prone to vulvovaginitis.
Table 1 -- Etiologic Factors of Premenarcheal Vulvovaginitis
Bacterial
A. Nonspecific
1. Poor perineal hygiene
2.
Intestinal parasitic invasion with pruritus
3.
Foreign bodies
Bacterial
4.
Urinary tract infections with irritation
B. Specific
Bacterial
1. Group A: β-hemolytic streptococci
2.
Streptococcus pneumoniae
3.
Haemophilus influenzae/parainfluenzae
4.
Staphylococcus aureus
5.
Neisseria meningitides
6.
Escherichia coli
7.
Shigella flexneri/sonnei
8.
Other enterics
9.
Neisseria gonorrhoeae
10. Chlamydia trachomatis
Protozoal—Trichomonas
Mycotic
1. Candida albicans
2.
Other
Helminthiasis—Enterobius vermicularis
Viral/Bacterial Systemic Illness
1. Chicken pox
2.
Measles
3.
Pityriasis rosea
4.
Mononucleosis
5.
Scarlet fever
6.
Kawasaki disease
Other Viral Illnesses
1. Molluscum contagiosum in genital area
2.
Condylomata acuminata
3.
Herpes simplex-type II
Physical/Chemical Agents
Bacterial
1.
Sandbox
2.
Trauma
3.
Bubble bath
4.
Other
Allergic/Skin Conditions
1. Serborrhea
2.
Lichen sclerosus
3.
Psoriasis
4.
Eczema
5.
Contact dermatitis
Tumors
Other
1. Prolapsed urethra
2.
Ectopic ureter
From Blythe MJ, Thompson L: Premenarchal vulvovaginitis.
Indiana Med 86:237, 1993.
In some cases nonspecific vulvovaginitis may be caused by
carrying viral infections from coughing into the hands directly to
the abraded vulvar epithelium. Similarly, a child with an upper
respiratory tract infection may autoinoculate her vulva, especially
with group A βhemolytic streptococci.
Vulvovaginitis in children may also be caused by specific
pathogens. Cultures of the vaginal discharge may identify specific
organisms such as group A or group B βhemolytic strepto-cocci,
Haemophilus. influenzae, Neisseria gonorrhoeae, Trichomonas
vaginalis, Chlamydia trachomatis, and Shigella boydii.
Pinworms are another cause of vulvovaginitis in prepubertal
children. Approximately 20% of female children infected with
pinworms (Enterobius vermicularis) develop vulvovaginitis. The
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. The tape is subsequently
examined under the microscope.
Mycotic vaginal infections are not common in prepubertal
children as the alkaline pH of the vagina does not support fungal
growth. Mycotic vaginal infections may be seen in
immunosuppressed prepubertal girls such as HIV patients or
patients on chronic steroid therapy. Other specific causes of
vulvovaginitis may include systemic diseases, chicken pox, and
herpes simplex infection.
There is nothing specific about the symptoms or signs of
childhood vulvovaginitis. Often the first awareness comes when the
mother notices staining of the child's underwear or the child
complains of itching or burning. There is a wide range in the
quantity of discharge, from minimal to copious. The color ranges
from white or gray to yellow or green. A discharge that is both
bloody and purulent is likely not from vulvovaginitis but from a
foreign body (see Prepubertal Bleeding without Puberty). A
purulent bloody discharge can also be seen in patients with shigella
vaginitis. The signs of vulvovaginitis are variable and not
diagnostic, but include vulvar erythema, edema, and excoriation.
The differential diagnosis of persistent or recurrent
vulvovaginitis not responsive to treatment should include
considerations of a foreign body, primary vulvar skin disease,
ectopic ureter, and child abuse. If the predominant symptom is
pruritus, then pinworms or an irritant vulvitis are 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 sclerosus. 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 foundation of treating childhood vulvovaginitis is the
improvement of local perineal hygiene. Both parent and child
should be instructed that the vulvar skin should be kept clean, dry,
and cool. For acute weeping lesions, wet compresses of Burow's
solution should be prescribed. An alternative is a sitz bath
containing 2 tablespoons of baking soda in the water. The child
should be instructed to void with her knees spread wide apart 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, and dryness of the
vulva should be maintained with calamine lotion or a nonirritating
cornstarch powder. Many episodes of childhood vulvovaginitis are
cured solely by improved local hygiene. The vast majority of cases
of persistent or recurrent nonspecific vulvovaginitis respond to a
combination of topical creams, low-potency steroids, or oral
antibiotics given for 10 to 14 days. If, however, the problem is
hygiene, then broad-spectrum antibiotics will only offer temporary
relief, and the problem is likely to recur. Relief of vulvar irritation
may be facilitated by using a bland cream, such as zinc oxide
creams or cod liver oil creams, both of which are readily available
in the infant's sections of drug stores. They should be applied
several times per day. Another approach is to utilize very low
potency steroid creams which are available over the counter.
If the initial therapy is not successful oral antibiotics should
be reconsidered. Vaginal cultures help to determine the choice of an
oral broad-spectrum antibiotic. Dosage of the selected antibiotic
depends on the child's weight. One method of obtaining a vaginal
culture in children is to use a nasopharyngeal small swab moistened
with nonbacterostatic saline. Pokorny has described another method
for collecting fluid from a child's vagina using a catheter within a
catheter. This easily assembled adaptation uses a No. 12 red rubber
bladder catheter for the outer catheter and the hub end of an
intravenous butterfly catheter for the inner catheter ( Fig. 13-6 ).
The outer catheter serves as an insulator, and the inner catheter is
used to instill a small amount of saline and aspirate the vaginal
fluid. The re-sults of the vaginal culture may demonstrate normal
flora or an overgrowth of a single organism that is a respiratory,
intestinal, or sexually transmitted disease pathogen. The presence of
any sexually transmitted organisms in a child is indication that
sexual abuse may have taken place and appropriate referral and
follow-up is necessary.
Other Prepubertal Gynecologic Problems
Labial Adhesions (Adhesive Vulvitis)
Labial adhesions literally mean the labia minora have
adhered or agglutinated together at the midline. Another term
sometimes used to describe this condition is adhesive vulvitis.
Denuded epithelium of adjacent labia minora agglutinates and fuses
the two labia together, creating a “flat appearance” of the vulvar
surface. A “tell-tale” somewhat translucent vertical midline line is
visible on physical exam at the site agglutination. This thin, narrow
line in a vertical direction is pathognomonic for adhe-sive vulvitis (
Fig. 13-7 ). Labial adhesions are often partial and only involve the
upper or lower aspects of the labia.
Inexperienced examiners may confuse labial adhesions for an
imperforate hymen or vaginal agenesis. Although the 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 appear normal 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 young girls between 2
and 6 years of age. Estrogen reaches a nadir during this time,
predisposing the nonestrogenized labia to denudation (see Fig. 13-7
). In one large study the average age of a child with agglutination of
the labia was 2½ years; 90% of cases appeared before age 6. There
is considerable variation in the length of agglutination of the two
labia minora. 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. How-ever, the partially fused labia may form a pouch in
which urine is caught and later dribbled, presenting as incontinence.
Associated urinary infections have been reported.
No treatment is absolutely necessary or ‘mandatory’ for
adhesive vulvitis unless the child is symptomatic. Symptoms
include voiding difficulties, recurrent vulvovaginitis, discomfort
from the labia pulling at the line of adhesions, and in rare cases
bleeding from the line of adhesion pulling apart. Jenkinson and
Mackinnon published results of a series of 10 girls who had no
therapy and noted spontaneous separation of the adhesive vulvitis
within 6 to 18 months. Attempts to separate the adhesions apart in
the office by pulling briskly on the labia minora should not be done.
It is very painful, and the raw edges are likely to readhese as the
child will be reticent to allow applica-tion of medication after being
subjected to this degree of pain. Some clinicians have also
recommended placing local anes-thetics on the adhesions and using
swabs to tweeze them apart. Many adhesions that would resolve
with this therapy are not symptomatic and may not require
treatment. However, in rare cases this is appropriate if the local
anesthesia (either lidocaine jelly or lidocaine/prilocaine) is
successful in preventing discomfort.
The most commonly utilized treatment of this condition is
topical estrogen cream dabbed onto the labia two times per day at
the site of fusion. This will usually result in spontaneous separation,
usually in approximately 2 weeks. In cases without resolution the
clinician can reexamine the patient. If increased pigmentation is
noted lateral to the midline line of agglutina-tion 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.
Recurrent adhesive vulvitis occurs in one of five children.
Once resolution has occurred reagglutination 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 one month.
A familial form of true posterior labial fusion has been
reported by several authors. Analysis of the pedigrees of these
children suggests that this congenital defect may be an auto-somal
dominant trait with incomplete penetrance. McCann and colleagues
(1988) reported the association between injuries of the posterior
fourchette and labial adhesions in sexually abused children. Labial
agglutination alone is so common that immediate suspicion of child
abuse based solely on this finding in 2- to 6-year-olds is
unwarranted. However, the combination of labial adhesions and
scarring of the posterior fourchette especially in children with newonset labial adhesions after age 6 should prompt the clinician to
consider sexual abuse in the differential diagnosis.
Physiologic Discharge of Puberty
In the early stages of puberty, children often develop a
physiologic vaginal discharge. This discharge is typically described
as having a gray-white coloration although it may appear slightly
yellow but is not purulent. The physiologic discharge represents
desquamation of the vaginal epithelium. The estrogenic
environment allows acid-producing bacilli to become part of the
nor-mal vaginal ecosystem. The acids the bacilli produce cause a
desquamation of the prepubertal vaginal epithelium. When the
physiologic discharge is examined with the microscope, sheets of
vaginal epithelial cells are identified.
Clinically there is usually very little symptomatology
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 maybe treated with sitz baths and frequent
changing of underwear.
Urethral Prolapse
Prolapse of the urethral mucosa is not a rare event in
children. The 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° 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 is conservative and noninteventional. Many
experts recommend application of various medications including
estrogen creams and antibiotic ointments. Although such
interventions appear appropriate, well-designed prospective studies
have not been done to confirm they are therapeutic. Surgery is
seldom necessary except in rare cases where necrosis is obviously
present.
Lichen Sclerosus
Lichen sclerosus (LS) formerly called lichen sclerosus atrophicus,
is a skin dystrophy most commonly seen in postmenopausal women
and prepubertal children. The cause is unclear although there is
some evidence that it may be associated with autoimmune
phenomena. This has not been confirmed by prospective studies.
Histologically there is thinning of the vulvar epithelium with loss of
the rete pegs. The most common symptoms are pruritus and vulvar
discomfort. Other presentations may include prepubertal bleeding,
constipation, and dysuria.
The appearance of LS varies, but lesions are always limited
by the labia majora. If lesions go beyond the labia majora is
unlikely to be LS. The lesion often appears in an hourglass or
figure-8 formation involving the genital and perianal area. The skin
may be lichenified with a parchment-like appearance. Parents may
note that the genital area appears “whitened.” Pruritus is typical but
not inevitable. In some cases the pruritus is so severe that patients
have used hair brushes to scratch the area in an attempt to stop the
pruritic sensation. Secondary changes may occur subsequent to the
patient excoriating the area. When this occurs, there are often blood
blisters and breaks in the integument. Patients with blood blisters
may present with prepubertal bleeding.
Given the abnormal appearance of LS with secondary
changes, clinicians unfamiliar with this skin dystrophy often arrive
at the misdiagnosis of sexual abuse. However, clinicians
experienced in pediatric or postmenopausal gynecology will usually
have no difficulty arriving at the correct diagnosis. In cases where
the diagnosis is unclear a small punch biopsy may confirm the
diagnosis. Performing a biopsy in prepubertal children is often
difficult. Many children will not tolerate a local injection, and
holding down children to perform a biopsy is clearly not acceptable.
General anesthesia is preferable in this situation. Some children will
tolerate a biopsy using local anesthesia, and 1% lidocaine without
epinephrine may be used.
The treatment of LS in children should always start with
avoiding irritation or trauma to the genital epithelium. LS is a skin
disorder in which lesions are most likely to occur in epithelium that
is irritated. 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 blue jeans may also
abrade and irritate the vulva.
Prepubertal Bleeding without Secondary Signs of Puberty
Puberty in the female is the process of biologic change and
physical development after which sexual reproduction becomes
possible. This is a time of accelerated linear skeletal growth and
development of secondary sexual characteristics, such as breast
development and the appearance of axillary and pubic hair. The
usual sequence of the physiologic events of puberty begins with
breast development, the subsequent appearance of pubic and
axillary hair, followed by the period of maximal growth velocity,
and lastly, menarche. Menarche may occur before the appearance of
axillary or pubic hair in 10% of normal females. Normal puberty
occurs over a wide range of ages (see Chapters 4 and 38
[Reproductive Endocrinology and Primary and Secondary
Amenorrhea and Precocious Puberty]). Precocious puberty is
arbitrarily defined as the appearance of any signs of secondary
sexual maturation at an early specific age more than 2.5 standard
deviations below the mean. Precocious puberty is covered in detail
in Chapter 38 . 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
The normal sequence of puberty is that thelarche precedes
menarche. In children with prepubertal bleeding without breast
budding there is almost never an endocrinologic cause, with the
exception being a very rare presentation of McCune–Albright
syndrome.
The 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. Rare causes include malignant tumors (sarcoma botryoides
and endodermal sinus tumors of the vagina) and an unusual
presentation of McCune–Albright syndrome. Lists of the
differential diagnosis of prepubertal bleeding often also include
accidental estrogen exposure (for example from ingestion of a
mother's birth control pills). However, in reality such exposure
would rarely provide enough endometrial stimulation to produce a
withdrawal bleed without breast budding. Neonates may develop a
white mucoid vaginal discharge or a small amount of vaginal
spotting. Both conditions are secondary to exposure during
pregnancy to the high levels of estrogen and are self-limited.
Foreign Bodies
Symptoms secondary to a vaginal foreign body are
responsible for approximately 4% of pediatric gynecologic
outpatient visits. The vast majority of foreign bodies are found in
girls between 3 and 9 years of age. The history is usually not
helpful because an adult has not witnessed, nor does the child
remember, putting a foreign object into the vagina. Many types of
foreign bodies have been discovered; however, the 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. Foreign bodies may be
inserted by children because the genital area is pruritic or when
naturally curious children are exploring their bodies.
The classic symptom is a foul, bloody vaginal discharge.
However, the 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. There is often a lag between insertion of the object and the
vaginal bleeding. Over time, the foreign body may become partially
“buried” into the vagina, and difficult to remove without
discomfort.
The presence of unexplained vaginal bleeding is one indication for
a vaginoscopy. In children younger than 6 years of age this should
be done expeditiously to rule out malignant vaginal tumors. 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. The catheter technique described previously may
be utilized. Often 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.
Children who insert foreign objects often are “repeat
performers.” This may be secondary to pain or pruritus in the
genital area, and the child uses the “object” (solid or toilet paper) to
rub or scratch the genital area. Sometimes this possibility may be
reduced by using wipes instead of toilet paper.
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 is a rare somatic mutation that
occurs during embryogenesis in neural crest cells. Since the
mutation does not occur in the germline, it is not inherited. The
mutation affects G protein receptors and has a quite variable
expression, depending on how many early cells are affected (an
example of mosaicism). The GNAS1 gene is the affected area. The
syndrome is manifested by 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, and endodermal sinus tumors occur
prior to age 2. Although these tumors are extremely rare causes of
prepubertal bleeding, they must be considered in every 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
Many times no clear cause of prepubertal bleeding is defined
at vaginoscopy. In these cases there likely was a small foreign
object that has been expelled from the vagina or disintegrated. Even
though many vaginoscopies are negative, it is especially important
for clinicians to perform them promptly in young prepubertal
bleeders to exclude the rare but very aggressive vaginal
malignancies.
ANOMALIES OF DEVELOPMENT
AND ABNORMAL POSITIONS
OF FEMALE GENITALIA
DEVELOPMENT OF FEMALE
GENITALIA IN PRENATAL
PERIOD
On the 3-4th weeks of embryo development on internal surface
of primary ddney a gonad germ is generated. Primary gonad has an
indifferent structure s identical for both genders) and consists of
celomic epithelium cells (external hyer), mesenchyme (internal
medullar layer) and gamete cells — gonocytes. Sexual
differentiation of indifferent glands is induced by sexual
chromosomes. Y-chromosome presence determines testicle
development, and X-chromosome presence determines ovarian
development.
External genitals of fetus also goes through the different stages of
development. у are germinated on the 6-7th weeks of the
development in the form of genital
inence and urethral fissure, bordered by urethral and labioscrotal
folds (fig. 46).
Forming of masculine sexual glands begins from the 7th week,
and masculine enitals — from the 8th week of fetal development.
Differentiation of female reproductive system takes place in later
terms. Funning of female-type gonads begins from 8-10th week of
pregnancy. Presence of 2 X-chromosomes in a zygote is necessary
for ovarian development. A gene inducing ovarian development is
localized in long shoulder of X-chromosome. Under its influence
gonocytes are transformed into ovogonies, then — into oocytes,
around which the primary granulous cells are generated from
mesenchimal cells. They are situated in the cortex of sexual gland
and intensively ieproduce themselves by means of mitotic division.
On the 5th month of embryonal development a number of primary
follicles reaches 4 mln., till the birth time of a girl their amount is
reduced to 1 mln. The ovary is morphologically formed.
Internal genitalia — uterine tubes, body, uterine cervix, upper
1/3 of vagina are formed from paramesonephral ducts. Process
starts on the 5-6th and finishes в 18th week of pregnancy. From
upper one-third of paramesonephral ducts uterine tubes are formed.
Lower and middle parts uniting together form a body and uterine
cervix. Lower department of paramesonephral ducts forms the upper
one-third of vagina, lower 2/3 are formed from urino-genital sinus
(fig. 44).
Common organ cavity is formed to 21-22 week of gestation.
The rest of mesonephral channels are preserved as paraovophorone,
epiovophorone and Gartner's passages on the lateral walls of vagina
(fig. 45). External female genitals are formed since the 17th week of
gestation. At first major labia are formed from labioscrotal folds, from
urethral folds minor labia are generated. Clitoris is formed from
genital prominence (fig. 46).
Fig. 45. The rest
of female
genitalia
embryonic
formation
Gartner s
passagesovary
epiovopho
rone
DEVELOPMENT
ANOMALIES OF
EXTERNAL GENITALIA
Most frequently defects of external genitalia development are
common at androgeny and adrenogenital syndrome. These defects of
external genitalia development are manifestations of genital glands
development violations and they will be discussed in the
corresponding chapter.
Fig. 46.
Development
of the male (A)
and female
(B) external
genitalia:
1— genital
tubercle
2— urogenital
folds
3—labioscrotal
folds
UTERINE AND VAGINA1
DEVELOPMENT
ANOMALIES
Genitalia formation in female fetus takes place during the first
months of gestation from the middle embryonic layer
(mesenchyme). From the same layer the organs of urinary system
are generated, that's why the uterine and vaginal anomalies can be
combined with urinary organs anomalies.
The ovaries are formed on the first weeks of gestation from
indifferent (identical for both genders) genital gland. On the third
month of gestation their differentiation starts. The ovaries dislocate
down and draw into the small pelvis.
Uterus, uterine tubes and vagina are developed from
mesodermal germs (MuUer's ducts). One uterine tube, a half of
uterus and vagina are generated from each of them. The middle and
lower third of these ducts are united on the second month of
gestation, forming the external organ contour, but on all the length
uterus and vagina are parted by membrane. During the 3rd month of
fetal development this membrane is dissolved, uterine cavity and
vagina are generated. Uterine tubes are formed from the upper parts
of MuUer's ducts that didn't join.
External genitalia are generated from urino-genital sinus.
If during the act of sexual organs forming harmful factors,
specifically medicinal ones (uncontrolled medicines reception),
would affect a pregnant woman, a differentiation process of the
genitals can be broken. Agenesy is the absence of the organ and
even of its rudiment. Aplasia is the absence of the organ's part.
Atresia is underdevelopment in the result of the prenatal cause.
Proceeding from mechanism of genitalia forming, such variants
of uterine and vaginal development defects are possible:
1.Both mesonephral ducts are formed properly, but they are not
joined together along the whole length. A full uterus and vagina
doubling (uterus didelfus) is generated: the patient has two vaginas
divided by a thin membrane. Uterine cervix opens into each vagina.
There are two uteruses (unicornous), in each uterus there is one tube
and one ovary. Both uteruses can function. In the patients with such
pathology pregnancy loss is more frequent. In most cases one half
of sexual apparatus is developed better than the other one (fig. 47
a).
2.Both mesonephral ducts are formed properly, but their uniting
takes place only at any interval. Other parts of uterus and vagina are
divided by a membrane. There can be following variants: membrane
in vagina (vagina septa); presence of one vagina, into which two
uterine cervices open (uterus bicornus bicollis); membrane in
uterine cavity (uterus septa), two-horned uterus (uterus bicornus);
saddle-like uterus (uterus arcuatus). At such anomalies genitals can
function normally, pregnancy can occur, but frequently pregnancy
loss takes place. When there is a saddle-like uterus the irregular
fetus positions are usually diagnosed (fig. 47 b, c, d).
3.One of MuUer's ducts develops properly, and the other one does
not develop at all. Vagina, single-horn uterus with one ovary and one
tube is formed (uterus unicornus). In such patient one should inspect
urinary system, because such defect correlates with the absence of
kidney on the affected side. Unicornous uterus can function,
menstrual function is usually for the type of hypomenstrual syndrome.
5
Fig. 47. Genitalia anomalies development:
a — complete double uterus, cervix and vagina; b —
complete double uterus, cervix с — bifid uterus with
single vagina; d — saddle-like uterus
The woman can become pregnant, however there exists high frequency
of pregnancy loss on the early terms.
4. One of the mesodermal ducts develops properly, the other—
insufficiently. The uterus with rudimentary horn is formed. These
cavities can be joined, that's why pregnancy in rudimentary horn is
possible. It develops as ectopic pregnancy. During its interruption a
considerable bleeding takes place (horn rupture) that's why the
surgical intervention is necessary. At presence of closed cavity of
rudimentary horn during menstruation blood can deposit inside, that
needs the removal of the horn.
If uterine development anomalies are combined with
underdevelopment of genitals it is followed by violation of
menstrual cycle, infertility.
Diagnosis is made after examination of external genitals,
uterine cervix examination in specula,
bimanual
examination.
Ultrasound
examination,
sounding,
hysterosalpingography or contrasting sonography
(contrasting substance "Echovist" is used)
are necessary for specification of
diagnosis.
Treatment of the development
anomalies is surgical. Doubling of uterus
and vagina, which does not disturb
woman's sexual and reproductive
functions,
Operative
doesn't
need
intervention.
Fig. 48. Bifid uterus
treatment is necessary at presence of ectopic preg(gyStcrosaipmgography)
nancy or agglomeration of menstrual blood in rudimentary horn.
Membranes in vagina are usually diagnosed during pregnancy or
delivery; if they prevent child's birth, they are lanced.
Absence of vagina (aplasia vagina) is a serious defect, which
makes impossible the realization of menstrual, sexual and
reproductive functions. It develops primarily (in fetus) or secondly in
the result of healing after the carried difficult inflammatory processes
in babyhood (smallpox, diphtheria, scarlet fever). It can rarely appear
in women after serious labour traumas.
Treatment is only surgical. It is a plastic
operation with vagina formation from
allotment of sigmoid bowel, recently
allopl'asty is common.
Gynatresy — violation of genital channel
permeability in some of its departments. Most
frequently atresia of hymen (fig. 49), vagina
and uterine cervix are present.
hig. 4V.
Hymen' atresia
Primary gynatresy develops in fetus in the result
Hematocolpos
of embryonic development defects. Secondary gynatresy (acquired)
develops in the result of inflammatory processes, carried in
childhood (fig. 51). In mature age vaginal atresia can occur in the
result of labour traumas, uterine cervix atresia after
diathermocoagulation, atresia of uterine cavity or adhesion in it after
surplus uterine curretage because of abortion.
Fig. 50. Hymen' atresia:
a — hematocolposhematometra
hematocolposhematometrahematosalpihx
Primary atresia of hymen ought to be diagnosed by medical personnel or by girl's
mother still in newborn period. Then all the further complications can be avoided. If
gynatresy is not found in time, then with the beginning of the first menses blood
begins to accumulate in vagina, straining it (haematocolpos). Girls complain of pain.
After finishing of such latent menses blood gemolizes, liquid part of it is absorbed,
volume is decreased, pain stops until the following menses begin. If the patient does
not apply for medical help, then blood, accumulating
:: ■■:..
Fig. 51. Labia major adhesion (a). The same patient after adhesion incision
(b)
more and more, gathers in the uterine cavity (haematometra), and in the uterine tubes
(haematosalpinx).
Diagnosis consists of examination of external genitals, during which one can see
obstructed hymen and blood, that has accumulated behind it. During the rectal
examination one can palpate tumorous formation in allotment of vagina, uterus and
uterine tubes.
Treatment Surgical incision of hymen is necessary. Hymen is crosswise incised.
Thick, brown-colored blood is removed from vagina. In order to prevent secondary
atresia they put several stitches in dissection edges.
Prognosis depends on the interm diagnostics of disease. At long illness duration
and development of haematometra and haematosalpinx later a woman can have
problems with pregnancy. Destructive process in uterine tubes leads to their
occlusion. Endometriosis of internal genitals develops frequently.
ABNORMALITES OF OVARIAN DEVELOPMENT
Hermaphroditism is a presence of signs of both sexes in one person. True
hermaphroditism is presence of genital glands of both sexes in one person on
condition of their simultaneous functioning. Such defects are almost not found in
practice, because children, sexual glands of which contain simultaneously the tissue of
ovary and testicle, are born with other different defects, and die during the first days
of their life.
False hermaphroditism (pseudoandrogeny) is a defect, at which the structure
of external genitalia does not correspond to the character of sexual gland. Human
sex is determined by chromosome set, according to which genital glands are
developed. At false female hermaphroditism internal genitals and sexual glands are
female (ovaries), and external sexual organs are developed like the male ones —
clitoris is enlarged and looks like penis, major labia are hypertrophied and look like
scrotum (fig. 52, 53). Sometimes after the birth such children's sex is mistakenly
determined and parents begin to bring a girl up as a boy. That's why in case of child
birth with anomaly of genitals development it is necessary to carry out careful
examination, including the genetic one.
At false male hermaphroditism genital glands are male (testicles), and structure of
sexual organs looks like the female ones.
The congenital adrenogenital syndrome is the disease that develops by reason
of adrenal glands cortical layer hyperfunction. It is followed by increasing in fetal
organism of female sex sexual hormones (androgens) and causes the formation of
female genitals according to the masculine type. It is very important to determine
correctly the child's sex at birth.
Clinic. In such girl the period of puberty begins at the age of 6-7 and it is
followed by virilization signs (appearance of masculine secondary sexual signs) —
hair growth, forming of skeleton and body building according to masculine type.
Children are of a low height, lower extremities are short because of the early
epiphisar cartilage closing. At postpubertal form, when the disease starts after the
period of the puberty beginning, amenorrhea or oligomenorrhea are found in girls.
Breasts, uterus and ovaries do not develop. Later the woman suffers from the primary
infertility.
Treatment is prescribed by an obstetrician-gynecologist together with endocrinologist. Medicines of glucocorticoid hormones (Prednizolone, Cortisone,
Dexametasone) are prescribed to decrease
androgens production by adrenal glands.
Owing to this the gonadotropic pituitary
function increases, ovarian stimulation and
production of own estrogens begins.
Ovarian absence — two or one —
happens rarely, predominantly in fetuses
having other severe development defects.
Ovarian hypoplasy is the insufficient
development of ovaries frequently
combined with
Fig. 52. Female external genitalia .
■ ,, ■. ■
of 20-year-old patient with false
mani-
uterme
,
,
,
,/-,,-
underdevelopment. Clinically it is
femaie hermaphroditism
fested as hypomenstrual syndrome.
Abnormalites of ovarian
development
89
Fig. 53. False female
hermaphroditism
(patient's appearance)
The gonads' dysgenesia (the Shereshevsky-Terner syndrome) is the disease,
associated with chromosome abnormalities (one X-chromosome is absent) that
causes ovarian tissue underdevelopment. The ovaries are represented by
connective tissue, their function is absent.
Diagnosis. These patients are of low hight (not higher 130-145 cm). Body
weight after birth is low even at interm pregnancy. During examination a short
neck with wing-like folds from ears to shoulders, wide shoulders and tubby
thorax are typical. The external eyes' corners are drawn down, palate is high,
that's why these patients have special timbre of voice. Psychic development is
normal, sexual orientation is female, but in the puberty period secondary sexual
signs develop not enough (fig. 54).
During gynecological examination highly expressed signs of genital infantilism are found. External genitalia are underdeveloped, there is a severe vagina,
uterine and ovarian hypoplasia. Genetic examination, that confirm the chromosome anomaly has a great importance for specification of diagnosis. Tests of
functional diagnostics give a picture of the
expressed lowering or practical absence of
hormones, the basal temperature is
permanently low, "fern" and "pupils"
symptoms are absent. There is 50% of
parabasal cells during colpocytological
investigation.
Treatment at prepuberty age is directed
on the growth stimulation. After 15-17 years
of age replacement t'herapy wifh "hormones is
prescribed: they are estrogens for 6-9
.months, after this the cyclic therapy with
Estrogens and Progesterone is indicated.
Such treatment leads to development of
secondary sexual signs, uterine cyclic
bleedins initate.
Polycystic ovarian disease (the SteinLeven-thal's syndrome). This is a genetically predisposed disease,
Fig. 54. Shereshevsky-Terner pathogenesis of which is a violation of
se-syndrome. A patient of 16 years old xuai hormones synthesis in ovaries
in the result of insufficiency of enzyme systems. Excess amount of
androgens is produced.
Clinically this disease is characterized by excessive hairiness
(hirsutism), by hypomenstrual syndrome or by amenorrhea and
infertility. Well-developed secondary sexual signs and enlarged twosided ovaries are found during the examination. During US-onografy
the presence of a great deal of follicular cysts, that is a cause of
ovarian enlargement is revealed. Excess androgen stimulation causes
thickening of albuminous ovarian envelope, that's why ovulation does
not come, and follicles, do not burst, transform into cysts.
Treatment of disease can be conservative (hormonal therapy) or
operative (wedge-shaped ovarian resection).
You can get more detailed information about the ShereshevskyTurner syndrome and the Stein-LeventhaFs syndrome from the
chapter "Menstrual function disorders".
DELAYED PUBERTY
Underdevelopment or absence of the secondary sexual signs at
the age of 13-14 and lack of menses at the age of 15-16 should be
considered as delay of sexual development (DSD).
There are central and ovarian form of delayed puberty. It
depends on the primary link of disease pathogenesis. At central
genesis the ovarian insufficiency
comes secondary in the result of insufficient gonadotropine stimulation. At
primary lesion of gonads a secretion of gonadotropic hormones is raised. It is
caused by the lack of inhibiting influence of sexual hormones on pituitary.
Central form of delayed puberty is most frequently caused by such factors, as
infectious-toxic diseases (rheumatism, viral influenza, chronic tonsillitis, tuberculosis), stress situations, excessive physical loading. These factors, acting in
the child age, give rise to functional immaturity of hypothalamic structures that
are responsible for sexual development, functional regulation of reproductive
system is disturbed. The lesion level at central form can be different. To genetically
predisposed forms delayed puberty at Lorenz-Munne-Barde-Bidle's syndrome is
refered. Delay of sexual maturity develops in patients with hypopituitarism of
organic origin.
Delay of sexual development of ovarian genesis most frequently appears in
patients with genetic defects. Hereditary factor is present in 2/3 of patients.
Damage of ovaries happens still in pre-natal period, damage degree of fetal ovaries
depends on the duration of pathogenic factor action such as taking of medicines
especially hormonal ones by mother, infectious mother's diseases, etc. In childhood epidemic parotitis and measles most frequently cause ovarian insufficiency.
Clinically a delay of sexual development is expressed by that or
other degree of sexual (genital) infantilism.
Genital infantilism is a such state, when in reproductive age
women anatomic and associated with them functional peculiarities
of genitalia, typical for child organism, are preserved.
Diagnosis. External examination of women reveals low hight,
frail body building, small breasts (fig. 55).
Hairiness on pubis is weakly developed, major
labia don't cover the minor ones. Vagina is
narrow, vaults are not expressed. Uterus is small,
2/3 of it is the cervix, 1/3 is the body. Taking into
consideration such anatomic peculiarity, the
expressed uterine bend to front — sharp-angle
anteflexion frequently occurs. Uterine cavity
length is always shorter than the norm (6 cm and
less). There are three degrees of uterine
underdevelopment for cavity lenght. They are:
• I degree — 7- cm
• II degree — 5-3,5 cm
• III degree — less then 3,5 cm
Uterine tubes are long and sinuous, ovaries
&
years old
'
„. „ .
Fig. 55. A patient of 18
are considerably smaller as compared with the with sexual
development retardation
92
Chapter
V
Functional changes are closely connected with the structural
ones. Menses in such women start lately at the age of 15-16.
Primary amenorrhea can appear in the result of considerable
underdevelopment. Amount of discharge is insignificant, menses
duration is 1-2 days (hypomenstrualsyndrome). Sometimes menses
comes not monthly, and is more rarely. Menses are followed by strong
pain (algo-dysmenorrhea), that is connected with uterine structure.
At expressed ovarian underdevelopment and considerable lowering
of their function, sexual desire is absent in women. If hormonal
background is moderately altered, sexual function is preserved.
Women with hypoplastic uterus can't become pregnant (primary
infertility) for a long time after marriage. If pregnancy comes, it can
be ectopic (because of uterine tubes' structure), or it interrupts in early
terms, because insufficient amount of hormones does not provide
normal pregnancy development. Such interruptions of pregnancy in
patients with genital infantilism can occur several times (regular
abortions), but pregnancy and associated with it intensive hormones
secretion always has positive influence on the patient's organism,
for it contributes to uterine development.
Treatment of such patients should be complex and includes
restorative therapy, physiotherapy, prescribing small doses of
hormones for ovarian function stimulation. Going in for sports,
sanatorium-health-resort cure, gynecological massage are also
recommended. The earlier the cure begins, the greater are the
chances for success.
At central genesis of disease Prephisone (25-50 AU i/m) is
indicated during the first phase of menstrual cycle (at amenorrhea the
first cycle day is considered the first day of cure) daily, 8-10 days.
Then Choriogonine (2500-3000 AU i/m) is prescribed during the 12,
14, 15, 16, 18th cycle day. Clostylbept (Clomiphen) — 50 mg per
day from 5th till 9th cycle day, then Microfolline — 0,05 mg 2 times
per day till 12-14th cycle day are also indicated. Treatment with
Clomiphen and estrogens takes 2-3 months, then synthetic
Progestines in cyclic mode during 2 courses with 7-days intervals
are taken. For better hormones' reception folic acid (0,06g per day)
in first phase, in ovulation period and second phase — Thyroidine
— 25-50 mg per day and vitamin E 50-100 mg per day are prescribed.
INCORRECT UTERINE POSITIONS
Physiological uterine position is considered to its situation in the
center of small pelvis on identical distance from symphysis, sacrum
and lateral walls of pelvis. Uterine fundus is situated beneath the
plane of inlet, external cervical os is on the level of ishial spines
(linea interspinalis).
Incorrect uterine positions
9
3
This situation is provided by sustaining fixative and suspentive
apparatus of uterus. Uterine and vaginal own tone, the tone of
frontal abdominal wall, diaphragm and muscles of pelvic floor have
a great importance.
Uterine position is uterine relation to the leading pelvis axis.
Uterus is able to displace as for its normal position. This
displacement can be physiological (uterus goes back to its previous
position) or pathological and fixed. For such conditions uterus is
immovably fixed to pelvis walls or adjacent organs by adhesions or
tumor.
Anteposition — uterine displacement considering to leading
axis to front.
Retroposition — uterine displacement backwards.
Lateroposition — (dextro- et sinistropositio) displacement of
uterus to correspond side.
Physiological retroposition of uterus happens at repletion of
urinary bladder (fig. 57). Anteposition appears at full rectum (fig.
56).
Pathological uterine displacement happens at tumors presence
or pus accumulation (fig. 58). Then uterus replaces to the healthy
side. After operative interventions or after the carried inflammatory
process with formation of adhesions, a connective cicatrix tissue
drags uterus into this side. Uterine movability is limited or absent
Fig. 56. Physiologic
Physiologic
Fig. 57.
anteposition
retroposition
Fig. 58. Front uterine
uterine
Fig. 59. Back
(fig. 59).
94
Chapter
V
Inclination of uterus (versio uteri) is a relation of vertical
uterine axis to horizontal plane. Inclination of uterus to front
(anteversio), aside (lateroversio), and also backwards (retroversio)
are distinguished.
Causes of pathological uterine inclinations may be the tumors
of genital organs (only uterine body is displaced, and cervix
remains in its place) and insufficiency of uterine ligaments.
Uterine flexion (flexio uteri) is the relation of uterine body to
its cervix. Normally between uterine body and cervix there exists an
obtuse angle (for about 120°), opened forward (anteflexio) (fig. 60).
If the angle is less than 120°, such anteflexion refers to the sharp
one (it is found at genital infantilism).
Fig. 60.
Uterine
anteversio,
anteflexio
Fig. 61.
Uterine
retroversi
o
Fig. 62.
Uterine
retrodevia
tion
Fig. 63. Uterine
reposition in the
case of
retrodeviation
Uterine flexion to back
(retroflexio), to the
right (lateroversio dextra), or to the left (lateroversio sinistra) is
pathological. Retroflexion is mobile and fixed. Fixation happens at
accretion of uterus with parietal peritoneum.
Uterine descense and
prolapse
95
Combination of retroversion and retroflexion is called uterine
retrodeviation (fig. 62). Retroflexion and uterine retrodeviation are
followed by aching dull pain in lower abdomen, painful menses
(algodysmenorrhea) and infertility. Uterine cervix erosions and
endocervicites can develop in the result of blood supply violation
and blood stagnation. Sometimes patients complain of frequent and
painful urination. There can be a delay of evacuation and pain
during it. These phenomena sometimes can be eliminated owing to
uterine reposition (fig. 63). Aged women with retrodeviation can have
uterine and vaginal prolapse often.
UTERINE DESCENSE AND PROLAPSE
Prolapse is a single pathological process based on a tight anatomic
tie between uterus, vagina, ovaries, urinary bladder and rectum.
Depending on the stage of this process, uterine descense and
prolapse are distinquished (fig. 64, 65).
96
Chapter
V
The vaginal walls descense — vaginal wall has lost its tone, they
are descent and do not leave the borders of vagina's introitus.
The vaginal walls prolapse — vaginal walls are beneath the
introitus of vagina.
Degrees of uterine displacement:
• I degree — vaginal part of uterine cervix is found lower, then
sciatic spines (linea interspinalis), however it stays inside of
pudendal cleft borders even during the exerting (uterine
descense) (fig. 64 b, c; 65)
• II degree — external cervical os goes beyond the borders of
pudendal cleft, it is found beneath vaginal introitus, and uterine
body is above it (incomplete uterine prolapse) (fig. 64 d)
• III degree — all the uterine and vaginal walls are found outside
of pudendal cleft (complete prolapse) (fig. 64 e; 66)
Fig. 65. Uterine descense and vaginal walls prolapse
Etiology and pathogenesis. Multiple deliveries, vaginal and
perineum raptures during the previous delivery, constipations,
weight lifting, hard work can cause weakening or violation of the
supportive, fixative and suspentive apparatus of the uterine structure
and uterine displacement. More frequently descense and prolapse of
uterus develops in women after 50 years in connection with the
beginning of age atrophy of sexual organs and ligament apparatus.
Uterine descense and
prolapse
97
Fig. 66.
Complete
uterine
prolapse:
a—
cystocele
b
Clinic. Uterine descense and prolapse is a long process.
— Woman
complain of dragging pain in lower abdomen and in sacrum
region,
rectocele
frequent urination, urine incontinence, that appears during the
smallest physical loads — cough, quick motions. Later a tumorous
formation — uterine cervix with external cervical os appears from
vagina. Menorrhage is possible if woman menstruates. Sexual life is
possible after uterine reposition. Woman can become pregnant.
During the first months of pregnancy cases of its spontaneous
interrupting are common. After the 12th week of gestation uterus
stops to prolapse because of its largeness, after delivery the prolapse
appears again.
Together with the anterior vaginal wall urinary bladder wall
discences and prolapses. Cystocele is formed. Descense and
prolapse of posterior vaginal wall causes formation of rectum hernia
(rectocele).
At complete uterine prolapse, its body together with cervix is
found beneath the introitus of vagina. Vagina is turned out by the
mucous membrane. Elongation of the cervix develops frequently.
Mucous membrane of vagina thins or thickens and dries out.
Secondary trophic changes can develop — trophic (decubital) ulcers
on cervix and vaginal walls, polyps near the external os are common
(fig. 66 b). Histologically micro-culation impairment, hyper- and
parakeratosis, inflammatory infiltration, sclerotic changes are found.
Changes in urinary system can also appear. Patients complain of
frequent
urination and urine incontinence. At urine analysis bacteriuria is
found. During
omocystoscopy trabecularity and cavities in mucous of urinary bladder,
ureters
^cation, cystitis, lowering of sphincters' tone are revealed. During
excretory
urography atony and dilation of ureters are present. At US
examination nephroptosis, dilation of kidney are observed. Changes
in this system are caused by violation of the blood circulation and
position of urinary bladder, ureters and associated with this urine
outflow.
Diagnosis. Diagnosis is not of a special difficulty because
prolapse is found during inspection of external genitalia. It is
important to determine whether it is a complete or incomplete
prolapse. Doctor takes the prolapsed organs with index and
forefingers on the level of vaginal introitus. If uterine body is palpated
between them, then the prolapse is incomplete. If fingers close
behind the uterine fundus — this is the complete uterine prolapse (p.
67). Perineum inspection is necessary to find scars and to estimate
the functional state of pelvic muscles.
Treatment Method of treatment for each patient is individually
selected. It depends on the age, general patient's state, presence of
menstrual and sexual functions.
Conservative treatment is indicated for women at small descense
of uterus, in reproductive age or for emaciated patients, the age or
general state of which does not allow to use a surgical intervention.
Medically-protective regimen with exclusion of physical loads is of
great importance. Medical physical training, directed to the
strengthening of abdominal press and pelvic floor muscles, rational
feeding for constipation prevention should be recommended.
Orthopedic method is in introduction of special devices into
vagina for support of uterus in its place (rubber rings). Great
attention is paid to hygiene of genitals during pessaries usage, taking
them out regularly and sterilizing by boiling.
Conservative treatment consist also of the treatment of trophic
ulcers and vaginitis, that develop in such patients rather frequently.
Doctor prescribes cure. Midwife or medical sister can fulfil it.
Vaginal walls, that have prolapsed and uterine cervix are processed
with antiseptic solutions (Potassium permanganate, Furacillin,
Hydrogenium peroxide, Chlorhexidin bigluconate). After
processing of the prolapsed tissues, they are dried up by a sterile gauze
serviette. On decubital ulcer's surface ointment or liniment with
antiseptics are applied, a surface is
covered by sterile serviette, and the uterine is replaced into vagina.
Then a tampon with aseptic remedy is inserted, tampon size depends
on the vaginal size. After elimination of inflammatory process and
vaginitis for acceleration of tissue regeneration on the region of
decubital ulcer there can be applied an ointment with Solcoseril,
Apilac, Methyluracil, Propoceum. Such procedures are hold daily
during 1-2 weeks to complete ulcer epithelization. If ulcer is not
healed up to that time, then biopsy for differential diagnostics with
cervix cancer is made.
Aged women which have used vaginal pessaries undergo careful
supervision by a doctor of female dyspansery, a midwife, and a
medical assistant. Their long usage can cause bedsores on the
uterine cervix and vagina.
Acute urine delay appears if tissues that have prolapsed squeeze.
Urine should be let out by catheter, and patient should be
hospitalized. During catheterization a catheter is directed not
upwards to symphysis but on the contrary downwards, because
urethra which is connected with anterior wall of vagina changes its
usual location.
Surgical treatment is the most radical method. The main aim of
cure is restoring of pelvic floor muscles integrity, creation of
uterine support, and also renewing of normal structure and function
of uterine ligaments.
Basic methods of surgical interventions are:
• the plastyc of frontal vaginal wall (anterior colporrhaphy), the
plastyc of posterior vaginal wall and perineum (posterior
colporrhaphy, colpoperineo-rrhaphy) — the plastyc of pelvic
floor and perineum is made
• shortening of round uterine ligaments (is used in women of
reproductive age);
• ventrofixation — uterus is fixed to the anterior abdominal wall (is
combined with frontal and posterior plastics of vagina)
• amputation of the cervix by Shturmdorf is made at uterine cervix
pathology
In senile age at complete uterine prolapse in combination with
concomitant pathology (uterine tumors etc), vaginal hysterectomy
through vagina is made. This operation is combined with the plastic
of posterior vaginal wall and levatoro-plastics.
Obligatory condition for surgical intervention is the complete
healing of decubital ulcers, absence of inflammatory process in
vagina.
Prevention of uterine and vaginal walls descense and prolapse is
necessary in medical and social aspects. It is important for woman to
do physical exercises, to go in for sport, to train abdominal press
and pelvic floor muscles. During delivery it is necessary to
diagnose interm and to restore perineal muscles in their rupture.
Doctor and midwife of postnatal department have to take careful
tendance for seams, to watch closely for the regimen and women's
conduct in postnatal period, not to allow woman with perineum
ruptures to get up and to sit down prematurely.
Uterine inversion
Uterine inversion is a state, in some causes of which uterine fundus is pressed
inside. So serous membrane is inside, and mucous one is outside. In this case the
ovaries get inside this formation, their blood supply violates. The stagnant
phenomena and uterine edema develop.
There are two forms of uterine inversion: puerperal (postnatal) and oncogenetic (caused by a tumor). The mechanism of puerperal inversion was described in
obstetrics course.
The oncogenetic uterine inversion is caused by the case of protruding myoma
placed on a short pedicle.
Clinically the oncogenetic uterine inversion is followed by extending strong
pain low in the abdomen like delivery. A fibromatous node, that is situated in
uterine cavity, more frequently near its fundus, descends into lower segment, is
perceived by it as a foreign body, and uterus begins to push it out. Uterine cervix is
dilated, node appears outside, but a pedicle stem does not allow it to be born.
Node's and uterine blood supply is disturbed, node necrosis is developed.
Hysterectomy is the treatment of oncogenetic uterine inversions. An attempt to
remove the node through the uterine cervix is dangerous because of uterine
perforation possibility, if nodes pedicle is short and wide.
The uterine torsion
The uterine torsion (torsio uteri) is turning of uterine body around vertical
axis. It happens extraordinarily rarely. Uterine and ovarian tumors, adhesions
process in small pelvis are the main causes of uterine torsion.
Assignments for Self - Assessment.
II. Multiple Choice.
Choose the correct answer / statement:
1 The most frequent type of endometriosis is:
A –Menstrual pain ;
B - Folate-deficiency anemia;
C - Vitamin B12-deficiency anemia,
2. Which of the following is Not characteristic of endometriosis?
A- Decreased factor VII;
B - Menstrual pain;
C - Family history of the disease;
D - Prolonged bleeding time.
3. Infants born to mothers with endometriosis are at higher risk for:
A - Neonatal hyperbilirubinemia;
B-Neonatal hypoglycemia;
C-Hypocalcemia;
D- Prolonged bleeding time;
III.Answers to the Self- Assessment. 1.A. 2.B. 3.D.
Students must know:
1.Etiology of endometriosis.
2. Classification of endometriosis.
3- Examination and urgency aid for women with endometriosis.
Students should be able to make:
l.Plan of management of patients with endometriosis.
2.Plan the treatment of patients with endometriosis .
3,Plan the delivery of patients with endometriosis.
4.Plan the postpartum care of patients with endometriosis .
References:
1.Danforth's Obstetrics and gynaecology. - Seventh edition.- 1994. - P. 351-464.
2.Obstetrics and gynaecology. Williams & Wilkins Waveriy Company. - Third
Edition.- 1998.-P." 196-236.
3. Basic Gynecology and Obstetrics. - Norman F. Gant, F. Gary Cuimingham.
1993.-P. 444-456.