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Fifth stage
gynecology
Lec-
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ﺩ.ﺍﺳﻣﺎء
/۷۲10/2015
GENITAL TRACT MALFORMATION
The student at the end of this lecture should be able to :
Describe the types of uterine anomalies and their clinical
presentations.
Exclude cryptomenorrhoea from a primary amenorrhoea
case with normal secondary sexual characteristics and
cyclical abdominal pain.
ANOMALIES OF THE PARAMESONEPHRIC DUCT
anomalies of the fallopian tubes, uterus, cervix
and vagina are not uncommon.
The exact aetiology is unknown ,the mostly
accepted three theories are: teratogenesis , genetic
inheritance and multifactorial expression.
Variation and combination of anomalies occur :
lack of development(agenesis),
incomplete development (hypoplasia),
incomplete canalization (atresia)
complete separate development.
UTERINE ANOMALIES
Absence of the uterus : uterus is absent or
rudimentary and incapable of function,
There are two conditions present with absent uterus .
1.Mayer Rokitansky Kuster Hauser syndrome (MRKH):
-absent uterus and vagina.
-46XX.
-normal ovarian function.
-presents as primary ammenorrhoea
OR 2. a state of androgen insensitivity
syndrome
- absent uterus
- blind end of the lower vagina
- XY
- absent axillary and pubic hair
- gonads are testis(histology) found in the
abdomen or the groin require removal
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because of long term malignancy.
No treatment is available for such uterine
anomalies (uterine transplantation)
Uterine fusion anomalies:
these are not uncommon
and lesser degrees of defect are quite common.
a - Uterus didelphys : complete lack of fusion of
the mullerian ducts with double uterus and
double vagina.
b - Uterus bicornis bicollis : the two horn of the
uterus have separate cavities with fused muscular
wall with or without vaginal septum.
c. Uterus bicornis unicollis: the uterine body is bifid and
the horns are unequal in size with normal cervix.
d. Uterus unicornis :when one mullerian canal develops
into fallopian tube, uterus and cervix while the second
duct is in rudimentary horn which its canal may not be
connected with the uterus causing hemihaematometra
with dysmenorrhoea.
e. Uterus septus: when the ducts fused and the cavity is
separated by a median septum either partial or
complete. There may be vaginal septum.
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Presentation of uterine anomalies
-symptomless discovered during HSG or during
operation.
-dysmenorrhoea(rudimentary horn)
-abortion
-malpresentation
-difficult labour
-rare presentation is ectopic pregnancy in a
rudimentary horn ,presented later than other
type and with rupture and sever haemorrhage.
Vaginal anomalies :(absent,atresia,septum)
absent vagina: usually occur with absent uterus
in MRKH syndrome ( rarely occur with the presence
of the uterus), there is normal secondary sexual
characteristics, presents between the age of twelve
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and sixten years as primary amenorrhoea which
suggest an anatomical abnormality.
Vulval and abdominal examination
ultrasound required to exclude any retained blood
inside the upper part of the genital tract,
karyotype is required to exclude androgen
insensitivity syndrome (XY ).
If the patient abnormality discovered after
marriage at attempt of intercoarse operation to
create a new vagina
(william operation:edge of the posterior parts of the
labia majora are sutured in the midline result into a
perineal puoch)is often satisfactory.
More extensive operation is MacIndoe operation
(aspace is dissected between therectum and the
bladder and urethra and this cavity is lined with a skin
graft from the thigh applied on amold which is held to
the introitus by stitches.
If there is a functioning uterus early operation is required
and the upper end of the vaginal epithelium is dissected
down to the introitus.
Always urinary tract abnormalities should be excluded.
Vaginal atresia: there is failure of fusion of the vaginal parts
derived from the mullerian duct and the urogenital sinus.
There is amembrane above the hymen obstruct the
entrance to the vagina or, less commonly, there is
a considerable length of the vagina may be un canalized.
As the uterus is often normal ,the menstrual blood
after puberty will be retained inside, a condition known as
cryptomenorrhoea.
THE VAGINA BECOME DISTENDED WITH BLOOD
(HAEMATOCOLPOS) THE BLOOD BECOME THICK AND CHOCKLATE
AFTER SLOW ABSORPTION OF FLUID .
LEAKING OF THE FLUID INTO THE PERITONEAL CAVITY MAY CAUSE
OBSTRUCTION OF THE FIMBIRIA OR ENDOMETRIOSIS.
The presentation is a female between 14-16 years .
primary amenorrhoea ,
cyclical discomfort then abdominal pain ,
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mass may be large and felt above the pubic symphysis ,
retention of urine may occur
even peritonitis.
Vulval inspection : a bulging membrane may be
seen and appear purple or red (thin or thick).
Rectal examination reveal a mass at the vagina.
Hydrocolpos is similar but its distention of the
vagina by the retained mucoid secretion of the
cervix present in the neonatal period.
Diagnosis needs US, laproscopy or even laparotomy.
Diagnosis is often obvious ,but difficult in case of
obstruction at higher level or in obstruction in one
of double system (normal menses at the patent side).
Treatment :
satisfactory drainage best is by excision of the
membrane.
There is high risk of infection need antibiotic
cover 24 hr. before and 5 days after,
meticulous surgery ,don’t use cotton swab for mopping
only suction .
Prognosis is good if the distention only involve
the vagina not the uterus nor the tubes.
There is risk of obstruction of the fimbria,
endometriosis and infertility.
There may be vaginal stenosis and dyspareunia later on .
Vaginal septum:
there is longitudinal anteroposterior septum
due to mullerian fusion anomalies.
One of them may be larger or both have equal
size.
May obstruct labour when it can be divided
easily if not give away .
FALLOPIAN TUBES ANOMALIES :
malformed or absent tube,
congenital diverticula or accessory ostia have no
clinical significance
Gonadal anomalies (gonadal dysgenesis)
Cervical anomalies (congenital elongation of
the cervix to reach the introitus causing
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discomfort and may require excision .
Cyst of the broad ligament:
Reminant of the wolffian duct
-hydatid of morgagni (pedinculated at the
outer end)
-koblet’s tubules (pronephric in origin)
-epoophoron and the paraphoron
(proximal) ,both are of mesodermal origin .
Reminant of ovary (fimbrial cyst)