The development anomalies of the external and internal genitalia are genetic in origin.
INTRODUCTION
From
the embryological considerations the facts can be deduced. Developmental anomalies of the external
genitalia along with atubignity of sex are usually genetic in origin. Major anatomic defect of the genital tract is
usually associated with urinary tract abnormality and normal gonadal
function. While minor abnormality
escapes attention, it is the moderate or severe form which will produce
gynaecologic and obstetric problem.
CLASSIFICATION
1.
Developmental anamolies of the
external genitalia
a.
Prineal of vestibular Anus
b.
Ectopic ureter
2.
Vaginal Abnormalities
a.
Narrow Introitus
b.
Hymen Abnormality
c.
Transverse Septum
d.
Partial agenesis of upper
vagina
e.
Complete Agenesis.
3.
Abnormalities of the Fallopian
tubes.
4.
Abnormalities of the Ovaries
5.
Walffian Remnant Abnormalities
6.
Fimbrial Cyst
7.
Uterine Anomalies
a.
Failure of development of one
or both mullerian duct.
b.
Failure of recanalisation of
the mullerian duct.
c.
Failure of fusion of mullerian
duct
i. Arcuate
ii. Uterus
didelphys
iii. Uterus
bicornis
iv. Uterus
Unicornis
v. DES
– Related Abnormalities
1.
Developmental Anomalies of the external genetalia
a.
Perineal or Vestibular Anus
1.
Definition:
The
anal opening is situated either close to the posterior end of the vestibule or
in the vestibule.
Rarely
it is situated in vagina termed as Congenital rectovaginal fistula.
2.
Clinical Manifestation
Ø Anal
opening is situated either close to the posterior end of the vestibule.
Ø Opening
is sufficiently big and continence is present
3.
Investigation
Ø Detected
at birth during physical examination
4.
Management
Surgical
management:
If
there are features of obstruction or the opening is situated high in the Vagina
– ‘Pull through operation’ is to be done bringing the anal end to the anal pit
with prior colostomy.
The
delivery should be by caesarean section.
Nursing
Management
Ø Give
psychological support that the condition can be recovered.
Ø Post
operative care such as
o
Regular checking of vital signs
o
Regular changing of dressing.
o
Administration of IV fluid and
medication.
Ø If
colostomy is present give colostomy care
o
The patient is monitored for
complications such as leakage from the site of the anastomosis, prolapse of
stoma, perforation stoma retraction, fecal impaction and skin irritation.
o
The abdominal dressing is
examined frequently to detect the signs of hemorrhage.
o
Teach the patient about its
care until the patient can take over.
Skin care must be taught along with how to apply and remove the drainage
pouch.
o
After cleansing the skin is
patted completely dry with a gauze pad, rubbing the area is avoided.
o
The patient is encouraged to
verbalize feeling and concerns about altered body image and the stoma. A supportive environmental should be given to
the patient.
o
Replace the colostomy dressing
or pouch.
ECTOPIC URETER
Definition
It
is a condition in which the additional ureteric opening is usually in the
vestibule close to the urethra or in the vagina.
Clinical
Manifestation
Uncontrolled wetness
Management
Ø Surgical
management
Ø Partial
nephrectomy and uretrectomy may be indicated / or implantation of the ectopic
ureter in to the bladder may be done.
Nursing
Management:
Ø Psychological
support to the patient must be given, tell the patient that she will be alright
after operation.
Ø Pre-operative
and post operative care should be given which include regular checking of vital
sign. NBM should be maintained,
pre-operative medication should be given.
Take the concent advice the patient not to do stenuous exercise for a
month. Advice to avoid heavy works. Advance about the follow up.
Ø Give
the post operative medication as per doctors order.
Ø Inspect
the site for any signs of infection such as redness, pus, fever etc.
Ø Maintain
aseptic technique in all procedures.
III. Vaginal Abnormalities
The deformities includes
Ø Narrow
introitus
Ø Hymen
abnormality
Ø Septum
Ø Atresia
a)
Narrow introitus
i)
Definition:
Introitus
means an opening entrance in a hallow organ or cavity.
ii)
Clinical Features:
Dyspareunia
iii)
Investigation:
After
marriage only its existence can be ruled out during pervaginal examination.
iv)
Management:
Surgical
management
Ø Manual
stretching under general anesthesia
Ø Perineoplasty
or Fentons operation
v)
Nursing Management
Ø Phychological
support
Ø Pre-Operative
nursing care should be given such as explain the patient about the important of
operation. NBM should be maintained, all
pre-operative medication should be given, and take the consent, cheek the vital
sign.
Ø Post-operative
nursing care such as proper position should be maintained after surgery. Inspect the site for any signs of infection,
cheek the vital sign.
HYMEN
ABNOEMALITIES:
a)
Imperforate Hymen
Definition:
It
is due to feature of disintegration of the central cells of the mullerian
eminence that project in to the urogenital sinus.
Clinical
features
Ø Cryptomenorrhiea
the menstrual blood is pent up inside the vagina behind the hymen as the uterus
is functionaing normally.
Ø Haematocolopos
– distends of the vagina due to the blood accumulated.
Ø Haematometra
– the uterine cavity is dilated.
Ø Haematosalpinx
– the distended fimbrial and become closed by adhesions.
Ø Periodic
lower abdominal pain which is continuous.
Ø Primary
anneorrhoea.
Ø Dysuria.
Ø Frequency
of urination
Diagnostic
evaluation
Ø Abdominal
examination reveals a suprapublic
swelling vulval inspection reveal a tense bulgins vagina.
Ø Ultrasonography reveal harmeto metra and haematocolpos
Management:
Surgical management
Ø Cruciate
incision is made in the hymen and the dark tarry colourd blood id allowed to
escape.
Nursing
Management:
Ø Pressure
from above not to be given.
Ø Internal
examination should not be done
Ø Head
end of the patient should be raised
Ø Antibiotics
should be given to prevent infection
Ø The
operative site should be checked for any signs of infection.
TRANSVERSE
SEPTUM (ABOVE THE LOWER ONE THIRD)
DEFINITION:
Transverse septum is the results of
failure of canalization of the fused mullerian duct and the urogenital sinus.
Clinical
manifestation:
Ø Cryptomenorrhoca
Ø Haematocolpos
Ø Haematometra
Ø Haematosalpinx
Ø Periodic
lower abdominal pain which is continuous.
Ø Primary
amenorrhea
Diagnostic
Evaluation:
Ø Abdominal
examination reveals a suprapublic swelling.
Ø Vulval
inspection reveals a tense bulging membrane of bluish discolouration.
Ø Rectal
examination reveal the bulged vagina.
Ø Ultra
sonography reveals haematometra and haematocolpos.
Management:
Surgical management:
Ø Cruciate
incision is made and blood is allowed to escape.
Nursing
Management:
Ø Psychological
support should be given
Ø Aseptic
technique should be maintained
Ø Pressure
should not be given
Ø Internal
examination should be done
Ø Head
end of the patient should be raised
Ø Antibiotic
should be given
Ø Internal
examination can be done only the next menstrual menstrual period is over.
PARTIAL
AGENESIS OF UPPER VAGINA
A
segment of vagina may be atretic in the upper third. It is often associated with hypoplasia or
even absence of cervix. Uterus may be
normal or functioning or malformation.
Clinical
Features
Ø Primary
amenorrhea
Ø Periodic
lower abdominal pain
Ø Presence
of lower abdominal mass
Management
Ø Surgical
management
Ø Hysterectomy
Nursing
management
Ø Asceptic
technique should be maintained
Ø Vital
signs should be checked
Ø Avoid
applying pressure
Ø Internal
examination should be avoided and should be done only after the next menstrual
period is over.
Ø Head
end of the patient should be raised
Ø Antibiotic
should be given to prevent infection
Ø Check
for any signs of infection
Ø Give
psychological support.
COMPLETE
AGENESIS
Definition
Complete
agenesis of the vagina is almost always associated with absence of uterus. The defect is usually associated with urinary
tract abnormality.
Clinical
Features:
Primary amenorrhoea
Dysparenunia
Management
Surgical management:
Reconstructive operation –
Vaginoplastry is done
Nursing
management
Ø Check
the vital signs
Ø Maintained
aseptic technique in all procedure
Ø Give
psychological support
Ø Advice
to avoid strenuous exercise to avoid to heavy work etc
Ø Head
end of the patient should be raised
Ø Check
the site for any signs of infection
Ø Antibiotics
should be given to prevent infection
Ø Advice
about regular follow up
In Certain Case the tubes may be
absent on one side
Clinical
features
Ø Tubes
may be elongated
Ø Accessory
ostia or diverticula’s may be present
Ø Tubes
may be absent
Ø Ectopic
pregnancy
Ø Lower
fertility rate
ANOMALIES
OF THE OVARIES
Definition:
There
may be steak gonads or gonadal dysgenesis which are usually associated with
errors of sex chromosomal pattern.
Ø Accessory
ovary (division of the original ovary in to two)
Ø Supernumerary
ovaries, may be found in the broad ligament or else where
Diagnostic
evaluation
Ø Ultra
sonography
Management:
Ø No
treatment is of any help
WOLFFIAN
REMNANT ABORMALITIES
Definition
The outers end of the wolffian
(gartner) duet may be cystic, size of pea, often pedunculated and is attached
near the outer end of the tube.
FIMBRIAL
CYST
Definition
It
probably arise4s from the small remnant of ovarian tissue in relation to the
ovarian fimbria. It can attain a big
size. The cyst is unilocular, wall is
thin and contain clear translucent fluid.
UTERINE MALFORMATIONS
Definition:
Uterine
malformations are defined as the malformations and displacement of the body of
uterus caused by the persistence to a greater or lesser extent of the septum
between the mullerian ducts.
Classification:
According
to American Fertility Society 9AFS)
1.
Uterine Atresia or Aplasia
These
anomalies are secondary to partial or complete absence of development of
Mullerian ducts. They are frequently
associated with renal anomalies.
Type
1
a)
Complete bilateral agenesis
Rate: Incompatible with life because associated
bilateral renal agenesis.
b) Incomplete bilateral agencies
It
is also known as mayer – Rokitansky Kuster – Hauser Syndrome. The women with this anomaly are is infertile
because of the absence of functionary uterus.
Type
II
a. Unilateral complete agenesis.
It
is also called true unicornuate uterus.
This form is characterized by missing of one uterine horn.
b. In complete unilateral agenesis
The
form is much more frequent than complete agenesis. In this case the rudimentary room of variable
development is present.
i.
Non communicating rudimentary
horn
ii.
Rudimentary horn without cavity
II.
Uterine Duplications
These
anomalies are related to defective fusion of the Mullerian ducts.
a. Uterus didelphys
It
is characterized by presence of two uterine horn and two cervixes, with or
without separate vagina.
b. Bicornulate uterus
In
this case the uterus consists of one cervix and two divergent horns
Iii. Uterine
Septations
Uterine
septations belong among most frequent malformations. Separations are secondary to the total or
partial defect of resorption of the sagittal septum.
a.
Complete septate uterus:
Through the mullerian ducts are fused, a median septum passes from the fundus
of the uterus through the cervix and may extend into the vagina.
b.
Partial septate: In the septum
is restricted to the body of the uterus.
IV.
Uterine Communications
These malformations are very
rate. The uterus has an isthemic
communication between the cavities of the bicornuate or septate uterus.
Clinical
features
Classified into gynecological and
obstetrical
a.
Gynecological
a.
Infertility and Dyspareunia
b.
Dysmenorrhoea
c.
Menorrhagia
b.
Obstetrical
a.
Mid trimester abortion
b.
Malpresentations
c.
Preterm labour
d.
Retained placenta
e.
Post partum Haemorrhage
f.
Prolonged and obstructed labour
Diagnostic
Evaluation:
a.
Internal Examination Reveals
septate vagina and two cervixes.
b.
Hysteroscopy
c.
Ultrasonography
d.
Magnetic Resonance Imaging
e.
Laparoscopy
Management
A.
Surgical Management
a.
Strassman utriculoplasty operation
b. Jone’s Metroplasty operation.
c. Tomkins Operation
d. Hysteroscopic resection
B.
Nursing Management
Preoperative
care
Ø Provide
psychological support
Ø Send
the blood and urine for routine examination
Ø Check
the vital signs
Ø Assess
for any systemic diseases such as DM, HTN, TB
Ø Obtain
consent for operation
Ø Mother
should be kept nil by mouth
Ø Pre-Operative
medication should be given such as Inj. TT, Inj Xylocaine
Ø Prophylactic
antibiotics such as cephalosporins to reduce the risk of infection.
Ø Preparation
of the part should be done with antiseptic solutions
Post
– operative care
Ø Provide
comfortable position.
Ø Monitor
the vital signs
Ø Observe
the patient for any signs of infection.
Ø Assess
the incision site for bleeding.
Ø Maintain
aseptic techniques in all procedure.
Ø Administer
antibiotics to prevent the infection
Ø Administer
analgesics to relieve pain
Ø Change
the soiled dressings at least twice a day
Ø Provide
calm and quite environment
CONCLUSION
The
development anomalies of the external and internal genitalia are genetic in
origin. The developmental anomalies of
the external genitalia include perineal or vestibular anus, ectopic ureter,
vaginal anomalies include narrow entroitus, hymen abnormality septum. Atresia, uterine anomalies include failure of
development of one or both mullerian ducts, failure of recanalisation of the
mullerian ducts. Failure of fusion or
mullerian duct. Type of fusion anomalies
include arcuate uterus didelphys, uterus bicornis, uterine unicornis, DES,
related abnormalities.
Abnormalities
of fallopian tube and ovaries are also delt.
BIBLIOGRAPHY
1.
D.c.Dutta, Text Book of
Gynaecology, 3rd Edition, New Central Book Agency (P) Ltd.,
culcutta., Pg.No.39-45.
2.
V.G.Padubidini, Shaw’s Textbook
of Gynaecology, Elsevier Company (P) Ltd., 13th Edition,
Pg.No.82-95.
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