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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