UTERINE MALFORMATION

INTRODUCTION

Congenital malformations are anomalies which may be either hereditary or occurring during
gestation and evident at the time of birth. Development of the female genital tract is an
complex process dependent upon a series of events involving cellular differentiation ,
migration , fusion and canalization. Failure of any one of these processes results in
congenital anomaly.

DEFINITION

A uterine malformation is a type of female genital malformation resulting from an

abnormal development of the mullerian ducts during embryogenesis.

CLASSIFICATION

American fertility society classification of mullerian anomalies (1988) are :

Class I : mullerian agenesis / hypoplasia – segmental

Class II : unicornuate uterus

Class III : didelphys uterus

Class IV : bicornuate uterus

Class V : septate uterus

Class VI : arcuate uterus

Class VII : diethyl stilboestrol related abnormality

INCIDENCE OF MULLARIAN ABNORMALITIES

It varies between 3 and 4 percent. The incidence is found to be high in women suffering
from recurrent miscarriage or preterm deliveries ( 5 - 20 % ).

Failure of development of one or both mullerian ducts

The absence of both ducts leads to absence of uterus including oviducts . There is absence
of vagina as well. Primary amenorrhoea is the chief complaint.The absence of one duct leads
to a unicornuate uterus with a single oviduct.

Failure of recanalisation of the mullerian ducts

Agenesis of the upper vagina or of the cervix – This may lead to haematometra as the uterus
is functioning.
Failure of fusion of mullarian ducts

In majority , the presence of deformity escapes attention.In some , the detection is made
accidentally during investigation of infertility or repeated pregnancy wastage.In others , the
diagnosis is made during D & E operation , manual removal of placenta or during caesarean
section.

TYPES OF FUSION ANOMALIES

Arcuate (18% ) – The corneal parts of the uterus remains separated.The uterine fundus
looks concave with heart shaped cavity outline.

Uterus didelphys ( 8 % ) – There is complete lack of fusion of the mullarian ducts with a
double uterus , double cervix and a double vagina.

Uterus bicornis (26 % ) – There is varying degree of fusion of the muscle walls of the two
ducts.

 Uterus bicornis bicollis – There are two uterine cavities with double cervix with or
without vaginal septum.
 Uterus bicornis unicollis – There are two uterine cavities with one cervix. The horns
may be equal or one horn may be rudimentary and have no communication with the
developed horn.

Sepatate uterus (35% ) – The two mullarian ducts are fused together but there is
persistence of septum in between the two either partially or completely.

Uterus unicornis (10% ) – Failure of development of one mullarian duct.

DES – related abnormality is due to DES exposure during intrauterine life. Varieties of
malformations are included.

Vagina : adenosis , adenocarcinoma.

Cervix : cockscomb cervix , cervical collar.

Uterus : hypoplasia , T shaped cavity , uterine synechiae.

Fallopian tube : corneal budding , abnormal fimbriae .such cases are not seen now.

CLINICAL FEATURES

GYNAECOLOGICAL

 Infertility and dyspareunia are often related in association with vaginal septum
 Dysmenorrhoea in bicornuate uterus or due to cryptomenorrhoea
 Menstrual disorders
OBSTETRICAL

 Midtrimester abortion which may be recurrent

 Rudimentary horn pregnancy
 Cervical incompetence
 Increased incidence of malpresentation
 Preterm labour , IUGR , IUD
 Prolonged labour
 Obstructed labour
 Retained placenta and postpartum haemorrhage where the placenta is implanted
over the uterine septum

DIAGNOSIS

 Hysterography
 Hysteroscopy
 Laproscopy
 Ultrasonography
 Magnetic resonance imaging

TREATMENT

Mere presence of any uterine malformation is not an indication of surgical intervention

REPRODUCTIVE OUTCOME

Unicornuate uterus has very poor (40%) pregnancy outcome. No treatment is generally
effective. Uterus didelphys has best possibility of successful pregnancy ( 64 % ) .Unification
operation is generally not needed.other causes of infertility or recurrent fetal loss must be
excluded.

RUDIMENTARY HORN should be excised to reduce the risk of ectopic pregnancy.

UNIFICATION OPERATION ( bicornuate / sepatate uterus ) is therefore indicated in

otherwise unexplained cases with uterine malformation. Abdominal metroplasty could be
done either by excising the septum or by incising the septum.Success rate of abdominal
metroplasty in terms of live birth is high ( 5- 75 % )

HYSTEROSCOPIC METROPLASTY is more commonly done .Resection of the septum can be

done either by a resectoscope or by laser

ADVANTAGES

 High success rate ( 80-89% )

 Short hospital stay
 Reduced postoperative morbidity
  Subsequent chance of vaginal delivery is high compared to abdominal metroplasty
where caesarean section is mandatory

ABNORMALITIES OF THE FALLOPIAN TUBES

The tubes may be unduly elongated; may have accessory ostia or diverticula . Rarely tube
may be absent on one side. These conditions may lower the fertility or favour ectopic
pregnancy.

ABNORMALITIES OF THE OVARIES

There may be streak gonads which are usually associated with errors of sex chromosomal
pattern. No treatment is of any help. Accessory ovary may be rarely present. Rarely
supernumerary ovaries may be found ( 1 in 29,000 ) in the broad ligament or elsewhere . In
this are event where menstruation continues even after removal of two ovaries.

WOLFFIAN REMNANT ABNORMALITIES

The outer end of the wolffian duct may be cystic , size of pea , often pedunculated and
attached near the outer end of the tube. Small cyst may arise from any of the tubules.A
cystic swelling from the gartner duct may appear in the anterolateral wall of the vagina
which may be confused with the cystocele.

PAROVARIAN CYST

It arises from the vestigial remnants of the wolffian tissue situated in the mesosalpinx
between the tube and ovary. This can attain a big size.This cyst is unilocular ; the wall is thin
and contains clear translucent fluid.The ovarian fimbria with the ovary is stretched over the
cyst . The wall consists of connective tissue lined by single layer of low columnar epithelium

OTHER ABNORMALITIES

LABIA MINORA : labial fusion

a) Due to developmental defect

b) Inflammatory

LABIA MAJORA

a) Hyperplastic or hypoplastic labia

b) Abnormal fusion in adrenogenital syndrome

CLITORIS : clitorial hypertrophy – often associated with various intersex problems.

PERINEUM

Imperforate anus , anal stenosis or fistula are the result of abnormal development of the
urorectal septum.
COMPLICATIONS

 Abortion
 Weak uterine action
 Post partum haemorrhage
 Adhesion of the placenta
 Malpresentations
 Prolonged or obstructed labour
 Uterine rupture

NURSING DIAGNOSIS

 Ineffective tissue perfusion related to excessive bleeding , hypotension and

decreased cardiac output
 Administer oxygen through a face mask
 Assess the blood loss
 Monitor the vital signs
 Administer the medications as per ordered
 Acute pain related to increase uterine activity
 Assess the degree of pain
 Administer pain medications as ordered
 Provide comfortable position
 Provide diversional therapy
 Fluid volume deficit related to excessive bleeding
 Monitor vaginal bleeding
 Evaluate fundal height to detect an increase bleeding
 Risk for infection related to excessive blood loss
 Assess the odor of vaginal bleeding
 Assess the vital signs
 Strict hand washing techniques
 Evaluate WBC and differential count
 Ensure that all the equipments used are sterile and clean
 Fear related excessive bleeding procedures and unknown outcome
 Assess the vebal and non verbal responses and the use of coping mechanisms
 Answer the questions in a calm manner , using simple terms
 Provide accurate and consistent information about the condition
SUMMARY

A uterine malformation is the result of an abnormal development of the mullerian ducts

during embryogenesis.Developmental anomalies of the external genitalia along with
ambiguity of sex are usually genetic in origin. Major anatomic defect of the genital tract is
usually associated with urinary tract abnormality , skeletal malformation and normal
gonadal function.

CONCLUSION

Congenital malformations are anomalies which may be either hereditary or occurring during
gestation and evident at the time of birth. Development of the female genital tract is an
complex process dependent upon a series of events involving cellular differentiation ,
migration , fusion and canalization.Failure of any one of these processes results in
congenital anomaly.

BILBLIOGRAPHY

 D.C DUTTA , Text book of gynaecology , 6th edition ,new central book publishers,
page No 56-63.
 Myles Text book for Midwives ,14th edition , Churchill Livingstone Publishers ,page
No 123-126
 Annamma Jacob , A comprehensive Text Book of Midwifery and Gynaecological
nursing , 3rd edition ,page No 144-146

NET REFERENCE

 uterine malformation: MedlinePlus

 www.congenital anomaly.com
 uterine anomaly.org