UTERINE MALFORMATIONS Aastha Singh - PowerPoint Presentation

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UTERINE MALFORMATIONS

Aastha Singh

Lecturer

Nightingale Institute of Nursing

Introduction

Uterine malformations result from partial or complete failure of one of three mechanisms either separately or combined - agenesis, fusion, and resorption.

Agenesis

results in either a complete absence of the uterus or a

unicornuate

uterus;

a

failure to fusion

gives rise to uterine

didelphys

or a

bicornuate

uterus;

and a

septated

uterus is due to a

failure of resorption

.

Clinical Presentation

Spontaneous Abortion

Premature Delivery

Infertility

Abnormal Fetal Lie

Dystocia at delivery

Dysmenorrhea

endometriosis

Cervical incompetence

Classification into 3 groups

Based on similar embryonic development defects and clinical presentation

Agenesis of uterus/vagina:

RokitanskyKuster

-Hauser Syndrome

Defects in Vertical Fusion (obstructive or non-obstructive)

Lateral Fusion defects (obstructive or non-obstructive)

Müllerian duct anomaly classification

 The American Fertility Society (AFS) classified

muellerian

anomalies according to the major uterine anatomic types:

 Hypoplasia/agenesis 

Unicornuate



Didelphys



Bicornuate



Septate



Arcuate

Arcuate:

The uterus appears normal from the outside but has a very small (1cm or less) indentation protruding from the top of the uterine cavity. This is generally unproblematic. It is simply considered a different, but perfectly normal, type of uterus.

Bicornuate

:

This uterus is considered heart-shaped. It has a deep indentation at the top of the uterus, forming an overall shape of a heart.

Septate

:

This uterus appears normal from the outside, but it contains an internal septum that divides the uterine cavity into 2 smaller cavities.

Didelphys

:

The two halves of the uterus are completely separate.

Unicornate

:

Only one-half of the uterus is well-developed.

CLASS I: UTERINE AGENESIS/UTERINE HYPOPLASIA

 a: vaginal (uterus: normal/ variety of abnormal forms) –

b: cervical

c: fundal

d: tubal

e: combined

 CLASS II: UNICORNUATE UTERUS/UNICORNIS UNICOLLI

 a: communicating contralateral rudimentary horn contains endometrium

b: non-communicating contralateral rudimentary horn contains endometrium

c: contralateral horn has no endometrial cavity

d: no horn

CLASS III: UTERUS DIDELPHYS

This condition exists when a woman has two separate uterine bodies, each one with a cervix. 

 CLASS IV: BICORNUATE UTERUS

A. complete division, all the way down to internal the

os

–

b: partial division, not extending to the

os



CLASS V: SEPTATE UTERUS

a: complete division, all the way down to the internal

os

b: incomplete division

This is the commonest anomaly

 CLASS VI: ARCUATE UTERUS

 An arcuate uterus is characterized by a mild indentation of the endometrium at the uterine fundus. It occurs due to failure of complete resorption of the

uterovaginal

septum

UTERINE AGENESIS

Uterine agenesis is the extreme of

Mullerian

duct anomalies (Class I) where there is complete absence of uterine tissue above the vagina. 

Epidemiology

:  The uterine agenesis-hypoplasia spectrum accounts for ~10-15% of all

Müllerian

duct anomalies. 

Clinical presentation

:  is

characterised

by primary

amenorrhoea

, with normal hormonal levels guaranteed by fully functional gonads.

Complete absence of the

Mullerian

ducts is termed

Mayer-

Rokitansky

-

Kuster

-Hauser (MRKH) syndrome

which includes absence of the vagina as well

Mayer-Rokitansky

-

Kuster

-Hauser (MRKH) syndrome (Utero vaginal

agensis

Expected Menarche 

Difficult to differentiate from imperforate hymen 

No uterus on exam, U/S, MRI, Laparoscopy, IVP 

Confused with Androgen Resistance Syndrome with shallow pouch and no uterus

UNICORNUATE UTERUS

Unicornuate

with uterine horn (not containing an endometrial cavity, not fused to cavity

Unicornuate

with communicating uterine horn

 Complications of the Mullerian

duct anomalies

Spontaneous abortion

Reported premature birth

Infertility

Symptoms of Uterine Malformations

Uterine malformations usually present with no symptoms at all. Some women with malformed uteruses don’t have difficulty getting pregnant and do not usually discover their unusually shaped uterus until they have a prenatal ultrasound.  Others may be diagnosed during an infertility evaluation.

Diagnosis

A complete medical history and physical examination may lead us to suspect that a congenital uterine anomaly is present. However, imaging studies, such as a

hysterosalpingogram

(HSG) and ultrasound, or an MRI are required to

visualise

the uterus and confirm that a congenital uterine anomaly is present

 MRI has been the "gold standard" for categorizing uterine

anomalie